The Full Wiki

More info on Landau–Kleffner syndrome

Landau–Kleffner syndrome: Wikis

Advertisements
  

Note: Many of our articles have direct quotes from sources you can cite, within the Wikipedia article! This article doesn't yet, but we're working on it! See more info or our list of citable articles.

Encyclopedia

From Wikipedia, the free encyclopedia

Landau–Kleffner syndrome
Classification and external resources

Landau–Kleffner syndrome is characterized by aphasia and an abnormal EEG
ICD-10 F80.3
ICD-9 345.8
OMIM 245570
DiseasesDB 31407
eMedicine neuro/182
MeSH D018887

Landau–Kleffner syndrome (LKS), also called infantile acquired aphasia, acquired epileptic aphasia[1] or aphasia with convulsive disorder, is a rare, childhood neurological syndrome.

It is named for William Landau and Frank Kleffner, who characterized it in 1957.[2]

Contents

Presentation

It is characterized by the sudden or gradual development of aphasia (the inability to understand or express language) and an abnormal electroencephalogram (EEG).[3] LKS affects the parts of the brain that control comprehension and speech (Broca's area and Wernicke's area). The disorder usually occurs in children between the ages of 5 and 7 years. Typically, children with LKS develop normally but then lose their language skills. While many of the affected individuals have clinical seizures, some only have electrographic seizures, including electrographic status epilepticus of sleep (ESES).

Differential diagnosis

The syndrome can be difficult to diagnose and may be misdiagnosed as autism, pervasive developmental disorder, hearing impairment, learning disability, auditory/verbal processing disorder, attention deficit disorder, mental retardation, childhood schizophrenia, or emotional/behavioral problems.

Treatment

Treatment for LKS usually consists of medications, such as anticonvulsants[4] and corticosteroids[5] (such as prednisone),[6] and speech therapy, which should be started early.

A controversial treatment option involves a surgical technique called multiple subpial transection[7] in which multiple incisions are made through the cortex of the affected part of the brain beneath the pia mater, severing the axonal tracts in the subjacent white matter.

Prognosis

The prognosis for children with LKS varies. Some affected children may have a permanent severe language disorder, while others may regain much of their language abilities (although it may take months or years). In some cases, remission and relapse may occur. The prognosis is improved when the onset of the disorder is after age 6 and when speech therapy is started early. Seizures generally disappear by adulthood.

References

  1. ^ Landau–Kleffner syndrome at Dorland's Medical Dictionary
  2. ^ Landau, W., & Kleffner, F. (1957). Syndrome of acquired aphasia with convulsive disorder in children. Neurology, 7, 523-530.
  3. ^ Pearl PL, Carrazana EJ, Holmes GL (November 2001). "The Landau–Kleffner Syndrome". Epilepsy Curr 1 (2): 39–45. doi:10.1046/j.1535-7597.2001.00012.x. PMID 15309183.  
  4. ^ Guevara-Campos J, González-de Guevara L (2007). "Landau–Kleffner syndrome: an analysis of 10 cases in Venezuela" (in Spanish; Castilian). Rev Neurol 44 (11): 652–6. PMID 17557221. http://www.revneurol.com/LinkOut/formMedLine.asp?Refer=2006465&Revista=RevNeurol.  
  5. ^ Sinclair DB, Snyder TJ (May 2005). "Corticosteroids for the treatment of Landau–Kleffner syndrome and continuous spike-wave discharge during sleep". Pediatr. Neurol. 32 (5): 300–6. doi:10.1016/j.pediatrneurol.2004.12.006. PMID 15866429. http://linkinghub.elsevier.com/retrieve/pii/S0887-8994(05)00030-5.  
  6. ^ Santos LH, Antoniuk SA, Rodrigues M, Bruno S, Bruck I (June 2002). "Landau–Kleffner syndrome: study of four cases". Arq Neuropsiquiatr 60 (2-A): 239–41. PMID 12068352. http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2002000200010&lng=en&nrm=iso&tlng=en.  
  7. ^ Grote CL, Van Slyke P, Hoeppner JA (March 1999). "Language outcome following multiple subpial transection for Landau–Kleffner syndrome". Brain 122 ( Pt 3): 561–6. PMID 10094262. http://brain.oxfordjournals.org/cgi/pmidlookup?view=long&pmid=10094262.  

External links

Advertisements

Advertisements






Got something to say? Make a comment.
Your name
Your email address
Message