Landau-Kleffner syndrome (LKS): clinical features

Evidence-based neurology checklist on landau-kleffner syndrome (lks): clinical features: Demographic features This is acquired aphasia of childhood with epilepsy It may be caused by mutations in the GRIN2A gene on chromosome 16p Boys are affected twice as often as girls The early development is…

Demographic features

  • This is acquired aphasia of childhood with epilepsy
  • It may be caused by mutations in the GRIN2A gene on chromosome 16p
  • Boys are affected twice as often as girls
  • The early development is normal
  • The onset age is 3-7 years
  • It develops over days or weeks

Language impairment

Seizure features

Behavioural disturbance

Possible associations

Overlap syndromes

Differential diagnoses

References

  1. Appleton RE. The Landau-Kleffner syndrome. Arch Dis Child 1995; 72:386–387.
  2. Nieuwenhuis L, Nicolai J. The pathophysiological mechanisms of cognitive and behavioral disturbances in children with Landau-Kleffner syndrome or epilepsy with continuous spike-and-waves during slow-wave sleep. Seizure 2006; 15:249-258.
  3. Kural Z, Ozer AF. Epileptic encephalopathies in adults and childhood. Epilepsy Res Treat 2012; 2012:205131.
  4. Carvill GL, Regan BM, Yendle SC, et al. GRIN2A mutations cause epilepsy-aphasia spectrum disorders. Nat Genet 2013; 45:1073-1076. 
  5. Gao K, Tankovic A, Zhang Y, et al. A de novo loss-of-function GRIN2A mutation associated with childhood focal epilepsy and acquired epileptic aphasia. PLoS One 2017; 12:e0170818.
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