[Miller-Dieker syndrome (type I lissencephaly) with specific EEG changes].

We present the case report of a girl with a subtype of Lissencephaly syndrome, type I, "Miller-Dieker syndrome", pointing out the specific EEG features in infancy and early childhood. The following pathognomonic EEG manifestations may confirm the diagnosis of an lissencephalic syndrome: abnormally fast background activity of an extraordinary high voltage increasing with age, missing topographic structuring, no reactivity to sleep or medication, unusually high-voltaged sharp-slow-wave complexes.