Moyamoya disease in young children

Abstract

Moyamoya disease (MMD) is characterized by the progressive occlusion of the bilateral distal internal carotid arteries or of the proximal portions of the middle and anterior cerebral arteries (MCAs and ACAs), which is accompanied by extensive collateral vessel formation at the base of the brain. In children, MMD frequently manifests itself as a transient ischemic attack (TIA) that is provoked by hyperventilation. As the cerebral perfusion gradually decreases, the frequency, extent, and duration of TIAs increase, which leads to cerebral infarction. The benefits of revascularization surgery, either direct or indirect, have been well established in patients with slow progression of ischemia, as this type of surgery is effective for the elimination of TIA and the prevention of cerebral infarction. © 2010 Springer-Verlag Tokyo.