Acquired aphasia of childhood with epilepsy: the Landau-Kleffner syndrome.
The Landau-Kleffner syndrome is an unusual childhood disorder characterised by an acquired aphasia and a paroxysmal electroencephalographic abnormality with spike and spike and wave discharges which are mostly multifocal and unstable in their course of evolution. Two accessory symptoms are frequently observed: psychomotor or behavioural disturbances and epilepsy. Epileptic seizures are most often generalised convulsive or partial motor and invariably disappear before the age of 15 years. A 20 year old woman is described who has been followed since the rarely observed onset of the Landau-Kleffner syndrome at the age of 3 years. At 4 years of age she was totally unable to communicate through speech and her EEG showed almost continuous bitemporal spike and spike and wave discharges. Maximum doses of diazepam, phenytoin, carbamazepine and sulthiame were used without effect. From the age of 14 years her ability to communicate through spoken and written language has improved and her EEG now shows no epileptiform activity.
Hankey
G J
GJ
Department of Neurology, Royal Perth Hospital, WA.
Gubbay
S S
SS
D000328:Adult; D001037:Aphasia / Q000150:complications*; D004569:Electroencephalography; D004827:Epilepsy / Q000150:complications* / Q000503:physiopathology; D005260:Female; D006801:Humans; D013577:Syndrome