From the Epilepsy Centre, Children’s Neuropsychiatric Unit (Drs Viani,

We studied the seizure and polygraphic patterns of 18 patients with Angelman’s syndrome. All patients showed move-ment problems. Eleven patients were also reported to have long-lasting periods of jerky movements. The polygraphic recording showed a myoclonic status epilepticus in nine of them. Seven patients had partial seizures with eye deviation and vomiting, similar to those of childhood occipital epilepsies. These seizures and electroencephalographic patterns suggest that Angelman’s syndrome occurs in most of the patients as a nonprogressive, age-dependent myoclonic encephalopathy with a prominent occipital involvement. These findings indicate that, whereas ataxia is a constant symptom in Angelman’s syndrome, the occurrence of a transient myoclonic status epilepticus may account for the recurrence of different abnormal movements, namely the jerky ones. (J Child Neurol 1995;10:467-471). Angelman’s syndrome, previously defined as &dquo;puppet&dquo; or &dquo;happy puppet&dquo; syndrome,l2 is characterized by neuro-logic, behavioral, ophthalmic, and pigmentary features and craniofacial abnormalities. About 65 % of patient