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Amyotrophic Lateral Sclerosis (Lou Gehrig's Disease)
Classification and external resources
ICD-10 G12.2
ICD-9 335.20
OMIM 105400
DiseasesDB 29148
MedlinePlus 000688
eMedicine neuro/14 emerg/24 pmr/10
MeSH D000690
.Amyotrophic lateral sclerosis is a form of motor neuron disease.^ Amyotrophic lateral sclerosis (ALS) is the most common form of motor neuron disease (MND).
  • PLoS ONE: Amyotrophic Lateral Sclerosis: An Emerging Era of Collaborative Gene Discovery 19 January 2010 9:52 UTC www.plosone.org [Source type: Academic]

^ Recent advances in amyotrophic lateral sclerosis.
  • Amyotrophic Lateral Sclerosis, Lead, and Genetic Susceptibility: Polymorphisms in the -Aminolevulinic Acid Dehydratase and Vitamin D Receptor Genes 19 January 2010 9:52 UTC ehp03.niehs.nih.gov [Source type: Academic]

^ Both patients were initially diagnosed as having lower motor neuron forms of amyotrophic lateral sclerosis.
  • Amyotrophic Lateral Sclerosis (ALS) - Abstracts : Online Reference for Health Concerns 19 January 2010 9:52 UTC www.lef.org [Source type: Academic]

.ALS, sometimes called Lou Gehrig's disease, is a progressive,[1] fatal, neurodegenerative disease caused by the degeneration of motor neurons, the nerve cells in the central nervous system that control voluntary muscle movement.^ Oliver D. Motor neurone disease.
  • Amyotrophic Lateral Sclerosis: Lou Gehrig's Disease - March 15, 1999 -American Academy of Family Physicians 20 September 2009 17:15 UTC www.aafp.org [Source type: Academic]

^ Brain's Diseases of the nervous system.
  • Amyotrophic Lateral Sclerosis: Lou Gehrig's Disease - March 15, 1999 -American Academy of Family Physicians 20 September 2009 17:15 UTC www.aafp.org [Source type: Academic]

^ ALS is a disease of the parts of the nervous system that control voluntary muscle movement.
  • Facts About ALS | MDA/ALS Publications 19 January 2010 9:52 UTC www.als-mda.org [Source type: Academic]

.The condition is often called Lou Gehrig's Disease in North America, after the New York Yankees baseball icon who was diagnosed with the disease in 1939 and died from it in 1941, at age thirty-seven.^ Lou Gehrig disease .
  • Lou Gehrig disease - definition of Lou Gehrig disease in the Medical dictionary - by the Free Online Medical Dictionary, Thesaurus and Encyclopedia. 20 September 2009 17:15 UTC medical-dictionary.thefreedictionary.com [Source type: Academic]

^ What are the causes of Lou Gehrig's disease?
  • amyotrophic lateral sclerosis Facts, information, pictures | Encyclopedia.com articles about amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.encyclopedia.com [Source type: Academic]

^ Why is amyotrophic lateral sclerosis called Lou Gehrig's disease?
  • amyotrophic lateral sclerosis Facts, information, pictures | Encyclopedia.com articles about amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.encyclopedia.com [Source type: Academic]

.Today, renowned physicist Stephen Hawking, British historian Tony Judt, guitar virtuoso Jason Becker and bass virtuoso Mike Porcaro are among the best-known living ALS patients.^ For example, British physicist Stephen Hawking has had ALS since the 1960s and is still able to write and practice his profession.
  • Facts About ALS | MDA/ALS Publications 19 January 2010 9:52 UTC www.als-mda.org [Source type: Academic]

^ Treatment of ALS patients is best provided at multidisciplinary clinics that have neurologists or physiatrists, speech language pathologists, occupational therapists, physiotherapists, and dietitians on staff.
  • Amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.cfpc.ca [Source type: Academic]

^ Care of ALS patients is best provided by multidisciplinary ALS clinics ( Table 4 ) in conjunction with family physicians.
  • Amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]
  • Amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.cfpc.ca [Source type: Academic]

.The disorder causes muscle weakness and atrophy throughout the body as both the upper and lower motor neurons degenerate, ceasing to send messages to muscles.^ The loss of lower motor neurons leads to weakness, twitching of muscle (fasciculation), and loss of muscle mass (muscle atrophy).
  • Amyotrophic Lateral Sclerosis Glossary of Terms with Definitions on MedicineNet.com 19 January 2010 9:52 UTC www.medicinenet.com [Source type: Academic]

^ None had definite upper motor neuron signs or muscle cramps.

^ Motor neuron disorders.
  • Amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

.Unable to function, the muscles gradually weaken, develop fasciculations (twitches) because of denervation, and eventually atrophy because of that denervation.^ When these cells gradually die, as in ALS, muscles atrophy (shrink) and become progressively weaker and eventually unable to contract, resulting in paralysis.
  • Facts About ALS | MDA/ALS Publications 19 January 2010 9:52 UTC www.als-mda.org [Source type: Academic]

^ The muscles atrophy quickly, causing weakness, paralysis, and eventual death, usually when the muscles that control respiration fail.
  • amyotrophic lateral sclerosis Facts, information, pictures | Encyclopedia.com articles about amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.encyclopedia.com [Source type: Academic]

^ The loss of lower motor neurons leads to weakness, twitching of muscle (fasciculation), and loss of muscle mass (muscle atrophy).
  • Amyotrophic Lateral Sclerosis Glossary of Terms with Definitions on MedicineNet.com 19 January 2010 9:52 UTC www.medicinenet.com [Source type: Academic]

.The patient may ultimately lose the ability to initiate and control all voluntary movement; bladder and bowel sphincters and the muscles responsible for eye movement are usually (but not always) spared.^ The motor neurons control the voluntary muscles, which are the muscles that allow movement.
  • Health: Amyotrophic Lateral Sclerosis (ALS); Amyotrophic lateral sclerosis (ALS) , also known as ... 19 January 2010 9:52 UTC www.mainegeneral.org [Source type: Academic]

^ Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's Disease, is a fatal neurological disease that rapidly attacks and destroys the nerve cells that are responsible for voluntary movement.
  • Biomarkers for amyotrophic lateral sclerosis and methods using the same - Patent Application 20070298998 19 January 2010 9:52 UTC www.freepatentsonline.com [Source type: Academic]

^ The destruction of the neurons in the brain and spinal cord that control movement eventually progresses to the point that all voluntary motor control is lost.
  • Biomarkers for amyotrophic lateral sclerosis and methods using the same - Patent Application 20070298998 19 January 2010 9:52 UTC www.freepatentsonline.com [Source type: Academic]

.Cognitive function is generally spared except in certain situations such as when ALS is associated with frontotemporal dementia.^ ALS/frontotemporal dementia (FTD) syndrome .
  • Amyotrophic Lateral Sclerosis Overview -- GeneReviews -- NCBI Bookshelf 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

^ Overlap syndromes exist, such as ALS-Parkinson-Dementia Complex of Guam where an association with a food chain concentration of the neurotoxic amino acid beta-methylamino-l-alanine (BMAA) [ 5 ] has been found.
  • BioMed Central | Full text | Widespread sensorimotor and frontal cortical atrophy in Amyotrophic Lateral Sclerosis 19 January 2010 9:52 UTC www.biomedcentral.com [Source type: Academic]

^ Patients meeting criteria for dementia not typical of a frontotemporal lobar degeneration shall be diagnosed with ALS-dementia (ie, ALS- Alzheimer disease , ALS-vascular dementia, ALS-mixed dementia).
  • Dementia associated with amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.medlink.com [Source type: Academic]

[2] .However, there are reports of more subtle cognitive changes of the frontotemporal type in many patients when detailed neuropsychological testing is employed.^ Traditionally, cognition was thought to be spared in ALS. Cognitive or behavioural features consistent with frontotemporal degeneration, however, have been observed, 10,11 and neuropsychologic evaluation can often identify personality changes, deficits in verbal fluency, and difficulty with planning and abstraction.
  • Amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.cfpc.ca [Source type: Academic]

^ As it was families and many of the athletes have been in the water this week, and there have been no reports of any illnesses.
  • SolarBee® Science Office 20 September 2009 17:15 UTC www.solarbee.com [Source type: News]

^ Formal neuropsychological testing can identify subtle alterations which may be masked by socially favorable traits such as empathy and optimism.
  • Amyotrophic Lateral Sclerosis Overview -- GeneReviews -- NCBI Bookshelf 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

.Sensory nerves and the autonomic nervous system, which controls functions like sweating, generally remain functional.^ Neurological: Having to do with the nerves or the nervous system.
  • Amyotrophic Lateral Sclerosis Glossary of Terms with Definitions on MedicineNet.com 19 January 2010 9:52 UTC www.medicinenet.com [Source type: Academic]

^ INTRODUCTION: The effects of Nerve Growth Factor (NGF) within and outside the nervous system have been amply discussed in recent decades.
  • Amyotrophic Lateral Sclerosis (ALS) - Abstracts : Online Reference for Health Concerns 19 January 2010 9:52 UTC www.lef.org [Source type: Academic]

^ Thus, they are also more likely to be recognized and degraded via the quality control systems and the proteasome.
  • Disulphide-reduced superoxide dismutase-1 in CNS of transgenic amyotrophic lateral sclerosis models -- Jonsson et al. 129 (2): 451 -- Brain 19 January 2010 9:52 UTC brain.oxfordjournals.org [Source type: Academic]

Contents

Symptoms

Initial symptoms

.The onset of ALS may be so subtle that the symptoms are frequently overlooked.^ In the case of ALS, putative causes need to precede not only clinical onset, but also biologic onset that may occur several years before the first clinical symptoms emerge.
  • Amyotrophic Lateral Sclerosis: eMedicine Neurology 19 January 2010 9:52 UTC emedicine.medscape.com [Source type: Academic]

^ By designing a careful regimen of nutrients and supplements, individuals with ALS may be able to blunt the effects of individual factors, thereby slowing the progression of the disease and lessening its symptoms.
  • Amyotrophic Lateral Sclerosis (ALS) - Lou Gehrig's Disease, Free Radical Damage, Glutamate - Life Extension Health Concern 19 January 2010 9:52 UTC www.lef.org [Source type: Academic]

^ Further, subjects with ALS may generally metabolize xenobiotics differently than healthy individuals or individuals suffering from illnesses that have symptoms similar to ALS symptoms.
  • Biomarkers for amyotrophic lateral sclerosis and methods using the same - Patent Application 20070298998 19 January 2010 9:52 UTC www.freepatentsonline.com [Source type: Academic]

[3] .The earliest symptoms are obvious weakness and/or muscle atrophy.^ A spontaneous disease of the horse, known as equine motoneuron disease, is characterized by a generalized weakness, progressive muscle atrophy and loss of motoneurons in the spinal cord and brainstem [ 148 ].
  • Molecular Neurodegeneration | Full text | Experimental models for the study of neurodegeneration in amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.molecularneurodegeneration.com [Source type: Academic]

^ Regardless of initial symptoms, atrophy and weakness eventually affect other muscles.
  • Amyotrophic Lateral Sclerosis Overview -- GeneReviews -- NCBI Bookshelf 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

^ The muscles atrophy quickly, causing weakness, paralysis, and eventual death, usually when the muscles that control respiration fail.
  • amyotrophic lateral sclerosis Facts, information, pictures | Encyclopedia.com articles about amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.encyclopedia.com [Source type: Academic]

.This is followed by twitching, cramping, or stiffness of affected muscles; muscle weakness affecting an arm or a leg; and/or slurred and nasal speech.^ Six of the 9 patients had asymmetric weakness; in 4 the distal arm muscles were affected.

^ This eventually leads to muscle weakening, twitching, and an inability to move the arms, legs, and body.
  • Amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.drexelmed.edu [Source type: Academic]

^ Medications such as baclofen and benzodiazepines can help relieve spasticity and muscle cramps; however, weakness and lethargy are common side effects.
  • Amyotrophic Lateral Sclerosis Overview -- GeneReviews -- NCBI Bookshelf 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

The twitching, cramping, etc. associated with ALS is a result of the dying motor neurons, therefore these symptoms without clinical weakness or atrophy of affected muscle is likely not ALS.
.The parts of the body affected by early symptoms of ALS depend on which motor neurons in the body are damaged first.^ ALS is a rapidly progerssive neuromuscular disease that destroys both upper and lower motor neurons, ultimately causing death, typically from respiratory failure.
  • CannabisMD Reports : Survey of Cannabis Use in Patients with Amyotrophic Lateral Sclerosis 19 January 2010 9:52 UTC www.cannabismd.org [Source type: Academic]

^ Because NAA is synthesized by mitochondria in an energy-dependent manner and the NAA/Cho metabolite ratios in the ALS motor cortices were found to be correlated to the degree of mitochondrial pathology in ALS skeletal muscle, our results can be explained by a deficiency of enzymes of mitochondrial respiratory chain in the ALS motor cortex which might affect motor neuron survival.

^ Misfolding and precipitation of the abnormal (and normal) SOD1 proteins are thought to be part of the pathophysiology of SOD1 ALS, but why the disease begins when it does, and how it causes the lower motor neuron ALS phenotype is not clear.
  • Amyotrophic Lateral Sclerosis: eMedicine Neurology 19 January 2010 9:52 UTC emedicine.medscape.com [Source type: Academic]

.About 75% of people experience "limb onset" ALS. In some of these cases, symptoms initially affect one of the legs, and patients experience awkwardness when walking or running or they notice that they are tripping or stumbling more often.^ The experience of ALS is unique to each patient and family.
  • Amyotrophic Lateral Sclerosis: Lou Gehrig's Disease - March 15, 1999 -American Academy of Family Physicians 20 September 2009 17:15 UTC www.aafp.org [Source type: Academic]

^ About 75% of patients present with onset in the limbs; about 21% present with onset in the bulbar area.
  • Amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]
  • Amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.cfpc.ca [Source type: Academic]

^ However, there are well-documented cases of patients with ALS having one or more of these "exceptions".
  • Introduction to ALS 19 January 2010 9:52 UTC www.als.ca [Source type: Academic]

.Other limb onset patients first see the effects of the disease on a hand or arm as they experience difficulty with simple tasks requiring manual dexterity such as buttoning a shirt, writing, or turning a key in a lock.^ They may have difficulty lifting objects or with tasks that require manual dexterity (e.g., buttoning a shirt, tying a shoe, turning a key).
  • Amyotrophic Lateral Sclerosis (ALS) 19 January 2010 9:52 UTC www.asha.org [Source type: Academic]

^ Other limb onset patients first see the effects of the disease on a hand or arm as they experience difficulty with simple tasks requiring manual dexterity such as buttoning a shirt, writing, or turning a key in a lock.

^ Some patients first see the effects of the disease on a hand or arm as they experience difficulty with simple tasks requiring manual dexterity such as buttoning a shirt, writing, or turning a key in a lock.
  • Amyotrophic Lateral Sclerosis Fact Sheet: National Institute of Neurological Disorders and Stroke (NINDS) 19 January 2010 9:52 UTC www.ninds.nih.gov [Source type: Academic]

.Occasionally the symptoms remain confined to one limb; this is known as monomelic amyotrophy.^ Monomelic amyotrophy — Monomelic amyotrophy, also known as focal amyotrophy, is a condition that presents clinically with juvenile onset of focal atrophy and weakness, most commonly of a single hand and arm and rarely of a leg.
  • Diagnosis of amyotrophic lateral sclerosis - a knol by Internet Medical Publishing 19 January 2010 9:52 UTC knol.google.com [Source type: Academic]

^ Monomelic amyotrophy — Monomelic amyotrophy, also known as focal amyotrophy, is a condition that presents clinically with juvenile onset of focal...
  • Diagnosis of amyotrophic lateral sclerosis - a knol by Internet Medical Publishing 19 January 2010 9:52 UTC knol.google.com [Source type: Academic]

^ Benign monomelic amyotrophy of lower limb: a rare entity with a characteristic muscular CT. J Neurol Sci 1994; 126:153.
  • Diagnosis of amyotrophic lateral sclerosis - a knol by Internet Medical Publishing 19 January 2010 9:52 UTC knol.google.com [Source type: Academic]

.About 25% of cases are "bulbar onset" ALS. These patients first notice difficulty speaking clearly.^ About 25% of cases are "bulbar onset" ALS. These patients first notice difficulty speaking clearly.

^ Approximately 25% of ALS patients have difficulty speaking as their first symptom.
  • Amyotrophic Lateral Sclerosis ALS: rockin for a cure 19 January 2010 9:52 UTC www.rockinforacure.org [Source type: Academic]

^ Bulbar ALS is the type of ALS where speaking and swallowing difficulties are the first symptoms.
  • Amyotrophic Lateral Sclerosis ALS: rockin for a cure 19 January 2010 9:52 UTC www.rockinforacure.org [Source type: Academic]

.Speech becomes garbled and slurred.^ Speech becomes garbled and slurred.

^ Speech may become slurred, and later there is difficulty chewing or swallowing.
  • Amyotrophic lateral sclerosis - Genetics Home Reference 19 January 2010 9:52 UTC ghr.nlm.nih.gov [Source type: Academic]

^ He or she may become hoarse or tired after speaking or may have slurred speech.
  • amyotrophic lateral sclerosis Facts, information, pictures | Encyclopedia.com articles about amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.encyclopedia.com [Source type: Academic]

.Nasality and loss of volume are frequently the first symptoms.^ Nasality and loss of volume are frequently the first symptoms.

^ First symptoms may be sleep disturbances, nightmares, frequent waking, morning headache and daytime sleepiness as well as shortness of breath on exertion.

^ Symptoms such as nausea, vomiting, loss of appetite, and increased frequency of bowel movements may occur with any cholinesterase inhibitor.
  • UCSF Memory and Aging Center | Treatments and Medications 19 January 2010 9:52 UTC memory.ucsf.edu [Source type: Academic]

.Difficulty swallowing, and loss of tongue mobility follow.^ Difficulty swallowing, and loss of tongue mobility follow.

^ There may be muscle spasms, weight loss, and difficulty in breathing, eating, and swallowing.
  • amyotrophic lateral sclerosis Facts, information, pictures | Encyclopedia.com articles about amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.encyclopedia.com [Source type: Academic]

^ Within a month, the patient had slurred speech, difficulty in swallowing liquids and fasciculations of the tongue.
  • Amyotrophic Lateral Sclerosis: Lou Gehrig's Disease - March 15, 1999 -American Academy of Family Physicians 20 September 2009 17:15 UTC www.aafp.org [Source type: Academic]
  • Amyotrophic Lateral Sclerosis: Lou Gehrig's Disease - CareCure Forums 19 January 2010 9:52 UTC sci.rutgers.edu [Source type: Academic]

Eventually total loss of speech and the inability to protect the airway when swallowing are experienced.
.Regardless of the part of the body first affected by the disease, muscle weakness and atrophy spread to other parts of the body as the disease progresses.^ As the disease progresses weakness tends to spread and become bilateral.

^ Regardless of the part of the body first affected by the disease, muscle weakness and atrophy spread to other parts of the body as the disease progresses.
  • Amyotrophic Lateral Sclerosis Fact Sheet: National Institute of Neurological Disorders and Stroke (NINDS) 19 January 2010 9:52 UTC www.ninds.nih.gov [Source type: Academic]
  • Amyotrophic Lateral Sclerosis -- TheBrainMatters.org 19 January 2010 9:52 UTC www.thebrainmatters.org [Source type: Academic]
  • Amyotrophic Lateral Sclerosis (ALS) 19 January 2010 9:52 UTC www.livingwithcerebralpalsy.com [Source type: Academic]

^ Regardless of the part of the body first affected by the disease, it is usual for muscle weakness and atrophy to spread to other parts of the body as the disease progresses.
  • Fighting Amyotrophic Lateral Sclerosis (ALS), alias Lou Gehrig's Disease 19 January 2010 9:52 UTC www.squidoo.com [Source type: Academic]

.Patients experience increasing difficulty moving, swallowing (dysphagia), and speaking or forming words (dysarthria).^ See the entire definition of Dysarthria Dysphagia: Difficulty in swallowing, swallowing problems .
  • Amyotrophic Lateral Sclerosis Glossary of Terms with Definitions on MedicineNet.com 19 January 2010 9:52 UTC www.medicinenet.com [Source type: Academic]

^ Difficulties in speaking (dysarthria) and swallowing (dysphagia) may occur in the course of the disease.

^ Individuals have increasing problems with moving, swallowing, and speaking or forming words.
  • Amyotrophic Lateral Sclerosis -- TheBrainMatters.org 19 January 2010 9:52 UTC www.thebrainmatters.org [Source type: Academic]

.Symptoms of upper motor neuron involvement include tight and stiff muscles (spasticity) and exaggerated reflexes (hyperreflexia) including an overactive gag reflex.^ Symptoms of upper motor neuron damage include stiffness (spasticity), muscle twitching (fasciculations), and muscle shaking (clonus).
  • Amyotrophic Lateral Sclerosis ALS: rockin for a cure 19 January 2010 9:52 UTC www.rockinforacure.org [Source type: Academic]

^ The earliest symptoms include muscle twitching, cramping, stiffness, or weakness.
  • Amyotrophic lateral sclerosis - Genetics Home Reference 19 January 2010 9:52 UTC ghr.nlm.nih.gov [Source type: Academic]

^ Symptoms of lower motor neuron damage include muscle weakness and muscle shrinking (atrophy).
  • Amyotrophic Lateral Sclerosis ALS: rockin for a cure 19 January 2010 9:52 UTC www.rockinforacure.org [Source type: Academic]

.An abnormal reflex commonly called Babinski's sign (the big toe extends upward and other toes spread out) also indicates upper motor neuron damage.^ None had definite upper motor neuron signs or muscle cramps.

^ Damage to upper motor neurons can result in spasticity and exaggerated reflexes.
  • Amyotrophic Lateral Sclerosis Glossary of Terms with Definitions on MedicineNet.com 19 January 2010 9:52 UTC www.medicinenet.com [Source type: Academic]

^ Upper motor neuron signs include the following: .
  • Diagnosis of amyotrophic lateral sclerosis - a knol by Internet Medical Publishing 19 January 2010 9:52 UTC knol.google.com [Source type: Academic]

.Symptoms of lower motor neuron degeneration include muscle weakness and atrophy, muscle cramps, and fleeting twitches of muscles that can be seen under the skin (fasciculations).^ Fasciculations are tiny movements of muscle beneath the skin while cramps are coarser.

^ Symptoms of upper motor neuron damage include stiffness (spasticity), muscle twitching (fasciculations), and muscle shaking (clonus).
  • Amyotrophic Lateral Sclerosis ALS: rockin for a cure 19 January 2010 9:52 UTC www.rockinforacure.org [Source type: Academic]

^ Fasciculation is a sign of muscle irritability, as the normal action of the lower motor neuron on the muscle is impaired.
  • Les Turner ALS Foundation - What Is ALS? 19 January 2010 9:52 UTC www.lesturnerals.org [Source type: Academic]

.Around 15–45% of patients experience pseudobulbar affect, also known as "emotional lability", which consists of uncontrollable laughter, crying or smiling, attributable to degeneration of bulbar upper motor neurons resulting in exaggeration of motor expressions of emotion.^ Damage to upper motor neurons can result in spasticity and exaggerated reflexes.
  • Amyotrophic Lateral Sclerosis Glossary of Terms with Definitions on MedicineNet.com 19 January 2010 9:52 UTC www.medicinenet.com [Source type: Academic]

^ ALS affects the motor neurons* of the body.
  • amyotrophic lateral sclerosis Facts, information, pictures | Encyclopedia.com articles about amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.encyclopedia.com [Source type: Academic]

^ Evidence of upper motor neuron (UMN) degeneration by clinical examination AND .
  • Amyotrophic Lateral Sclerosis Overview -- GeneReviews -- NCBI Bookshelf 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

.To be diagnosed with ALS, patients must have signs and symptoms of both upper and lower motor neuron damage that cannot be attributed to other causes.^ Other ALS-causing genes .
  • Facts About ALS | MDA/ALS Publications 19 January 2010 9:52 UTC www.als-mda.org [Source type: Academic]

^ Lou Gehrig's disease; ALS; Upper and lower motor neuron disease; Motor neuron disease .
  • Amyotrophic Lateral Sclerosis - Symptoms, Diagnosis, Treatment of Amyotrophic Lateral Sclerosis - NY Times Health Information 19 January 2010 9:52 UTC health.nytimes.com [Source type: FILTERED WITH BAYES]
  • Amyotrophic Lateral Sclerosis - Symptoms, Diagnosis, Treatment of Amyotrophic Lateral Sclerosis - NY Times Health Information 19 January 2010 9:52 UTC health.nytimes.com [Source type: FILTERED WITH BAYES]
  • Amyotrophic lateral sclerosis - Health Information 19 January 2010 9:52 UTC www.qualityhealth.com [Source type: Academic]
  • Amyotrophic lateral sclerosis: MedlinePlus Medical Encyclopedia 19 January 2010 9:52 UTC www.nlm.nih.gov [Source type: Academic]
  • Amyotrophic lateral sclerosis - Lifespan 19 January 2010 9:52 UTC www.lifespan.org [Source type: Academic]
  • Walgreens.com | Health Library | Print - Amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.walgreens.com [Source type: Academic]
  • Saint Thomas Health Services - Health Information - Amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.sths.com [Source type: Academic]
  • Amyotrophic lateral sclerosis - Baylor Health 19 January 2010 9:52 UTC info.baylorhealth.com [Source type: Academic]
  • DHMC | Health Encyclopedia | Amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.dhmc.org [Source type: Academic]
  • Amyotrophic lateral sclerosis - Hillcrest Health System 19 January 2010 9:52 UTC hillcrest2.adam.com [Source type: Academic]
  • Amyotrophic lateral sclerosis - Kootenai Health 19 January 2010 9:52 UTC kootenaihealth.adam.com [Source type: Academic]
  • Amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.drexelmed.edu [Source type: Academic]
  • Amyotrophic lateral sclerosis - Lifespan 19 January 2010 9:52 UTC wwwwww.nwcr.ws [Source type: Academic]
  • Amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.pennmedicine.org [Source type: Academic]

^ ALS has features of both upper and lower motor neuron damage.
  • Otolaryngology Aspects of Amyotrophic Lateral Sclerosis 19 January 2010 9:52 UTC www.bcm.edu [Source type: Academic]

Emerging symptoms

.Although the sequence of emerging symptoms and the rate of disease progression vary from person to person, eventually most patients are not able to stand or walk, get in or out of bed on their own, or use their hands and arms.^ It is a degenerative disease of upper and lower motor neurons that produces progressive weakness of voluntary muscles, with eventual death.
  • Use of pramipexole to treat amyotrophic lateral sclerosis - Patent 7157480 19 January 2010 9:52 UTC www.freepatentsonline.com [Source type: Academic]

^ Interpretation of the significance of an SOD1 mutation regarding disease severity and progression depends on the specific mutation identified because of wide variability in genotype / phenotype correlations.
  • Amyotrophic Lateral Sclerosis Overview -- GeneReviews -- NCBI Bookshelf 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

^ The disease progresses most slowly in those who are young and have their first symptoms in the arms and legs.
  • amyotrophic lateral sclerosis Facts, information, pictures | Encyclopedia.com articles about amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.encyclopedia.com [Source type: Academic]

.Difficulty swallowing and chewing impair the patient's ability to eat normally and increase the risk of choking.^ How do ALS patients eat when they lose their ability to chew and swallow?
  • Amyotrophic Lateral Sclerosis ALS: rockin for a cure 19 January 2010 9:52 UTC www.rockinforacure.org [Source type: Academic]

^ Difficulty swallowing and chewing impair the patient's ability to eat normally and increase the risk of choking.
  • Amyotrophic Lateral Sclerosis Fact Sheet: National Institute of Neurological Disorders and Stroke (NINDS) 19 January 2010 9:52 UTC www.ninds.nih.gov [Source type: Academic]
  • Fighting Amyotrophic Lateral Sclerosis (ALS), alias Lou Gehrig's Disease 19 January 2010 9:52 UTC www.squidoo.com [Source type: Academic]

^ Difficulty chewing and swallowing .
  • Living with Amyotrophic Lateral Sclerosis (ALS) 19 January 2010 9:52 UTC www.med-help.net [Source type: Academic]

Maintaining weight can then become a problem. .Because the disease usually does not affect cognitive abilities, patients are aware of their progressive loss of function and may become anxious and depressed.^ The disease does not affect a person's mental abilities.

^ Cognitive abilities usually are not affected.
  • CIGNA - Amyotrophic Lateral Sclerosis 19 January 2010 9:52 UTC www.cigna.com [Source type: Academic]

^ Because the disease usually does not affect cognitive abilities, patients are aware of their progressive loss of function and may become anxious and depressed.
  • Amyotrophic Lateral Sclerosis Fact Sheet: National Institute of Neurological Disorders and Stroke (NINDS) 19 January 2010 9:52 UTC www.ninds.nih.gov [Source type: Academic]
  • Fighting Amyotrophic Lateral Sclerosis (ALS), alias Lou Gehrig's Disease 19 January 2010 9:52 UTC www.squidoo.com [Source type: Academic]

.A small percentage of patients go on to develop frontotemporal dementia characterized by profound personality changes; this is more common among those with a family history of dementia.^ Frontotemporal dementia (FTD) is a profound alteration in personality and social conduct characterized by loss of volition and insight, social disinhibition, and distractibility, with preservation of memory function [ Neary et al 1998 ].
  • Amyotrophic Lateral Sclerosis Overview -- GeneReviews -- NCBI Bookshelf 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

^ A clinical and pathological study of a Japanese case of amyotrophic lateral sclerosis/parkinsonism-dementia complex with family history.
  • Dementia associated with amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.medlink.com [Source type: Academic]

^ About 10 percent of familial ALS patients have been associated with more than 50 mutations of the gene of the Cu/Zn superoxide dismutase (SOD1).

.A larger proportion of patients experience mild problems with word-generation, attention, or decision-making.^ A larger proportion of patients experience mild problems with word-generation, attention, or decision-making.
  • Fighting Amyotrophic Lateral Sclerosis (ALS), alias Lou Gehrig's Disease 19 January 2010 9:52 UTC www.squidoo.com [Source type: Academic]

^ Patients experience increasing difficulty moving, swallowing (dysphagia), and speaking or forming words (dysarthria).

^ This years total brings to over $700,000 in research money generated by Ride for Life  patients working together to make a difference.
  • Ride For Life | Helping People Living With ALS - Lou Gehrig's Disease 19 January 2010 9:52 UTC rideforlife.com [Source type: Academic]

.Cognitive function may be affected as part of the disease process or could be related to poor breathing at night (nocturnal hypoventilation).^ Cognitive function may be affected as part of the disease process or could be related to poor breathing at night (nocturnal hypoventilation).
  • Fighting Amyotrophic Lateral Sclerosis (ALS), alias Lou Gehrig's Disease 19 January 2010 9:52 UTC www.squidoo.com [Source type: Academic]

^ Often referred to as "Lou Gehrig's disease" (a famous baseball player who died from the disease), it is one of the most devastating of the disorders that affects the function of nerves and muscles.
  • Amyotrophic Lateral Sclerosis (ALS) 19 January 2010 9:52 UTC www.healthsystem.virginia.edu [Source type: Academic]
  • Amyotrophic Lateral Sclerosis (ALS) 19 January 2010 9:52 UTC www.montefiore.org [Source type: Academic]
  • Main Line Health - Amyotrophic Lateral Sclerosis (ALS) 19 January 2010 9:52 UTC www.mainlinehealth.org [Source type: Academic]

^ Dysarthria, exaggeration of motor expressions, and emotional lability (pseudobulbar affect) may occur when the disease process involves the corticobulbar projections to the brainstem.
  • Amyotrophic Lateral Sclerosis: eMedicine Emergency Medicine 19 January 2010 9:52 UTC emedicine.medscape.com [Source type: Academic]

.Health care professionals need to explain the course of the disease and describe available treatment options so that patients can make informed decisions in advance.^ Health care professionals need to explain the course of the disease and describe available treatment options so that patients can make informed decisions in advance.
  • Amyotrophic Lateral Sclerosis Fact Sheet: National Institute of Neurological Disorders and Stroke (NINDS) 19 January 2010 9:52 UTC www.ninds.nih.gov [Source type: Academic]
  • Fighting Amyotrophic Lateral Sclerosis (ALS), alias Lou Gehrig's Disease 19 January 2010 9:52 UTC www.squidoo.com [Source type: Academic]

^ Always seek the advice of your physician or other qualified health professional before starting any new treatment, making any changes to existing treatment, or altering in any way your current exercise or diet regimen.
  • Amyotrophic Lateral Sclerosis (ALS) - Topic Overview - Yahoo! Health 20 September 2009 17:15 UTC health.yahoo.com [Source type: Academic]

^ The Lois Insolia ALS Center provides a multidisciplinary approach to patient care, including an individualized treatment plan guided by the patient's personal preferences and wishes.
  • Les Turner ALS Foundation - What Is ALS? 19 January 2010 9:52 UTC www.lesturnerals.org [Source type: Academic]

.As the diaphragm and intercostal muscles (rib cage) weaken, forced vital capacity and inspiratory pressure diminish.^ Forced vital capacity was measured and smoking status was noted.
  • Factors correlated with hypermetabolism in patients with amyotrophic lateral sclerosis -- Desport et al. 74 (3): 328 -- American Journal of Clinical Nutrition 19 January 2010 9:52 UTC www.ajcn.org [Source type: Academic]

^ The metabolite changes were also evaluated relative to changes in the Forced Vital Capacity (FVC), a clinical measurement that indicates disease severity.
  • Biomarkers for amyotrophic lateral sclerosis and methods using the same - Patent Application 20070298998 19 January 2010 9:52 UTC www.freepatentsonline.com [Source type: Academic]

^ Another random coefficient regression was performed for each compound on the FVC (forced vital capacity).
  • Biomarkers for amyotrophic lateral sclerosis and methods using the same - Patent Application 20070298998 19 January 2010 9:52 UTC www.freepatentsonline.com [Source type: Academic]

.In bulbar onset ALS, this may occur before significant limb weakness is apparent.^ This is called limb-onset ALS. .
  • Amyotrophic Lateral Sclerosis ALS: rockin for a cure 19 January 2010 9:52 UTC www.rockinforacure.org [Source type: Academic]

^ In 90 to 95% of all ALS cases, the disease occurs apparently at random with no clearly associated risk factors.
  • Amyotrophic Lateral Sclerosis (ALS) Support Group - DailyStrength 19 January 2010 9:52 UTC www.dailystrength.org [Source type: FILTERED WITH BAYES]

^ Those with upper limb onset may first notice difficulty in actions such as buttoning clothes, picking up small objects or turning keys.
  • Amyotrophic Lateral Sclerosis: Lou Gehrig's Disease - March 15, 1999 -American Academy of Family Physicians 20 September 2009 17:15 UTC www.aafp.org [Source type: Academic]
  • Amyotrophic Lateral Sclerosis: Lou Gehrig's Disease - CareCure Forums 19 January 2010 9:52 UTC sci.rutgers.edu [Source type: Academic]

.Bilevel positive pressure ventilation (frequently referred to by the tradename BiPAP) is frequently used to support breathing, first at night, and later during the daytime as well.^ Another form of breathing support, known as invasive ventilation , delivers air through a hole in the trachea, or windpipe.
  • Facts About ALS | MDA/ALS Publications 19 January 2010 9:52 UTC www.als-mda.org [Source type: Academic]

^ Bach JR, Alba AS. Intermittent abdominal pressure ventilator in a regimen of noninvasive ventilatory support.
  • Dr. John R. Bach - The DMD Doc 19 January 2010 9:52 UTC www.doctorbach.com [Source type: Academic]

^ This type of machine is often called a BiPAP, for bilevel positive airway pressure.
  • Facts About ALS | MDA/ALS Publications 19 January 2010 9:52 UTC www.als-mda.org [Source type: Academic]

.It is recommended that long before BiPAP becomes insufficient, patients must decide whether to have a tracheostomy and long term mechanical ventilation.^ They also discussed questions concerning the benefits of this type of support, with the aim of letting patients decide on their manner of treatment, with full awareness and authorization regarding whether or not their suffering should be prolonged by using this ventilation support.
  • Sao Paulo Medical Journal - Physiotherapeutic conduct in amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.scielo.br [Source type: Academic]

^ Patients with amyotrophic lateral sclerosis receiving long-term mechanical ventilation: advance care planning and outcomes.
  • Dr. John R. Bach - The DMD Doc 19 January 2010 9:52 UTC www.doctorbach.com [Source type: Academic]

^ Blexrud, MD, Windebank, AJ, Daube, JR. Long-term follow-up of 121 patients with benign fasciculations.
  • Diagnosis of amyotrophic lateral sclerosis - a knol by Internet Medical Publishing 19 January 2010 9:52 UTC knol.google.com [Source type: Academic]

.At this point, some patients choose palliative hospice care.^ Recently, it was found that some patients with autosomal-dominant familial ALS (FALS) have point mutations in the gene that encodes Cu/Zn superoxide dismutase (SOD1).
  • Amyotrophic Lateral Sclerosis (ALS) - Abstracts : Online Reference for Health Concerns 19 January 2010 9:52 UTC www.lef.org [Source type: Academic]

^ Some ALS patients choose to use them for only short periods of time or not at all.
  • amyotrophic lateral sclerosis Facts, information, pictures | Encyclopedia.com articles about amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.encyclopedia.com [Source type: Academic]

.Most people with ALS die of respiratory failure or pneumonia.^ ALS is a rapidly progerssive neuromuscular disease that destroys both upper and lower motor neurons, ultimately causing death, typically from respiratory failure.
  • CannabisMD Reports : Survey of Cannabis Use in Patients with Amyotrophic Lateral Sclerosis 19 January 2010 9:52 UTC www.cannabismd.org [Source type: Academic]

^ Since severe bulbar dysfunction is essentially inevitable in ALS, respiratory failure has to eventually develop from saliva obstructing the airways or during intercurrent chest infections.
  • Dr. John R. Bach - The DMD Doc 19 January 2010 9:52 UTC www.doctorbach.com [Source type: Academic]

^ In the majority of ALS patients, death is due to respiratory failure caused by the denervation of the respiratory muscles and diaphragm.
  • Molecular Neurodegeneration | Full text | Experimental models for the study of neurodegeneration in amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.molecularneurodegeneration.com [Source type: Academic]

.Death usually occurs within two to five years of diagnosis.^ The evolution of the disease is rapid, with death generally occurring between two and five years after diagnosis.
  • Sao Paulo Medical Journal - Physiotherapeutic conduct in amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.scielo.br [Source type: Academic]

^ Death, usually from respiratory insufficiency or aspiration, generally occurs within 3 to 5 years ( 1 ).
  • Resilience and Distress Among Amyotrophic Lateral Sclerosis Patients and Caregivers -- Rabkin et al. 62 (2): 271 -- Psychosomatic Medicine 19 January 2010 9:52 UTC www.psychosomaticmedicine.org [Source type: Academic]

^ Once diagnosed, the disease progresses rapidly, and most patients die within three to five years after symptoms appear.
  • Amyotrophic Lateral Sclerosis (ALS) - Lou Gehrig's Disease, Free Radical Damage, Glutamate - Life Extension Health Concern 19 January 2010 9:52 UTC www.lef.org [Source type: Academic]

.Although the disease can strike at any age, most people are between forty and seventy years of age when the disease strikes and men are affected slightly more frequently than women.^ Men are affected more frequently than women.
  • Amyotrophic Lateral Sclerosis: eMedicine Neurology 19 January 2010 9:52 UTC emedicine.medscape.com [Source type: Academic]

^ Men are somewhat more likely to develop ALS than are women.
  • Facts About ALS | MDA/ALS Publications 19 January 2010 9:52 UTC www.als-mda.org [Source type: Academic]

^ In Brazil, the characteristics are similar to those found in Europe and North America, with a higher prevalence in men (gender ratio of 3:2) and an average onset age of 57 years.
  • Sao Paulo Medical Journal - Physiotherapeutic conduct in amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.scielo.br [Source type: Academic]

.An estimated 5,000 people in the United States are diagnosed with the disease each year [4].^ Approximately 20,000 people in the United States have ALS, and 5,000 people are diagnosed with ALS each year.
  • Biomarkers for amyotrophic lateral sclerosis and methods using the same - Patent Application 20070298998 19 January 2010 9:52 UTC www.freepatentsonline.com [Source type: Academic]

^ Therefore, disease prevalence is estimated at 6 per 100,000 population.
  • Amyotrophic Lateral Sclerosis: eMedicine Neurology 19 January 2010 9:52 UTC emedicine.medscape.com [Source type: Academic]

^ In the United States, ALS also is called Lou Gehrig’s disease, named for the Yankees baseball player who died of it in 1941.
  • Facts About ALS | MDA/ALS Publications 19 January 2010 9:52 UTC www.als-mda.org [Source type: Academic]

.ALS, a progressive disease, leads to death in half of the people diagnosed within three years and ninety percent within six years.^ Death usually occurs within 2 to 5 years.
  • SOD1 Gene - GeneCards | SODC Protein | SODC Antibody 19 January 2010 9:52 UTC www.genecards.org [Source type: Academic]

^ Most people die within 3 years of being diagnosed.
  • Natural & Alternative Treatments - Hartford Hospital, Connecticut 19 January 2010 9:52 UTC www.harthosp.org [Source type: Academic]

^ ALS is a rapidly progerssive neuromuscular disease that destroys both upper and lower motor neurons, ultimately causing death, typically from respiratory failure.
  • CannabisMD Reports : Survey of Cannabis Use in Patients with Amyotrophic Lateral Sclerosis 19 January 2010 9:52 UTC www.cannabismd.org [Source type: Academic]

.ALS predominantly affects the motor neurons, and in the majority of cases the disease does not impair a patient's mind, personality, intelligence, or memory.^ Diagnosis and management of motor neurone disease.
  • NEJM -- Whole-Genome Analysis of Sporadic Amyotrophic Lateral Sclerosis 19 January 2010 9:52 UTC content.nejm.org [Source type: Academic]

^ In other countries, ALS is sometimes called Motor Neuron Disease.
  • ALS Information 20 September 2009 17:15 UTC www.cwfo.org [Source type: Academic]

^ ALS is a neurodegenerative disease that affects both upper and lower motor neurons.
  • ATSDR - Multiple Sclerosis and Amyotrophic Lateral Sclerosis 19 January 2010 9:52 UTC www.atsdr.cdc.gov [Source type: Academic]

.Nor does it affect a person's ability to see, smell, taste, hear, or feel touch.^ ALS does not affect a person's ability to see, smell, taste, hear, or recognize touch.
  • Amyotrophic Lateral Sclerosis Fact Sheet: National Institute of Neurological Disorders and Stroke (NINDS) 19 January 2010 9:52 UTC www.ninds.nih.gov [Source type: Academic]

^ ALS does not affect the senses (sight, smell, taste, hearing, touch), bladder or bowel function, or a person's ability to think or reason.
  • Amyotrophic lateral sclerosis Medical Information 19 January 2010 9:52 UTC www.drugs.com [Source type: Academic]
  • GAMC Glendale, CA - Amyotrophic lateral sclerosis. Amyotrophic lateral sclerosis, or ALS, is ... 19 January 2010 9:52 UTC www.glendaleadventist.com [Source type: Academic]
  • Amyotrophic lateral sclerosis - Baylor Health 19 January 2010 9:52 UTC info.baylorhealth.com [Source type: Academic]
  • Amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.drexelmed.edu [Source type: Academic]
  • Amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.pennmedicine.org [Source type: Academic]
  • Amyotrophic lateral sclerosis Information at myOptumHealth 19 January 2010 9:52 UTC www.myoptumhealth.com [Source type: Academic]

^ The disease does not affect a person's mental abilities.

.Control of eye muscles is the most preserved function, although some patients with an extremely long duration of disease (20+ years) may lose eye control too.^ Cannabis may be an option for some patients.
  • Symptom Management of the Patient With Amyotrophic Lateral Sclerosis: A Guide for Hospice Nurses 19 January 2010 9:52 UTC www.nursingcenter.com [Source type: Academic]
  • Symptom Management of the Patient With Amyotrophic Lateral Sclerosis: A Guide for Hospice Nurses 19 January 2010 9:52 UTC www.nursingcenter.com [Source type: Academic]

^ Some people with the disease may lose some control over their emotional responses.
  • Amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.alzheimer-adna.com [Source type: Academic]
  • Cure Amyotrophic Lateral Sclerosis (ALS), Lou Gehrig's disease, pseudobulbar palsy 19 January 2010 9:52 UTC www.damo-qigong.net [Source type: Academic]

^ Baclofen may reduce muscle spasms in some patients.
  • CIGNA - Amyotrophic Lateral Sclerosis 19 January 2010 9:52 UTC www.cigna.com [Source type: Academic]

.Unlike multiple sclerosis, bladder and bowel control are usually preserved in ALS, although as a result of immobility and diet changes, intestinal problems such as constipation can require intensive management.^ Does ALS affect the bladder and bowel?
  • Amyotrophic Lateral Sclerosis ALS: rockin for a cure 19 January 2010 9:52 UTC www.rockinforacure.org [Source type: Academic]

^ No single cause for  amyotrophic lateral sclerosis (ALS) explains its entire pathology; indeed, there may be multiple causes resulting in phenotypic similarity.
  • Amyotrophic Lateral Sclerosis: eMedicine Emergency Medicine 19 January 2010 9:52 UTC emedicine.medscape.com [Source type: Academic]

^ Constipation results from involvement of abdominal and pelvic muscles, compounded by limited physical exercise, certain medical treatments, and a diet lacking in fiber ( 5 , 10 ).
  • Factors correlated with hypermetabolism in patients with amyotrophic lateral sclerosis -- Desport et al. 74 (3): 328 -- American Journal of Clinical Nutrition 19 January 2010 9:52 UTC www.ajcn.org [Source type: Academic]

Cause and risk factors

.Evidence favors the idea of a neurodegenerative chain-reaction in all neurodegenerative disorders, in which initial cellular 'faults' produce or induce some version of a cascade failure that leads to progressive neuronal loss, eventual loss of function, and progressively more serious consequences.^ The destruction of the neurons in the brain and spinal cord that control movement eventually progresses to the point that all voluntary motor control is lost.
  • Biomarkers for amyotrophic lateral sclerosis and methods using the same - Patent Application 20070298998 19 January 2010 9:52 UTC www.freepatentsonline.com [Source type: Academic]

^ It is a degenerative disease of upper and lower motor neurons that produces progressive weakness of voluntary muscles, with eventual death.
  • Use of pramipexole to treat amyotrophic lateral sclerosis - Patent 7157480 19 January 2010 9:52 UTC www.freepatentsonline.com [Source type: Academic]

^ All cases will end fatally, usually due to the natural course of the motor neuron disorder.
  • Dementia associated with amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.medlink.com [Source type: Academic]

.Given that familial ALS has been linked to a mutation on the gene coding for superoxide dismutase (a critical enzyme involved in the protection of mitochondria against oxidative stress - see next section), initial theorizing has focused on oxidative stress, a probable but nonspecific mechanism in virtually all neurodegenerative disorders.^ Mutations in superoxide dismutase 1 as cause of one form of familial ALS .
  • Molecular Neurodegeneration | Full text | Experimental models for the study of neurodegeneration in amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.molecularneurodegeneration.com [Source type: Academic]

^ Oxidative stress in ALS .
  • Molecular Neurodegeneration | Full text | Experimental models for the study of neurodegeneration in amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.molecularneurodegeneration.com [Source type: Academic]

^ A later discovery pinpointed a mutation in the gene that codes for an enzyme, superoxide dismutase (SOD), as responsible for a percentage of familial cases.
  • amyotrophic lateral sclerosis Facts, information, pictures | Encyclopedia.com articles about amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.encyclopedia.com [Source type: Academic]

.Contrary to popular conceptions, oxidative stress is probably not uniform at the cellular level, and much of it is probably created (and managed) within the mitochondria, the primary chemical energy generation system in the cell.^ These mtDNA mutations lead to abnormalities in functioning of energy production pathways in mitochondria, resulting in an excessive generation of damaging oxygen derivatives known as “reactive oxygen species” (ROS), including entities called “oxygen free radicals.” When ROS production exceeds the capacity of cellular mechanisms to remove/inactivate ROS, the condition known as “oxidative stress” exists.
  • Use of pramipexole to treat amyotrophic lateral sclerosis - Patent 7157480 19 January 2010 9:52 UTC www.freepatentsonline.com [Source type: Academic]

^ S (−) PPX has been shown by several groups to be neuroprotective in cellular and animal models of increased oxidative stress, including MPTP toxicity to dopamine neurons (see U.S. Pat.
  • Use of pramipexole to treat amyotrophic lateral sclerosis - Patent 7157480 19 January 2010 9:52 UTC www.freepatentsonline.com [Source type: Academic]

^ Accumulating evidence provides compelling support that a primary etiologic component of sporadic adult NDD relates to mitochondrial dysfunction and the resulting increased cellular oxidative stress.
  • Use of pramipexole to treat amyotrophic lateral sclerosis - Patent 7157480 19 January 2010 9:52 UTC www.freepatentsonline.com [Source type: Academic]

.Most so-called free radicals are indeed produced in the mitochondria, but there are extensive cellular defenses against oxidative stress in the mitochondria (in which superoxide dismutase plays an important but not exclusive role).^ These mtDNA mutations lead to abnormalities in functioning of energy production pathways in mitochondria, resulting in an excessive generation of damaging oxygen derivatives known as “reactive oxygen species” (ROS), including entities called “oxygen free radicals.” When ROS production exceeds the capacity of cellular mechanisms to remove/inactivate ROS, the condition known as “oxidative stress” exists.
  • Use of pramipexole to treat amyotrophic lateral sclerosis - Patent 7157480 19 January 2010 9:52 UTC www.freepatentsonline.com [Source type: Academic]

^ Cu 2+ /Zn 2+ superoxide dismutase 1 (SOD1) is an ubiquitously expressed cytoplasmic enzyme that catalyzes the dismutation of the superoxide radical (O 2 - ) into hydrogen peroxide and molecular oxygen and is an important free radical scavenging enzyme that protects cells against oxidative stress.
  • Molecular Neurodegeneration | Full text | Experimental models for the study of neurodegeneration in amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.molecularneurodegeneration.com [Source type: Academic]

^ S (−) PPX has been shown by several groups to be neuroprotective in cellular and animal models of increased oxidative stress, including MPTP toxicity to dopamine neurons (see U.S. Pat.
  • Use of pramipexole to treat amyotrophic lateral sclerosis - Patent 7157480 19 January 2010 9:52 UTC www.freepatentsonline.com [Source type: Academic]

.A critical issue is the emerging evidence that in aging, there is probably progressive damage to mitochondrial DNA and progressive failure of the mechanisms by which the mitochondria are protected from oxidative stress.^ Work by the inventor has shown that a critical cell component damaged by oxidative stress in cell models of AD and PD is a particular mitochondrial protein complex known as the “mitochondrial transition pore complex” (MTPC).
  • Use of pramipexole to treat amyotrophic lateral sclerosis - Patent 7157480 19 January 2010 9:52 UTC www.freepatentsonline.com [Source type: Academic]

^ How mitochondrial DNA becomes defective in SALS is not known; both maternally inherited and sporadically acquired mechanisms are possible.
  • Use of pramipexole to treat amyotrophic lateral sclerosis - Patent 7157480 19 January 2010 9:52 UTC www.freepatentsonline.com [Source type: Academic]

^ Creatine administration protected G93A transgenic mice from increases in biochemical indices of oxidative damage.
  • Amyotrophic Lateral Sclerosis (ALS) - Abstracts : Online Reference for Health Concerns 19 January 2010 9:52 UTC www.lef.org [Source type: Academic]

.There is work suggesting that progressive damage to the mitochondria is at the central mechanism in age-related change, implying that damage to the mitochondria may be a common denominator in all age-related neurodegenerative diseases.^ Oxidative damage in neurodegenerative disease.
  • Amyotrophic Lateral Sclerosis (ALS) - Abstracts : Online Reference for Health Concerns 19 January 2010 9:52 UTC www.lef.org [Source type: Academic]

^ The role of mitochondria in the pathogenesis of neurodegenerative diseases.
  • Amyotrophic Lateral Sclerosis (ALS) - Abstracts : Online Reference for Health Concerns 19 January 2010 9:52 UTC www.lef.org [Source type: Academic]

^ Common variation in three genes, including a noncoding variant in CFH, strongly influences risk of age-related macular degeneration.
  • PLoS ONE: Amyotrophic Lateral Sclerosis: An Emerging Era of Collaborative Gene Discovery 19 January 2010 9:52 UTC www.plosone.org [Source type: Academic]

.Most scientists and researchers however believe that this set of ideas is probably too nonspecific to explain individual neurodegenerative disorders such as ALS, and that more specific degenerative cascades at a cellular level, explaining the unique features and unique protein depositions of each neurodegenerative disorder, have yet to be outlined.^ The phenotypic data collected on individual controls in this collection is designed for use in gene discovery efforts in ALS as well as other neurodegenerative disorders.
  • PLoS ONE: Amyotrophic Lateral Sclerosis: An Emerging Era of Collaborative Gene Discovery 19 January 2010 9:52 UTC www.plosone.org [Source type: Academic]

^ Additional research is now being conducted to find out more about the cause or causes of ALS. .
  • amyotrophic lateral sclerosis Facts, information, pictures | Encyclopedia.com articles about amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.encyclopedia.com [Source type: Academic]

^ In another neurodegenerative disorder, Alzheimer's disease, the enzyme glutamine synthetase, which normally catalyzes glutamine formation, is elevated (Tzika et al., 1993 ).
  • Rats Expressing Human Cytosolic Copper-Zinc Superoxide Dismutase Transgenes with Amyotrophic Lateral Sclerosis: Associated Mutations Develop Motor Neuron Disease -- Nagai et al. 21 (23): 9246 -- Journal of Neuroscience 19 January 2010 9:52 UTC www.jneurosci.org [Source type: Academic]

.However, failures in the management of oxidative stress within the mitochondria can lead to a whole cascade of undesirable effects, including acceleration of apoptosis (programmed cell death), damage to other cellular organelles, and disorders of protein folding (an area of increasing interest in ALS and many other neurodegenerative disorders).^ Apoptosis is also called programmed cell death or cell suicide.
  • Amyotrophic Lateral Sclerosis Glossary of Terms with Definitions on MedicineNet.com 19 January 2010 9:52 UTC www.medicinenet.com [Source type: Academic]

^ Oxidative stress in ALS .
  • Molecular Neurodegeneration | Full text | Experimental models for the study of neurodegeneration in amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.molecularneurodegeneration.com [Source type: Academic]

^ But in ALS and other degenerative diseases, it’s possible that the cell death program is activated inappropriately.
  • Facts About ALS | MDA/ALS Publications 19 January 2010 9:52 UTC www.als-mda.org [Source type: Academic]

.Since neurons for the most part cannot be replaced (with some partial exceptions), any process that substantially up-regulates programmed cell death in aging within motor neuron populations will eventually lead to motor symptoms and eventual motor system failure.^ Under some circumstances, programmed cell death is normal.
  • Facts About ALS | MDA/ALS Publications 19 January 2010 9:52 UTC www.als-mda.org [Source type: Academic]

^ Most cells have a built-in “suicide” program known as programmed cell death , or apoptosis .
  • Facts About ALS | MDA/ALS Publications 19 January 2010 9:52 UTC www.als-mda.org [Source type: Academic]

^ ALS is a rapidly progerssive neuromuscular disease that destroys both upper and lower motor neurons, ultimately causing death, typically from respiratory failure.
  • CannabisMD Reports : Survey of Cannabis Use in Patients with Amyotrophic Lateral Sclerosis 19 January 2010 9:52 UTC www.cannabismd.org [Source type: Academic]

Evidence suggests that programmed cell death may be upregulated (as a primary cause of atrophy) in both upper and lower motor neuron populations in ALS.
.A major challenge for these perspectives however is the evidence that calorie restriction (thought to protect cells from oxidative stress through a variety of mechanisms but including enhanced mitochondrial biogenesis) actually accelerates ALS (see below), suggesting the apparently paradoxical possibility that upregulation of defenses against oxidative stress may play a role in neurodegeneration in ALS. All of this underlines that a final explanation for the degenerative processes of ALS remains to be established.^ But in ALS, free radicals may build to toxic levels and damage cells, through an attack process called oxidative stress .
  • Facts About ALS | MDA/ALS Publications 19 January 2010 9:52 UTC www.als-mda.org [Source type: Academic]

^ The association of viruses and ALS remains to be established.
  • Amyotrophic Lateral Sclerosis (ALS) - Abstracts : Online Reference for Health Concerns 19 January 2010 9:52 UTC www.lef.org [Source type: Academic]

^ Oxidative stressors are well established in sporadic ALS, however the etiology of increased oxidative stress remains obscure.
  • Use of pramipexole to treat amyotrophic lateral sclerosis - Patent 7157480 19 January 2010 9:52 UTC www.freepatentsonline.com [Source type: Academic]

.Despite not finding a definitive or primary cause for ALS, the onset of the disease has been linked to several factors, including: a virus; exposure to various neurotoxins or heavy metals; DNA defects; immune system abnormalities; occupational involvement in military service and elite sports (where the issue again may be toxic exposure); extreme stress; and enzyme abnormalities.^ Testing for heavy metals if exposure is suspected .
  • Amyotrophic Lateral Sclerosis Overview -- GeneReviews -- NCBI Bookshelf 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

^ Immune system abnormalities .
  • Facts About ALS | MDA/ALS Publications 19 January 2010 9:52 UTC www.als-mda.org [Source type: Academic]

^ ALS patients were diagnosed on the basis of characteristic clinical course, physical and electrodiagnostic findings, and absence of evidence of spondylotic myelopathy, paraproteinemias, hyperparathyroidism, Lyme disease, glycoprotein antibodies, and vitamin E toxicity.
  • Dr. John R. Bach - The DMD Doc 19 January 2010 9:52 UTC www.doctorbach.com [Source type: Academic]

.Surgeries involving the spinal cord have also been thought to play a role in the onset of ALS, possibly due to the increased burden of oxidative stress and inflammation after spinal cord injury or stress.^ Oxidative stress in ALS .
  • Molecular Neurodegeneration | Full text | Experimental models for the study of neurodegeneration in amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.molecularneurodegeneration.com [Source type: Academic]

^ A combination of oxidative stress, glutamate excitotoxicity, mitochondrial dysfunction, inflammation, and apoptosis has repeatedly been proposed [ Cleveland & Rothstein 2001 ] but not validated: .
  • Amyotrophic Lateral Sclerosis Overview -- GeneReviews -- NCBI Bookshelf 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

^ SOD1 is ubiquitously expressed, in many tissues at higher levels than in the brain and spinal cord (Marklund, 1984 ; Jonsson et al ., 2004 ).
  • Disulphide-reduced superoxide dismutase-1 in CNS of transgenic amyotrophic lateral sclerosis models -- Jonsson et al. 129 (2): 451 -- Brain 19 January 2010 9:52 UTC brain.oxfordjournals.org [Source type: Academic]

.There is a known hereditary factor in familial ALS (FALS); however, there is no known hereditary component in the 90–95% cases diagnosed as sporadic ALS. An inherited genetic defect on chromosome 21 (coding for superoxide dismutase) is associated with approximately 20% of familial cases of ALS.[5][6] This mutation is believed to be autosomal dominant.^ Familial ALS Of ALS cases, 5-10% are familial with a Mendelian pattern of inheritance.
  • Amyotrophic Lateral Sclerosis: eMedicine Neurology 19 January 2010 9:52 UTC emedicine.medscape.com [Source type: Academic]

^ About 10% of ALS cases are hereditary.
  • amyotrophic lateral sclerosis Facts, information, pictures | Encyclopedia.com articles about amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.encyclopedia.com [Source type: Academic]

^ Familial cases constitute 5 – 10% of cases which are caused by mutations of the human superoxide dismutase in 20% of familial ALS [ 6 , 7 ].
  • BioMed Central | Full text | Widespread sensorimotor and frontal cortical atrophy in Amyotrophic Lateral Sclerosis 19 January 2010 9:52 UTC www.biomedcentral.com [Source type: Academic]

.The children of those diagnosed with familial ALS have a higher risk factor for developing the disease; however, those who have close family members diagnosed with sporadic ALS have no greater a risk factor than the general population, suggesting again an environmental or other non-genetic cause.^ Other ALS-causing genes .
  • Facts About ALS | MDA/ALS Publications 19 January 2010 9:52 UTC www.als-mda.org [Source type: Academic]

^ However, most cases of ALS are sporadic, and genetic factors may contribute to the risk for disease.
  • PLoS ONE: Amyotrophic Lateral Sclerosis: An Emerging Era of Collaborative Gene Discovery 19 January 2010 9:52 UTC www.plosone.org [Source type: Academic]

^ Other mutations as cause of familial ALS .
  • Molecular Neurodegeneration | Full text | Experimental models for the study of neurodegeneration in amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.molecularneurodegeneration.com [Source type: Academic]

[7]
.Some environmental causative factors have been suggested for the increased incidence in the western Pacific.^ Hyperthyroidism, infection, and inflammation can cause an increase in REE; however, these conditions were not a factor in our study because none of the hypermetabolic patients was hyperthyroid and patients with clinical infection were excluded.
  • Factors correlated with hypermetabolism in patients with amyotrophic lateral sclerosis -- Desport et al. 74 (3): 328 -- American Journal of Clinical Nutrition 19 January 2010 9:52 UTC www.ajcn.org [Source type: Academic]

^ Environmental exposures have been proposed as the explanation for an increased incidence of ALS in Gulf War veterans [ Haley 2003 , Horner et al 2003 ].
  • Amyotrophic Lateral Sclerosis Overview -- GeneReviews -- NCBI Bookshelf 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

^ Our data indicate that high-incidence ALS in the Western Pacific may result from Ca-Mg dysmetabolism with resultant deposition of Al .
  • Amyotrophic Lateral Sclerosis (ALS) - Abstracts : Online Reference for Health Concerns 19 January 2010 9:52 UTC www.lef.org [Source type: Academic]

.Prolonged exposure to a dietary neurotoxin called BMAA is one suspected risk factor in Guam; the neurotoxin is a compound found in the seed of the cycad Cycas circinalis,[8] a tropical plant found in Guam, which was used in the human food supply during the 1950s and early 1960s.^ Overlap syndromes exist, such as ALS-Parkinson-Dementia Complex of Guam where an association with a food chain concentration of the neurotoxic amino acid beta-methylamino-l-alanine (BMAA) [ 5 ] has been found.
  • BioMed Central | Full text | Widespread sensorimotor and frontal cortical atrophy in Amyotrophic Lateral Sclerosis 19 January 2010 9:52 UTC www.biomedcentral.com [Source type: Academic]

^ A high incidence of ALS on the island of Guam has led to the idea that the cycad seed, ingested on the island, could be an ALS trigger.
  • Facts About ALS | MDA/ALS Publications 19 January 2010 9:52 UTC www.als-mda.org [Source type: Academic]

^ Unproven risk factors include ingestion of lead or agricultural chemicals, physical prowess (excellence in athletics), and intake of dietary glutamate.
  • Amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

.The very high incidence of the disease among Italian soccer players (more than five times higher than normally expected) has raised the concern of a possible link between the disease and the use of pesticides on the soccer fields (several of which have been linked to neuronal toxicity).^ The hypothesis that cytokines play a role in the genesis of neuronal damage in ALS was raised by several authors ( 42 , 43 ).
  • Factors correlated with hypermetabolism in patients with amyotrophic lateral sclerosis -- Desport et al. 74 (3): 328 -- American Journal of Clinical Nutrition 19 January 2010 9:52 UTC www.ajcn.org [Source type: Academic]

^ Chancellor AM, Warlow CP: Adult onset motor neuron disease: worldwide mortality, incidence and distribution since 1950.
  • Molecular Neurodegeneration | Full text | Experimental models for the study of neurodegeneration in amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.molecularneurodegeneration.com [Source type: Academic]

^ For example, it is known that certain doses of selegiline (higher than those used in the study) can lead to serious interactions with some types of foods and certain medications.
  • UCSF Memory and Aging Center | Treatments and Medications 19 January 2010 9:52 UTC memory.ucsf.edu [Source type: Academic]

[9][10]
.According to the ALS Association, military veterans are at an increased risk of contracting ALS (again, possibly implying a link to neurotoxic chemical exposure).^ In contrast, a prospective study in a contemporary cohort of veterans 53 that alleged an increased risk of ALS associated with deployment to the Persian Gulf in the first Gulf War (see discussion below) showed no overall increased incidence of ALS associated with contemporary military service (Class I Evidence).
  • Amyotrophic Lateral Sclerosis: eMedicine Neurology 19 January 2010 9:52 UTC emedicine.medscape.com [Source type: Academic]

^ While mutations in ANG have not been robustly linked with ALS as a Mendelian disorder, they most likely constitute a risk factor in certain populations.
  • Amyotrophic Lateral Sclerosis Overview -- GeneReviews -- NCBI Bookshelf 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

^ Family history is associated with an increased risk of ALS, and many Mendelian causes have been discovered.
  • PLoS ONE: Amyotrophic Lateral Sclerosis: An Emerging Era of Collaborative Gene Discovery 19 January 2010 9:52 UTC www.plosone.org [Source type: Academic]

.In its report ALS in the Military,[11] the group pointed to an almost 60% greater chance of the disease in military veterans than the general population.^ In general, individuals younger than age 55 years at onset of symptoms survive longer, regardless of gender [ Magnus et al 2002 ].
  • Amyotrophic Lateral Sclerosis Overview -- GeneReviews -- NCBI Bookshelf 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

^ The finding of an mREE value that was 10.1% greater in the ALS patients than in the control group confirmed the existence of hypermetabolism in ALS patients.
  • Factors correlated with hypermetabolism in patients with amyotrophic lateral sclerosis -- Desport et al. 74 (3): 328 -- American Journal of Clinical Nutrition 19 January 2010 9:52 UTC www.ajcn.org [Source type: Academic]

^ FLAIR images displayed a hyperintense signal with a higher specificity than other modalities along the descending pyramidal tract of ALS patients [ 11 ].
  • BioMed Central | Full text | Widespread sensorimotor and frontal cortical atrophy in Amyotrophic Lateral Sclerosis 19 January 2010 9:52 UTC www.biomedcentral.com [Source type: Academic]

.The former Indian Army Chief of Staff General K.Sunderji also suffered from ALS before passing away.^ Further, subjects with ALS may generally metabolize xenobiotics differently than healthy individuals or individuals suffering from illnesses that have symptoms similar to ALS symptoms.
  • Biomarkers for amyotrophic lateral sclerosis and methods using the same - Patent Application 20070298998 19 January 2010 9:52 UTC www.freepatentsonline.com [Source type: Academic]

.For Gulf War veterans, the chance is seen as twice that of veterans not deployed to the Persian Gulf in a joint study by the Veterans Affairs Administration and the DOD, another epidemiologic association suggesting a link to toxic exposure.^ In contrast, a prospective study in a contemporary cohort of veterans 53 that alleged an increased risk of ALS associated with deployment to the Persian Gulf in the first Gulf War (see discussion below) showed no overall increased incidence of ALS associated with contemporary military service (Class I Evidence).
  • Amyotrophic Lateral Sclerosis: eMedicine Neurology 19 January 2010 9:52 UTC emedicine.medscape.com [Source type: Academic]

^ Armon C. Occurrence of amyotrophic lateral sclerosis among Gulf War veterans.
  • Amyotrophic Lateral Sclerosis: eMedicine Neurology 19 January 2010 9:52 UTC emedicine.medscape.com [Source type: Academic]

^ Haley RW. Excess incidence of ALS in young Gulf War veterans.
  • Amyotrophic Lateral Sclerosis: eMedicine Neurology 19 January 2010 9:52 UTC emedicine.medscape.com [Source type: Academic]

[12][13]
Dietary intake of polyunsaturated fatty acids (PUFA) has been shown in several studies to decrease the risk of developing ALS and other neurodegenerative disorders, probably through several mechanisms, including promotion of neurotrophins such as BDNF [14][15]

Pathophysiology

SOD1

.The cause of ALS is not known, though an important step toward determining the cause came in 1993 when scientists discovered that mutations in the gene that produces the Cu/Zn superoxide dismutase (SOD1) enzyme were associated with some cases (approximately 20%) of familial ALS. This enzyme is a powerful antioxidant that protects the body from damage caused by superoxide, a toxic free radical generated in the mitochondria.^ The finding of a single-site mutation in the Cu,Zn-superoxide dismutase (SOD1) gene in familial ALS (FALS) patients (2 , 3) linked this disease to free radicals (4) .
  • The roles of free radicals in amyotrophic lateral sclerosis: reactive oxygen species and elevated oxidation of protein, DNA, and membrane phospholipids -- LIU et al. 13 (15): 2318 -- The FASEB Journal 19 January 2010 9:52 UTC www.fasebj.org [Source type: Academic]

^ In ~25% of familial cases, the disease is caused by mutations in the gene encoding cytosolic copper-zinc superoxide dismutase ( SOD1 ) (Rosen et al., 1993 ).
  • Rats Expressing Human Cytosolic Copper-Zinc Superoxide Dismutase Transgenes with Amyotrophic Lateral Sclerosis: Associated Mutations Develop Motor Neuron Disease -- Nagai et al. 21 (23): 9246 -- Journal of Neuroscience 19 January 2010 9:52 UTC www.jneurosci.org [Source type: Academic]

^ Recently, it was found that some patients with autosomal-dominant familial ALS (FALS) have point mutations in the gene that encodes Cu/Zn superoxide dismutase (SOD1).
  • Amyotrophic Lateral Sclerosis (ALS) - Abstracts : Online Reference for Health Concerns 19 January 2010 9:52 UTC www.lef.org [Source type: Academic]

.Free radicals are highly reactive molecules produced by cells during normal metabolism again largely by the mitochondria.^ Mitochondria, free radicals, and neurodegeneration.
  • Amyotrophic Lateral Sclerosis (ALS) - Abstracts : Online Reference for Health Concerns 19 January 2010 9:52 UTC www.lef.org [Source type: Academic]

^ Spontaneous swallows with the same characteristics have been observed and produced in normal subjects under some special conditions or during sleep (Fig.
  • Pathophysiological mechanisms of oropharyngeal dysphagia in amyotrophic lateral sclerosis -- Ertekin et al. 123 (1): 125 -- Brain 19 January 2010 9:52 UTC brain.oxfordjournals.org [Source type: Academic]

^ Brain cell damage caused by free radicals may play a role in Alzheimer’s disease.
  • UCSF Memory and Aging Center | Treatments and Medications 19 January 2010 9:52 UTC memory.ucsf.edu [Source type: Academic]

.Free radicals can accumulate and cause damage to both mitochondrial and nuclear DNA and proteins within cells.^ Free radicals are very active chemicals that can damage living cells.
  • amyotrophic lateral sclerosis Facts, information, pictures | Encyclopedia.com articles about amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.encyclopedia.com [Source type: Academic]

^ Free radical damage is implicated in the course of many diseases, including age-related dementias.
  • Amyotrophic Lateral Sclerosis (ALS) - Abstracts : Online Reference for Health Concerns 19 January 2010 9:52 UTC www.lef.org [Source type: Academic]

^ Brain cell damage caused by free radicals may play a role in Alzheimer’s disease.
  • UCSF Memory and Aging Center | Treatments and Medications 19 January 2010 9:52 UTC memory.ucsf.edu [Source type: Academic]

.Evidence suggests that failure of defenses against oxidative stress up-regulates programmed cell death (apoptosis), among many other possible consequences.^ Apoptosis is also called programmed cell death or cell suicide.
  • Amyotrophic Lateral Sclerosis Glossary of Terms with Definitions on MedicineNet.com 19 January 2010 9:52 UTC www.medicinenet.com [Source type: Academic]

^ But in ALS and other degenerative diseases, it’s possible that the cell death program is activated inappropriately.
  • Facts About ALS | MDA/ALS Publications 19 January 2010 9:52 UTC www.als-mda.org [Source type: Academic]

^ Most cells have a built-in “suicide” program known as programmed cell death , or apoptosis .
  • Facts About ALS | MDA/ALS Publications 19 January 2010 9:52 UTC www.als-mda.org [Source type: Academic]

.Although it is not yet clear how the SOD1 gene mutation leads to motor neuron degeneration, researchers have theorized that an accumulation of free radicals may result from the faulty functioning of this gene.^ Although it is not yet clear how the SOD1 gene mutation leads to motor neuron degeneration, researchers have theorized that an accumulation of free radicals may result from the faulty functioning of this gene.
  • Amyotrophic Lateral Sclerosis Fact Sheet: National Institute of Neurological Disorders and Stroke (NINDS) 19 January 2010 9:52 UTC www.ninds.nih.gov [Source type: Academic]

^ Motor neuron disease and exposure to lead.
  • Association of Lead Exposure with Survival in Amyotrophic Lateral Sclerosis 19 January 2010 9:52 UTC ehp03.niehs.nih.gov [Source type: Academic]

^ The progressive degeneration of the motor neurons in ALS eventually lead to their death.
  • Fighting Amyotrophic Lateral Sclerosis (ALS), alias Lou Gehrig's Disease 19 January 2010 9:52 UTC www.squidoo.com [Source type: Academic]

.Current research, however, indicates that motor neuron death is not likely a result of lost or compromised dismutase activity, suggesting mutant SOD1 induces toxicity in some other way (a gain of function).^ Researchers have been unsuccessful in finding the cause of motor neuron death.
  • amyotrophic lateral sclerosis Facts, information, pictures | Encyclopedia.com articles about amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.encyclopedia.com [Source type: Academic]

^ Mutant dynactin in motor neuron disease.
  • PLoS ONE: Amyotrophic Lateral Sclerosis: An Emerging Era of Collaborative Gene Discovery 19 January 2010 9:52 UTC www.plosone.org [Source type: Academic]

^ It is a degenerative disease of upper and lower motor neurons that produces progressive weakness of voluntary muscles, with eventual death.
  • Use of pramipexole to treat amyotrophic lateral sclerosis - Patent 7157480 19 January 2010 9:52 UTC www.freepatentsonline.com [Source type: Academic]

[16][17]
.Studies involving transgenic mice have yielded several theories about the role of SOD1 in mutant SOD1 familial amyotrophic lateral sclerosis.^ Chronic treatment with L-threonine in amyotrophic lateral sclerosis: a pilot study.
  • Natural & Alternative Treatments - Hartford Hospital, Connecticut 19 January 2010 9:52 UTC www.harthosp.org [Source type: Academic]

^ PubMed ] Siddique T. Molecular genetics of familial amyotrophic lateral sclerosis.
  • Amyotrophic Lateral Sclerosis Overview -- GeneReviews -- NCBI Bookshelf 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

^ Familial amyotrophic lateral sclerosis.
  • Dementia associated with amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.medlink.com [Source type: Academic]

.Mice lacking the SOD1 gene entirely do not customarily develop ALS, although they do exhibit an acceleration of age-related muscle atrophy (sarcopenia) and a shortened lifespan (see article on superoxide dismutase).^ Find this article online Ignacio S, Moore DH, Smith AP, Lee NM (2005) Effect of neuroprotective drugs on gene expression in G93A/SOD1 mice.
  • PLoS ONE: Beyond Parkinson Disease: Amyotrophic Lateral Sclerosis and the Axon Guidance Pathway 19 January 2010 9:52 UTC www.plosone.org [Source type: Academic]

^ The finding of a single-site mutation in the Cu,Zn-superoxide dismutase (SOD1) gene in familial ALS (FALS) patients (2 , 3) linked this disease to free radicals (4) .
  • The roles of free radicals in amyotrophic lateral sclerosis: reactive oxygen species and elevated oxidation of protein, DNA, and membrane phospholipids -- LIU et al. 13 (15): 2318 -- The FASEB Journal 19 January 2010 9:52 UTC www.fasebj.org [Source type: Academic]

^ In ~25% of familial cases, the disease is caused by mutations in the gene encoding cytosolic copper-zinc superoxide dismutase ( SOD1 ) (Rosen et al., 1993 ).
  • Rats Expressing Human Cytosolic Copper-Zinc Superoxide Dismutase Transgenes with Amyotrophic Lateral Sclerosis: Associated Mutations Develop Motor Neuron Disease -- Nagai et al. 21 (23): 9246 -- Journal of Neuroscience 19 January 2010 9:52 UTC www.jneurosci.org [Source type: Academic]

.This indicates that the toxic properties of the mutant SOD1 are a result of a gain in function rather than a loss of normal function.^ Second, the nature of the pathology is dependent on the properties of the mutant SOD1 protein.
  • Rats Expressing Human Cytosolic Copper-Zinc Superoxide Dismutase Transgenes with Amyotrophic Lateral Sclerosis: Associated Mutations Develop Motor Neuron Disease -- Nagai et al. 21 (23): 9246 -- Journal of Neuroscience 19 January 2010 9:52 UTC www.jneurosci.org [Source type: Academic]

^ The duration of the CP-EMG pause was also shorter than in normal subjects, probably as a result of these abnormal changes.
  • Pathophysiological mechanisms of oropharyngeal dysphagia in amyotrophic lateral sclerosis -- Ertekin et al. 123 (1): 125 -- Brain 19 January 2010 9:52 UTC brain.oxfordjournals.org [Source type: Academic]

^ That the primary abnormality is not loss of SOD1 dismutation activity is supported by the observation that many mutant forms of SOD1 retain nearly normal SOD1 activity.
  • Rats Expressing Human Cytosolic Copper-Zinc Superoxide Dismutase Transgenes with Amyotrophic Lateral Sclerosis: Associated Mutations Develop Motor Neuron Disease -- Nagai et al. 21 (23): 9246 -- Journal of Neuroscience 19 January 2010 9:52 UTC www.jneurosci.org [Source type: Academic]

.In addition, aggregation of proteins has been found to be a common pathological feature of both familial and sporadic ALS (see article on proteopathy).^ ALS occurs in both sporadic and familial forms.
  • Use of pramipexole to treat amyotrophic lateral sclerosis - Patent 7157480 19 January 2010 9:52 UTC www.freepatentsonline.com [Source type: Academic]

^ Protein aggregation in ALS .
  • Molecular Neurodegeneration | Full text | Experimental models for the study of neurodegeneration in amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.molecularneurodegeneration.com [Source type: Academic]

^ Recently, it was found that some patients with autosomal-dominant familial ALS (FALS) have point mutations in the gene that encodes Cu/Zn superoxide dismutase (SOD1).
  • Amyotrophic Lateral Sclerosis (ALS) - Abstracts : Online Reference for Health Concerns 19 January 2010 9:52 UTC www.lef.org [Source type: Academic]

.Interestingly, in mutant SOD1 mice, aggregates (misfolded protein accumulations) of mutant SOD1 were found only in diseased tissues, and greater amounts were detected during motor neuron degeneration.^ Motor neuron disease: G12.2 .
  • Dementia associated with amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.medlink.com [Source type: Academic]

^ SOD1-ALS is a lower motor neuron form of the disease.
  • Amyotrophic Lateral Sclerosis: eMedicine Neurology 19 January 2010 9:52 UTC emedicine.medscape.com [Source type: Academic]

^ Mutant dynactin in motor neuron disease.
  • PLoS ONE: Amyotrophic Lateral Sclerosis: An Emerging Era of Collaborative Gene Discovery 19 January 2010 9:52 UTC www.plosone.org [Source type: Academic]

[18] .It is speculated that aggregate accumulation of mutant SOD1 plays a role in disrupting cellular functions by damaging mitochondria, proteasomes, protein folding chaperones, or other proteins.^ SOD1 proteins are degraded by the proteasome complex.
  • Oxidative stress causes abnormal accumulation of familial amyotrophic lateral sclerosis-related mutant SOD1 in transgenic Caenorhabditis elegans -- Oeda et al. 10 (19): 2013 -- Human Molecular Genetics 19 January 2010 9:52 UTC hmg.oxfordjournals.org [Source type: Academic]

^ Creatine plays a role in cellular energy metabolism and potentially has a role in protein metabolism.
  • Amyotrophic Lateral Sclerosis (ALS) - Abstracts : Online Reference for Health Concerns 19 January 2010 9:52 UTC www.lef.org [Source type: Academic]

^ In conclusion, we found that oxidative damage inhibits the degradation of FALS-related mutant human SOD1 proteins, resulting in the aberrant accumulation of mutant proteins and aggregate formation.
  • Oxidative stress causes abnormal accumulation of familial amyotrophic lateral sclerosis-related mutant SOD1 in transgenic Caenorhabditis elegans -- Oeda et al. 10 (19): 2013 -- Human Molecular Genetics 19 January 2010 9:52 UTC hmg.oxfordjournals.org [Source type: Academic]

[19] .Any such disruption, if proven, would lend significant credibility to the theory that aggregates are involved in mutant SOD1 toxicity.^ Increased oxidative damage in macromolecules has also been demonstrated in the transgenic mutant SOD1 mouse, [ 100 - 104 ] suggesting that oxidative stress could be involved in FALS pathogenesis.
  • Molecular Neurodegeneration | Full text | Experimental models for the study of neurodegeneration in amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.molecularneurodegeneration.com [Source type: Academic]

^ Our results suggest that the specific interaction and aggregation of CCS-SOD1 (probably CCS-mutant SOD1) in SOD1-mutated FALS patients may amplify the formation of inclusions and emphasize a more marked mutant SOD1-mediated toxicity.

^ Mutations in the superoxide dismutase 1 (sod1) gene cause familial amyotrophic lateral sclerosis (FALS), likely due to the toxic properties of misfolded mutant SOD1 protein.
  • First 'Neuroprotective' Gene In Patients With Amyotrophic Lateral Sclerosis Isolated - ALS/MND Support Group Forums 19 January 2010 9:52 UTC www.alsforums.com [Source type: Academic]

.Critics have noted that in humans, SOD1 mutations cause only 2% or so of overall cases and the etiological mechanisms may be distinct from those responsible for the sporadic form of the disease.^ The main limitation of our model is that, although glutamate-mediated overactivation of AMPA receptors may be a relevant mechanism of spinal motoneuron degeneration in ALS, convincing evidence that this occurs in the human disease has not yet been obtained.
  • Molecular Neurodegeneration | Full text | Experimental models for the study of neurodegeneration in amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.molecularneurodegeneration.com [Source type: Academic]

^ By simple inference from the data of this study, one can predict that humans heterozygous for SOD1 mutations (50% of the control synthesis rate), would develop terminal disease at 25/0.5 x 1 year = 50 years of age, as is actually the case (Andersen et al ., 2003 ).
  • Disulphide-reduced superoxide dismutase-1 in CNS of transgenic amyotrophic lateral sclerosis models -- Jonsson et al. 129 (2): 451 -- Brain 19 January 2010 9:52 UTC brain.oxfordjournals.org [Source type: Academic]

^ Mutations in superoxide dismutase 1 as cause of one form of familial ALS .
  • Molecular Neurodegeneration | Full text | Experimental models for the study of neurodegeneration in amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.molecularneurodegeneration.com [Source type: Academic]

.To date, the ALS-SOD1 mice remain the best model of the disease for preclinical studies but it is hoped that more useful models will be developed.^ Experimental models for the study of ALS .
  • Molecular Neurodegeneration | Full text | Experimental models for the study of neurodegeneration in amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.molecularneurodegeneration.com [Source type: Academic]

^ To date, 114 different SOD1 mutations have been identified in ALS patients, with all but one, D90A, showing a dominant mode of inheritance (Andersen et al ., 2003 ).
  • Disulphide-reduced superoxide dismutase-1 in CNS of transgenic amyotrophic lateral sclerosis models -- Jonsson et al. 129 (2): 451 -- Brain 19 January 2010 9:52 UTC brain.oxfordjournals.org [Source type: Academic]

^ Diagnosis is best made by a neurologist, as there is no one specific test for ALS. Rather, a combination of signs and symptoms suggest the disease.
  • Amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

Other factors

.Studies also have focused on the role of glutamate in motor neuron degeneration.^ Studies also have focused on the role of glutamate in motor neuron degeneration.
  • Amyotrophic Lateral Sclerosis Fact Sheet: National Institute of Neurological Disorders and Stroke (NINDS) 19 January 2010 9:52 UTC www.ninds.nih.gov [Source type: Academic]

^ The disease is characterized by muscular atrophy and weakness due to degeneration of spinal motor neurons and by hyperreflexia after loss of cerebral cortical motor neurons.
  • Amyotrophic Lateral Sclerosis, Lead, and Genetic Susceptibility: Polymorphisms in the -Aminolevulinic Acid Dehydratase and Vitamin D Receptor Genes 19 January 2010 9:52 UTC ehp03.niehs.nih.gov [Source type: Academic]

^ Researchers are conducting studies to increase their understanding of genes that may cause the disease, mechanisms that can trigger motor neurons to degenerate in ALS, and approaches to stop the progress leading to cell death.
  • Amyotrophic Lateral Sclerosis (ALS), Robert Wood Johnson University Hospital, New Brunswick, NJ, 08903 19 January 2010 9:52 UTC www.rwjuh.edu [Source type: Academic]
  • Amyotrophic Lateral Sclerosis (ALS) 19 January 2010 9:52 UTC www.montefiore.org [Source type: Academic]

.Glutamate is one of the chemical messengers or neurotransmitters in the brain.^ Glutamate is the main excitatory neurotransmitter in the brain.

^ Glutamate is one of the chemical messengers or neurotransmitters in the brain.
  • Amyotrophic Lateral Sclerosis Fact Sheet: National Institute of Neurological Disorders and Stroke (NINDS) 19 January 2010 9:52 UTC www.ninds.nih.gov [Source type: Academic]

^ Glutamate and ascorbate (vitamin C) are the two main excitatory neurotransmitters in the brain with glutamate being responsible for 75% of it.
  • ~Amyotrophic Lateral Sclerosis (ALS), Part 3 - Diet and Supplements 19 January 2010 9:52 UTC www.lifeextensionvitamins.com [Source type: Academic]

.Scientists have found that, compared to healthy people, ALS patients have higher levels of glutamate in the serum and spinal fluid.^ ALS and were compared to healthy controls.
  • ~Amyotrophic Lateral Sclerosis (ALS), Part 3 - Diet and Supplements 19 January 2010 9:52 UTC www.lifeextensionvitamins.com [Source type: Academic]

^ Scientists have found that, compared to healthy people, ALS patients have higher levels of glutamate in the serum and spinal fluid.
  • Amyotrophic Lateral Sclerosis Fact Sheet: National Institute of Neurological Disorders and Stroke (NINDS) 19 January 2010 9:52 UTC www.ninds.nih.gov [Source type: Academic]

^ Researchers found a set of 11 proteins that were significantly higher in the spinal fluid of ALS patients.
  • Amyotrophic lateral sclerosis W::PROTEomicsSURF 19 January 2010 9:52 UTC proteomicssurf.wcyte.com [Source type: Academic]

[6] .Laboratory studies have demonstrated that neurons begin to die off when they are exposed over long periods to excessive amounts of glutamate (excitotoxicity).^ As muscle cells are not used, they begin to die off.
  • amyotrophic lateral sclerosis Facts, information, pictures | Encyclopedia.com articles about amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.encyclopedia.com [Source type: Academic]

^ Laboratory studies have demonstrated that neurons begin to die off when they are exposed over long periods to excessive amounts of glutamate.
  • Amyotrophic Lateral Sclerosis Fact Sheet: National Institute of Neurological Disorders and Stroke (NINDS) 19 January 2010 9:52 UTC www.ninds.nih.gov [Source type: Academic]

^ Early studies show that stem cells may have the ability to repair or replace the motor neurons damaged by ALS, but clinical use or clinical trials with stem cell therapy is still a long way off.
  • MayoClinic.com Health Library - Amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.riversideonline.com [Source type: FILTERED WITH BAYES]

.Now, scientists are trying to understand what mechanisms lead to a buildup of unneeded glutamate in the spinal fluid and how this imbalance could contribute to the development of ALS. Failure of astrocytes to sequester glutamate from the extracellular fluid surrounding the neurones has been proposed as a possible cause of this glutamate-mediated neurodegeneration.^ The leading cause of death for ALS patients is respiratory failure.
  • Amyotrophic Lateral Sclerosis: Part 1 of 2 19 January 2010 9:52 UTC www.massagetoday.com [Source type: FILTERED WITH BAYES]

^ Now, scientists are trying to understand what mechanisms lead to a buildup of unneeded glutamate in the spinal fluid and how this imbalance could contribute to the development of ALS. .
  • Amyotrophic Lateral Sclerosis Fact Sheet: National Institute of Neurological Disorders and Stroke (NINDS) 19 January 2010 9:52 UTC www.ninds.nih.gov [Source type: Academic]

^ It is proposed that excess glutamate (an excitatory neurotransmitter) reaches toxic concentrations in the synapses, causing the neurons in the central nervous system to die.
  • USPharmacist.com > The Management of Amyotrophic Lateral Sclerosis 19 January 2010 9:52 UTC www.uspharmacist.com [Source type: Academic]

.Riluzole is currently the only FDA approved drug for ALS and targets glutamate transporters.^ Riluzole is the only currently FDA-approved drug for the treatment of ALS. Its mechanism of action is thought to be glutamate inhibition.
  • Amyotrophic Lateral Sclerosis Overview -- GeneReviews -- NCBI Bookshelf 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

^ Ceftriaxone was identified after a search through already approved drugs for a compound that was effective at stimulating expression of astrocytic glutamate transporter, which could reduce excitotoxicity by inactivating synaptic glutamate.
  • Amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

^ Riluzole is the only currently FDA-approved drug for treatment of ALS. Oral secretions in those with bulbar symptoms can be reduced with tricylic antidepressants and other anticholinergic agents.
  • Amyotrophic Lateral Sclerosis Overview -- GeneReviews -- NCBI Bookshelf 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

.Its very modest benefit to patients has bolstered the argument that glutamate is not a primary cause of the disease.^ The disease is inherited in 5-10% of cases leading to familial forms Defects in ALS2 are the cause of juvenile primary lateral sclerosis (JPLS) [MIM:606353].
  • ALS2 Gene - GeneCards | ALS2 Protein | ALS2 Antibody 19 January 2010 9:52 UTC www.genecards.org [Source type: Academic]

^ In humans, subacute combined degeneration of the spinal cord and brain, a primary demyelinating disease, is caused by cobalamin or methyltetrahydrofolate deficiency.
  • Amyotrophic Lateral Sclerosis (ALS) - Abstracts : Online Reference for Health Concerns 19 January 2010 9:52 UTC www.lef.org [Source type: Academic]

^ Many patients with amyotrophic lateral sclerosis (ALS; motor neuron disease) use natural or traditional therapies of unproven benefit.
  • Amyotrophic Lateral Sclerosis (ALS) - Abstracts : Online Reference for Health Concerns 19 January 2010 9:52 UTC www.lef.org [Source type: Academic]

.The antibiotic ceftriaxone has demonstrated an unexpected effect on glutamate and appears to be a beneficial treatment for ALS in animal models.^ Riluzole, a glutamate antagonist, is the only pharmacologic treatment for ALS approved by Health Canada and the United States Food and Drug Administration.
  • Amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

^ In this framework, the purpose of the present article is to review the available experimental animal models of ALS. .
  • Molecular Neurodegeneration | Full text | Experimental models for the study of neurodegeneration in amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.molecularneurodegeneration.com [Source type: Academic]

^ Riluzole is the only currently FDA-approved drug for the treatment of ALS. Its mechanism of action is thought to be glutamate inhibition.
  • Amyotrophic Lateral Sclerosis Overview -- GeneReviews -- NCBI Bookshelf 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

.Ceftriaxone is currently being tested in clinical trials.^ See the entire definition of Clinical trials Cognitive: Pertaining to cognition , the process of knowing and, more precisely, the process of being aware, knowing, thinking, learning and judging.
  • Amyotrophic Lateral Sclerosis Glossary of Terms with Definitions on MedicineNet.com 19 January 2010 9:52 UTC www.medicinenet.com [Source type: Academic]

^ Currently, doses of 240 mg/day of GB are being studied in a multi-site trial.
  • UCSF Memory and Aging Center | Treatments and Medications 19 January 2010 9:52 UTC memory.ucsf.edu [Source type: Academic]

^ Compounds currently being evaluated in phase III trials include minocycline, IGF-1 polypeptide, ceftriaxone, and ONO-2506.
  • Amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

.Autoimmune responses which occur when the body's immune system attacks normal cells have been suggested as one possible cause for motor neuron degeneration in ALS. Some scientists theorize that antibodies may directly or indirectly impair the function of motor neurons, interfering with the transmission of signals between the brain and muscles.^ The altered axons may impair transmission of impulses from nerves to muscles.
  • Amyotrophic Lateral Sclerosis - Medpedia 19 January 2010 9:52 UTC wiki.medpedia.com [Source type: Academic]

^ The progressive degeneration of the motor neurons in ALS eventually lead to their death.
  • Fighting Amyotrophic Lateral Sclerosis (ALS), alias Lou Gehrig's Disease 19 January 2010 9:52 UTC www.squidoo.com [Source type: Academic]

^ One cause of brain cell death is glutamate toxicity.
  • ~Amyotrophic Lateral Sclerosis (ALS), Part 3 - Diet and Supplements 19 January 2010 9:52 UTC www.lifeextensionvitamins.com [Source type: Academic]

.More recent evidence indicates that the nervous system's immune cells, microglia, are heavily involved in the later stages of the disease.^ Since it is more active in the central nervous system and less in peripheral tissues, donepezil produces fewer side effects than tacrine.
  • UCSF Memory and Aging Center | Treatments and Medications 19 January 2010 9:52 UTC memory.ucsf.edu [Source type: Academic]

^ Bergmann M, Volpel M, Kuchelmeister K: Onuf's nucleus is frequently involved in motor neuron disease/amyotrophic lateral sclerosis.
  • BioMed Central | Full text | Widespread sensorimotor and frontal cortical atrophy in Amyotrophic Lateral Sclerosis 19 January 2010 9:52 UTC www.biomedcentral.com [Source type: Academic]

^ Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease involving both upper motor neurons (UMNs) and lower motor neurons (LMNs).
  • Amyotrophic Lateral Sclerosis Overview -- GeneReviews -- NCBI Bookshelf 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

.In searching for the cause of ALS, researchers have also studied environmental factors such as exposure to toxic or infectious agents.^ Researchers are studying several possible causes of ALS, including: .
  • Amyotrophic lateral sclerosis 19 January 2010 9:52 UTC cgi.cnn.com [Source type: General]
  • Amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.cnn.com [Source type: General]

^ In searching for the cause of ALS, researchers have also studied environmental factors such as exposure to toxic or infectious agents.
  • Amyotrophic Lateral Sclerosis Fact Sheet: National Institute of Neurological Disorders and Stroke (NINDS) 19 January 2010 9:52 UTC www.ninds.nih.gov [Source type: Academic]

^ Is ALS caused by environmental factors?
  • Amyotrophic Lateral Sclerosis ALS: rockin for a cure 19 January 2010 9:52 UTC www.rockinforacure.org [Source type: Academic]

.Other research has examined the possible role of dietary deficiency or trauma.^ Other research has examined the possible role of dietary deficiency or trauma.
  • Amyotrophic Lateral Sclerosis Fact Sheet: National Institute of Neurological Disorders and Stroke (NINDS) 19 January 2010 9:52 UTC www.ninds.nih.gov [Source type: Academic]

^ By exploring these and other possible factors, researchers hope to find the cause or causes of motor neuron degeneration in ALS and develop therapies to slow the progression of the disease.
  • Amyotrophic Lateral Sclerosis Fact Sheet: National Institute of Neurological Disorders and Stroke (NINDS) 19 January 2010 9:52 UTC www.ninds.nih.gov [Source type: Academic]

^ Prions seem to have a preference for the nervous system, so they’re being examined for a possible role in ALS. .
  • Facts About ALS | MDA/ALS Publications 19 January 2010 9:52 UTC www.als-mda.org [Source type: Academic]

However, while the personal histories of many people who develop als do include these types of factors, as of yet, no study has produced evidence sufficient to directly implicate these factors as causes of ALS.
One possible exception is smoking which, in a study the results of which were published in 2009, was determined to be an "established" risk factor for ALS.[20]
Future research may show that many factors, including a genetic predisposition, are involved in the development of ALS.

Diagnosis

.No test can provide a definite diagnosis of ALS, although the presence of upper and lower motor neuron signs in a single limb is strongly suggestive.^ Diagnosis and management of motor neurone disease.
  • NEJM -- Whole-Genome Analysis of Sporadic Amyotrophic Lateral Sclerosis 19 January 2010 9:52 UTC content.nejm.org [Source type: Academic]

^ The hallmark of ALS is the combination of upper motor neuron (UMN) and lower motor neuron (LMN) involvement.
  • Pharmacologic treatment of amyotrophic lateral sclerosis - a knol by iMedPub 19 January 2010 9:52 UTC knol.google.com [Source type: Academic]

^ Diagnosis of ALS is made on the basis of a combination of upper motor neuron (UMN) and lower motor neuron (LMN) findings.
  • Amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]
  • Amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.cfpc.ca [Source type: Academic]

.Instead, the diagnosis of ALS is primarily based on the symptoms and signs the physician observes in the patient and a series of tests to rule out other diseases.^ Symptoms Early signs and symptoms of ALS include: .
  • Amyotrophic lateral sclerosis 19 January 2010 9:52 UTC cgi.cnn.com [Source type: General]
  • Amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.cnn.com [Source type: General]

^ Only through clinical examination and a series of diagnostic tests to rule out other diseases can a diagnosis be established.
  • AMYOTROPHIC LATERAL SCLEROSIS 19 January 2010 9:52 UTC www.dhs.state.or.us [Source type: Academic]

^ Tests to rule out other conditions may include: .
  • Amyotrophic lateral sclerosis 19 January 2010 9:52 UTC cgi.cnn.com [Source type: General]
  • Amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.cnn.com [Source type: General]
  • Amyotrophic lateral sclerosis (ALS): Tests and diagnosis - MayoClinic.com 19 January 2010 9:52 UTC www.mayoclinic.com [Source type: Academic]
  • georgiahealthinfo.gov 19 January 2010 9:52 UTC georgiahealthinfo.gov [Source type: FILTERED WITH BAYES]

.Physicians obtain the patient's full medical history and usually conduct a neurologic examination at regular intervals to assess whether symptoms such as muscle weakness, atrophy of muscles, hyperreflexia, and spasticity are getting progressively worse.^ Muscle weakness in ALS patients .
  • ALS - Amyotrophic Lateral Sclerosis 19 January 2010 9:52 UTC www.1888discuss.com [Source type: Academic]

^ A diagnosis is made by patient history and a neurological examination.
  • Amyotrophic Lateral Sclerosis -- eCureMe.com 19 January 2010 9:52 UTC www.ecureme.com [Source type: Academic]

^ The patient or physician usually notices fasciculation, or muscle twitching.
  • Les Turner ALS Foundation - What Is ALS? 19 January 2010 9:52 UTC www.lesturnerals.org [Source type: Academic]

.Because symptoms of ALS can be similar to those of a wide variety of other, more treatable diseases or disorders, appropriate tests must be conducted to exclude the possibility of other conditions.^ Because symptoms of ALS can be similar to those of a wide variety of other, more treatable diseases or disorders, appropriate tests must be conducted to exclude the possibility of other conditions.
  • Amyotrophic Lateral Sclerosis Fact Sheet: National Institute of Neurological Disorders and Stroke (NINDS) 19 January 2010 9:52 UTC www.ninds.nih.gov [Source type: Academic]

^ Blood tests to rule out other conditions that may cause similar symptoms .

^ Are there diseases similar to ALS? .
  • Les Turner ALS Foundation - What Is ALS? 19 January 2010 9:52 UTC www.lesturnerals.org [Source type: Academic]

.One of these tests is electromyography (EMG), a special recording technique that detects electrical activity in muscles.^ Electromyography, or EMG, involves testing the electrical activity of muscles.
  • Amyotrophic Lateral Sclerosis (Lou Gehrig Disease) Causes, Symptoms, Diagnosis, Treatment, and Prognosis Information on eMedicineHealth.com 19 January 2010 9:52 UTC www.emedicinehealth.com [Source type: FILTERED WITH BAYES]

^ One of these tests is electromyography (EMG), a special recording technique that detects electrical activity in muscles.
  • Amyotrophic Lateral Sclerosis Fact Sheet: National Institute of Neurological Disorders and Stroke (NINDS) 19 January 2010 9:52 UTC www.ninds.nih.gov [Source type: Academic]

^ Diagnostic tests include MRI (magnetic resonance imaging), EMG (electromyogram), muscle biopsy, and blood tests.
  • Amyotrophic Lateral Sclerosis ALS: rockin for a cure 19 January 2010 9:52 UTC www.rockinforacure.org [Source type: Academic]

.Certain EMG findings can support the diagnosis of ALS. Another common test measures nerve conduction velocity (NCV).^ This finding is supported by histological evidence of reduced expression of the alpha1-subunit of the GABA A receptor in ALS motor cortex [ 19 ].
  • BioMed Central | Full text | Widespread sensorimotor and frontal cortical atrophy in Amyotrophic Lateral Sclerosis 19 January 2010 9:52 UTC www.biomedcentral.com [Source type: Academic]

^ ELECTRODIAGNOSTIC STUDIES Electromyography — The EMG findings in ALS combine features of acute and chronic denervation [12,13] .
  • Diagnosis of amyotrophic lateral sclerosis - a knol by Internet Medical Publishing 19 January 2010 9:52 UTC knol.google.com [Source type: Academic]

^ Electromyography — The EMG findings in ALS combine features of acute and chronic denervation [ 12,13 ] .
  • Diagnosis of amyotrophic lateral sclerosis - a knol by Internet Medical Publishing 19 January 2010 9:52 UTC knol.google.com [Source type: Academic]

.Specific abnormalities in the NCV results may suggest, for example, that the patient has a form of peripheral neuropathy (damage to peripheral nerves) or myopathy (muscle disease) rather than ALS. The physician may order magnetic resonance imaging (MRI), a noninvasive procedure that uses a magnetic field and radio waves to take detailed images of the brain and spinal cord.^ MRI: Abbreviation and nickname for magnetic resonance imaging .
  • Amyotrophic Lateral Sclerosis Glossary of Terms with Definitions on MedicineNet.com 19 January 2010 9:52 UTC www.medicinenet.com [Source type: Academic]

^ Neuroimaging of brain and/or spinal cord .
  • Amyotrophic Lateral Sclerosis Overview -- GeneReviews -- NCBI Bookshelf 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

^ The spinal cord consists of nerve fibers that transmit impulses to and from the brain.
  • Amyotrophic Lateral Sclerosis Glossary of Terms with Definitions on MedicineNet.com 19 January 2010 9:52 UTC www.medicinenet.com [Source type: Academic]

.Although these MRI scans are often normal in patients with ALS, they can reveal evidence of other problems that may be causing the symptoms, such as a spinal cord tumor, multiple sclerosis, a herniated disk in the neck, syringomyelia, or cervical spondylosis.^ Other mutations as cause of familial ALS .
  • Molecular Neurodegeneration | Full text | Experimental models for the study of neurodegeneration in amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.molecularneurodegeneration.com [Source type: Academic]

^ Some patients were also investigated by cranial and/or cervical MRI and by CT scanning.
  • Pathophysiological mechanisms of oropharyngeal dysphagia in amyotrophic lateral sclerosis -- Ertekin et al. 123 (1): 125 -- Brain 19 January 2010 9:52 UTC brain.oxfordjournals.org [Source type: Academic]

^ See the entire definition of NIH NINDS: Stands for the National Institute of Neurological Disorders and Stroke, one of the National Institutes of Health in the U.S., whose mission is to "support and conduct research and research training on the normal structure and function of the nervous system and on the causes, prevention, diagnosis, and treatment of more than 600 nervous system disorders including stroke , epilepsy , multiple sclerosis , Parkinson's disease, head and spinal cord injury, Alzheimer's disease, and brain tumors."
  • Amyotrophic Lateral Sclerosis Glossary of Terms with Definitions on MedicineNet.com 19 January 2010 9:52 UTC www.medicinenet.com [Source type: Academic]

.Based on the patient's symptoms and findings from the examination and from these tests, the physician may order tests on blood and urine samples to eliminate the possibility of other diseases as well as routine laboratory tests.^ The ALS test requires a blood sample.

^ Routine laboratory findings are not helpful.
  • Pathology of Degenerative CNS Diseases 19 January 2010 9:52 UTC library.med.utah.edu [Source type: Academic]

^ Blood and urine tests.
  • Amyotrophic lateral sclerosis 19 January 2010 9:52 UTC cgi.cnn.com [Source type: General]
  • Amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.cnn.com [Source type: General]
  • MayoClinic.com Health Library - Amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.riversideonline.com [Source type: FILTERED WITH BAYES]
  • georgiahealthinfo.gov 19 January 2010 9:52 UTC georgiahealthinfo.gov [Source type: FILTERED WITH BAYES]

.In some cases, for example, if a physician suspects that the patient may have a myopathy rather than ALS, a muscle biopsy may be performed.^ Cannabis may be an option for some patients.
  • Symptom Management of the Patient With Amyotrophic Lateral Sclerosis: A Guide for Hospice Nurses 19 January 2010 9:52 UTC www.nursingcenter.com [Source type: Academic]
  • Symptom Management of the Patient With Amyotrophic Lateral Sclerosis: A Guide for Hospice Nurses 19 January 2010 9:52 UTC www.nursingcenter.com [Source type: Academic]

^ Some of the effects of ALS may be painful.
  • Amyotrophic Lateral Sclerosis ALS: rockin for a cure 19 January 2010 9:52 UTC www.rockinforacure.org [Source type: Academic]

^ If your doctor believes you may have a muscle disease rather than ALS, you may undergo a muscle biopsy.
  • Amyotrophic lateral sclerosis 19 January 2010 9:52 UTC cgi.cnn.com [Source type: General]
  • Amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.cnn.com [Source type: General]
  • MayoClinic.com Health Library - Amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.riversideonline.com [Source type: FILTERED WITH BAYES]
  • georgiahealthinfo.gov 19 January 2010 9:52 UTC georgiahealthinfo.gov [Source type: FILTERED WITH BAYES]

.Infectious diseases such as human immunodeficiency virus (HIV), human T-cell leukaemia virus (HTLV), Lyme disease,[21] syphilis[22] and tick-borne encephalitis[23] viruses can in some cases cause ALS-like symptoms.^ The types of neuromuscular diseases associated with human immunodeficiency virus (HIV) infection are described.
  • Amyotrophic Lateral Sclerosis (ALS) - Abstracts : Online Reference for Health Concerns 19 January 2010 9:52 UTC www.lef.org [Source type: Academic]

^ HTLV: Human T-lymphotropic virus .
  • Amyotrophic Lateral Sclerosis Glossary of Terms with Definitions on MedicineNet.com 19 January 2010 9:52 UTC www.medicinenet.com [Source type: Academic]

^ Neuromuscular diseases associated with human immunodeficiency virus infection.
  • Amyotrophic Lateral Sclerosis (ALS) - Abstracts : Online Reference for Health Concerns 19 January 2010 9:52 UTC www.lef.org [Source type: Academic]

.Neurological disorders such as multiple sclerosis, post-polio syndrome, multifocal motor neuropathy, CIDP, and spinal muscular atrophy can also mimic certain facets of the disease and should be considered by physicians attempting to make a diagnosis.^ ALS1 is a familial form of amyotrophic lateral sclerosis, a neurodegenerative disorder affecting upper and lower motor neurons and resulting in fatal paralysis.
  • SOD1 Gene - GeneCards | SODC Protein | SODC Antibody 19 January 2010 9:52 UTC www.genecards.org [Source type: Academic]

^ See the entire definition of NIH NINDS: Stands for the National Institute of Neurological Disorders and Stroke, one of the National Institutes of Health in the U.S., whose mission is to "support and conduct research and research training on the normal structure and function of the nervous system and on the causes, prevention, diagnosis, and treatment of more than 600 nervous system disorders including stroke , epilepsy , multiple sclerosis , Parkinson's disease, head and spinal cord injury, Alzheimer's disease, and brain tumors."
  • Amyotrophic Lateral Sclerosis Glossary of Terms with Definitions on MedicineNet.com 19 January 2010 9:52 UTC www.medicinenet.com [Source type: Academic]

^ Genetic testing is not a routine part of the diagnostic evaluation in ALS but can be helpful in making the diagnosis in two genetic disorders (Kennedy syndrome and adult-onset Tay-Sachs disease) that mimic ALS. Muscle biopsy is not a routine part of the diagnostic evaluation of ALS but should be performed if there is clinical suspicion of inflammatory myopathy.
  • Diagnosis of amyotrophic lateral sclerosis - a knol by Internet Medical Publishing 19 January 2010 9:52 UTC knol.google.com [Source type: Academic]

.Because of the prognosis carried by this diagnosis and the variety of diseases or disorders that can resemble ALS in the early stages of the disease, patients should always obtain a second neurological opinion.^ Genetic testing is not a routine part of the diagnostic evaluation in ALS but can be helpful in making the diagnosis in two genetic disorders (Kennedy syndrome and adult-onset Tay-Sachs disease) that mimic ALS. Muscle biopsy is not a routine part of the diagnostic evaluation of ALS but should be performed if there is clinical suspicion of inflammatory myopathy.
  • Diagnosis of amyotrophic lateral sclerosis - a knol by Internet Medical Publishing 19 January 2010 9:52 UTC knol.google.com [Source type: Academic]

^ We examined the family history and associated diseases in 58 patients with amyotrophic lateral sclerosis (ALS), as well as the T-cell phenotypes and functions in 46 consecutive patients with this disorder.
  • Amyotrophic Lateral Sclerosis (ALS) - Abstracts : Online Reference for Health Concerns 19 January 2010 9:52 UTC www.lef.org [Source type: Academic]

^ In another neurodegenerative disorder, Alzheimer's disease, the enzyme glutamine synthetase, which normally catalyzes glutamine formation, is elevated (Tzika et al., 1993 ).
  • Rats Expressing Human Cytosolic Copper-Zinc Superoxide Dismutase Transgenes with Amyotrophic Lateral Sclerosis: Associated Mutations Develop Motor Neuron Disease -- Nagai et al. 21 (23): 9246 -- Journal of Neuroscience 19 January 2010 9:52 UTC www.jneurosci.org [Source type: Academic]

.A recent study identified three proteins that are found in significantly lower concentrations in the cerebral spinal fluid of patients with ALS than in healthy individuals [24] .^ To date, 114 different SOD1 mutations have been identified in ALS patients, with all but one, D90A, showing a dominant mode of inheritance (Andersen et al ., 2003 ).
  • Disulphide-reduced superoxide dismutase-1 in CNS of transgenic amyotrophic lateral sclerosis models -- Jonsson et al. 129 (2): 451 -- Brain 19 January 2010 9:52 UTC brain.oxfordjournals.org [Source type: Academic]

^ Further studies and analysis of pathology tissue from a p.Arg521Gly-positive individual showed diffuse ubiquitin positivity indicating cytoplasmic retention and aggregation of the mutant protein.
  • Amyotrophic Lateral Sclerosis Overview -- GeneReviews -- NCBI Bookshelf 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

^ SOD1 is ubiquitously expressed, in many tissues at higher levels than in the brain and spinal cord (Marklund, 1984 ; Jonsson et al ., 2004 ).
  • Disulphide-reduced superoxide dismutase-1 in CNS of transgenic amyotrophic lateral sclerosis models -- Jonsson et al. 129 (2): 451 -- Brain 19 January 2010 9:52 UTC brain.oxfordjournals.org [Source type: Academic]

.Evaluating the levels of these three proteins proved 95% accurate for diagnosing ALS. Two of the three protein were identified as cystatin C (13.4 kD) and a 4.8 kD peptide fragment of VEGF (Vascular Endothelial Growth Factor).^ To test these hypotheses, we analyzed two whole-genome association datasets, one for ALS and one for PD [19] , [20] .
  • PLoS ONE: Beyond Parkinson Disease: Amyotrophic Lateral Sclerosis and the Axon Guidance Pathway 19 January 2010 9:52 UTC www.plosone.org [Source type: Academic]

^ The method of claim 1, wherein the ALS-negative reference levels of the one or more biomarkers comprise levels of the one or more biomarkers in one or more samples from one or more subjects not having ALS and the ALS-positive reference levels of the one or more biomarkers comprise levels of the one or more biomarkers in one or more samples from one or more subjects diagnosed with ALS. 3.
  • Biomarkers for amyotrophic lateral sclerosis and methods using the same - Patent Application 20070298998 19 January 2010 9:52 UTC www.freepatentsonline.com [Source type: Academic]

^ Patients meeting criteria for dementia not typical of a frontotemporal lobar degeneration shall be diagnosed with ALS-dementia (ie, ALS- Alzheimer disease , ALS-vascular dementia, ALS-mixed dementia).
  • Dementia associated with amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.medlink.com [Source type: Academic]

A third 6.7 kD cationic peptide has not yet been identified. .These diagnostic biomarkers may be useful in making earlier diagnoses of ALS than previously possible.^ The level(s) of the one or more biomarkers in the biological sample may also be compared to ALS-positive and/or ALS-negative reference levels using one or more statistical analyses (e.g., t-test, Welch's T-test, Wilcoxon's rank sum test, random forest).
  • Biomarkers for amyotrophic lateral sclerosis and methods using the same - Patent Application 20070298998 19 January 2010 9:52 UTC www.freepatentsonline.com [Source type: Academic]

^ These increases are far less than the 8–24-fold increases in hSOD1 protein, suggesting that CCS may be limiting with regard to Cu-charging in the transgenic mice.
  • Disulphide-reduced superoxide dismutase-1 in CNS of transgenic amyotrophic lateral sclerosis models -- Jonsson et al. 129 (2): 451 -- Brain 19 January 2010 9:52 UTC brain.oxfordjournals.org [Source type: Academic]

^ The method of claim 1, wherein the ALS-negative reference levels of the one or more biomarkers comprise levels of the one or more biomarkers in one or more samples from one or more subjects not having ALS and the ALS-positive reference levels of the one or more biomarkers comprise levels of the one or more biomarkers in one or more samples from one or more subjects diagnosed with ALS. 3.
  • Biomarkers for amyotrophic lateral sclerosis and methods using the same - Patent Application 20070298998 19 January 2010 9:52 UTC www.freepatentsonline.com [Source type: Academic]

Treatment

Medication

Approved drugs

.The Food and Drug Administration (FDA) has approved the first drug treatment for the disease: Riluzole (Rilutek).^ Riluzole, a glutamate antagonist, is the only pharmacologic treatment for ALS approved by Health Canada and the United States Food and Drug Administration.
  • Amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

^ Riluzole, the only drug launched for treatment ALS is currently undergoing industrial trials for Alzheimer's, Parkinson's, Huntington disease, stroke and head injury.
  • Amyotrophic Lateral Sclerosis (ALS) - Abstracts : Online Reference for Health Concerns 19 January 2010 9:52 UTC www.lef.org [Source type: Academic]

^ Coenzyme Q10 administration and its potential for treatment of neurodegenerative diseases.
  • Amyotrophic Lateral Sclerosis (ALS) - Abstracts : Online Reference for Health Concerns 19 January 2010 9:52 UTC www.lef.org [Source type: Academic]

.Riluzole is believed to reduce damage to motor neurons by decreasing the release of glutamate via activation of glutamate transporters.^ Rothstein JD, Martin LJ, Kuncl RW: Decreased glutamate transport by the brain and spinal cord in amyotrophic lateral sclerosis.
  • Molecular Neurodegeneration | Full text | Experimental models for the study of neurodegeneration in amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.molecularneurodegeneration.com [Source type: Academic]

^ Persephin, in contrast, does not appear to have neurotrophic or neurite-promoting effects on mature motor neurons and may instead worsen the glutamate injury of motor neurons.
  • Amyotrophic Lateral Sclerosis (ALS) - Abstracts : Online Reference for Health Concerns 19 January 2010 9:52 UTC www.lef.org [Source type: Academic]

^ Shaw PJ, Ince PG, Falkous G, Mantle D: Oxidative damage to protein in sporadic motor neuron disease spinal cord.
  • Molecular Neurodegeneration | Full text | Experimental models for the study of neurodegeneration in amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.molecularneurodegeneration.com [Source type: Academic]

.In addition, the drug offers a wide array of other neuroprotective effects, by means of sodium and calcium channel blockades,[25] inhibition of protein kinase C,[26] and the promotion of NMDA (N-methyl d-aspartate) receptor antagonism.^ The aim of the study was to assess the effect of pimozide voltage-dependent calcium channel blocker on the progression of ALS patients as compared to the potentially neuroprotective drugs, selegiline and vitamin E. There were 44 patients (17 females and 27 males, aged from 30 to 80 years, mean age: 56.2 years) diagnosed as either definite or possible ALS. The study design was open randomised.
  • Amyotrophic Lateral Sclerosis (ALS) - Abstracts : Online Reference for Health Concerns 19 January 2010 9:52 UTC www.lef.org [Source type: Academic]

^ Memantine is a clinically well tolerated N-methyl-D-aspartate (NMDA) receptor antagonist--a review of preclinical data.
  • Amyotrophic Lateral Sclerosis (ALS) - Abstracts : Online Reference for Health Concerns 19 January 2010 9:52 UTC www.lef.org [Source type: Academic]

^ Other novel drug targets include caspaces, protein kinases and other molecules influencing apoptosis.
  • Amyotrophic Lateral Sclerosis (ALS) - Abstracts : Online Reference for Health Concerns 19 January 2010 9:52 UTC www.lef.org [Source type: Academic]

[25][27] .Clinical trials with ALS patients showed that Riluzole lengthens survival by several months, and may have a greater survival benefit for those with a bulbar onset.^ A clinical trial of creatine in ALS. Neurology .
  • Natural & Alternative Treatments - Hartford Hospital, Connecticut 19 January 2010 9:52 UTC www.harthosp.org [Source type: Academic]

^ To date, 114 different SOD1 mutations have been identified in ALS patients, with all but one, D90A, showing a dominant mode of inheritance (Andersen et al ., 2003 ).
  • Disulphide-reduced superoxide dismutase-1 in CNS of transgenic amyotrophic lateral sclerosis models -- Jonsson et al. 129 (2): 451 -- Brain 19 January 2010 9:52 UTC brain.oxfordjournals.org [Source type: Academic]

^ Armon C, Brandstater ME. Motor unit number estimate-based rates of progression of ALS predict patient survival.
  • Amyotrophic Lateral Sclerosis: eMedicine Neurology 19 January 2010 9:52 UTC emedicine.medscape.com [Source type: Academic]

.The drug also extends the time before a patient needs ventilation support.^ The drug also extends the time before a patient needs ventilation support.
  • Amyotrophic Lateral Sclerosis Fact Sheet: National Institute of Neurological Disorders and Stroke (NINDS) 19 January 2010 9:52 UTC www.ninds.nih.gov [Source type: Academic]
  • Amyotrophic Lateral Sclerosis (ALS) Support Group - DailyStrength 19 January 2010 9:52 UTC www.dailystrength.org [Source type: FILTERED WITH BAYES]

^ Treatment by Western Medicine 1.Riluzole At present, Riluzole is the only drug approved by the FDA for the treatment of ALS. It is also the only drug shown to extend the survival of ALS patients.
  • Cure Amyotrophic Lateral Sclerosis (ALS), Lou Gehrig's disease, pseudobulbar palsy 19 January 2010 9:52 UTC www.damo-qigong.net [Source type: Academic]

^ Patients may have more trouble with swallowing of thin liquids then thick, and they may need to swallow multiple times to complete transport of liquids through the esophagus to the stomach.
  • Amyotrophic Lateral Sclerosis: eMedicine Emergency Medicine 19 January 2010 9:52 UTC emedicine.medscape.com [Source type: Academic]

.Riluzole does not reverse the damage already done to motor neurons, and patients taking the drug must be monitored for liver damage and other possible side effects.^ All patients were on riluzole; none were taking psychoactive drugs.
  • BioMed Central | Full text | Widespread sensorimotor and frontal cortical atrophy in Amyotrophic Lateral Sclerosis 19 January 2010 9:52 UTC www.biomedcentral.com [Source type: Academic]

^ Riluzole does not reverse the damage already done to motor neurons, and patients taking the drug must be monitored for liver damage and other possible side effects.
  • Amyotrophic Lateral Sclerosis Fact Sheet: National Institute of Neurological Disorders and Stroke (NINDS) 19 January 2010 9:52 UTC www.ninds.nih.gov [Source type: Academic]
  • Fighting Amyotrophic Lateral Sclerosis (ALS), alias Lou Gehrig's Disease 19 January 2010 9:52 UTC www.squidoo.com [Source type: Academic]
  • Amyotrophic Lateral Sclerosis (ALS) Support Group - DailyStrength 19 January 2010 9:52 UTC www.dailystrength.org [Source type: FILTERED WITH BAYES]

^ In an early study, a family history of thyroid disease was present in 19% of ALS patients, and an additional 21% of patients described family members with other possible autoimmune disorders.

.However, this first disease-specific therapy offers hope that the progression of ALS may one day be slowed by new medications or combinations of drugs.^ With inherited ALS, the disease may progress more slowly.

^ However, this first disease-specific therapy offers hope that the progression of ALS may one day be slowed by new medications or combinations of drugs.
  • Amyotrophic Lateral Sclerosis Fact Sheet: National Institute of Neurological Disorders and Stroke (NINDS) 19 January 2010 9:52 UTC www.ninds.nih.gov [Source type: Academic]
  • Fighting Amyotrophic Lateral Sclerosis (ALS), alias Lou Gehrig's Disease 19 January 2010 9:52 UTC www.squidoo.com [Source type: Academic]

^ Studies have shown the drug to slow down the progression on ALS symptoms.
  • Cure Amyotrophic Lateral Sclerosis (ALS), Lou Gehrig's disease, pseudobulbar palsy 19 January 2010 9:52 UTC www.damo-qigong.net [Source type: Academic]

.A small, open-label study recently suggested that the drug lithium which traditionally is used for the treatment of bipolar affective disorder may slow progression in both animal models and the human form of the disease.^ These findings may be of relevance for future therapeutic strategies aimed at slowing the progression of this chronic neurodegenerative disease, as recently suggested by the beneficial therapeutic effects of an IGF-I treatment in ALS patients .
  • Amyotrophic Lateral Sclerosis (ALS) - Abstracts : Online Reference for Health Concerns 19 January 2010 9:52 UTC www.lef.org [Source type: Academic]

^ Cyanobacteria may contain algae species that can produce toxins harmful to humans and animals.
  • SolarBee® Science Office 20 September 2009 17:15 UTC www.solarbee.com [Source type: News]

^ Memantine has been investigated extensively in animal studies and following this, its efficacy and safety has been established and confirmed by clinical experience in humans.
  • Amyotrophic Lateral Sclerosis (ALS) - Abstracts : Online Reference for Health Concerns 19 January 2010 9:52 UTC www.lef.org [Source type: Academic]

[28] .However, further studies were terminated early for failure to replicate the initial success of the original Fornai et al study.^ To further confirm the assignment of the bands, recombinant mutant hSOD1s coexpressed with CCS in E.coli (Ahl et al ., 2004 ) were used as reference materials ( Fig.
  • Disulphide-reduced superoxide dismutase-1 in CNS of transgenic amyotrophic lateral sclerosis models -- Jonsson et al. 129 (2): 451 -- Brain 19 January 2010 9:52 UTC brain.oxfordjournals.org [Source type: Academic]

^ Cholinergic activity, especially in the nucleus basalis, has been normal in several studies (Horoupian et al 1984; Gilbert et al 1988) .
  • Dementia associated with amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.medlink.com [Source type: Academic]

^ The one important exception is the study of Ganzini et al.
  • Resilience and Distress Among Amyotrophic Lateral Sclerosis Patients and Caregivers -- Rabkin et al. 62 (2): 271 -- Psychosomatic Medicine 19 January 2010 9:52 UTC www.psychosomaticmedicine.org [Source type: Academic]

[29][1][2]

Experimental drugs

A number of clinical trials are underway globally for ALS.
.KNS-760704 is under clinical investigation in ALS patients.^ Care of ALS patients is provided collaboratively by multidisciplinary ALS clinics and patients’ family physicians.
  • Amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

^ BACKGROUND: Preclinical and clinical studies of gabapentin in patients with ALS led the authors to undertake a phase III randomized clinical trial.
  • Amyotrophic Lateral Sclerosis (ALS) - Abstracts : Online Reference for Health Concerns 19 January 2010 9:52 UTC www.lef.org [Source type: Academic]

^ In the clinical literature, some commentators have noted the lack of distress and the presence of unexpected cheerfulness and stoicism among patients with ALS ( 2 , 3 ), although others qualify such observations.
  • Resilience and Distress Among Amyotrophic Lateral Sclerosis Patients and Caregivers -- Rabkin et al. 62 (2): 271 -- Psychosomatic Medicine 19 January 2010 9:52 UTC www.psychosomaticmedicine.org [Source type: Academic]

It is hoped that the drug will have a neuroprotective effect. .It is the enantiomer of pramipexole, which is approved for the treatment of Parkinson's disease and restless legs syndrome.^ The syndrome corresponded to Lyme's Disease and improved after treatment with ceftriaxona.
  • Amyotrophic Lateral Sclerosis (ALS) - Abstracts : Online Reference for Health Concerns 19 January 2010 9:52 UTC www.lef.org [Source type: Academic]

^ Riluzole, the only drug launched for treatment ALS is currently undergoing industrial trials for Alzheimer's, Parkinson's, Huntington disease, stroke and head injury.
  • Amyotrophic Lateral Sclerosis (ALS) - Abstracts : Online Reference for Health Concerns 19 January 2010 9:52 UTC www.lef.org [Source type: Academic]

^ Parkinson's disease, Alzheimer's disease, Huntington's disease, ALS) to symptomatic treatment (e.g.
  • Amyotrophic Lateral Sclerosis (ALS) - Abstracts : Online Reference for Health Concerns 19 January 2010 9:52 UTC www.lef.org [Source type: Academic]

[30] However, KNS-760704, which has been manufactured to a high degree of enantiomeric purity and which is essentially inactive at dopamine receptors, is not dose limited by the potent dopaminergic properties of pramipexole.[31] .The potential utility of KNS-760704 in ALS is being advanced in clinical studies by Knopp Neurosciences Inc.^ BACKGROUND: Preclinical and clinical studies of gabapentin in patients with ALS led the authors to undertake a phase III randomized clinical trial.
  • Amyotrophic Lateral Sclerosis (ALS) - Abstracts : Online Reference for Health Concerns 19 January 2010 9:52 UTC www.lef.org [Source type: Academic]

^ Clinical studies have suggested that the beneficial effects of neuroprotection in human amyotrophic lateral sclerosis (ALS) may be due to a preferential effect on early phases of the disease.
  • Amyotrophic Lateral Sclerosis (ALS) - Abstracts : Online Reference for Health Concerns 19 January 2010 9:52 UTC www.lef.org [Source type: Academic]

^ See more articles cited in this paragraph Effect of a multidisciplinary amyotrophic lateral sclerosis (ALS) clinic on ALS survival: a population based study, 1996-2000.
  • Amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

of Pittsburgh, PA.
.The tetracycline antibiotic minocycline is also under investigation for the treatment of ALS among other neurological disorders.^ Other agents approved or under investigation for treating amyotrophic lateral sclerosis include glutamate antagonists, neurotrophic factors , protease inhibitors, and antioxidants.
  • Dementia associated with amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.medlink.com [Source type: Academic]

^ A diagnostic feature of ALS, typically not seen in other disorders, is the presence of hyperreflexia in segmental regions of muscle atrophy, unaccompanied by sensory disturbance.
  • Amyotrophic Lateral Sclerosis Overview -- GeneReviews -- NCBI Bookshelf 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

^ On the other hand, the same abnormalities have been described by means of radiological and manometric studies in various non-neurological disorders which is known as `cricopharyngeal achalasia' (Goyal, 1984 ; Dantas et al ., 1990 ).
  • Pathophysiological mechanisms of oropharyngeal dysphagia in amyotrophic lateral sclerosis -- Ertekin et al. 123 (1): 125 -- Brain 19 January 2010 9:52 UTC brain.oxfordjournals.org [Source type: Academic]

.In rodents with the SOD1 gene mutation that has been associated with ALS, Minocycline was as effective as Riluzole in extending survival, and it delayed the onset of movement problems.^ To date, 114 different SOD1 mutations have been identified in ALS patients, with all but one, D90A, showing a dominant mode of inheritance (Andersen et al ., 2003 ).
  • Disulphide-reduced superoxide dismutase-1 in CNS of transgenic amyotrophic lateral sclerosis models -- Jonsson et al. 129 (2): 451 -- Brain 19 January 2010 9:52 UTC brain.oxfordjournals.org [Source type: Academic]

^ The onset of FALS in middle-aged patients with a SOD1 mutation could be attributed to the accumulation of oxidative damage with aging.
  • Oxidative stress causes abnormal accumulation of familial amyotrophic lateral sclerosis-related mutant SOD1 in transgenic Caenorhabditis elegans -- Oeda et al. 10 (19): 2013 -- Human Molecular Genetics 19 January 2010 9:52 UTC hmg.oxfordjournals.org [Source type: Academic]

^ In ~25% of familial cases, the disease is caused by mutations in the gene encoding cytosolic copper-zinc superoxide dismutase ( SOD1 ) (Rosen et al., 1993 ).
  • Rats Expressing Human Cytosolic Copper-Zinc Superoxide Dismutase Transgenes with Amyotrophic Lateral Sclerosis: Associated Mutations Develop Motor Neuron Disease -- Nagai et al. 21 (23): 9246 -- Journal of Neuroscience 19 January 2010 9:52 UTC www.jneurosci.org [Source type: Academic]

[32] .It is thought to exert its neuroprotective effects not by affecting glutamate release as with Riluzole, but by inhibiting the release of a mitochondrial protein called cytochrome c into the body of the cell.^ Effects of methylcobalamin on mitosis of Schwann cells and incorporation of labeled amino acid into protein fractions of crushed sciatic nerve in rats].
  • Amyotrophic Lateral Sclerosis (ALS) - Abstracts : Online Reference for Health Concerns 19 January 2010 9:52 UTC www.lef.org [Source type: Academic]

^ Riluzole is the only currently FDA-approved drug for the treatment of ALS. Its mechanism of action is thought to be glutamate inhibition.
  • Amyotrophic Lateral Sclerosis Overview -- GeneReviews -- NCBI Bookshelf 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

^ We investigated whether it can exert neuroprotective effects in a variety of animal models.
  • Amyotrophic Lateral Sclerosis (ALS) - Abstracts : Online Reference for Health Concerns 19 January 2010 9:52 UTC www.lef.org [Source type: Academic]

.The new discovery of RNAi has some promise in treating ALS. In recent studies, RNAi has been used in lab rats to shut off specific genes that lead to ALS. Cytrx Corporation has sponsored ALS research using RNAi gene silencing technology targeted at the mutant SOD1 gene.^ Generation of transgenic rats with mutant SOD1 .
  • Rats Expressing Human Cytosolic Copper-Zinc Superoxide Dismutase Transgenes with Amyotrophic Lateral Sclerosis: Associated Mutations Develop Motor Neuron Disease -- Nagai et al. 21 (23): 9246 -- Journal of Neuroscience 19 January 2010 9:52 UTC www.jneurosci.org [Source type: Academic]

^ The ratios of human mutant to rat endogenous SOD1 were determined using an anti-human polyclonal antibody against a peptide sequence identical in human and rat SOD1 (Calbiochem).
  • Rats Expressing Human Cytosolic Copper-Zinc Superoxide Dismutase Transgenes with Amyotrophic Lateral Sclerosis: Associated Mutations Develop Motor Neuron Disease -- Nagai et al. 21 (23): 9246 -- Journal of Neuroscience 19 January 2010 9:52 UTC www.jneurosci.org [Source type: Academic]

^ In ~25% of familial cases, the disease is caused by mutations in the gene encoding cytosolic copper-zinc superoxide dismutase ( SOD1 ) (Rosen et al., 1993 ).
  • Rats Expressing Human Cytosolic Copper-Zinc Superoxide Dismutase Transgenes with Amyotrophic Lateral Sclerosis: Associated Mutations Develop Motor Neuron Disease -- Nagai et al. 21 (23): 9246 -- Journal of Neuroscience 19 January 2010 9:52 UTC www.jneurosci.org [Source type: Academic]

[33] .The mutant SOD1 gene is responsible for causing ALS in a subset of the 10% of all ALS patients who suffer from the familial, or genetic, form of the disease.^ Mutations in superoxide dismutase 1 as cause of one form of familial ALS .
  • Molecular Neurodegeneration | Full text | Experimental models for the study of neurodegeneration in amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.molecularneurodegeneration.com [Source type: Academic]

^ Do all ALS patients become bedridden?
  • Amyotrophic Lateral Sclerosis ALS: rockin for a cure 19 January 2010 9:52 UTC www.rockinforacure.org [Source type: Academic]

^ Other ALS-causing genes .
  • Facts About ALS | MDA/ALS Publications 19 January 2010 9:52 UTC www.als-mda.org [Source type: Academic]

Cytrx's orally-administered drug Arimoclomol is currently in clinical evaluation as a therapeutic treatment for ALS.
.Insulin-like growth factor 1 has also been studied as treatment for ALS. Cephalon and Chiron conducted two pivotal clinical studies of IGF-1 for ALS, and although one study demonstrated efficacy, the second was equivocal, and the product has never been approved by the FDA. In January 2007, the Italian Ministry of Health has requested INSMED corporation's drug, IPLEX, which is a recombinant IGF-1 with Binding Protein 3(IGF1BP3) to be used in a clinical trial for ALS patients in Italy.^ Effect of recombinant human insulin-like growth factor-I on progression of ALS. A placebo-controlled study.
  • Amyotrophic Lateral Sclerosis (ALS) - Abstracts : Online Reference for Health Concerns 19 January 2010 9:52 UTC www.lef.org [Source type: Academic]

^ Recently clinical studies have shown the effectiveness of this growth factor in the treatment of neurodegenerative disorders.
  • Amyotrophic Lateral Sclerosis (ALS) - Abstracts : Online Reference for Health Concerns 19 January 2010 9:52 UTC www.lef.org [Source type: Academic]

^ Cost effectiveness of recombinant human insulin-like growth factor I therapy in patients with ALS. .
  • Amyotrophic Lateral Sclerosis (ALS) - Abstracts : Online Reference for Health Concerns 19 January 2010 9:52 UTC www.lef.org [Source type: Academic]

.Methylcobalamin is being studied in Japan;[34] preliminary results show it significantly lengthens survival time of ALS patients.^ To date, 114 different SOD1 mutations have been identified in ALS patients, with all but one, D90A, showing a dominant mode of inheritance (Andersen et al ., 2003 ).
  • Disulphide-reduced superoxide dismutase-1 in CNS of transgenic amyotrophic lateral sclerosis models -- Jonsson et al. 129 (2): 451 -- Brain 19 January 2010 9:52 UTC brain.oxfordjournals.org [Source type: Academic]

^ We could locate only two studies that examined quality of life of spousal caregivers of ALS patients.
  • Resilience and Distress Among Amyotrophic Lateral Sclerosis Patients and Caregivers -- Rabkin et al. 62 (2): 271 -- Psychosomatic Medicine 19 January 2010 9:52 UTC www.psychosomaticmedicine.org [Source type: Academic]

^ This older age group is the group that is similar in age to the ALS patients and control subjects for the ALS studies.
  • Biomarkers for amyotrophic lateral sclerosis and methods using the same - Patent Application 20070298998 19 January 2010 9:52 UTC www.freepatentsonline.com [Source type: Academic]

Other

.In addition to the use of medical intervention to treat ALS, there are other approaches that people apply to solving the problem.^ When such a method is used to aid in the diagnosis of ALS, the results of the method may be used along with other methods (or the results thereof) useful in the clinical determination of whether a subject has ALS. .
  • Biomarkers for amyotrophic lateral sclerosis and methods using the same - Patent Application 20070298998 19 January 2010 9:52 UTC www.freepatentsonline.com [Source type: Academic]

^ The proteins identified as potential drug targets may then be used to identify compositions that may be potential candidates for treating ALS, including compositions for gene therapy.
  • Biomarkers for amyotrophic lateral sclerosis and methods using the same - Patent Application 20070298998 19 January 2010 9:52 UTC www.freepatentsonline.com [Source type: Academic]

^ There are other differences between this system and the previous one, and for now the author believes forgoing the use of the revised WFN criteria is premature.
  • Amyotrophic Lateral Sclerosis: eMedicine Neurology 19 January 2010 9:52 UTC emedicine.medscape.com [Source type: Academic]

.These include mind body methods, holistic problem solving methods, psychotherapy, hypnotherapy and nutrition related methods.^ Common features include cognitive deficits in attention, abstraction, planning, and problem-solving with preservation of perception and spatial functions.
  • Amyotrophic Lateral Sclerosis Overview -- GeneReviews -- NCBI Bookshelf 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

.Other treatments for ALS are designed to relieve symptoms and improve the quality of life for patients.^ Other treatments for ALS are designed to relieve symptoms and improve the quality of life for patients.
  • Amyotrophic Lateral Sclerosis Fact Sheet: National Institute of Neurological Disorders and Stroke (NINDS) 19 January 2010 9:52 UTC www.ninds.nih.gov [Source type: Academic]
  • Fighting Amyotrophic Lateral Sclerosis (ALS), alias Lou Gehrig's Disease 19 January 2010 9:52 UTC www.squidoo.com [Source type: Academic]

^ What can I do at home to relieve symptoms of ALS? .
  • Health: Amyotrophic Lateral Sclerosis (ALS); Amyotrophic lateral sclerosis (ALS) , also known as ... 19 January 2010 9:52 UTC www.mainegeneral.org [Source type: Academic]
  • CIGNA - Amyotrophic Lateral Sclerosis (ALS) 19 January 2010 9:52 UTC stage.cigna.com [Source type: FILTERED WITH BAYES]

^ Psychotherapy is an essential part of any treatment for depression and can help improve a patient’s quality of life.
  • USPharmacist.com > The Management of Amyotrophic Lateral Sclerosis 19 January 2010 9:52 UTC www.uspharmacist.com [Source type: Academic]

.This supportive care is best provided by multidisciplinary teams of health care professionals such as physicians; pharmacists; physical, occupational, and speech therapists; acupuncturists; nutritionists; social workers; and home care and hospice nurses.^ Individuals with ALS may benefit from care by a multidisciplinary team that includes a neurologist, specially trained nurses, pulmonologist, speech therapist, physical therapist, occupational therapist, respiratory therapist, nutritionist, psychologist, social worker, and genetics professional.
  • Amyotrophic Lateral Sclerosis Overview -- GeneReviews -- NCBI Bookshelf 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

^ Treatment is palliative and many individuals benefit from care by a multidisciplinary team including: a neurologist, specially trained nurses, pulmonologist, speech therapist, physical therapist, occupational therapist, respiratory therapist, nutritionist, psychologist, social worker, and genetic counselor.
  • Amyotrophic Lateral Sclerosis Overview -- GeneReviews -- NCBI Bookshelf 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

^ Care of ALS patients is best provided by multidisciplinary ALS clinics ( Table 4 ) in conjunction with family physicians.
  • Amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

.Working with patients and caregivers, these teams can design an individualized plan of medical and physical therapy and provide special equipment aimed at keeping patients as mobile and comfortable as possible.^ Working with patients and caregivers, these teams can design an individualized plan of medical and physical therapy and provide special equipment aimed at keeping patients as mobile and comfortable as possible.
  • Amyotrophic Lateral Sclerosis Fact Sheet: National Institute of Neurological Disorders and Stroke (NINDS) 19 January 2010 9:52 UTC www.ninds.nih.gov [Source type: Academic]
  • Fighting Amyotrophic Lateral Sclerosis (ALS), alias Lou Gehrig's Disease 19 January 2010 9:52 UTC www.squidoo.com [Source type: Academic]
  • Amyotrophic Lateral Sclerosis (ALS) Support Group - DailyStrength 19 January 2010 9:52 UTC www.dailystrength.org [Source type: FILTERED WITH BAYES]

^ Physical therapy and special equipment can enhance patients' independence and safety throughout the course of ALS. Gentle, low-impact aerobic exercise such as walking, swimming, and stationary bicycling can strengthen unaffected muscles, improve cardiovascular health, and help patients fight fatigue and depression.
  • Amyotrophic Lateral Sclerosis Fact Sheet: National Institute of Neurological Disorders and Stroke (NINDS) 19 January 2010 9:52 UTC www.ninds.nih.gov [Source type: Academic]
  • Amyotrophic Lateral Sclerosis (ALS) Support Group - DailyStrength 19 January 2010 9:52 UTC www.dailystrength.org [Source type: FILTERED WITH BAYES]

^ Treatment Treatment of adult polyglucosan body disease generally requires a team approach and may include physicians, physical therapists, medical social workers, and nurses.
  • CIGNA - Polyglucosan Body Disease, Adult 19 January 2010 9:52 UTC www.cigna.com [Source type: Academic]

.Physicians can prescribe medications to help reduce fatigue, ease muscle cramps, control spasticity, and reduce excess saliva and phlegm.^ Medications such as baclofen and benzodiazepines can help relieve spasticity and muscle cramps.
  • Amyotrophic Lateral Sclerosis Overview -- GeneReviews -- NCBI Bookshelf 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

^ Physicians can prescribe medications to help reduce fatigue, ease muscle cramps, control spasticity, and reduce excess saliva and phlegm.
  • Amyotrophic Lateral Sclerosis Fact Sheet: National Institute of Neurological Disorders and Stroke (NINDS) 19 January 2010 9:52 UTC www.ninds.nih.gov [Source type: Academic]
  • Amyotrophic Lateral Sclerosis (ALS) Support Group - DailyStrength 19 January 2010 9:52 UTC www.dailystrength.org [Source type: FILTERED WITH BAYES]

^ Early in the course of the disease, light aerobic exercise and slow, easy stretching may help you stay strong, reduce fatigue and depression, and prevent muscle cramps and spasms.
  • Health: Amyotrophic Lateral Sclerosis (ALS); Amyotrophic lateral sclerosis (ALS) , also known as ... 19 January 2010 9:52 UTC www.mainegeneral.org [Source type: Academic]

.Drugs also are available to help patients with pain, depression, sleep disturbances, and constipation.^ Drugs also are available to help patients with pain, depression, sleep disturbances, and constipation.
  • Amyotrophic Lateral Sclerosis Fact Sheet: National Institute of Neurological Disorders and Stroke (NINDS) 19 January 2010 9:52 UTC www.ninds.nih.gov [Source type: Academic]

^ These include drugs to ease cramps and muscle twitches, help in handling saliva, reduce anxiety and depression, treat constipation, help with sleep problems, and alleviate pain associated with prolonged immobility and joint displacements.
  • Facts About ALS | MDA/ALS Publications 19 January 2010 9:52 UTC www.als-mda.org [Source type: Academic]
  • Charity.com: Amyotrophic Lateral Sclerosis (ALS), Lou Gehrig's disease- August 2004 Charity-of-the-Month 19 January 2010 9:52 UTC www.charity.com [Source type: Academic]

^ Muscle cramps Constipation Fatigue Excessive salivation Excessive phlegm Pain Depression Therapy .
  • OhioHealth - Amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.ohiohealth.com [Source type: FILTERED WITH BAYES]

Pharmacists can advise on best use of medications. .This is particularly helpful with regards to patients with dysphagia, which many ALS patients experience.^ The dysphagia and related aspiration pneumonia are usually the major handicaps to the quality of life, in addition to the risk of poor nutrition and dehydration, which occur particularly in older patients (Hillel and Miller, 1989 ; Strand et al ., 1996 ).
  • Pathophysiological mechanisms of oropharyngeal dysphagia in amyotrophic lateral sclerosis -- Ertekin et al. 123 (1): 125 -- Brain 19 January 2010 9:52 UTC brain.oxfordjournals.org [Source type: Academic]

^ We report the presence of serum antibodies directed against GM1 ganglioside, a defined neural antigen, in many patients with amyotrophic lateral sclerosis (ALS).
  • Amyotrophic Lateral Sclerosis (ALS) - Abstracts : Online Reference for Health Concerns 19 January 2010 9:52 UTC www.lef.org [Source type: Academic]

^ While the symptoms of motor dysfunction are best recognized, affecting all patients with ALS, a fair proportion of patients also experience emotional and special cognitive difficulties that are part of the disease.
  • Amyotrophic Lateral Sclerosis: eMedicine Neurology 19 January 2010 9:52 UTC emedicine.medscape.com [Source type: Academic]

.They would also monitor a patient's medications to reduce risk of drug interactions.^ Although it was difficult for these patients to swallow solid food, they were at much greater risk of aspirating liquid material.
  • Pathophysiological mechanisms of oropharyngeal dysphagia in amyotrophic lateral sclerosis -- Ertekin et al. 123 (1): 125 -- Brain 19 January 2010 9:52 UTC brain.oxfordjournals.org [Source type: Academic]

^ Several ongoing drug trials are evaluating medications for reducing mortality and treating symptoms in ALS. 17 It is unlikely, however, that a single medication will stop disease progression.
  • Amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

.Physical therapy and special equipment such as assistive technology can enhance patients' independence and safety throughout the course of ALS. Gentle, low-impact aerobic exercise such as walking, swimming, and stationary bicycling can strengthen unaffected muscles, improve cardiovascular health, and help patients fight fatigue and depression.^ Physical therapy can help the muscles maintain strength and flexibility for a time.
  • Natural & Alternative Treatments - Hartford Hospital, Connecticut 19 January 2010 9:52 UTC www.harthosp.org [Source type: Academic]

^ See the entire definition of Peripheral neuropathy Physical therapy: A branch of rehabilitative health that uses specially designed exercises and equipment to help patients regain or improve their physical abilities.
  • Amyotrophic Lateral Sclerosis Glossary of Terms with Definitions on MedicineNet.com 19 January 2010 9:52 UTC www.medicinenet.com [Source type: Academic]

^ Woolley JM, Ringel S. Caregiver quality of life in ALS: relationship between patient health status and caregiver health-related quality of life, role satisfaction and workforce participation [abstract].
  • Resilience and Distress Among Amyotrophic Lateral Sclerosis Patients and Caregivers -- Rabkin et al. 62 (2): 271 -- Psychosomatic Medicine 19 January 2010 9:52 UTC www.psychosomaticmedicine.org [Source type: Academic]

.Range of motion and stretching exercises can help prevent painful spasticity and shortening (contracture) of muscles.^ Range of motion and stretching exercises can help prevent painful spasticity and shortening (contracture) of muscles.
  • Amyotrophic Lateral Sclerosis Fact Sheet: National Institute of Neurological Disorders and Stroke (NINDS) 19 January 2010 9:52 UTC www.ninds.nih.gov [Source type: Academic]
  • Amyotrophic Lateral Sclerosis (ALS) Support Group - DailyStrength 19 January 2010 9:52 UTC www.dailystrength.org [Source type: FILTERED WITH BAYES]

^ Early in the course of the disease, light aerobic exercise and slow, easy stretching may help you stay strong, reduce fatigue and depression, and prevent muscle cramps and spasms.
  • CIGNA - Amyotrophic Lateral Sclerosis (ALS) 19 January 2010 9:52 UTC stage.cigna.com [Source type: FILTERED WITH BAYES]

^ Physical therapy can be helpful in performing range of motion activity and exercises to maintain strength and flexibility.
  • Amyotrophic Lateral Sclerosis - Medpedia 19 January 2010 9:52 UTC wiki.medpedia.com [Source type: Academic]

.Physical therapists can recommend exercises that provide these benefits without overworking muscles.^ Physical therapists can recommend exercises that provide these benefits without overworking muscles.
  • Amyotrophic Lateral Sclerosis Fact Sheet: National Institute of Neurological Disorders and Stroke (NINDS) 19 January 2010 9:52 UTC www.ninds.nih.gov [Source type: Academic]
  • Amyotrophic Lateral Sclerosis (ALS) Support Group - DailyStrength 19 January 2010 9:52 UTC www.dailystrength.org [Source type: FILTERED WITH BAYES]

^ Constipation results from involvement of abdominal and pelvic muscles, compounded by limited physical exercise, certain medical treatments, and a diet lacking in fiber ( 5 , 10 ).
  • Factors correlated with hypermetabolism in patients with amyotrophic lateral sclerosis -- Desport et al. 74 (3): 328 -- American Journal of Clinical Nutrition 19 January 2010 9:52 UTC www.ajcn.org [Source type: Academic]

^ A physical therapist can recommend low-impact exercises to maintain your muscle strength and range of motion as long as possible, helping you preserve a sense of independence.
  • OhioHealth - Amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.ohiohealth.com [Source type: FILTERED WITH BAYES]

.Physiotherapists can suggest devices such as ramps, braces, walkers, and wheelchairs that help patients remain mobile.^ Assistive devices, such as walkers or wheelchairs, can aid mobility; and others, such as bathroom installments, hospital bed, and Hoyer lift, can aid in activities of daily living at home.
  • Amyotrophic Lateral Sclerosis Overview -- GeneReviews -- NCBI Bookshelf 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

^ Other assistive devices such as walkers, wheelchairs, bathroom modifications, hospital beds, and hoyer lifts can aid in activities of daily life.
  • Amyotrophic Lateral Sclerosis Overview -- GeneReviews -- NCBI Bookshelf 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

.Occupational therapists can provide or recomment equipment and adaptations to enable people to retain as much independence in activities of daily living as possible.^ Assistive devices, such as walkers or wheelchairs, can aid mobility; and others, such as bathroom installments, hospital bed, and Hoyer lift, can aid in activities of daily living at home.
  • Amyotrophic Lateral Sclerosis Overview -- GeneReviews -- NCBI Bookshelf 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

.ALS patients who have difficulty speaking may benefit from working with a speech-language pathologist.^ Many patients with amyotrophic lateral sclerosis (ALS; motor neuron disease) use natural or traditional therapies of unproven benefit.
  • Amyotrophic Lateral Sclerosis (ALS) - Abstracts : Online Reference for Health Concerns 19 January 2010 9:52 UTC www.lef.org [Source type: Academic]

^ Individuals with ALS may benefit from care by a multidisciplinary team that includes a neurologist, specially trained nurses, pulmonologist, speech therapist, physical therapist, occupational therapist, respiratory therapist, nutritionist, psychologist, social worker, and genetics professional.
  • Amyotrophic Lateral Sclerosis Overview -- GeneReviews -- NCBI Bookshelf 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

^ Patients’ ability to swallow should be evaluated by speech-language pathologists using bedside swallowing assessments and modified barium swallows.
  • Amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

.These health professionals can teach patients adaptive strategies such as techniques to help them speak louder and more clearly.^ A speech therapist can help teach techniques to make your speech more clearly understood.
  • OhioHealth - Amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.ohiohealth.com [Source type: FILTERED WITH BAYES]
  • Amyotrophic lateral sclerosis 19 January 2010 9:52 UTC cgi.cnn.com [Source type: General]
  • Amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.cnn.com [Source type: General]

^ These health professionals can teach patients adaptive strategies such as techniques to help them speak louder and more clearly.
  • Amyotrophic Lateral Sclerosis Fact Sheet: National Institute of Neurological Disorders and Stroke (NINDS) 19 January 2010 9:52 UTC www.ninds.nih.gov [Source type: Academic]
  • Amyotrophic Lateral Sclerosis (ALS) Support Group - DailyStrength 19 January 2010 9:52 UTC www.dailystrength.org [Source type: FILTERED WITH BAYES]

^ Occupational therapists can suggest devices such as ramps, braces, walkers, and wheelchairs that help patients conserve energy and remain mobile.
  • Amyotrophic Lateral Sclerosis Fact Sheet: National Institute of Neurological Disorders and Stroke (NINDS) 19 January 2010 9:52 UTC www.ninds.nih.gov [Source type: Academic]
  • Amyotrophic Lateral Sclerosis (ALS) Support Group - DailyStrength 19 January 2010 9:52 UTC www.dailystrength.org [Source type: FILTERED WITH BAYES]

.As ALS progresses, speech-language pathologists can recommend the use of augmentative and alternative communication such as voice amplifiers, speech-generating devices (or voice output communication devices) and/or low tech communication techniques such as alphabet boards or yes/no signals.^ Alphabet boards and computer-assisted devices can aid communication.
  • Amyotrophic Lateral Sclerosis Overview -- GeneReviews -- NCBI Bookshelf 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

^ Analytical techniques such as GC-MS (gas chromatography-mass spectrometry) and LC-MS (liquid chromatography-mass spectrometry) were used to analyze the metabolites.
  • Biomarkers for amyotrophic lateral sclerosis and methods using the same - Patent Application 20070298998 19 January 2010 9:52 UTC www.freepatentsonline.com [Source type: Academic]

^ As with the other methods described herein, the comparisons made in the methods of monitoring progression/regression of ALS in a subject may be carried out using various techniques, including simple comparisons, one or more statistical analyses, and combinations thereof.
  • Biomarkers for amyotrophic lateral sclerosis and methods using the same - Patent Application 20070298998 19 January 2010 9:52 UTC www.freepatentsonline.com [Source type: Academic]

.These methods and devices help patients communicate when they can no longer speak or produce vocal sounds.^ These methods and devices help patients communicate when they can no longer speak or produce vocal sounds.
  • Amyotrophic Lateral Sclerosis Fact Sheet: National Institute of Neurological Disorders and Stroke (NINDS) 19 January 2010 9:52 UTC www.ninds.nih.gov [Source type: Academic]
  • Amyotrophic Lateral Sclerosis (ALS) Support Group - DailyStrength 19 January 2010 9:52 UTC www.dailystrength.org [Source type: FILTERED WITH BAYES]

^ Occupational therapists can suggest devices such as ramps, braces, walkers, and wheelchairs that help patients conserve energy and remain mobile.
  • Amyotrophic Lateral Sclerosis Fact Sheet: National Institute of Neurological Disorders and Stroke (NINDS) 19 January 2010 9:52 UTC www.ninds.nih.gov [Source type: Academic]
  • Amyotrophic Lateral Sclerosis (ALS) Support Group - DailyStrength 19 January 2010 9:52 UTC www.dailystrength.org [Source type: FILTERED WITH BAYES]

^ Communication devices can be useful for individuals with amyotrophic lateral sclerosis who have difficulty speaking (dysarthria).
  • CIGNA - Amyotrophic Lateral Sclerosis 19 January 2010 9:52 UTC www.cigna.com [Source type: Academic]

.With the help of occupational Therapists, speech-generating devices can be activated by switches or mouse emulation techniques controlled by small physical movements of, for example, the head, finger or eyes.^ Individuals with ALS may benefit from care by a multidisciplinary team that includes a neurologist, specially trained nurses, pulmonologist, speech therapist, physical therapist, occupational therapist, respiratory therapist, nutritionist, psychologist, social worker, and genetics professional.
  • Amyotrophic Lateral Sclerosis Overview -- GeneReviews -- NCBI Bookshelf 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

^ Treatment is palliative and many individuals benefit from care by a multidisciplinary team including: a neurologist, specially trained nurses, pulmonologist, speech therapist, physical therapist, occupational therapist, respiratory therapist, nutritionist, psychologist, social worker, and genetic counselor.
  • Amyotrophic Lateral Sclerosis Overview -- GeneReviews -- NCBI Bookshelf 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

^ The recent development of the eye movement-controlled on-screen keyboard may enable communication for individuals without any remaining limb function.
  • Amyotrophic Lateral Sclerosis Overview -- GeneReviews -- NCBI Bookshelf 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

.Patients and caregivers can learn from speech-language pathologists and nutritionists how to plan and prepare numerous small meals throughout the day that provide enough calories, fiber, and fluid and how to avoid foods that are difficult to swallow.^ Patients and caregivers can learn from speech therapists and nutritionists how to plan and prepare numerous small meals throughout the day that provide enough calories, fiber, and fluid and how to avoid foods that are difficult to swallow.
  • Amyotrophic Lateral Sclerosis Fact Sheet: National Institute of Neurological Disorders and Stroke (NINDS) 19 January 2010 9:52 UTC www.ninds.nih.gov [Source type: Academic]

^ Speech pathologists can also assist patients who have problems in swallowing.
  • Amyotrophic Lateral Sclerosis: Lou Gehrig's Disease - March 15, 1999 -American Academy of Family Physicians 20 September 2009 17:15 UTC www.aafp.org [Source type: Academic]
  • Amyotrophic Lateral Sclerosis: Lou Gehrig's Disease - CareCure Forums 19 January 2010 9:52 UTC sci.rutgers.edu [Source type: Academic]

^ Social workers provide support such as assistance in obtaining financial aid, arranging durable power of attorney, preparing a living will, and finding support groups for patients and caregivers.
  • Amyotrophic Lateral Sclerosis Fact Sheet: National Institute of Neurological Disorders and Stroke (NINDS) 19 January 2010 9:52 UTC www.ninds.nih.gov [Source type: Academic]

.Patients may begin using suction devices to remove excess fluids or saliva and prevent choking.^ Later, additional devices may be useful.
  • Facts About ALS | MDA/ALS Publications 19 January 2010 9:52 UTC www.als-mda.org [Source type: Academic]
  • Charity.com: Amyotrophic Lateral Sclerosis (ALS), Lou Gehrig's disease- August 2004 Charity-of-the-Month 19 January 2010 9:52 UTC www.charity.com [Source type: Academic]

^ Mechanical suction devices are useful in preventing aspiration.
  • Amyotrophic Lateral Sclerosis: Lou Gehrig's Disease - March 15, 1999 -American Academy of Family Physicians 20 September 2009 17:15 UTC www.aafp.org [Source type: Academic]
  • Amyotrophic Lateral Sclerosis: Lou Gehrig's Disease - CareCure Forums 19 January 2010 9:52 UTC sci.rutgers.edu [Source type: Academic]

^ Patients may begin using suction devices to remove excess fluids or saliva and prevent choking.
  • Amyotrophic Lateral Sclerosis Fact Sheet: National Institute of Neurological Disorders and Stroke (NINDS) 19 January 2010 9:52 UTC www.ninds.nih.gov [Source type: Academic]

.When patients can no longer get enough nourishment from eating, doctors may advise inserting a feeding tube into the stomach.^ The tube, referred to as a PEG, is surgically inserted into the person's stomach.

^ Patients may decide to have a tube placed into their stomach for feeding.
  • Amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.drexelmed.edu [Source type: Academic]

^ Research has indicated, however, that this is no longer true and that more than 50% of all people with ALS may have some cognitive impairment.
  • Symptom Management of the Patient With Amyotrophic Lateral Sclerosis: A Guide for Hospice Nurses 19 January 2010 9:52 UTC www.nursingcenter.com [Source type: Academic]
  • Symptom Management of the Patient With Amyotrophic Lateral Sclerosis: A Guide for Hospice Nurses 19 January 2010 9:52 UTC www.nursingcenter.com [Source type: Academic]

.The use of a feeding tube also reduces the risk of choking and pneumonia that can result from inhaling liquids into the lungs.^ A feeding tube can reduce these risks.
  • OhioHealth - Amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.ohiohealth.com [Source type: FILTERED WITH BAYES]
  • Amyotrophic lateral sclerosis 19 January 2010 9:52 UTC cgi.cnn.com [Source type: General]
  • Amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.cnn.com [Source type: General]

^ The use of a feeding tube also reduces the risk of choking and pneumonia that can result from inhaling liquids into the lungs.
  • Amyotrophic Lateral Sclerosis Fact Sheet: National Institute of Neurological Disorders and Stroke (NINDS) 19 January 2010 9:52 UTC www.ninds.nih.gov [Source type: Academic]

^ Patients may decide to have a tube placed into their stomach for feeding.
  • Amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.drexelmed.edu [Source type: Academic]

.The tube is not painful and does not prevent patients from eating food orally if they wish.^ Although it was difficult for these patients to swallow solid food, they were at much greater risk of aspirating liquid material.
  • Pathophysiological mechanisms of oropharyngeal dysphagia in amyotrophic lateral sclerosis -- Ertekin et al. 123 (1): 125 -- Brain 19 January 2010 9:52 UTC brain.oxfordjournals.org [Source type: Academic]

^ How do ALS patients eat when they lose their ability to chew and swallow?
  • Amyotrophic Lateral Sclerosis ALS: rockin for a cure 19 January 2010 9:52 UTC www.rockinforacure.org [Source type: Academic]

^ If it's still possible to swallow some foods or liquids safely, the ALS patient can continue to eat and drink after placement of a feeding tube, but the swallowing mechanism no longer has to be relied on as the sole method of obtaining adequate nutrition.
  • Charity.com: Amyotrophic Lateral Sclerosis (ALS), Lou Gehrig's disease- August 2004 Charity-of-the-Month 19 January 2010 9:52 UTC www.charity.com [Source type: Academic]

.When the muscles that assist in breathing weaken, use of ventilatory assistance (intermittent positive pressure ventilation (IPPV), bilevel positive airway pressure (BIPAP), or biphasic cuirass ventilation (BCV)) may be used to aid breathing.^ When such a method is used to aid in the diagnosis of ALS, the results of the method may be used along with other methods (or the results thereof) useful in the clinical determination of whether a subject has ALS. .
  • Biomarkers for amyotrophic lateral sclerosis and methods using the same - Patent Application 20070298998 19 January 2010 9:52 UTC www.freepatentsonline.com [Source type: Academic]

^ This method may provide a clinically useful measure to improve or retard muscle wasting, if a larger extended trial fulfills its promise .
  • Amyotrophic Lateral Sclerosis (ALS) - Abstracts : Online Reference for Health Concerns 19 January 2010 9:52 UTC www.lef.org [Source type: Academic]

^ As described herein, ratios of biomarkers (e.g., a ratio of paraxanthine to caffeine) may be used as ALS-positive and/or ALS-negative reference levels.
  • Biomarkers for amyotrophic lateral sclerosis and methods using the same - Patent Application 20070298998 19 January 2010 9:52 UTC www.freepatentsonline.com [Source type: Academic]

.Such devices artificially inflate the patient's lungs from various external sources that are applied directly to the face or body.^ Such devices artificially inflate the patient's lungs from various external sources that are applied directly to the face or body.
  • Amyotrophic Lateral Sclerosis Fact Sheet: National Institute of Neurological Disorders and Stroke (NINDS) 19 January 2010 9:52 UTC www.ninds.nih.gov [Source type: Academic]

^ Occupational therapists can suggest devices such as ramps, braces, walkers, and wheelchairs that help patients conserve energy and remain mobile.
  • Amyotrophic Lateral Sclerosis Fact Sheet: National Institute of Neurological Disorders and Stroke (NINDS) 19 January 2010 9:52 UTC www.ninds.nih.gov [Source type: Academic]
  • Amyotrophic Lateral Sclerosis (ALS) Support Group - DailyStrength 19 January 2010 9:52 UTC www.dailystrength.org [Source type: FILTERED WITH BAYES]

^ There is no cure for ALS. Devices such as wheelchairs and speech synthesizers can help patients maintain independence.
  • amyotrophic lateral sclerosis Facts, information, pictures | Encyclopedia.com articles about amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.encyclopedia.com [Source type: Academic]

.When muscles are no longer able to maintain oxygen and carbon dioxide levels, these devices may be used full-time.^ When muscles are no longer able to maintain oxygen and carbon dioxide levels, these devices may be used full-time.
  • Amyotrophic Lateral Sclerosis Fact Sheet: National Institute of Neurological Disorders and Stroke (NINDS) 19 January 2010 9:52 UTC www.ninds.nih.gov [Source type: Academic]

^ Later, additional devices may be useful.
  • Facts About ALS | MDA/ALS Publications 19 January 2010 9:52 UTC www.als-mda.org [Source type: Academic]
  • Charity.com: Amyotrophic Lateral Sclerosis (ALS), Lou Gehrig's disease- August 2004 Charity-of-the-Month 19 January 2010 9:52 UTC www.charity.com [Source type: Academic]

^ These methods and devices help patients communicate when they can no longer speak or produce vocal sounds.
  • Amyotrophic Lateral Sclerosis Fact Sheet: National Institute of Neurological Disorders and Stroke (NINDS) 19 January 2010 9:52 UTC www.ninds.nih.gov [Source type: Academic]
  • Amyotrophic Lateral Sclerosis (ALS) Support Group - DailyStrength 19 January 2010 9:52 UTC www.dailystrength.org [Source type: FILTERED WITH BAYES]

BCV has the added advantage of being able to assist in clearing secretions by using high-frequency oscillations followed by several positive expiratory breaths. .[35] Patients may eventually consider forms of mechanical ventilation (respirators) in which a machine inflates and deflates the lungs.^ Invasive ventilation involves tracheostomy and mechanical ventilation and is declined by most ALS patients at our clinic.
  • Amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

^ CONCLUSIONS: It is concluded that in some ALS cases and also in some patients with other motor neuron diseases an autoimmune mechanism may contribute to motor neuron injury .
  • Amyotrophic Lateral Sclerosis (ALS) - Abstracts : Online Reference for Health Concerns 19 January 2010 9:52 UTC www.lef.org [Source type: Academic]

.To be effective, this may require a tube that passes from the nose or mouth to the windpipe (trachea) and for long-term use, an operation such as a tracheostomy, in which a plastic breathing tube is inserted directly in the patient's windpipe through an opening in the neck.^ Delpoplo said he used to see about 150 boaters near Long Point State Park, now, he said, there may be six or seven.
  • SolarBee® Science Office 20 September 2009 17:15 UTC www.solarbee.com [Source type: News]

^ We examined the cortical concentrations of glutamate using in vivo microdialysis and in vivo nuclear magnetic resonance (NMR) spectroscopy, and the effect of long-term creatine supplementation.
  • Amyotrophic Lateral Sclerosis (ALS) - Abstracts : Online Reference for Health Concerns 19 January 2010 9:52 UTC www.lef.org [Source type: Academic]

^ The impact of potential long-term exposure to low levels of MMT combustion products that may be present in emissions from automobiles has yet to be fully evaluated.
  • Amyotrophic Lateral Sclerosis (ALS) - Abstracts : Online Reference for Health Concerns 19 January 2010 9:52 UTC www.lef.org [Source type: Academic]

.Patients and their families should consider several factors when deciding whether and when to use one of these options.^ Patients and their families should consider several factors when deciding whether and when to use one of these options.
  • Amyotrophic Lateral Sclerosis Fact Sheet: National Institute of Neurological Disorders and Stroke (NINDS) 19 January 2010 9:52 UTC www.ninds.nih.gov [Source type: Academic]

^ This information should not be considered complete, up to date, and is not intended to be used in place of a visit, consultation, or advice of a legal, medical, or any other professional.
  • Lou Gehrig disease - definition of Lou Gehrig disease in the Medical dictionary - by the Free Online Medical Dictionary, Thesaurus and Encyclopedia. 20 September 2009 17:15 UTC medical-dictionary.thefreedictionary.com [Source type: Academic]

^ They also discussed questions concerning the benefits of this type of support, with the aim of letting patients decide on their manner of treatment, with full awareness and authorization regarding whether or not their suffering should be prolonged by using this ventilation support.
  • Sao Paulo Medical Journal - Physiotherapeutic conduct in amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.scielo.br [Source type: Academic]

.Ventilation devices differ in their effect on the patient's quality of life and in cost.^ Ventilation devices differ in their effect on the patient's quality of life and in cost.
  • Amyotrophic Lateral Sclerosis Fact Sheet: National Institute of Neurological Disorders and Stroke (NINDS) 19 January 2010 9:52 UTC www.ninds.nih.gov [Source type: Academic]

^ We could locate only two studies that examined quality of life of spousal caregivers of ALS patients.
  • Resilience and Distress Among Amyotrophic Lateral Sclerosis Patients and Caregivers -- Rabkin et al. 62 (2): 271 -- Psychosomatic Medicine 19 January 2010 9:52 UTC www.psychosomaticmedicine.org [Source type: Academic]

^ Other treatments for ALS are designed to relieve symptoms and improve the quality of life for patients.
  • Amyotrophic Lateral Sclerosis Fact Sheet: National Institute of Neurological Disorders and Stroke (NINDS) 19 January 2010 9:52 UTC www.ninds.nih.gov [Source type: Academic]
  • Fighting Amyotrophic Lateral Sclerosis (ALS), alias Lou Gehrig's Disease 19 January 2010 9:52 UTC www.squidoo.com [Source type: Academic]
  • Amyotrophic Lateral Sclerosis (ALS) Support Group - DailyStrength 19 January 2010 9:52 UTC www.dailystrength.org [Source type: FILTERED WITH BAYES]

.Although ventilation support can ease problems with breathing and prolong survival, it does not affect the progression of ALS. Patients need to be fully informed about these considerations and the long-term effects of life without movement before they make decisions about ventilation support.^ The ventilator then does the person's breathing for him or her.

^ ALS progression can vary considerably with different patients.
  • Amyotrophic Lateral Sclerosis ALS: rockin for a cure 19 January 2010 9:52 UTC www.rockinforacure.org [Source type: Academic]

^ Does ALS affect speech?
  • Amyotrophic Lateral Sclerosis ALS: rockin for a cure 19 January 2010 9:52 UTC www.rockinforacure.org [Source type: Academic]

.Some patients under long-term tracheostomy intermittent positive pressure ventilation with deflated cuffs or cuffless tracheostomy tubes (leak ventilation) are able to speak, provided their bulbar muscles are strong enough.^ In most patients with bulbar/suprabulbar involvement (26 out of 35), the perioral muscles, tongue and submental-suprahyoid muscles were often weak.
  • Pathophysiological mechanisms of oropharyngeal dysphagia in amyotrophic lateral sclerosis -- Ertekin et al. 123 (1): 125 -- Brain 19 January 2010 9:52 UTC brain.oxfordjournals.org [Source type: Academic]

.This technique preserves speech in some patients with long-term mechanical ventilation.^ When muscles in the diaphragm and chest wall fail, patients lose the ability to breathe without help from mechanical ventilation.
  • Amyotrophic Lateral Sclerosis (ALS) Support Group - DailyStrength 19 January 2010 9:52 UTC www.dailystrength.org [Source type: FILTERED WITH BAYES]

^ Gourie-Devi, M, Nalini, A. Long-term follow-up of 44 patients with brachial monomelic amyotrophy.
  • Diagnosis of amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.uptodate.com [Source type: Academic]
  • Diagnosis of amyotrophic lateral sclerosis - a knol by Internet Medical Publishing 19 January 2010 9:52 UTC knol.google.com [Source type: Academic]

^ Invasive ventilation involves tracheostomy and mechanical ventilation and is declined by most ALS patients at our clinic.
  • Amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]
  • Amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.cfpc.ca [Source type: Academic]

.Social workers and home care and hospice nurses help patients, families, and caregivers with the medical, emotional, and financial challenges of coping with ALS, particularly during the final stages of the disease.^ Hospice care in terminal stages is beneficial.
  • Amyotrophic Lateral Sclerosis Overview -- GeneReviews -- NCBI Bookshelf 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

^ Care of ALS patients is provided collaboratively by multidisciplinary ALS clinics and patients’ family physicians.
  • Amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

^ Recently, it was found that some patients with autosomal-dominant familial ALS (FALS) have point mutations in the gene that encodes Cu/Zn superoxide dismutase (SOD1).
  • Amyotrophic Lateral Sclerosis (ALS) - Abstracts : Online Reference for Health Concerns 19 January 2010 9:52 UTC www.lef.org [Source type: Academic]

.Social workers provide support such as assistance in obtaining financial aid, arranging durable power of attorney, preparing a living will, and finding support groups for patients and caregivers.^ Charlotte Area ALS Caregiver Support Group (ALS Association ) http://www.catfishchapter.org/events/charlotte-area-als-caregiver-support-group (704) 315-5627 .
  • Amyotrophic Lateral Sclerosis -- Web Sites Located in Charlotte -- NC Health Info 19 January 2010 9:52 UTC www.nchealthinfo.org [Source type: Academic]

^ End-of-life care can be provided in various settings depending on patient preferences and caregiver capacities.
  • Amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

.Home nurses are available not only to provide medical care but also to teach caregivers about tasks such as maintaining respirators, giving feedings, and moving patients to avoid painful skin problems and contractures.^ Respiratory therapists can help caregivers with tasks such as operating and maintaining respirators, and home care nurses are available not only to provide medical care but also to teach caregivers about giving tube feedings and moving patients to avoid painful skin problems and contractures.
  • Amyotrophic Lateral Sclerosis Fact Sheet: National Institute of Neurological Disorders and Stroke (NINDS) 19 January 2010 9:52 UTC www.ninds.nih.gov [Source type: Academic]

^ Social workers and home care and hospice nurses help patients, families, and caregivers with the medical, emotional, and financial challenges of coping with ALS, particularly during the final stages of the disease.
  • Amyotrophic Lateral Sclerosis Fact Sheet: National Institute of Neurological Disorders and Stroke (NINDS) 19 January 2010 9:52 UTC www.ninds.nih.gov [Source type: Academic]

^ Pharmacists can give advice on the proper use of medications and monitor a patient's prescriptions to avoid risks of drug interactions.
  • Amyotrophic Lateral Sclerosis Fact Sheet: National Institute of Neurological Disorders and Stroke (NINDS) 19 January 2010 9:52 UTC www.ninds.nih.gov [Source type: Academic]
  • Amyotrophic Lateral Sclerosis (ALS) Support Group - DailyStrength 19 January 2010 9:52 UTC www.dailystrength.org [Source type: FILTERED WITH BAYES]

.Home hospice nurses work in consultation with physicians to ensure proper medication, pain control, and other care affecting the quality of life of patients who wish to remain at home.^ Home hospice nurses work in consultation with physicians to ensure proper medication, pain control, and other care affecting the quality of life of patients who wish to remain at home.
  • Amyotrophic Lateral Sclerosis Fact Sheet: National Institute of Neurological Disorders and Stroke (NINDS) 19 January 2010 9:52 UTC www.ninds.nih.gov [Source type: Academic]

^ This supportive care is best provided by multidisciplinary teams of health care professionals such as physicians; pharmacists; physical, occupational, and speech therapists; nutritionists; social workers; and home care and hospice nurses.
  • Amyotrophic Lateral Sclerosis Fact Sheet: National Institute of Neurological Disorders and Stroke (NINDS) 19 January 2010 9:52 UTC www.ninds.nih.gov [Source type: Academic]
  • Amyotrophic Lateral Sclerosis (ALS) Support Group - DailyStrength 19 January 2010 9:52 UTC www.dailystrength.org [Source type: FILTERED WITH BAYES]

^ Most of this, in the later stages, requires nursing management of a patient who is alert but functionally quadriplegic with intact sensory function, bedridden and aware he or she is going to die."
  • AMYOTROPHIC LATERAL SCLEROSIS 19 January 2010 9:52 UTC www.dhs.state.or.us [Source type: Academic]

.The home hospice team can also counsel patients and caregivers about end-of-life issues.^ The home hospice team can also counsel patients and caregivers about end-of-life issues.
  • Amyotrophic Lateral Sclerosis Fact Sheet: National Institute of Neurological Disorders and Stroke (NINDS) 19 January 2010 9:52 UTC www.ninds.nih.gov [Source type: Academic]

^ Social workers and home care and hospice nurses help patients, families, and caregivers with the medical, emotional, and financial challenges of coping with ALS, particularly during the final stages of the disease.
  • Amyotrophic Lateral Sclerosis Fact Sheet: National Institute of Neurological Disorders and Stroke (NINDS) 19 January 2010 9:52 UTC www.ninds.nih.gov [Source type: Academic]

^ We could locate only two studies that examined quality of life of spousal caregivers of ALS patients.
  • Resilience and Distress Among Amyotrophic Lateral Sclerosis Patients and Caregivers -- Rabkin et al. 62 (2): 271 -- Psychosomatic Medicine 19 January 2010 9:52 UTC www.psychosomaticmedicine.org [Source type: Academic]

Both animal and human research suggest calorie restriction (CR) may be contraindicated for those with ALS. Research on a transgenic mouse model of ALS demonstrates that CR may hasten the onset of death in ALS.[36] In that study, Hamadeh et al. also note two human studies[37][38] that they indicate show "low energy intake correlates with death in people with ALS." However, in the first study, Slowie, Paige, and Antel state: "The reduction in energy intake by ALS patients did not correlate with the proximity of death but rather was a consistent aspect of the illness." They go on to conclude: "We conclude that ALS patients have a chronically deficient intake of energy and recommended augmentation of energy intake."[37]
.Previously, Pedersen and Mattson also found that in the ALS mouse model, CR "accelerates the clinical course" of the disease and had no benefits.^ ALS patients were diagnosed on the basis of characteristic clinical course, physical and electrodiagnostic findings, and absence of evidence of spondylotic myelopathy, paraproteinemias, hyperparathyroidism, Lyme disease, glycoprotein antibodies, and vitamin E toxicity.
  • Dr. John R. Bach - The DMD Doc 19 January 2010 9:52 UTC www.doctorbach.com [Source type: Academic]

^ In 90 to 95% of all ALS cases, the disease occurs apparently at random with no clearly associated risk factors.
  • Amyotrophic Lateral Sclerosis (ALS) Support Group - DailyStrength 19 January 2010 9:52 UTC www.dailystrength.org [Source type: FILTERED WITH BAYES]

^ No cure has yet been found for ALS. However, the Food and Drug Administration (FDA) has approved the first drug treatment for the disease: riluzole (Rilutek).
  • Amyotrophic Lateral Sclerosis (ALS) Support Group - DailyStrength 19 January 2010 9:52 UTC www.dailystrength.org [Source type: FILTERED WITH BAYES]

[39] .Suggesting that a calorically dense diet may slow ALS, a ketogenic diet in the ALS mouse model has been shown to slow the progress of disease.^ The ALS-positive and/or ALS-negative reference levels may be levels that are specific for comparison with another particular neurodegenerative disease (e.g., reference levels of biomarkers for ALS that distinguish between peripheral neuropathy or myopathy).
  • Biomarkers for amyotrophic lateral sclerosis and methods using the same - Patent Application 20070298998 19 January 2010 9:52 UTC www.freepatentsonline.com [Source type: Academic]

^ Several ongoing drug trials are evaluating medications for reducing mortality and treating symptoms in ALS. 17 It is unlikely, however, that a single medication will stop disease progression.
  • Amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

^ Clinical trials have shown marginal slowing of disease progression in some but not all individuals, [ Riviere et al 1998 ].
  • Amyotrophic Lateral Sclerosis Overview -- GeneReviews -- NCBI Bookshelf 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

[40]

Prognosis

.Regardless of the part of the body first affected by the disease, it is usual for muscle weakness and atrophy to spread to other parts of the body as the disease progresses.^ It usually begins with weakness in the hands or feet, which then spreads to the rest of the body.
  • Natural & Alternative Treatments - Hartford Hospital, Connecticut 19 January 2010 9:52 UTC www.harthosp.org [Source type: Academic]

^ Regardless of initial symptoms, atrophy and weakness eventually affect other muscles.
  • Amyotrophic Lateral Sclerosis Overview -- GeneReviews -- NCBI Bookshelf 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

^ Various subtypes of ALS may be identified: "progressive bulbar palsy," which presents with speech disturbance and swallowing difficulties; limb-onset ALS; progressive muscular atrophy in which only lower motor neurons are involved; and UMN-predominant ALS. Regardless of initial symptoms, atrophy and weakness eventually spread to affect other muscles.
  • Amyotrophic Lateral Sclerosis Overview -- GeneReviews -- NCBI Bookshelf 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

.It is important to remember that some patients with ALS have an arrested course with no progression beyond a certain point despite extensive follow-up.^ Armon C, Brandstater ME. Motor unit number estimate-based rates of progression of ALS predict patient survival.
  • Amyotrophic Lateral Sclerosis: eMedicine Neurology 19 January 2010 9:52 UTC emedicine.medscape.com [Source type: Academic]

^ Recently, it was found that some patients with autosomal-dominant familial ALS (FALS) have point mutations in the gene that encodes Cu/Zn superoxide dismutase (SOD1).
  • Amyotrophic Lateral Sclerosis (ALS) - Abstracts : Online Reference for Health Concerns 19 January 2010 9:52 UTC www.lef.org [Source type: Academic]

^ There was no difference in the rate of progression as measured by the Appel ALS total score, showing an average increase of 22 points in 6 months.
  • Amyotrophic Lateral Sclerosis (ALS) - Abstracts : Online Reference for Health Concerns 19 January 2010 9:52 UTC www.lef.org [Source type: Academic]

.Such a pattern is particularly true for young males with predominant upper limb weakness especially on one side (so-called monomelic or Hirayama type motor neuron disease).^ Moreover, mice with targeted inactivation of the SOD1 gene do not develop motor neuron disease (Reaume et al., 1996 ).
  • Rats Expressing Human Cytosolic Copper-Zinc Superoxide Dismutase Transgenes with Amyotrophic Lateral Sclerosis: Associated Mutations Develop Motor Neuron Disease -- Nagai et al. 21 (23): 9246 -- Journal of Neuroscience 19 January 2010 9:52 UTC www.jneurosci.org [Source type: Academic]

^ However, in patients with pure lower motor neuron disorders, both voluntary and reflexive movements are expected to be similarly weak.
  • Pathophysiological mechanisms of oropharyngeal dysphagia in amyotrophic lateral sclerosis -- Ertekin et al. 123 (1): 125 -- Brain 19 January 2010 9:52 UTC brain.oxfordjournals.org [Source type: Academic]

^ Wild-type nonneuronal cells extend survival of SOD1 mutant motor neurons in ALS mice.
  • Disulphide-reduced superoxide dismutase-1 in CNS of transgenic amyotrophic lateral sclerosis models -- Jonsson et al. 129 (2): 451 -- Brain 19 January 2010 9:52 UTC brain.oxfordjournals.org [Source type: Academic]

.Eventually many people with ALS are not able to stand or walk, get in or out of bed on their own, or use their hands and arms.^ Many patients with amyotrophic lateral sclerosis (ALS; motor neuron disease) use natural or traditional therapies of unproven benefit.
  • Amyotrophic Lateral Sclerosis (ALS) - Abstracts : Online Reference for Health Concerns 19 January 2010 9:52 UTC www.lef.org [Source type: Academic]

^ The best procedure is to let them die out on their own as they use up the nutrients,” Lanfear said.
  • SolarBee® Science Office 20 September 2009 17:15 UTC www.solarbee.com [Source type: News]

^ Evidence from animal and open human trials had suggested that creatine improved strength and slowed the progression of the disease, and for these reasons many people with ALS have tried it.
  • Natural & Alternative Treatments - Hartford Hospital, Connecticut 19 January 2010 9:52 UTC www.harthosp.org [Source type: Academic]

.In later stages of the disease, individuals have difficulty breathing as the muscles of the respiratory system weaken.^ The toxin found in the water causes almost immediate clinical signs, such as muscle tremors, respiratory disease and convulsions.
  • SolarBee® Science Office 20 September 2009 17:15 UTC www.solarbee.com [Source type: News]

^ Ben Hamida M, Hentati F, Ben Hamida C: Hereditary motor system diseases (chronic juvenile amyotrophic lateral sclerosis).
  • Molecular Neurodegeneration | Full text | Experimental models for the study of neurodegeneration in amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.molecularneurodegeneration.com [Source type: Academic]

^ To establish the extent of disease in an individual diagnosed with amyotrophic lateral sclerosis (ALS), the following evaluations are recommended: .
  • Amyotrophic Lateral Sclerosis Overview -- GeneReviews -- NCBI Bookshelf 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

.Although ventilation support can ease problems with breathing and prolong survival, it does not affect the progression of ALS. Most people with ALS die from respiratory failure, usually within three to five years from the onset of symptoms.^ Most people die within 3 years of being diagnosed.
  • Natural & Alternative Treatments - Hartford Hospital, Connecticut 19 January 2010 9:52 UTC www.harthosp.org [Source type: Academic]

^ Amyotrophic lateral sclerosis, described in 1869 by the French neurologist Jean-Martin Charcot, is a fatal adult-onset neurodegenerative disease characterized by the selective and progressive death of both upper and lower motoneurons, leading to a progressive paralysis, respiratory depression and death usually within 2–5 years after onset.
  • Molecular Neurodegeneration | Full text | Experimental models for the study of neurodegeneration in amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.molecularneurodegeneration.com [Source type: Academic]

^ Note: Although most individuals diagnosed with autosomal dominant ALS have an affected parent, the family history may appear to be negative because of failure to recognize the disorder in family members, early death of the parent before the onset of symptoms, or late onset or reduced penetrance of the disease in the affected parent.
  • Amyotrophic Lateral Sclerosis Overview -- GeneReviews -- NCBI Bookshelf 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

.However, about 10–20 percent[41] of those individuals with ALS survive 10 or more years.^ In middle of Kii peninsula, one of the biggest mercury mine in Japan had been present until about 10 years ago.
  • Amyotrophic Lateral Sclerosis (ALS) - Abstracts : Online Reference for Health Concerns 19 January 2010 9:52 UTC www.lef.org [Source type: Academic]

^ Of the 4,133 SNPs within brain-expressed genes of the axon-guidance pathway, 442 SNPs (10.7%) were individually associated with susceptibility to ALS, as detailed in Text S1 .
  • PLoS ONE: Beyond Parkinson Disease: Amyotrophic Lateral Sclerosis and the Axon Guidance Pathway 19 January 2010 9:52 UTC www.plosone.org [Source type: Academic]

^ In CSF of patients with ALS, however, thiamin monophosphate values decreased much more than thiamin levels, so that the T/TMP ratio was significantly increased.
  • Amyotrophic Lateral Sclerosis (ALS) - Abstracts : Online Reference for Health Concerns 19 January 2010 9:52 UTC www.lef.org [Source type: Academic]

Epidemiology

.ALS is one of the most common neuromuscular diseases worldwide, and people of all races and ethnic backgrounds are affected.^ Lou Gehrig's Disease is one of the most common neuromuscular diseases worldwide, and people of all races and ethnic backgrounds are affected.
  • Leu Gehrig's Disease: symptoms, cause, treatments, risks, complications, long-term outlook 19 January 2010 9:52 UTC mamashealth.com [Source type: Academic]

^ Unfortunately, in all parts of the world, ALS happens to be one of the most common diseases of the neuromuscular system.
  • ALS - Amyotrophic Lateral Sclerosis 19 January 2010 9:52 UTC www.mahalo.com [Source type: Academic]

^ ALS occurs worldwide and is equally common in all races and ethnic background.
  • Amyotrophic lateral sclerosis (Lou Gehrig's Disease, Charcot's Disease) 19 January 2010 9:52 UTC www.virtualmedicalcentre.com [Source type: General]

.One or two out of 100,000 people develop ALS each year.^ The number of individuals newly diagnosed with ALS each year is 1-3:100,000.
  • Amyotrophic Lateral Sclerosis Overview -- GeneReviews -- NCBI Bookshelf 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

^ Orrell et al [1997] reported a range of duration of 2.5 to 20 years in individuals with the p.Ile113Thr mutation and of two to 12 years in individuals with the p.Gly93Arg mutation .
  • Amyotrophic Lateral Sclerosis Overview -- GeneReviews -- NCBI Bookshelf 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

^ In one large pedigree , presentation typical of ALS began in the legs for the majority of individuals; onset was age 45 years and duration was five years.
  • Amyotrophic Lateral Sclerosis Overview -- GeneReviews -- NCBI Bookshelf 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

[42] .ALS most commonly strikes people between 40 and 60 years of age, but younger and older people can also develop the disease.^ As many as 30,000 Americans have ALS at any given time and the disease most often strikes between the ages of 40 and 70.

^ ALS most commonly strikes people between 40 and 60 years of age, but younger and older people also can develop the disease.
  • Amyotrophic Lateral Sclerosis Fact Sheet: National Institute of Neurological Disorders and Stroke (NINDS) 19 January 2010 9:52 UTC www.ninds.nih.gov [Source type: Academic]

^ Most people who develop ALS are between the ages of 40 and 70, although the disease can occur at a younger age.
  • Amyotrophic Lateral Sclerosis (ALS) - New York Presbyterian Hospital 19 January 2010 9:52 UTC nyp.org [Source type: Academic]
  • Amyotrophic Lateral Sclerosis (ALS) - New York Presbyterian Hospital 19 January 2010 9:52 UTC nyp.org [Source type: Academic]
  • Amyotrophic Lateral Sclerosis (ALS), Robert Wood Johnson University Hospital, New Brunswick, NJ, 08903 19 January 2010 9:52 UTC www.rwjuh.edu [Source type: Academic]
  • Amyotrophic Lateral Sclerosis (ALS) 19 January 2010 9:52 UTC www.lancastergeneral.org [Source type: Academic]
  • Amyotrophic Lateral Sclerosis (ALS) 19 January 2010 9:52 UTC www.montefiore.org [Source type: Academic]
  • Amyotrophic Lateral Sclerosis (ALS) 19 January 2010 9:52 UTC healthlibrary.brighamandwomens.org [Source type: Academic]
  • GWDOCS - Amyotrophic Lateral Sclerosis (ALS) 19 January 2010 9:52 UTC www.gwdocs.com [Source type: Academic]
  • http://medicalcenter.osu.edu/patientcare/healthcare_services/nervous_system/als/Pages/index.aspx 19 January 2010 9:52 UTC medicalcenter.osu.edu [Source type: Academic]
  • Amyotrophic Lateral Sclerosis (ALS) - Nervous System Disorders - The Nebraska Medical Center 19 January 2010 9:52 UTC www.nebraskamed.com [Source type: Academic]
  • Howard County General Hospital - Amyotrophic Lateral Sclerosis (ALS) 19 January 2010 9:52 UTC www.hcgh.org [Source type: Academic]
  • Amyotrophic Lateral Sclerosis (ALS) | Cooper University Hospital - South Jersey, New Jersey & Philadelphia 19 January 2010 9:52 UTC www.cooperhealth.org [Source type: Academic]
  • Norwood Hospital - Amyotrophic Lateral Sclerosis (ALS) 19 January 2010 9:52 UTC www.caritasnorwood.org [Source type: Academic]
  • Amyotrophic Lateral Sclerosis (ALS) 19 January 2010 9:52 UTC www.memorialhealth.com [Source type: Academic]
  • ALS, Amyotrophic Lateral Sclerosis, Lou Gehrig's disease 19 January 2010 9:52 UTC www.hopkinsmedicine.org [Source type: Academic]
  • Amyotrophic Lateral Sclerosis (ALS) - Carilion Clinic 19 January 2010 9:52 UTC www.carilionclinic.org [Source type: Academic]
  • Main Line Health - Amyotrophic Lateral Sclerosis (ALS) 19 January 2010 9:52 UTC www.mainlinehealth.org [Source type: Academic]
  • Amyotrophic Lateral Sclerosis (ALS) - Online Medical Encyclopedia - University of Rochester Medical Center 19 January 2010 9:52 UTC www.urmc.rochester.edu [Source type: Academic]
  • Amyotrophic Lateral Sclerosis (ALS) | Health Library | Weill Cornell Physicians | Advancing Science. Enhancing Life. 19 January 2010 9:52 UTC www.weillcornell.org [Source type: Academic]
  • Akron General Medical Center -- Amyotrophic Lateral Sclerosis (ALS) 19 January 2010 9:52 UTC healthinfo.akrongeneral.org [Source type: Academic]
  • Neurological Disorders:Amyotrophic Lateral Sclerosis (ALS): Resurrection Health Care 19 January 2010 9:52 UTC www.reshealth.jobs [Source type: Academic]

.Men are affected slightly more often than women.^ Men have it slightly more often than women.
  • Amyotrophic Lateral Sclerosis: Part 1 of 2 19 January 2010 9:52 UTC www.massagetoday.com [Source type: FILTERED WITH BAYES]

^ Men are affected more often than women.
  • Amyotrophic Lateral Sclerosis (Lou Gehrig Disease) Causes, Symptoms, Diagnosis, Treatment, and Prognosis Information on eMedicineHealth.com 19 January 2010 9:52 UTC www.emedicinehealth.com [Source type: FILTERED WITH BAYES]
  • Amyotrophic Lateral Sclerosis Fact Sheet: National Institute of Neurological Disorders and Stroke (NINDS) 19 January 2010 9:52 UTC www.ninds.nih.gov [Source type: Academic]
  • Amyotrophic Lateral Sclerosis (ALS) 19 January 2010 9:52 UTC www.depression-guide.com [Source type: Academic]
  • Leu Gehrig's Disease: symptoms, cause, treatments, risks, complications, long-term outlook 19 January 2010 9:52 UTC mamashealth.com [Source type: Academic]
  • Amyotrophic Lateral Sclerosis (ALS) Support Group - DailyStrength 19 January 2010 9:52 UTC www.dailystrength.org [Source type: FILTERED WITH BAYES]

^ ALS occurs more frequently in men than in women.

."Familial ALS" accounts for approximately 5%–10% of all ALS cases and is caused by genetic factors.^ Approximately 10% of ALS patients are familial cases exhibiting an autosomal dominant inheritance ( 1 ).
  • Oxidative stress causes abnormal accumulation of familial amyotrophic lateral sclerosis-related mutant SOD1 in transgenic Caenorhabditis elegans -- Oeda et al. 10 (19): 2013 -- Human Molecular Genetics 19 January 2010 9:52 UTC hmg.oxfordjournals.org [Source type: Academic]

^ Other mutations as cause of familial ALS .
  • Molecular Neurodegeneration | Full text | Experimental models for the study of neurodegeneration in amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.molecularneurodegeneration.com [Source type: Academic]

^ In ~25% of familial cases, the disease is caused by mutations in the gene encoding cytosolic copper-zinc superoxide dismutase ( SOD1 ) (Rosen et al., 1993 ).
  • Rats Expressing Human Cytosolic Copper-Zinc Superoxide Dismutase Transgenes with Amyotrophic Lateral Sclerosis: Associated Mutations Develop Motor Neuron Disease -- Nagai et al. 21 (23): 9246 -- Journal of Neuroscience 19 January 2010 9:52 UTC www.jneurosci.org [Source type: Academic]

.Of these, approximately 1 in 10 are linked to a mutation in copper/zinc superoxide dismutase (SOD1), an enzyme responsible for scavenging free radicals.^ The copper/zinc SOD1 gene is mutated in 10-20% of familial cases.
  • Amyotrophic Lateral Sclerosis: eMedicine Neurology 19 January 2010 9:52 UTC emedicine.medscape.com [Source type: Academic]

^ Cu 2+ /Zn 2+ superoxide dismutase 1 (SOD1) is an ubiquitously expressed cytoplasmic enzyme that catalyzes the dismutation of the superoxide radical (O 2 - ) into hydrogen peroxide and molecular oxygen and is an important free radical scavenging enzyme that protects cells against oxidative stress.
  • Molecular Neurodegeneration | Full text | Experimental models for the study of neurodegeneration in amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.molecularneurodegeneration.com [Source type: Academic]

^ About 10% of ALS cases are familial (Haverkamp et al ., 1995 ) and in some of these the disease has been linked to mutations in the CuZn-superoxide dismutase (SOD1) gene (Rosen et al ., 1993 ).
  • Disulphide-reduced superoxide dismutase-1 in CNS of transgenic amyotrophic lateral sclerosis models -- Jonsson et al. 129 (2): 451 -- Brain 19 January 2010 9:52 UTC brain.oxfordjournals.org [Source type: Academic]

A recent study has identified a gene called FUS ("Fused in Sarcoma", ALS6) as being responsible for 1 in 20 cases of fALS.[43][44]
.Although the incidence of ALS is thought to be regionally uniform, there are three regions in the West Pacific where there has in the past been an elevated occurrence of ALS. This seems to be declining in recent decades.^ Although respiratory function declines faster in ALS with bulbar onset, overall muscle weakness declines more slowly [ Magnus et al 2002 ].
  • Amyotrophic Lateral Sclerosis Overview -- GeneReviews -- NCBI Bookshelf 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

^ Clinically definite ALS. The presence of UMN and LMN signs in three regions .
  • Amyotrophic Lateral Sclerosis Overview -- GeneReviews -- NCBI Bookshelf 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

^ Anticipation is not thought to be a feature, although intrafamilial and interfamilial variability in age of onset and disease progression is not uncommon [ Appelbaum et al 1992 ].
  • Amyotrophic Lateral Sclerosis Overview -- GeneReviews -- NCBI Bookshelf 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

.The largest is the area of Guam inhabited by the Chamorro people, who have historically had a high incidence (as much as 143 cases per 100,000 people per year) of a condition called Lytico-Bodig disease which is a combination of ALS, Parkinsonism, and dementia.^ The number of individuals newly diagnosed with ALS each year is 1-3:100,000.
  • Amyotrophic Lateral Sclerosis Overview -- GeneReviews -- NCBI Bookshelf 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

^ In ~25% of familial cases, the disease is caused by mutations in the gene encoding cytosolic copper-zinc superoxide dismutase ( SOD1 ) (Rosen et al., 1993 ).
  • Rats Expressing Human Cytosolic Copper-Zinc Superoxide Dismutase Transgenes with Amyotrophic Lateral Sclerosis: Associated Mutations Develop Motor Neuron Disease -- Nagai et al. 21 (23): 9246 -- Journal of Neuroscience 19 January 2010 9:52 UTC www.jneurosci.org [Source type: Academic]

^ I must have lost about $5,000 from the more than 12 people who cancelled."
  • SolarBee® Science Office 20 September 2009 17:15 UTC www.solarbee.com [Source type: News]

[45] .Two more areas of increased incidence are the Kii peninsula of Japan and West Papua.^ In middle of Kii peninsula, one of the biggest mercury mine in Japan had been present until about 10 years ago.
  • Amyotrophic Lateral Sclerosis (ALS) - Abstracts : Online Reference for Health Concerns 19 January 2010 9:52 UTC www.lef.org [Source type: Academic]

^ High aluminum deposition in the central nervous system of patients with amyotrophic lateral sclerosis from the Kii Peninsula, Japan: two case reports.
  • Amyotrophic Lateral Sclerosis (ALS) - Abstracts : Online Reference for Health Concerns 19 January 2010 9:52 UTC www.lef.org [Source type: Academic]

^ We report two cases of ALS from the Kii Peninsula of Japan with markedly elevated concentrations of Al in central nervous system (CNS) tissues.
  • Amyotrophic Lateral Sclerosis (ALS) - Abstracts : Online Reference for Health Concerns 19 January 2010 9:52 UTC www.lef.org [Source type: Academic]

[46][47]
.Although there have been reports of several "clusters" including three American football players from the San Francisco 49ers, more than fifty soccer players in Italy [9], three soccer-playing friends in the south of England,[48] and reports of conjugal (husband and wife) cases in the south of France,[49][50][51][52][53] these are statistically plausible chance events.^ Wilhelmsen et al [2004] reported a family with ALS/FTD from San Francisco with α-synuclein and 4R/0N tau pathology linked to chromosome 17q.
  • Amyotrophic Lateral Sclerosis Overview -- GeneReviews -- NCBI Bookshelf 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

^ San Marcos — It’s as yucky to look at as anything in the San Marcos River; and it’s much more than just a slimy face.
  • SolarBee® Science Office 20 September 2009 17:15 UTC www.solarbee.com [Source type: News]

^ Small children and pets are affected quicker and more severely from exposure to the blue-green algae because their systems are smaller than adults.
  • SolarBee® Science Office 20 September 2009 17:15 UTC www.solarbee.com [Source type: News]

.Although many authors consider ALS to be caused by a combination of genetic and environmental risk factors, so far the latter have not been firmly identified, other than a higher risk with increasing age.^ Other ALS-causing genes .
  • Facts About ALS | MDA/ALS Publications 19 January 2010 9:52 UTC www.als-mda.org [Source type: Academic]

^ Lou Gehrig's disease; ALS Causes, incidence, and risk factors .
  • HowStuffWorks "Amyotrophic Lateral Sclerosis - Medical Dictionary" 19 January 2010 9:52 UTC healthguide.howstuffworks.com [Source type: Academic]

^ Family history is associated with an increased risk of ALS, and many Mendelian causes have been discovered.
  • PLoS ONE: Amyotrophic Lateral Sclerosis: An Emerging Era of Collaborative Gene Discovery 19 January 2010 9:52 UTC www.plosone.org [Source type: Academic]

Etymology

.Amyotrophic comes from the Greek language: A- means "no", myo refers to "muscle", and trophic means "nourishment"; amyotrophic therefore means "no muscle nourishment," which describes the characteristic atrophication of the sufferer's disused muscle tissue.^ Both transgenes were controlled by the myo-3 gene promoter, which constitutively induces high-level protein expression in all of the muscle tissues except for the pharynx ( 24 ).
  • Oxidative stress causes abnormal accumulation of familial amyotrophic lateral sclerosis-related mutant SOD1 in transgenic Caenorhabditis elegans -- Oeda et al. 10 (19): 2013 -- Human Molecular Genetics 19 January 2010 9:52 UTC hmg.oxfordjournals.org [Source type: Academic]

.Lateral identifies the areas in a person's spinal cord where portions of the nerve cells that are affected are located.^ Schnaar RI, Schaffner AE: Separation of cell types from embryonic chicken and rat spinal cord: characterization of motoneuron-enriched fractions.
  • Molecular Neurodegeneration | Full text | Experimental models for the study of neurodegeneration in amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.molecularneurodegeneration.com [Source type: Academic]

^ Concentrations of 15 elements were determined by instrumental neutron activation analysis in brain, spinal cord, blood cells, serum and nails of Amyotrophic Lateral Sclerosis (ALS) patients and appropriately matched control subjects.
  • Amyotrophic Lateral Sclerosis (ALS) - Abstracts : Online Reference for Health Concerns 19 January 2010 9:52 UTC www.lef.org [Source type: Academic]

^ Rothstein JD, Martin LJ, Kuncl RW: Decreased glutamate transport by the brain and spinal cord in amyotrophic lateral sclerosis.
  • Molecular Neurodegeneration | Full text | Experimental models for the study of neurodegeneration in amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.molecularneurodegeneration.com [Source type: Academic]

As this area degenerates it leads to scarring or hardening ("sclerosis") in the region.

History

Timeline
Year Event
1850 English scientist Augustus Waller describes the appearance of shriveled nerve fibers
1869 French doctor Jean-Martin Charcot first describes ALS in scientific literature
1881 "On Amyotrophic Lateral Sclerosis" is translated into English and published in a three-volume edition of Lectures on the Diseases of the Nervous System
1939 ALS becomes a .cause célèbre in the United States when baseball legend Lou Gehrig's career—and, two years later, his life—are ended by the disease.^ Later in the disease, when the motor neurons that supply the large motor units die, group atrophy ensues.
  • Amyotrophic Lateral Sclerosis: eMedicine Neurology 19 January 2010 9:52 UTC emedicine.medscape.com [Source type: Academic]

^ These results point to two pathophysiological mechanisms that operate to cause dysphagia in amyotrophic lateral sclerosis patients.
  • Pathophysiological mechanisms of oropharyngeal dysphagia in amyotrophic lateral sclerosis -- Ertekin et al. 123 (1): 125 -- Brain 19 January 2010 9:52 UTC brain.oxfordjournals.org [Source type: Academic]

^ ALS is also known as motor neurone disease (MND), Charcot’s disease, and Lou Gehrig disease.
  • Amyotrophic Lateral Sclerosis: eMedicine Neurology 19 January 2010 9:52 UTC emedicine.medscape.com [Source type: Academic]

He gives his farewell speech on July 4.
1950s ALS epidemic occurs among the Chamorro people on Guam
1991 Researchers link chromosome 21 to FALS (Familial ALS)
1993 SOD1 gene on chromosome 21 found to play a role in some cases of FALS
1996 Rilutek becomes the first FDA-approved drug for ALS
1998 The El Escorial criteria is developed as the standard for classifying ALS patient in clinical research

Notable people affected by ALS

Notable people living with ALS include:
.
  • Jason Becker - American guitarist and composer
  • Stefano Borgonovo - Former Italian soccer player [54]
  • O.J. Brigance - American football star
  • Ward Halstead - American neuropsychologist (Halstead-Reitan Neuropsycholological Test Battery)[55]
  • Stephen Hawking - British theoretical physicist
  • Tony Judt - British-born, US-based public intellectual and historian .^ Belli S, Vanacore N. Proportionate mortality of Italian soccer players: is amyotrophic lateral sclerosis an occupational disease?.
    • Amyotrophic Lateral Sclerosis: eMedicine Neurology 19 January 2010 9:52 UTC emedicine.medscape.com [Source type: Academic]

    ^ Three putative risk factors that have gained recent attention include service in the US military, deployment to the Persian Gulf in the First Persian Gulf War, and being an Italian soccer player.
    • Amyotrophic Lateral Sclerosis: eMedicine Neurology 19 January 2010 9:52 UTC emedicine.medscape.com [Source type: Academic]

    ^ Chiò A, Benzi G, Dossena M, Mutani R, Mora G. Severely increased risk of amyotrophic lateral sclerosis among Italian professional football players.
    • Amyotrophic Lateral Sclerosis: eMedicine Neurology 19 January 2010 9:52 UTC emedicine.medscape.com [Source type: Academic]

    [56]
  • Jeff Kaufman - American trial lawyer, writer, University of Wisconsin track athlete, major fundraiser of ALS research, founder of the Evening of Hope event in Milwaukee, WI
  • Scott LeDoux - American politician and former boxer[57]
  • Eric Lowen - American musician, in Lowen & Navarro[58]
  • Richard Olney - ALS researcher
  • Mike Porcaro - Bass player
  • Tony Proudfoot - Former Canadian Football League player
  • Tony "Tempt" Quan - American Graffiti Artist and first Amyotrophic Lateral Sclerosis sufferer to use the EyeWriter
  • Washington César Santos - Brazilian former football player[59]
  • Tucker Stilley, aka New Jack Rasputin - American artist[60]
  • Orlando Thomas - American football star[61]
  • Charlie Wedemeyer - Younger brother of American football star-turned actor Herman Wedemeyer
  • Chris Woodhead - Former head of the Office for Standards in Education
Notable people who have died of ALS include:

See also

References

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Further reading

  • "ALS Hope Foundation". http://www.alshopefoundation.org/. Retrieved 2008-06-21. ."Dedicated to the care and cure of people with Lou Gehrig's Disease.^ Amyotrophic Lateral Sclerosis (ALS) - Lou Gehrig's Disease, Free Radical Damage, Glutamate Health Concern Search For This Topic Search For This Topic Search For This Topic .
    • Amyotrophic Lateral Sclerosis (ALS) - Abstracts : Online Reference for Health Concerns 19 January 2010 9:52 UTC www.lef.org [Source type: Academic]

    ^ Amyotrophic Lateral Sclerosis Overview [ Lou Gehrig's Disease ] .
    • Amyotrophic Lateral Sclerosis Overview -- GeneReviews -- NCBI Bookshelf 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

    (from site page us.php)"
     
  • "Lou Gehrig: The Official Web Site". CMG Worldwide. .http://www.lougehrig.com.^ The electronic version of this article is the complete one and can be found online at: http://www.molecularneurodegeneration.com/content/4/1/31 .
    • Molecular Neurodegeneration | Full text | Experimental models for the study of neurodegeneration in amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.molecularneurodegeneration.com [Source type: Academic]

    Retrieved 2008-06-21
    . "The Official Web site of Lou Gehrig is an informational Web site intended to honor the life, the legend and the career of Lou Gehrig. (from site page siteinfo/index.htm)"
     
  • Patrick Aebischer; Ann C. Kato (November 2007). ."Playing defense against Lou Gehrig's Disease" (Paper).^ Amyotrophic Lateral Sclerosis Overview [ Lou Gehrig's Disease ] .
    • Amyotrophic Lateral Sclerosis Overview -- GeneReviews -- NCBI Bookshelf 19 January 2010 9:52 UTC www.ncbi.nlm.nih.gov [Source type: Academic]

    ^ Amyotrophic Lateral Sclerosis (ALS) - Lou Gehrig's Disease, Free Radical Damage, Glutamate Health Concern Search For This Topic Search For This Topic Search For This Topic .
    • Amyotrophic Lateral Sclerosis (ALS) - Abstracts : Online Reference for Health Concerns 19 January 2010 9:52 UTC www.lef.org [Source type: Academic]

    Scientific American (Verlagsgruppe Georg von Holtzbrinck): pp. 86–93. .http://www.sciam.com/article.cfm?id=playing-defense-against-l.^ The electronic version of this article is the complete one and can be found online at: http://www.molecularneurodegeneration.com/content/4/1/31 .
    • Molecular Neurodegeneration | Full text | Experimental models for the study of neurodegeneration in amyotrophic lateral sclerosis 19 January 2010 9:52 UTC www.molecularneurodegeneration.com [Source type: Academic]

    Retrieved 2008-06-21
    . "Researchers have proposed potential therapies for a paralyzing disorder once thought to be untreatable (sub-title)"
     

External links


Simple English

Amyotrophic lateral sclerosis (ALS, sometimes called Lou Gehrig’s disease or motor neurone disease) is a chronic, progressive, almost always fatal neurological disease. ALS is marked by slow but steady death of the nerve cells in the central nervous system that control voluntary muscle movement. That is, people with ALS can no longer move, but their heart still beats. The disorder causes muscle weakness and muscle shrinking (atrophy) throughout the body. In ALS, both the upper motor neurons and the lower motor neurons die, making them stop sending messages to the muscles. Unable to function, the muscles gradually weaken and waste away (atrophy). Eventually, the ability of the brain to start and control voluntary movement is lost. However, even patients in later stages of the disease may still have the same intelligence, memory, and personality they had before it started.

Symptoms

ALS does not show many symptoms, making it very hard to diagnose. It usually affects people ages 40-60. The earliest symptoms may include twitching, cramping, or stiffness of muscles; muscle weakness affecting an arm or a leg; slurred and strange-sounding nasal speech; or having a difficult time chewing or swallowing. These general symptoms then develop into clear weakness or atrophy that may cause a physician to believe that a person has ALS.

The parts of the body affected by early symptoms of ALS depend on which muscles in the body are affected first. About 75% of people have limb onset ALS. In some of these cases, symptoms first affect one of the legs, and patients have awkwardness when walking or running or they notice that they are tripping or stumbling more often. Other limb onset patients first see the effects of the disease on a hand or arm as they have a difficult time with simple tasks requiring hand dexterity, or ability to move small things, such as buttoning a shirt, writing, or turning a key in a lock.

About 25% of cases are bulbar onset ALS. These patients first have difficulty speaking clearly. Speech becomes hard to understand and slurred. Speaking through the nose and softer talking are often the first symptoms. Difficulty swallowing, and loss of tongue movement follow. Eventually total loss of speech and ability to keep the airway clear when swallowing are experienced.

Treatment

No cure has been found for ALS. However, the Food and Drug Administration (FDA) has approved the first drug treatment for the disease: Riluzole (Rilutek). Riluzole is believed to make damage smaller to motor neurons by decreasing the release of glutamate, a substance that is found in most ALS patients. Clinical trials with ALS patients showed that riluzole gives a better chance of survival by several months, mainly in those with a hard time swallowing. The drug also allows more time before a patient needs breathing help. Riluzole does not heal the damage already done to motor neurons, and patients taking the drug must be watched for liver damage and other side-effects. However, this first ALS therapy offers hope that the assault of ALS may one day be slowed by new medications or combinations of drugs.

Other treatments for ALS are designed to make symptoms less painful and improve the quality of life for patients. This care is best given by teams of health care professionals such as physicians; pharmacists; physical, occupational, and speech therapists; nutritionists; social workers; and home care and hospice nurses. Working with patients and people who take care of the patient, these teams can make a personalized plan of medical and physical therapy and give special equipment that can be used to keep patients as mobile and comfortable as possible.


Citable sentences

Up to date as of December 14, 2010

Here are sentences from other pages on Amyotrophic lateral sclerosis, which are similar to those in the above article.








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