RESUMEN
<p><b>OBJECTIVE</b>To study the clinical manifestations and neuroimaging characteristics of pediatric moyamoya disease.</p><p><b>METHODS</b>The clinical data of 17 children with moyamoya disease were retrospectively studied.</p><p><b>RESULTS</b>The onset age was between 3 and 14 years. The main clinical manifestations included motor weakness of extremities or hemiplegia, sensory disturbance and headache. Cranial CT or/and MRI examinations predominately showed cerebral infarct. Magnetic resonance angiography (MRA) and digital subtraction angiography (DSA) showed stenosis or occlusion at the terminus of the siphon portions of internal carotid arteries and proximal portions of anterior or middle cerebral arteries, and abnormal vascular networks at the base of brain.</p><p><b>CONCLUSIONS</b>Cerebral ischemia is main clinical manifestations in children with moyamoya disease, presenting motor weakness of extremities or hemiplegia, sensory disturbance and headache. DSA is essential to the diagnosis of the disease.</p>