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Vogt-Koyanagi-Harada syndrome (VKH) is a disease distinguished by a triad of uveitis, dermatologic findings, and CNS involvement. [1,2] Although the etiology of VKH is unknown, abnormalities including increased interleukins in the aqueous humor, ocular lymphocytic infiltrates. Increased peripheral blood CD4plus /CD8plus T-cell ratios have been seen in VKH, suggesting an immune-mediated disease mechanism. [3,4] We describe a patient with steroid intractable VKH who responded to a combination of azothioprine and intravenous immunoglobulin (IVIg) therapy.
Report of a case.
A 57-year-old man with no family history of neurologic disease was healthy until age 40 years, when he gradually experienced bilateral eye pain and ``blurred'' vision. An ophthalmologist diagnosed uveitis and treated with low-dose prednisone, with resultant improvement. Prednisone was then discontinued after a 2-month taper.
At age 44 years, after a subacute decrease in visual acuity, oral prednisone was restarted at 100 mg/d. Vision improved, but hyperglycemia 1 year later prompted a discontinuation. He progressively developed unsteadiness of gait, paraparesis (R more than L), hand numbness bilaterally (R more than L), and a 70-pound weight loss. Patchy depigmentation of the hair and eyebrows (poliosis) and …
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