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Abstract #50604 Published in IGR 14-2
Sturge-Weber syndrome -clinical and neuroimaging variability
Rios M; Barbot C; Pinto PS; Salí,cio L; Santos M; Carrilho I; Temudo TAnales de pediatria (Barcelona, Spain : 2003) 2012; 77: 397-402
Sturge Weber Syndrome is a rare neurocutaneous syndrome in which the severity is determined by degree of brain involvement and control of epilepsy. The authors describe and analyse clinical and imaging features of this syndrome, through a retrospective study of 13 patients (8 girls; aged between 15 days and 9 years at first visit). Twelve had facial angioma and one had atrichia corresponding to the area of brain involvement. Epilepsy was diagnosed in 6 cases, hemiplegia in 4, psychomotor delay in 7, and glaucoma in 4. Cerebral abnormalities were found in 10 children, 3 without neurological symptoms. The clinical signs and symptoms vary and there is not always a relationship between the severity of the clinical and neuroimaging abnormalities, which may occur even in the absence of neurological symptoms.
Servicio de Neuropediatría, Centro Hospitalar do Porto, Oporto, Portugal.
Full articleClassification:
9.4.15 Glaucoma in relation to systemic disease (Part of: 9 Clinical forms of glaucomas > 9.4 Glaucomas associated with other ocular and systemic disorders)
