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Sturge-Weber Syndrome Coexisting With Moyamoya Disease in the Fifth Decade

A Case Report and Literature Review

Hao, Zilong, MD, PhD; Lai, Xiaohui, MD, PhD

doi: 10.1097/NRL.0000000000000207
Case Report/Case Series

Sturge-Weber syndrome (SWS) is a sporadically occurring neurocutaneous syndrome characterized by port-wine stain over the face, ocular abnormalities (glaucoma and choroidal hemangioma), and leptomeningeal angiomas. It is usually diagnosed in infancy, but it may occasionally present in adulthood with seizures or stroke-like episodes. Here, we report a 46-year-old male patient, having SWS coexisting with moyamoya disease, attending our hospital due to sudden loss of consciousness. We also searched PubMed (from its earliest date to August 2014) for case reports mentioning that SWS presents in adulthood. We identified 31 patients. The common clinical manifestation are seizures, stroke-like episodes, and migraine-like headaches.On the basis of our findings in this patient, we would recommend that patients with a port-wine nevus of the face should be given further investigation to rule out an intracranial vascular malformation, especially if seizures, stroke-like episodes, or migraine-like headaches are present.

Department of Neurology, West China Hospital, Sichuan University, Guo Xue Xiang, Chengdu, China

Supported by the National Natural Science Foundation of China (81601012) and the Science and Technology Program of Sichuan Province (No. 2016JY0151).

The authors declare no conflict of interest.

Correspondence to: Xiaohui Lai, MD, PhD, Department of Neurology, West China Hospital, Sichuan University, No. 37, Guo Xue Xiang, Chengdu 610041, China. E-mail:

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