Absence epilepsy with multiple daily seizures and onset at the age of 6 and 1/2 months in a healthy female child with normal development is described. EEG-video recording revealed typical absence seizures (vacant staring and interruption of motor activity) and complex absences (as above, plus raising of the eyeballs, slight beatings of the eyebrows, and forward propulsion of head and shoulders). The absences were accompanied by bilateral symmetrical 3-Hz spike-wave discharges preceded, and at times followed, by bilateral frontoparietal theta activity coinciding with onset and termination of the absence seizures. The seizures regressed with nitrazepam therapy. At age 3-years, the child is seizure-free and shows normal neurologic development.

In view of the therapeutic efficacy of adrenocorticotropic hormone (ACTH) in the treatment of infantile spasms (IS) with hypsarrhythmia, we studied the cerebrospinal fluid (CSF) levels of ACTH in 15 children (4-10 months) affected by IS with hypsarrhythmia (eight cryptogenic forms, seven secondary to perinatal distress) and in age-matched controls. Lumbar puncture was performed in all but one case before any kind of treatment. In another case, CSF was collected 3 weeks after a spontaneous remission. Both ACTH and beta-endorphin (beta-EP), the other peptide related to the same precursor (proopiomelanocortin), were measured by specific radioimmunoassay after gel chromatography. While beta-EP levels were unchanged in the two groups of patients, ACTH concentrations of cryptogenic (3.75 +/- 2.40 fmol/ml, Mean +/- SD p less than 0.05) and secondary (6.36 +/- 3.70, NS) forms were lower than in controls (10.90 +/- 5.79). On the other hand, ACTH was higher in the case studied after therapy (9.0) and in the case presenting a spontaneous clinical and EEG remission (15.0). These data indicate that in children affected by IS with hypsarrhythmia (mainly of cryptogenic type), CSF levels of ACTH are lower, while levels of beta-EP remain normal. It would therefore appear that central ACTH content may play a possible role in the pathogenesis of IS with hypsarrhythmia.

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