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Infantile spasms in the setting of Sturge–Weber syndrome

Authors :: Martino Ruggieri
Gemma Incorpora
Massimo Barbagallo
Lorenzo Pavone
Piero Pavone
Agata Polizzi
Caterina Nucifora
Andrea D. Praticò
Paola Iannetti
Alberto Spalice
Source :: (2008)., info:cnr-pdr/source/autori:Barbagallo M, Ruggieri M, Incorpora G, Pavone P, Nucifora C, Spalice A, Praticò AD, Polizzi A, Pavone L, Iannetti P./titolo:Infantile spasms in the setting of Sturge-Weber syndrome./doi:/rivista:/anno:2008/pagina_da:/pagina_a:/intervallo_pagine:/volume
Publication Year :: 2008
Publisher :: Springer Science and Business Media LLC, 2008.
Abstract: 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).