Document Detail

Infantile spasms in the setting of Sturge-Weber syndrome.
MedLine Citation:
PMID:  18830609     Owner:  NLM     Status:  MEDLINE    
INTRODUCTION: The prevalence and outcome of the most frequent type of epilepsy in infancy-infantile spasms (IS)-are well characterized in the setting of most neurocutaneous disorders. By contrast, still there is no study describing the natural history of IS in the setting of Sturge-Weber syndrome (SWS). MATERIALS AND METHODS: Two patients with SWS and IS were identified in our series and five in the literature. The aim of study is to evaluate the clinical, electroencephalographic (EEG) and imaging features of our cases and to compare our cases with those described in the literature. IS in the setting of SWS is an uncommon but possible event (2/19 patients seen over 13 years in our institutions). RESULTS: We confirmed the correlation between IS and severity of SWS cutaneous and neural (extension of leptomeningeal capillary malformation) phenotype. IS in SWS seems to be atypical both from a clinical viewpoint (they are asymmetric) and from a laboratory viewpoint (EEG is not classically hypsarrhythmic).
Massimo Barbagallo; Martino Ruggieri; Gemma Incorpora; Piero Pavone; Caterina Nucifora; Alberto Spalice; Andrea Domenico Praticò; Agata Polizzi; Lorenzo Pavone; Paola Iannetti
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Publication Detail:
Type:  Case Reports; Journal Article     Date:  2008-10-02
Journal Detail:
Title:  Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery     Volume:  25     ISSN:  1433-0350     ISO Abbreviation:  Childs Nerv Syst     Publication Date:  2009 Jan 
Date Detail:
Created Date:  2008-12-03     Completed Date:  2009-02-20     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8503227     Medline TA:  Childs Nerv Syst     Country:  Germany    
Other Details:
Languages:  eng     Pagination:  111-8     Citation Subset:  IM    
Department of Pediatrics, University of Catania, Catania, Italy.
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MeSH Terms
Child, Preschool
Electroencephalography / methods
Severity of Illness Index
Spasms, Infantile / etiology,  pathology*,  physiopathology
Sturge-Weber Syndrome / complications,  pathology*,  physiopathology

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