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Ann Indian Acad Neurol. 2013 Jan;16(1):118-20. doi: 10.4103/0972-2327.107725.

A clinician's dilemma: Sturge-Weber syndrome 'without facial nevus'!!

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  • 1Department of Neurology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, Kerala, India.

Abstract

Sturge-Weber syndrome (SWS) is a rare, sporadic neurocutaneous syndrome characterized by a classical triad of facial port wine nevus, ipsilateral leptomeningeal angiomatosis (LAM) and glaucoma. The incidence of SWS is 1/50,000 live births, although it is more often underreported. The incidence of SWS without facial nevus is not known, although very few patients without facial nevus have been reported. In these patients, the diagnosis of SWS is made by the findings of computed tomography, magnetic resonance imaging, and histopathology. Here, we report three patients with SWS from our cohort of 28 patients with SWS without facial nevus and discuss their clinical profile and outcome.

KEYWORDS:

Leptomeningeal angiomatosis; Sturge-Weber syndrome; seizure

PMID:
23661980
[PubMed]
PMCID:
PMC3644771
Free PMC Article
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