[A special case of 'deafness'; Landau-Kleffner syndrome].

Abstract

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.

• Authors: H Stroink, H R Van Dongen, J Meulstee, M Scheltens-de Boer, H H Geesink
• Journal: Nederlands tijdschrift voor geneeskunde (Ned Tijdschr Geneeskd) Vol. 141 Issue 33 Pg. 1623-5 (Aug 16 1997) ISSN: 0028-2162 [Print] Netherlands
• Vernacular Title: Een bijzonder geval van 'doofheid'; het syndroom van Landau-Kleffner.
• PMID: 9543768 (Publication Type: Case Reports, English Abstract, Journal Article)

Chemical References

• Glucocorticoids
• Prednisone

Topics

• Child, Preschool
• Glucocorticoids (therapeutic use)
• Hearing Disorders (diagnosis, etiology, psychology)
• Humans
• Landau-Kleffner Syndrome (complications, diagnosis, drug therapy)
• Male
• Neuropsychological Tests
• Prednisone (therapeutic use)
• Speech Discrimination Tests
• Speech Perception