Infantile spasms in the setting of Sturge-Weber syndrome.
BACKGROUND
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).


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).
Barbagallo
Massimo
M
Department of Pediatrics, University of Catania, Catania, Italy.
Ruggieri
Martino
M
Incorpora
Gemma
G
Pavone
Piero
P
Nucifora
Caterina
C
Spalice
Alberto
A
Praticò
Andrea Domenico
AD
Polizzi
Agata
A
Pavone
Lorenzo
L
Iannetti
Paola
P
D002648:Child; D002675:Child, Preschool; D004569:Electroencephalography / Q000379:methods; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D012720:Severity of Illness Index; D013036:Spasms, Infantile / Q000209:etiology / Q000473:pathology* / Q000503:physiopathology; D013341:Sturge-Weber Syndrome / Q000150:complications / Q000473:pathology* / Q000503:physiopathology