Document Detail

Evolution of epilepsy and EEG findings in Angelman syndrome.
MedLine Citation:
PMID:  9048672     Owner:  NLM     Status:  MEDLINE    
PURPOSE: To evaluate the evolution of epileptic seizures and EEG features in a large group of patients with Angelman syndrome (AS). METHODS: Thirty-six patients with AS with a proven chromosome 15q11-13 deletion were retrospectively analyzed with regard to their epilepsy and EEG findings by examination of patient files and EEGs. AIJ EEGs were reviewed by one of the authors. A logistic regression model, with a follow-up from 1 to 39 years (mean, 15 years), was used for statistical analysis. RESULTS: Epileptic seizures had occurred in 30 (83%) patients. In 43% of them, the initial symptoms of epilepsy were febrile convulsions in infancy. In childhood, epilepsy could start with almost any type of seizure. Atypical absences and myoclonic seizures prevailed in adulthood. Epileptic seizures were present in 92% of the adult patients. The most typical EEG findings were rhythmic triphasic delta waves of high amplitude with a maximum over the frontal regions, identified in 99 (66%) of 150 EEGs, and continuously or intermittently, in 30 (83%) of 36 patients with AS. In 47% it was present even before a clinical diagnosis of AS was considered. High-amplitude rhythmic 4-6/s slow activity, seen in 44 (29%) of 150 EEGs, was not present after the age of 12 years. CONCLUSIONS: In contrast to previous reports suggesting a decreasing frequency of epileptic seizures with age, we found that 92% of the adult patients with AS continued to have epileptic seizures. The most typical EEG finding in AS, in both children and adults, was the presence of frontal triphasic delta waves. In mentally retarded patients, this EEG pattern should point the physician in the direction of AS.
L A Laan; W O Renier; W F Arts; I M Buntinx; I J vd Burgt; H Stroink; J Beuten; K H Zwinderman; J G van Dijk; O F Brouwer
Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Epilepsia     Volume:  38     ISSN:  0013-9580     ISO Abbreviation:  Epilepsia     Publication Date:  1997 Feb 
Date Detail:
Created Date:  1997-03-19     Completed Date:  1997-03-19     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  2983306R     Medline TA:  Epilepsia     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  195-9     Citation Subset:  IM    
Department of Neurology, Leiden University Hospital, The Netherlands.
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MeSH Terms
Age Factors
Age of Onset
Angelman Syndrome / diagnosis*,  physiopathology
Brain / physiopathology
Child, Preschool
Delta Rhythm
Electroencephalography* / statistics & numerical data
Follow-Up Studies
Logistic Models
Retrospective Studies

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