(Acquired aphasia of the child with epilepsy)

[MIM 245 570]

Rare neurological disorder of the child which combines the sudden or gradual onset of aphasia and paroxysmal EEG anomalies predominantly in the parieto-parietal area (typically: spike and wave seizure activity during sleep). The cause of the condition is unknown but autosomal transmission of heterozygous mutations of the GRIN2A (16p13.2) gene could be associated. The condition usually begins between the age of 3 and 7 years: the first sign is severe impairment of auditory language comprehension followed with progressive speech difficulties (jargon, paraphasias). About 20 % of patients do not present with  seizures (which are sometimes very localized): there is then a risk of misdiagnosis with autism, childhood schizophrenia, behavioural  or learning disorders.

Treatment: antiepileptics, sometimes corticosteroid therapy, speech rehabilitation. In case of unilateral localization, some teams propose a subpial resection.

Anesthetic implications:

management of a mute patient, suffering from epilepsy; risk of behavioural disorders.

References : 

• Gosh B, Carsten D. 
  General anesthesia for a child with Landau-Kleffner syndrome : a case report. 
  Anesth Prog 2010; 57: 109-11.

Updated: February 2019