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 male patient, without relevant personal or family history, was admitted to the emergency room after he suddenly developed severe headache with dysarthria, altered state of consciousness and syncope. At the time of admission, he was found drowsy with global aphasia, stiff neck, right hemiparesis and right Babinski's sign. Kernig's sign was present too. Fundoscopy did not show retinal hemorrhages. Autoimmune diseases, brain tumors, actinic encephalopathy, Von Recklinghausen's disease and Down syndrome were clinically ruled out.\nA non-contrast brain computed tomography revealed bleeding in the subarachnoid space over the left frontal and parietal cortex []. Four-vessel cerebral angiography showed bilateral stenosis of the supraclinoid segment of both internal carotid arteries and multiple tortuous vessels branching from the anterior and middle cerebral arteries (moyamoya vessels). These abnormal vessels were anastomosing with branches from the posterior cerebral and middle meningeal arteries. With this information, MMD diagnosis was made. An extensive search for aneurysms and vascular malformations was done, including a three-dimensional reconstruction from each projection of the four-vessel angiography, with no other abnormalities found []. Taking into account the possibility of an angiographically occult aneurysm, digital subtraction angiography was repeated after 4 weeks and remained without changes from the previous.\nDuring hospitalization, the patient developed a massive pulmonary embolism and subsequently died.