Headache, Uveitis, and Leptomeningeal Enhancement

A 35-year-old man sought care for a severe, acute-onset, pounding, bifrontal headache, photopsias, and nausea for 1 day. Initially, bilateral red eyes developed, and within 24 hours he had central blurred vision problems in the left eye. He reported that objects had a yellow tint with the left eye and looked “wavy” supranasally. An emergent evaluation documented bilateral red eyes, and an initial diagnosis of bilateral panuveitis was given. By 48 hours after symptom onset, he started vomiting. He also was feeling feverish and off-balance. He reported no tinnitus or hearing loss, any change in color of his eyelashes or eyebrows, alopecia, poliosis, or cognitive difficulties. An initial work-up for infectious processes was negative. Given the patient’s ethnic background, including Chinese, Japanese, and Filipino origin, and typical findings of uveomeningitis, he was diagnosed with probable Vogt-Koyanagi-Harada syndrome. There is no specific diagnostic test for this entity, and the diagnosis remains reliant on a combined interpretation of clinical and ancillary testing. The patient was kept on oral prednisone daily, and azathioprine was initiated, as well as prophylaxis against Pneumocystis carinii pneumonia and gastrointestinal tract hemorrhage. During the tapering phase of prednisone, liver function test abnormalities were found, so azathioprine was discontinued. At 1-year follow-up, he had some mild skin flaking and weight gain from the corticosteroid therapy but no other symptoms, despite having discontinued azathioprine for 3 months. He continued to taper off prednisone. He had development of bilateral hip pain; imaging showed bilateral aseptic hip necrosis. A decision was made to initiate tumor necrosis factor (TNF)-α‎ inhibitors. He lost all the weight gained and recovered from the aseptic necrosis of the hip to be able to continue running. The final diagnosis was recurrent Vogt-Koyanagi-Harada syndrome. Vogt-Koyanagi-Harada syndrome is an idiopathic inflammatory disease with panuveitis and neurologic involvement in the form of aseptic meningitis and/or hearing loss. Although the full spectrum of the disorder may involve many skin changes and additional findings, most patients have incomplete disease because they are urgently treated with corticosteroids and the disorder is steroid responsive.