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.
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.