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.
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.