Landau–Kleffner syndrome: Difference between revisions
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==External links== |
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* {{cite web |title= Landau Kleffner syndrome: abilities, behaviour and education |publisher= [[Great Ormond Street Hospital]] |url=http://www.gosh.nhs.uk/gosh_families/information_sheets/landau_kleffner_abilities/landau_kleffner_abilities_families.html |accessdate=2011-02-28 |date=2010-11-17}} |
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* {{cite web |title= Landau–Kleffner syndrome information page |publisher= [[National Institute of Neurological Disorders and Stroke]] |url=http://www.ninds.nih.gov/disorders/landaukleffnersyndrome/landaukleffnersyndrome.htm |accessdate=2007-08-23 |date=2007-02-13}} |
* {{cite web |title= Landau–Kleffner syndrome information page |publisher= [[National Institute of Neurological Disorders and Stroke]] |url=http://www.ninds.nih.gov/disorders/landaukleffnersyndrome/landaukleffnersyndrome.htm |accessdate=2007-08-23 |date=2007-02-13}} |
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* {{cite web |url=http://www.nidcd.nih.gov/health/voice/landklfs.htm |accessdate=2007-08-23 |publisher= National Institute on Deafness and other Communication Disorders |title= Landau–Kleffner syndrome |year=2002}} |
* {{cite web |url=http://www.nidcd.nih.gov/health/voice/landklfs.htm |accessdate=2007-08-23 |publisher= National Institute on Deafness and other Communication Disorders |title= Landau–Kleffner syndrome |year=2002}} |
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Revision as of 14:58, 28 February 2011
| Landau–Kleffner syndrome | |
|---|---|
| Specialty | Neurology, psychiatry  |
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]
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
- ^ "Landau–Kleffner syndrome" at Dorland's Medical Dictionary
- ^ Landau WM, Kleffner FR (1957). "Syndrome of acquired aphasia with convulsive disorder in children". Neurology. 7 (8): 523–30. PMID 13451887.
{{cite journal}}: Unknown parameter|month=ignored (help) Reproduced as Landau WM, Kleffner FR (1998). "Syndrome of acquired aphasia with convulsive disorder in children. 1957". Neurology. 51 (5): 1241, 8 pages following 1241. PMID 9867583.{{cite journal}}: Unknown parameter|month=ignored (help) - ^ Pearl PL, Carrazana EJ, Holmes GL (2001). "The Landau–Kleffner Syndrome". Epilepsy Curr. 1 (2): 39–45. doi:10.1046/j.1535-7597.2001.00012.x. PMC 320814. PMID 15309183.
{{cite journal}}: Unknown parameter|month=ignored (help)CS1 maint: multiple names: authors list (link) - ^ Guevara-Campos J, González-de Guevara L (2007). "Landau–Kleffner syndrome: an analysis of 10 cases in Venezuela". Rev Neurol (in Spanish; Castilian). 44 (11): 652–6. PMID 17557221.
{{cite journal}}: CS1 maint: unrecognized language (link) - ^ Sinclair DB, Snyder TJ (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.
{{cite journal}}: Unknown parameter|month=ignored (help) - ^ Santos LH, Antoniuk SA, Rodrigues M, Bruno S, Bruck I (2002). "Landau–Kleffner syndrome: study of four cases". Arq Neuropsiquiatr. 60 (2-A): 239–41. PMID 12068352.
{{cite journal}}: Unknown parameter|month=ignored (help)CS1 maint: multiple names: authors list (link) - ^ Grote CL, Van Slyke P, Hoeppner JA (1999). "Language outcome following multiple subpial transection for Landau–Kleffner syndrome". Brain. 122 ( Pt 3): 561–6. PMID 10094262.
{{cite journal}}: Unknown parameter|month=ignored (help)CS1 maint: multiple names: authors list (link)
External links
- "Landau Kleffner syndrome: abilities, behaviour and education". Great Ormond Street Hospital. 2010-11-17. Retrieved 2011-02-28.
- "Landau–Kleffner syndrome information page". National Institute of Neurological Disorders and Stroke. 2007-02-13. Retrieved 2007-08-23.
- "Landau–Kleffner syndrome". National Institute on Deafness and other Communication Disorders. 2002. Retrieved 2007-08-23.
- Rotenberg J, Pearl PL (2003). "Landau–Kleffner syndrome". Arch Neurol. 60 (7): 1019–21. doi:10.1001/archneur.60.7.1019. PMID 12873863.