Four children between five and nine years of age with Landau-Kleffner syndrome were treated with ACTH or corticosteroids at the Department of Pediatrics, Beilinson Medical Centre, and Sackler Faculty of Medicine, Tel Aviv University, Israel. ACTH in one patient was given for three months starting with 80 units/day followed by a gradual reduction of the dose. An EEG three weeks after the start of the treatment showed complete disappearance of epileptic activity but the aphasia was initially unchanged. Complete remission of the aphasia occurred eight months after the completion of the ACTH course. Two years later the aphasia recurred with the concomitant appearance of multiple spike discharges on the EEG. Prompt prescription of ACTH therapy led to a recovery of speech and a normal EEG within a few weeks. Prednisone 60 mg/day for two to three months in two patients caused a prompt improvement in the EEG followed by normal speech. Dexamethasone 4 mg/day resulted in full recovery of speech and a completely normal EEG within two weeks of starting treatment in a nine year old child. Three to six years after discontinuing therapy the children are off anticonvulsant medication and free from seizures and language disability. [1]

COMMENT: These results are encouraging and warrant further trials of hormone therapy in children with the aphasic epilepsy syndrome. As in the treatment of West syndrome, corticosteroids administered early are more effective than delayed treatments.

Hypsarrhythmia is not a sine qua non for ACTH therapy. Two children, aged 3 years and 2 years 8 months with status absence and generalized tonic-clonic seizures refractory to anticonvulsant medication were benefitted by ACTH 10 units intramuscular daily. Control of seizures, improvement in the EEG, and a dramatic recovery of mental and motor abilities occurred within two hours of the first injection in one patient and seizure control and EEG improvement occurred in the second after a three week course of therapy. Further trials of ACTH in children with seizures other than infantile spasms are recommended. [2]