The long-term follow up of 6 patients and a review of the recent literature on the Landau-Kleffner syndrome (“acquired aphasia with convulsive disorder”) are reported from the Department of Neurology, University Hospital Dijkzigt-Rotterdam, the Netherlands. The age at onset was 3-5 ½ years, with epilepsy as the first sign in 3 and comprehension deficit in 3. Seizures were absence, generalized tonic-clonic, partial and complex partial in patterns. Seizures had disappeared at follow-up in 3 and epileptic activity in the EEG was absent in 4. The course of the aphasia was linked to the appearance and disappearance of electrical status epilepticus during slow sleep (ESES) which was found in 3 patients. Lasting EEG abnormalities and persisting clinical seizures, combined with the presence and duration of ESES, may impede language recovery. The disappearance of ESES may be associated with an improvement of language functions. The course of the aphasia was variable: slow and continuous improvement in 3, rapid recovery in 1, fluctuating in 1, and deterioration followed by slow improvement in 1. The follow-up period varied from 3 to 19 years. The response of the seizures and aphasia to anticonvulsants was variable; some benefit was obtained with phenytoin and phenobarbital in 1 patient, ethosuximide and valproate were helpful in 2. Prednisone in 1 patient had a variable and inconclusive effect. [1]

COMMENT. The correlation between ESES and acquired epileptic aphasia has been emphasized previously (See also Ped Neur Briefs Sept 1991; 5:71; Jan 1991; 5:4-5) [2, 3]. The beneficial effects of ACTH and cortico steroids have been reported from Tel Aviv University (Lerman P et al. See Ped Neur Briefs April 1991; 5:28-29). Cortico steroids administered early were more effective than delayed treatments.