Sturge-Weber Syndrome: Clinical Spectrum, Disease Course, and Outcome of 30 Patients

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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.date.accessioned2017-03-10T03:28:42Z
dc.date.available2017-03-10T03:28:42Z
dc.date.issued2013
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.identifier.citation28 ,6;722-728en_US
dc.identifier.uri10.1177/0883073812451326
dc.identifier.urihttps://dspace.sctimst.ac.in/handle/123456789/10492
dc.publisherJOURNAL OF CHILD NEUROLOGY
dc.subjectNeurosciences & Neurology; Pediatrics
dc.titleSturge-Weber Syndrome: Clinical Spectrum, Disease Course, and Outcome of 30 Patients
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