2007 Volume 25 Issue 2 Pages 94-99
We present a favorable effect of ACTH therapy on a girl with early myoclonus encephalopathy (EME) with infantile spasms. Hydrops fetalis and intrauterine growth retardation had been evident from fetal period, and she was born with severe asphyxia at 31 gestational weeks. Erratic myoclonia with partial seizures developed at one month of age, and EEG showed suppression-burst patterns. She was diagnosed as having EME, and treated with anticonvulsant agents. Her seizures were intractable and persisted all day long. Erratic myoclonia with partial seizures progressed to infantile spasms at five months. ACTH therapy was initiated at six months. This ceased her infantile spasms, and also improved of erratic myoclonia and partial seizures. Erratic myoclonia during sleep disappeared, and decreased while awake. EME is one of the severe intractable types of epilepsy during early stage of life. It is characterized by myoclonus such as erratic myoclonia, and partial seizures. Few agents are effective. In EME with infantile spasms, ACTH therapy might be one of choices to be tried.
