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17-month old male with newly diagnosed MoyaMoya after presenting with new onset seizures and right-sided weakness.

Case Description

MoyaMoya is a rare cerebrovascular condition predisposing patients to stroke in association with progressive stenosis of the intracranial internal carotid arteries and their proximal branches.

The patient presented initially after a fall with subsequent inability to bear weight on his right leg along with minimized use of his right arm. Patient was treated for a suspected tibial Salter-Harris II and nursemaid’s elbow. He presented four days later to the ED with new onset seizures. CT of the brain showed loss of left frontal gray-white matter. MRI showed bilateral MoyaMoya and a saccular aneurysm at the left internal carotid bifurcation. Patient underwent bilateral encephaloarterial synangiosis procedure. Four days post-op, new left-sided deficits were noted and stat MRI showed new acute infarcts. Once medically stable, patient was transferred to acute inpatient rehabilitation.

MoyaMoya is associated with approximately 6% of childhood strokes. Females are affected twice as often as males and more common in those of Asian or African American descent. The highest rate of diagnosis is in the first decade (average age 5 years). Inherited gene defect associated with sickle cell disease, Down Syndrome, or a history of surgery for congenital heart disease. However, environmental factors precipitate clinical emergence in susceptible patients.

MoyaMoya is a progressive disease due to increased narrowing of arteries. Without surgery, 50-60% of individuals will experience gradual deterioration of cognitive function from recurring strokes. MoyaMoya should be suspected in any patient, particularly children, presenting with a stroke of unclear etiology. The standard diagnosis of MoyaMoya is cerebral angiography (Figure 1).