Document Detail

Ictal and nonictal paroxysmal events in infantile neuroaxonal dystrophy: polygraphic study of a case.
MedLine Citation:
PMID:  11554895     Owner:  NLM     Status:  MEDLINE    
A 7.5-year-old girl, with infantile neuroaxonal dystrophy (INAD), showed a gradual deterioration from 16 months; at age 5 years she was bedridden, with severe tetraplegia, strabismus, nystagmus and optic atrophy, and dementia. From age 5.5 years, she had paroxysmal tonic events. Videopolygraphic recordings disclosed two different kinds of motor events: (a) epileptic tonic seizures, in wakefulness and sleep, associated with autonomic changes and ictal EEG discharges; and (b) nonepileptic prolonged clusters of brief tonic spasms, without ictal modifications of the EEG. Both motor events were characterized by a minimal and clinically similar tonic contraction of the upper extremities. Video-polygraphic studies are mandatory for a correct paroxysmal event classification and treatment in INAD patients.
M Santucci; G Ambrosetto; M C Scaduto; M Morbin; E V Tzolas; P G Rossi
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Epilepsia     Volume:  42     ISSN:  0013-9580     ISO Abbreviation:  Epilepsia     Publication Date:  2001 Aug 
Date Detail:
Created Date:  2001-09-13     Completed Date:  2001-12-04     Revised Date:  2007-01-25    
Medline Journal Info:
Nlm Unique ID:  2983306R     Medline TA:  Epilepsia     Country:  United States    
Other Details:
Languages:  eng     Pagination:  1074-7     Citation Subset:  IM    
Department of Child Neurology and Psychiatry, Neurological Institute, University of Bologna, Italy.
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MeSH Terms
Arm / physiopathology
Electroencephalography / statistics & numerical data*
Electromyography / statistics & numerical data
Epilepsy / diagnosis*,  epidemiology,  physiopathology
Monitoring, Physiologic / statistics & numerical data*
Muscle Contraction / physiology
Muscle, Skeletal / physiopathology
Neuroaxonal Dystrophies / diagnosis*,  epidemiology,  physiopathology
Polysomnography / statistics & numerical data
Sleep / physiology
Videotape Recording
Wakefulness / physiology

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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