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Year : 2013  |  Volume : 2  |  Issue : 3  |  Page : 206-208

Encephalotrigeminal angiomatosis

Department of Pediatrics, JSS Medical College and Hospital, JSS University, Mysore, Karnataka, India

Correspondence Address:
N Rashmi
Department of Pediatrics, JSS Medical College, Hospital, Ramanuja Road, Agrahara, Mysore - 570 009, Karnataka
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2278-344X.120593

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Encephalotrigeminal angiomatosis, more commonly known as Sturge-Weber syndrome (SWS), is a rare disorder occurring sporadically with a frequency of 1:50,000. It is characterized by facial nevus, seizures, hemiparesis, intracranial calcification, and mental retardation. We report here a young infant presenting with port-wine stain of one side of the face and transient hemiparesis (stroke-like episode) of the opposite side, seizures, and intracranial calcification, suggesting the diagnosis of SWS. The child reported here presented with a stroke-like event at a relatively young age, which is unusual. The baby was given supportive therapy.

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