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 13-month-old Syrian baby boy born to a non-consanguineous couple was admitted to our hospital with complaints of sudden onset focal tonic-clonic seizure, mainly on the right side, for further evaluation and management. The seizure attack lasted approximately a few minutes. The second attack was associated with loss of consciousness and uprolling of the eyes. There was a history of sudden falls during playing. However, there was no history of fever, head trauma, headache, vomiting, or ear discharge. He had normal growth parameters. there was no significant family history of early stroke, ischemic heart disease, or autoimmune disease. His past medical history was significant for right arm weakness one month before his presentation and resolved without treatment. Upon presentation, he was vitally normal except for HR of 128, and RR of 28.\nA head CT revealed nonspecific hypodensity, bilateral periventricular white matter, and the left parietofrontal subcortical area requiring further workup.\nAn MRI (Figure ) revealed acute infarction involving the left frontal lobe cortical and subcortical region and multiple bilateral periventricular white matter changes likely due to leu-co-encephalopathy/chronic ischemic. MRA (Figure ) showed attenuated and irregular distal intracranial ICAs at the cavernous and supraclinoid segments and bilateral stenosis seen at both MCAs, almost occluded on the right side, and are surrounded by extensive lenticulostriate collaterals.\nEncephalo-duro-arterio-synangiosis (EDAS) surgery was performed due to failure of the first attempt of cerebral angiography. The baby was discharged home in a stable condition on Tegretol and aspirin for follow-up in the clinic and revascularization surgery on the right side was scheduled three months after the first one.\nThree weeks later, the child presented with lethargy, weakness in his left arm, and vomiting. Upon admission, he was afebrile, maintaining room air. Physical examination findings were normal apart from bilateral chest rhonchi, and weakness of the left upper and lower limbs. Hematologists recommended starting the patient on clexane and continuing with aspirin.\nConventional cerebral angiography (Figure ) was performed post left EDAS surgery and demonstrated bilateral ICAs stenosis seen at the carotid terminus with multiple collateral formation compatible with the history of MMD. There is evidence of right MCA M2 segment thrombosis compatible with MRI finding of acute infarction, left distal MCA branches thrombosis, and posterior circulation collateral formation synangiosis to the anterior circulation. Neurologists were consulted, and right-side revascularization was scheduled.\nA post-operative MRI brain (Figure ) was performed because the patient still had upper and lower left side weakness, and it revealed a large right parietal-temporal-occipital area cortical and sub-cortical area of restricted diffusion consistent with acute infarct. The previously seen large right cerebral diffusion restriction now shows residual small areas of cortical and adjacent subcortical spaces.\nThe clinical and radiological findings were suggestive of a progressive vascular occlusive disease that could be seen in MMD. Several investigations including factor V, factor VII, antithrombin activity, protein C, protein S, and EEG were carried to rule out associated medical conditions. The patient was discharged in a stable condition with residual left side weakness. Therefore, an appointment with physiotherapy was reserved.