Moyamoya Disease Presenting as Transient Amnesia with Cortical Blindness

Abstract

Moyamoya disease (MMD) is a rare progressive occlusive cerebrovascular disease characterized by occlusion or stenosis of the internal carotid artery and the compensatory development of abnormal Moyamoya vessels at the base of brain, originating either from the basilar artery or extracranial blood vessels. 1 The annual prevalence and incidence of MMD are estimated to be 3.16 and 0.35 per 100,000 individuals, respectively. 2,3 Symptoms and signs of MMD are presented as variable etiological categories. Symptoms of MMD are typically associated with the regions of the brain supplied by the internal carotid arteries and middle cerebral arteries. 2 Patients with bilateral occipital and temporal lesions typically present with acute onset of cortical blindness, anosognosia, and amnesia. We now describe a patient with MMD and unusual symptoms who presented with transient cortical blindness, anosognosia, and amnesia after experiencing emotional stress.

Neuropsychology of Moyamoya Disease

“…These deficits continued to be present or had improved slightly when follow-up was carried out [78]. Hong et al [79] described a patient (a 22-year-old, right-handed woman) who presented with transient cortical blindness, anosognosia, and global transitory amnesia associated with MMD. The woman completely denied blindness and recent memory disturbances with confabulation.…”