[Landau-kleffner syndrome]

ABSTRACT

Landau-Kleffner syndrome is a rare disorder caraterised by acquired aphasia of children, paroxysmal electroencephalographic disturbance and in 75% of cases epileptic seizures and cognitives disturbances. We report a 6 year old daughter who presented epileptic seizures and six months later regression langage and a behaviour of deaf. This patient had a global aphasia. The understanding was affected because of the cortical deafness. The electroencephalogramme showed discharges in the temporal bilateral regions. The cerebral scan and the potential evoked auditory were normal. The Landau-Kleffner syndrome's diagnosis was confirmed. The patient was treated by valproic acid and corticosteroids with good evolution. The stoopage of treatment has brought more seizure and the worsening aphasia. The authors discussed the pathogenic hypothesis, the semiology and the treatment of the Landau-Kleffner syndrome