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Seizure and EEG Patterns in Angelman's Syndrome

Epilepsy Centre, Children's Neuropsychiatric Unit

Antonino Romeo, MD

Epilepsy Centre, Children's Neuropsychiatric Unit

Maurizio Viri, MD

Epilepsy Centre, Children's Neuropsychiatric Unit

Massimo Mastrangelo, MD

Ospedale Regina Elena, the Service of Neurophysiology

Faustina Lalatta, MD

Laboratory of Cytogenetics, Istituti Clinici di Perfezionamento

Angelo Selicorni, MD

Department of Pediatrics, University of Milano

Giuseppe Gobbi, MD

Children's Neuropsychiatric Unit, Reggio Emilia

Giovanni Lanzi, MD

Children's Neuropsychiatric Clinic, University of Pavia, Pavia, Italy

Daniela Bettio, PhD

Laboratory of Cytogenetics, Centro Auxologico Italiano, Milano

Vincenza Briscioli, MD

Laboratory of Cytogenetics, Istituti Clinici di Perfezionamento

Marina Di Segni, PhD

Laboratory of Cytogenetics

Rossella Parini, MD

Department of Pediatrics, University of Milano

Gianluigi Terzoli

Laboratory of Cytogenetics

We studied the seizure and polygraphic patterns of 18 patients with Angelman's syndrome. All patients showed movement 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).

Journal of Child Neurology, Vol. 10, No. 6, 467-471 (1995)
DOI: 10.1177/088307389501000609


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