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.
A 53-year-old Arab woman living in Saudi Arabia presented to the emergency department with an acute onset of unsteadiness, a tendency to fall forward present for one week, and slurred speech present for one day. She had a negative history of loss of consciousness, abnormal movement, weakness, numbness, and vertigo. She had a history of chronic headaches. She has diabetes and suffered a stroke two years ago. She had one abortion and twelve successful pregnancies; the last two were C-section. She had no history of sickle-cell disease or other chronic diseases. Her neurological assessment was normal with no focal signs. Other examinations were unremarkable. A head computed tomography (CT) scan and a magnetic resonance imaging (MRI) of her brain (Figure ) upon presentation revealed acute corpus callosum hemorrhage.\nHer brain CT venogram was normal. Other diagnostic studies were normal. A cerebral angiogram (Figures -) showed severe narrowing or occlusion of both distal internal carotid arteries (ICA) with multiple intracranial collaterals, including the choroidal arteries and the posterior pericallosal artery as well as the lenticulostriate arteries; this was a classical presentation of the moyamoya pattern.