A 58-year-old Hispanic man with a history of coronary artery disease and three episodes of strokes with residual left upper and lower extremity weakness presented to our facility with a new onset left-sided facial droop. He reported facial numbness, dysarthria, and blurred vision. His neurological examination revealed left-sided facial droop, positive pronator drift, and left-sided hemiparesis, as well as positive left Babinski sign. Brain magnetic resonance imaging (MRI) without contrast described a patchy region of acute ischemia in the mid and posterior right frontal lobe, predominantly peripherally and cortically based, measuring 3.8 cm x 3 cm (Figure ).\nNeck and head computed tomography (CT) angiography revealed bilateral supraclinoid ICA occlusions (Figure ).\nCerebral angiography demonstrated diffuse intracranial vascular irregularity with stenosis, more pronounced above the bilateral supraclinoid ICAs and on the right middle cerebral artery (MCA) (Figure ).\nImaging findings were suggestive of moyamoya phenomenon. Superficial temporal artery (STA)-MCA vascular bypass was not an option due to the lack of right MCA patency. Therefore, he underwent right-sided EDAS procedure. STA was dissected inferiorly to see it at just above the root of the zygoma. Superiorly, three branches were identified: one frontally, one temporally, and one superiorly. Even after full dissection, the STA was still not completely movable. The smallest posterior branch was bipolared and cut, and therefore there was more mobility in the STA. The other branches were all dopplerable. The STA was then again dissected completely free and moved anteriorly. Subperiosteal dissection was done. A perforator drill was used to makde a bur hole at the superior portion of the incision, and then one at the inferior portion of incision along the course of the STA. The burr holes were connected with a footplate, and the bone was removed in one piece. The dura was tented up and opened. The STA was brought on top of the brain. There was still some tension on the STA. Therefore, STA was further dissected at the level of the root of zygoma and at the superior branches. There was still tension on the STA, and therefore again it was dopplered inferiorly and superiorly. The frontal branch of the STA was bipolared and cut. At this point, the STA was completely free superiorly and inferiorly with good Doppler signals superiorly and inferiorly. The STA was brought in and laid down on top of the brain. The STA was on top of the pia without tension. No complications noted intraoperatively. Postoperatively, his facial droop gradually improved, but the residual left-sided weakness remained unchanged. He was discharged home with levetiracetam and aspirin. Follow-up of nine months later revealed no evidence of new stroke recurrence.
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.