[A special case of 'deafness'; Landau-Kleffner syndrome].
A boy aged 5 had displayed from the age of 3 a fluctuating deficit in understanding spoken language. Audiometric testing proved his hearing to be normal. After 18 months, the parents noticed minor attacks of 'absence'. An EEG showed a normal background pattern and frequent spikes and spike waves complexes; CT-scanning of the brain revealed no abnormalities. On the basis of the acquired language disorder, the seizures and the features of the nocturnal EEG, the diagnosis the syndrome of Landau-Kleffner was made. After treatment with ethosuximide a temporary improvement of the aphasia occurred. A permanent improvement however was realized by treatment with prednisone. The boy could attend a primary school. The syndrome runs a fluctuating course; the patient may recover, but he may also undergo general mental deterioration. Antiepileptic agents only may have a brief favorable effect on the aphasia. In case of failure corticosteroids or even subpial cortical transsection are indicated.
Stroink
H
H
Afd. Kinderneurologie, Academisch Ziekenhuis Rotterdam-Sophia Kinderziekenhuis.
Van Dongen
H R
HR
Meulstee
J
J
Scheltens-de Boer
M
M
Geesink
H H
HH
D002675:Child, Preschool; D005938:Glucocorticoids / Q000627:therapeutic use; D006311:Hearing Disorders / Q000175:diagnosis / Q000209:etiology* / Q000523:psychology; D006801:Humans; D018887:Landau-Kleffner Syndrome / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D008297:Male; D009483:Neuropsychological Tests; D011241:Prednisone / Q000627:therapeutic use; D013063:Speech Discrimination Tests; D013067:Speech Perception
D005938:Glucocorticoids; D011241:Prednisone