Seizure and EEG patterns in Angelman's syndrome

F. Viani, A. Romeo, M. Viri, M. Mastrangelo, F. Lalatta, A. Selicorni, G. Gobbi, G. Lanzi, D. Bettio, V. Briscioli, M. Di Segni, R. Parini, G. Terzoli

Research output: Contribution to journalArticlepeer-review

Abstract

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.

Original languageEnglish
Pages (from-to)467-471
Number of pages5
JournalJournal of Child Neurology
Volume10
Issue number6
Publication statusPublished - 1995

ASJC Scopus subject areas

  • Clinical Neurology
  • Pediatrics, Perinatology, and Child Health

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