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

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 SW...

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Bibliographic Details
Published in:Annals of the Indian Academy of Neurology 2013-01, Vol.16 (1), p.118-120
Main Authors: Jagtap, Sujit A, Srinivas, G, Radhakrishnan, Ashalatha, Harsha, K J
Format: Article
Language:English
Subjects:
Care and treatment
Case Report
Case studies
Causes of
Development and progression
Diagnosis
Face
Neurological disorders
Neurology
Sturge-Weber syndrome
Vitaceae
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Summary: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.
ISSN:0972-2327
1998-3549
DOI:10.4103/0972-2327.107725