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 23-month-old full-term Moroccan baby boy born to a non-consanguineous couple was referred to our hospital with the complaint of sudden onset left-sided hemiparesis evolving for 2 days. There was no history of fever, seizure, head injury, ear discharge, headache, visual disturbances, or vomiting. No family history of early stroke, ischemic heart disease, or autoimmune disease was reported. At presentation, he was conscious and well orientated. There was no dysmorphism, no neurocutaneous markers, and he had normal growth parameters. He was afebrile with a pulse of 110 beats per minute and a respiratory rate of 26 breaths per minute. On neurological examination, there were no signs of meningeal irritation, his gait was hemiplegic, tone was decreased over left side, power was 2/5 over left upper and lower limb using Medical Research Council (MRC) muscle strength scale, and deep tendon reflexes were exaggerated. There were no abnormalities on cardiac auscultation or hepatosplenomegaly. Brain computed tomography (CT) showed a low density area in the right cortical and subcortical frontotemporoparietal regions and right insular cortex representing ischemic lesions in the right middle cerebral artery (MCA) territory (Fig. ). Laboratory investigations, including hemogram, prothrombin, partial thromboplastin, erythrocyte sedimentation rate (ESR), fibrinogen, proteins C and S, antithrombin III activity, and antinuclear antibody tests were all within limits. Renal ultrasonography with Doppler flow study showed no evidence of renal artery stenosis. Electroencephalography revealed no epileptic discharges, only a diffuse slow rhythm over the left hemisphere with slow waves at the temporal regions.\nOur patient was booked for cerebral angiography with contrast, which revealed a proximal obliteration in his MCA with a fine anastomotic moyamoya network and typical “puff of smoke” appearance (Fig. ). Urine for metabolic screening, including homocysteine levels, was normal. A normal hemoglobin electrophoresis ruled out sickle cell disease. An electrocardiogram and echocardiogram revealed no abnormalities. He was finally diagnosed as a case of MMD.\nWe treated our patient conservatively with acetylsalicylic acid 5 mg/kg per day. He showed slight improvement in power over left side (3/5). He was then discharged to follow-up with pediatric neurosurgeon. Cerebral revascularization surgery using encephaloduroarteriosynangiosis (EDAS) was performed. At 8-month follow-up, his hemiparesis was improved and no further ischemic events had occurred.