A 32-year-old Saudi Arabian male sickler patient presented to the emergency department with a six-hour history of sudden and continuous shortness of breath. The history was obtained from the mother. The patient’s current presentation was associated with agitation and a sudden change in behavior. The patient was shouting and refusing to communicate with doctors. The mother denied any history of orthopnea, cough or fever during the patient’s current presentation. Past medical history was remarkable for SCD since childhood with infrequent hospital visits. Socially, the patient was single, non-smoker and living with his family.\nOn general examination, the patient looked ill, anxious and sitting on the bed lying forward connected to an oxygen mask. The body mass index (BMI) was 18.5 kg/m2. Vital signs showed blood pressure: 125/80 mmHg, pulse: 110 beats/min, respiratory rate: 28 breaths/min, temperature: 39 degrees Celsius and oxygen saturation: 87% on room air and 94% on 5 liters of oxygen. Respiratory and cardiovascular examinations were normal. Hemoglobin electrophoresis showed a hemoglobin S (Hb S) level of 78%. Laboratory testing for D-dimer showed 12 ng/L (normal range is < 0.5 ng/L). Pulmonary embolism was suspected and chest spiral computed tomography (CT) scan showed bilateral consolidation without acute pulmonary embolism. In consideration of the fever and agitation, the patient received ceftriaxone, vancomycin and diazepam.\nCT scan of the head without contrast showed left frontal subcortical deep white matter hypodensity, most likely representing chronic watershed infarction. Moreover, there was left frontal cortical gray matter hypodensity, most likely representing cerebral atrophy (Figure ). There were no signs of acute hemorrhage or ischemic infarction. Magnetic resonance angiography (MRA) showed severely hypoplastic left ICA and abnormal tortuous collateral vessels around the circle of Willis (Figure ). No arteriovenous malformation or aneurysm was noted.\nBased on the clinical and radiological findings, the final differential diagnosis was suggestive of a progressive vascular occlusive process which could be seen in MMD. Hematology team was consulted for further evaluation. The patient received a blood transfusion three times daily for 16 days. The patient dramatically improved and returned to his regular behavior. The patient was discharged home on aspirin 81 mg daily. The patient was seen four weeks later at the neurology clinic and he was doing well.
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.