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Ohtahara syndrome
Classification and external resources
ICD-10 G40.4
OMIM 308350
DiseasesDB 33878

Ohtahara syndrome (OS), also known as Early Infantile Epileptic Encephalopathy with Burst-Suppression (EIEE), is a progressive epileptic encephalopathy. The syndrome is outwardly characterized by tonic spasms and partial seizures, and receives its more elaborate name from the pattern of burst activity on an electroencephalogram (EEG). It is an extremely debilitating progressive neurological disorder, involving intractable seizures and severe mental retardation. No single cause has been identified, although in many cases structural brain damage is present.[1]

Contents

Characteristics

Ohtahara syndrome is rare and the earliest-appearing age-related epileptic encephalophy, with seizure onset occurring within the first three months of life, and often in the first ten days.[2] Many, but not all, cases of OS evolve into other seizure disorders, namely West syndrome and Lennox-Gastaut syndrome.[1]

The primary outward manifestation of OS is seizures, usually presenting as tonic seizures (a generalized seizure involving a sudden stiffening of the limbs).[3] Other seizure types that may occur include partial seizures and, rarely, myoclonic seizures. In addition to seizures, children with OS exhibit profound mental and physical retardation.

Clinically, OS is characterized by a “burst suppression” pattern on an EEG. This pattern involves high voltage spike wave discharge followed by little brain wave activity.[1]

Causes and treatment

No single cause of OS has been identified. In most cases, there is severe atrophy of both hemispheres of the brain. Less often, the root of the disorder is an underlying metabolic syndrome. No genetic connection has been established.[4]

Treatment outlook is poor. Anticonvulsant drugs and glucocorticoid steroids may be used to try to control the seizures, but their effectiveness is limited. Most therapies are related to symptoms and day-to-day living.[1]

Notable sufferers

References

  1. ^ a b c d National Institute of Neurological Disorders and Stroke (5 December 2008). "NINDS Ohtahara Syndrome Information Page". http://www.ninds.nih.gov/disorders/ohtahara/ohtahara.htm. Retrieved 2009-03-10.  
  2. ^ Ohtahara S, Ishida T, Oka E, et al. (1976). "[On the specific age dependent epileptic syndrome: the early-infantile epileptic encephalopathy with suppression-burst]" (in Japanese). No to Hattatsu 8: 270–9.  
  3. ^ Holmes, Gregory L. (January 2004). "Tonic". Epilepsy.com/Professionals. http://professionals.epilepsy.com/page/generalized_tonic.html. Retrieved 2007-11-26.  
  4. ^ Nabbout, Rima (July 2004). "Early infantile epileptic encephalopathy" (PDF). http://www.orpha.net/data/patho/Pro/en/EarlyInfantileEpilepticEncephalopathy-FRenPro889.pdf.  
  5. ^ "Cameron's 'beautiful boy' dies". BBC News Online. 25 February 2009. http://news.bbc.co.uk/1/hi/uk_politics/7909562.stm. Retrieved 2009-03-10.  

External links

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Wiktionary

Up to date as of January 15, 2010

Definition from Wiktionary, a free dictionary

English

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Noun

Singular
Ohtahara syndrome

Plural
Ohtahara syndromes

Ohtahara syndrome (plural Ohtahara syndromes)

  1. (neurology, pathology) A progressive condition with a combination of severe epilepsy and cerebral palsy. It is characterised by severe physical and mental retardation and partial seizures.

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