[ACTH therapy for two cases of epileptic spasms without hypsarrhythmia]

We present two patients with epileptic spasms without hypsarrhythmia (ESWoH) who were successfully treated with ACTH. One patient was an 18-month-old boy who had normal development for the first 16 months of his life. Thereafter, he experienced sudden flexion of the axial muscles, resulting in head nodings, or epileptic spasms (ES). EEG tracings showed generalized polyspikes that appeared frequently during sleep without associated hypsarrhythmia. We were thus able to diagnose him with ESWoH and treated him with ACTH, which resolved his seizures. Another patient was a 7-year-old boy. At 18 months of age, the patient began to sufferdil from sudden ES. Anti-epileptic drugs were administered individually and in combination but were proved ineffective. Like the first case, this child's EEG tracing showed generalized polyspikes during sleep without associated hypsarrhythmia. We diagnosed him with ESWoH and treated him with ACTH. His ES disappeared soon after the initiation of treatment. These two cases demonstrate the difficulty of treating ESWoH only with standard anti-epileptic drugs, we emphasize the importance of early treatment with ACTH.