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 35-year-old Hispanic male presented to the emergency department for sudden onset worsening headaches over the past four days. Headaches were diffusely felt, not localized to a specific head region, and not relieved by over the counter pain medication. There was no associated trauma, fever, night sweats, loss of consciousness, photophobia, neck stiffness, or visual disturbances. His past medical history was significant for migraine headaches, type 2 diabetes mellitus, and benign essential hypertension. Physical exam revealed Glasgow Coma Scale score of 3T on 15L of oxygen, pupils 2-2.5 mm bilaterally, inability to arouse by voice or painful stimulation, and paralysis of upper and lower extremities bilaterally. Vitals include (heart rate = 89/min, blood pressure = 159/78 mmHg, temperature = 37°C, respiratory rate = 22/min, and oxygen saturation = 99% on 15L of oxygen). Labs were significant for respiratory alkalosis (blood pH 7.54, pCO2 27.5 mmHg), normal oxygenation (159.3 mmHg), and negative urinary toxicology screen.\nA head CT scan revealed a 1.5 cm hematoma in the left thalamus with expansion into the lateral ventricle (Figure ). Cerebral angiogram demonstrated complete stenosis of the left internal carotid artery (ICA) and partial stenosis of the right ICA (Figures -). Diagnostic studies coupled with clinical presentation were compatible with a diagnosis of MMD. Patient treatment was initialized with emergent bedside right ventriculostomy (with opening pressure of 11 cmH2O) for intracranial pressure monitoring and for diversion of cerebrospinal fluid and hematoma. This patient ultimately underwent surgical revascularization. The procedure included a left superficial temporal artery-left middle cerebral artery bypass without any complications.