A 36 year old African American female presented to the Out Patient Office with headache which was on and off for last 4–6 months. The headache presented was of piercing type with moderate intensity not relieved by butalbital, acetaminophen. It was associated with weakness on right hand side for last few days, which evolved into dizziness and speech difficulty. There was no associated fever, night sweats, loss of consciousness, vomiting, ataxia, photophobia, tingling, numbness, difficulty swallowing, difficulty hearing, neck stiffness, vision problems. Her family history was significant for hypertension, diabetes and coronary artery disease. General physical examination showed no abnormalities. She is obese with BMI of 36.4; Vitals include [Heart Rate = 93/min, Blood Pressure = 165/75 mm Hg, Temp. = 36.67°C, Resp. Rate = 18/min]. Neurological examination showed intact 2–12 cranial nerve function, motor strength grossly intact with strength 3/5 on the right side of hand. No pronator drift, normal muscular tone, and gait was normal. Laboratory investigations, complete blood count, electroencephalogram and echocardiogram were normal.\nA CT angiogram head with contrast shows one sagittal [\n] and two axial views [\n,\n] at the level of the lateral ventricle and Circle of Willis, demonstrate mild proliferation of collateral vessels emanating from the distal internal carotid artery particularly on the left side. In addition, the visualized portion of the distal internal carotid artery and M1 segment of the left middle cerebral arteries appeared somewhat diminutive in caliber. Findings were generally compatible with progressive vascular occlusive process which can be seen in Moyamoya disease.\nSince the patient was refractory to the pain medications, surgical revascularization was done and the procedure involved left superficial temporal artery-left middle cerebral artery bypass without any complications. Following surgery, she was prescribed 81mg aspirin and discharged home and was followed up initially after surgery once every three months for one year followed by every 6 months for next four years. She did not complain of any headaches or focal neurological symptoms.
A 53-year-old Arab woman living in Saudi Arabia presented to the emergency department with an acute onset of unsteadiness, a tendency to fall forward present for one week, and slurred speech present for one day. She had a negative history of loss of consciousness, abnormal movement, weakness, numbness, and vertigo. She had a history of chronic headaches. She has diabetes and suffered a stroke two years ago. She had one abortion and twelve successful pregnancies; the last two were C-section. She had no history of sickle-cell disease or other chronic diseases. Her neurological assessment was normal with no focal signs. Other examinations were unremarkable. A head computed tomography (CT) scan and a magnetic resonance imaging (MRI) of her brain (Figure ) upon presentation revealed acute corpus callosum hemorrhage.\nHer brain CT venogram was normal. Other diagnostic studies were normal. A cerebral angiogram (Figures -) showed severe narrowing or occlusion of both distal internal carotid arteries (ICA) with multiple intracranial collaterals, including the choroidal arteries and the posterior pericallosal artery as well as the lenticulostriate arteries; this was a classical presentation of the moyamoya pattern.