Brain single photon emission tomography and hypercapnia test in testing cerebrovascular reserve capacity, in Moya moya disease.

Moya moya is a progressive cerebral occlusive vasculopathy, rare in European countries. We describe a case of a young woman with right-hand side hemiparesis, mixed expressive aphasia, organic psychosyndrome and cognitive malfunction. Detailed imaging methods displayed bilateral stenosis of the internal carotid artery, bilateral ischemic cerebral changes and bilateral perfusion deficit, which guided us to the final diagnosis. Before the bypass surgery, cerebrovascular reserve capacity (vasoreactivity), by the brain single photon emission tomography and hypercapnia, were assessed and the lower cerebrovascular reserve was demonstrated. Bilateral bypass surgery with extracranial-intracranial anastomosis, improved the neurological deficit. Diagnosis was confirmed by histological examination of the vessel wall specimen.