Neuro-Ophthalmic Vascular Disease

This 23-year-old woman has had insulin-dependent diabetes mellitus since age 3. She was diagnosed with Sydenham's chorea in early childhood and had grand mal seizures from age 13 to 15. She has been hypertensive since age 18. Her vision was 20/25 OD and 20/40 OS, with dyschromatopsia OS, and a 1.8 log unit APD OS. The field OD was normal: the field OS showed a temporal scotoma originating out of the blind spot. The fundus OD was small and crowded, with a cup:disc ratio of 0.1 and a few dot hemorrhages. The left eye showed regressed neovascularization and a dragged macula. Her antiphospholipid antibodies were negative. The MRI showed multiple foci of increased signal on T2 and proton density images. This was felt to be moyamoya disease, with stenosis of the middle cerebrals and dilated vascular channels penetrating the microglia. The MR angiogram (MRA) showed occlusion of both internal carotids and the left posterior cerebral arteries. Pair with Images 95_59, 95_61, and 95_62.