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Response to immunotherapy in a patient with Landau-Kleffner syndrome and GRIN2A mutation Volume 18, numéro 1, March 2016

TEST YOURSELF

(1) What are the cardinal features of Landau-Kleffner syndrome?


(2) What treatments are used in Landau-Kleffner syndrome?


(3) What is the function of the GRIN2A gene and what epileptic disorders are associated with it?

 

 

 

 

 

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Answers

(1) Acute or gradual onset of auditory agnosia, loss of expressive speech abilities in previously normal children, epileptiform discharges that increase during sleep, and occasionally clinical seizures.


(2) Antiepileptic drugs such as nocturnal diazepam, immunotherapy with steroids, and at times intravenous immunoglobulin.


(3) GRIN2A encodes for the NMDA 2A receptor. Dysfunction of this gene is associated with certain epilepsy syndromes including electrical status epilepticus in sleep, Landau-Kleffner syndrome, atypical benign partial epilepsy, and benign epilepsy with centro-temporal spikes.

 

 

 

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