Photoclinic Diagnosis: Moyamoya Disease Associated with Neurofibromatosis 1

A38-year-old woman presented with sudden weakness of the left upper and lower extremities, severe headache, repeated vomiting, and decreased level of consciousness since five to six hours prior to admission. She had no history of diabetes mellitus, hypertension, or any other remarkable diseases. She was normotensive at the time of admission. On general examination, the patient had some characteristic cutaneous lesions on her trunk and extremities. On neurologic examination, she was in a stupor state with a dense left hemiplegia, and had a positive Babinski sign in the left side and a gaze deviation to the right side. Brain computed tomography (CT) revealed a large area of hemorrhage in the right fronto-temporal area. A digital subtraction angiography of cerebral vessels with intravenous contrast was obtained.