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 10-year-old Saudi girl was referred from a local hospital with right side weakness, which was of sudden onset, started in the right lower limb, and 2 days later involved the right upper limb. She had a history of 4 months of pallor, and 2 weeks skin bruising prior to the onset. It was not associated with facial weakness, headache, fever, convulsion, visual disturbance, or loss of consciousness, and no history of preceding head trauma. She had a similar attack of left side weakness 5 months earlier, which resolved spontaneously without medical evaluation at a local hospital. Her mother noticed that she is shorter than her other siblings. Her immunization was completed, and her physical, and cognitive development was normal. Her parents were first degree cousin, and had 6 other siblings. Her physical examination revealed a well looking girl, with pallor and skin bruising on lower limbs, with 2x3cm scattered café au lait spots on the back, and inguinal region. Her weight was 25 kg, below 5th centile for her age, and her height was 118 cm, below the 3rd centile for her age. She had right hemiparesis, there were no hepatosplenomegaly, and the examination of other systems were unremarkable.\nHer investigations showed white blood cell count 2600/mm3 (normal value [NV] - 4000-10500/mm3), neutrophil count 500/mm3 (NV - 3000-5000/mm3), hemoglobin 6.3 g/dl (NV - 12.5-16.1 g/dl), and platelet count 33000/mm3 (NV - 150-400/mm3). Her liver and renal profile were within normal limits. Bone marrow aspiration showed hypocellular marrow. Chromosomal analysis confirmed chromosomal breakage. Brain MRI showed a left frontal focal area of acute ischemic infarction, and an area of right parietal periventricular white matter old infarction, with multiple small white matter lacunar infarcts (). The brain MRA showed poor arterial circulation, and a narrow distal internal carotid artery with puff of smoke sign (arrow) on the right side suggestive of moyamoya disease (). The diagnosis was confirmed as FA, with association of moyamoya disease, presenting with right hemiplegia. She was managed initially by supportive care in the form of a blood transfusion, and physical therapy for hemiplegia, later she underwent partial match related donor stem cell transplantation (SCT), but unfortunately, she died after 3 months from post SCT complications.