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dc.contributor.authorJagtap, S-
dc.contributor.authorSrinivas, G-
dc.contributor.authorHarsha, KJ-
dc.contributor.authorRadhakrishnan, N-
dc.contributor.authorRadhakrishnan, A-
dc.identifier.citation28 ,6;722-728en_US
dc.description.abstractSturge-Weber syndrome is a heterogeneous neurocutaneous syndrome with facial and leptomeningeal angiomas, glaucoma, seizures, stroke-like episodes, and mental retardation. The authors critically evaluated the clinical manifestations, outcome, and natural history in 30 patients with Sturge-Weber syndrome followed up from January 1985 to May 2010. Of the patients, 15 were males, age at diagnosis ranged from 1 month to 43 years. Typical port-wine stain nevus occurred in 26 (86%), it was bilateral in 2 (8%), and it was absent in 4 (4%). Nine patients had glaucoma (30%), 3 required surgery. Four had transient hemiparesis. All patients had seizures; they were well controlled in 22 (73.3%); in 8 they remained drug resistant. Three patients underwent surgery and became seizure-free. Of the 17 who had mental subnormality, 14 (82.4%) had seizure onset before 2 years. An early age at seizure onset and those with drug-resistant seizures had more severe degree of mental subnormality. Uncontrolled seizures, mental subnormality, visual handicap, and cosmetic disfiguration were the major impediments in life.-
dc.subjectNeurosciences & Neurology; Pediatrics-
dc.titleSturge-Weber Syndrome: Clinical Spectrum, Disease Course, and Outcome of 30 Patients-
Appears in Collections:Journal Articles

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