Moyamoya disease (MMD), a chronic disease of unknown cause, is characterized by abnormal vascularization at the base of the brain and bilateral steno-occlusive changes in the terminal portion of the internal carotid artery (ICA).1-4 The standard of care for patients with MMD with ischemic symptoms is revascularization surgery, including superficial temporal artery–middle cerebral artery (MCA) anastomosis,1,5which aims to prevent additional cerebral ischemic attacks.1,6,7 In pediatric MMD patients with ischemic symptoms of all severities, revascularization surgery is recommended for all such patients, because the brain of pediatric patients is not completely developed.8-11 In contrast, although several investigators have recommended selective revascularization surgery for adult patients with ischemic presentation and hemodynamic compromise,12,13 no clear guidelines are available for this selection.
Two recent studies have shown the natural clinical course of nonsurgically treated patients with adult-onset MMD presenting with ischemia and stable hemodynamics.13,14 Those 2 studies included a relatively large number of patients (140 and 144 patients, respectively), but retrospectively reviewed the medical records. Furthermore, they assessed only the incidence of stroke recurrence. Revascularization surgery for patients with adult-onset MMD augments cerebral blood flow (CBF), often leading to improvement of cognitive function.12,15 However, in nonsurgically treated adult MMD patients with ischemic onset and stable hemodynamics, the cerebral hemodynamic and cognitive course remains unclear.
To see the results of their study, click here.