Landau-Kleffner syndrome (LKS): clinical features

Written and reviewed by Dr Ibrahim Imam, Consultant Neurologist. Last reviewed .

Landau-Kleffner syndrome (LKS): clinical features. This is acquired aphasia of childhood with epilepsy. Fully referenced neurology checklist.

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

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Seizure features

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Behavioural disturbance

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Possible associations

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Overlap syndromes

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Differential diagnoses

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Poor prognostic factors

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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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