Many Small Lesions, One Big Problem - Presentation PPT

A 73-year old hypertensive and hyperlipidemic man presented with 18 months of horizontal diplopia, worse at distance and gaze right, oscillopsia on left head turn, and unsteady gait. Six months prior to consult, he developed dysphagia, worse with liquids, and recurrent falls forced him to use a walker. He had memory impairment for three months. On exam, MoCA testing revealed score of 20/30, with both language and visuo-spatial deficits. Visual acuity was 20/40 OD, 20/30- OS. Mydriatic pupil OS, due to old injury, was present; no RAPD was found. Color plates were normal. Visual fields were full to confrontation. Left ptosis from old inury was present.He had nuclear sclerotic cataracts. Optic discs were pink & flat, with 0.2 cup/disc ratio. EOM revealed partial right abduction deficit, comitant left hypertropia, and upbeat nystagmus. Motor and sensory exams were unremarkable. He had dysmetria in his upper extremities, left worse than right. He had an ataxic gait, with tendency to fall to the left, and could not tandem walk. MRI of the brain showed numerous contrast-enhancing lesions, hyperintense on FLAIR, widely distributed in the brainstem, basal ganglia, cerebral and cerebellar hemispheres, in patterns suggesting perivascular localization. CSF analysis revealed elevated protein at 69 mg/dl, with 7 leukocytes per cmm. CSF cultures, cytology, and demyelination studies were all negative. No malignancies were discovered by CT scans of the chest, abdomen, and pelvis, or by whole-body PET scanning. A battery of autoimmune markers was negative. Vitamin E and B12 levels were normal. Lactic dehydrogenase, beta 2 microglobulin, ANCA, and HIV testing were all normal. Serum IgE was elevated, and absolute CD4 and CD8 counts were roughly at a third of low normal, and the CD4:CD8 ratio was high. A diagnostic procedure was performed