Ocular symptoms of moyamoya disease

We examined four patients with moyamoya disease who had ocular symptoms. The diagnosis of the disease was based on an abnormal vascular network in the cerebral basilar region, observed by carotid angiography. One patient had amaurosis fugax. A second patient demonstrated bilaterally decreased visual acuity and visual spatial agnosia. The other two patients had homonymous hemianopsia; one also had transient diplopia and the other had temporal pallor of the optic disks. We found that patients with moyamoya disease exhibited various ocular symptoms but had few abnormal intraocular findings.