A 27-year-old obese female presented with acute onset, rapidly progressing binocular visual disturbance.  Ophthalmological examination revealed bilateral conjunctival injection and optic disk edema with macular detachment.  There was no reported history of trauma, infectious exposure, or prior autoimmune disease.  Initially, a diagnosis of idiopathic intracranial hypertension was considered.  Magnetic resonance imaging of the Brain and cerebral venous system was unremarkable, and lumbar puncture revealed only borderline elevated opening pressure.  Given the acute onset, atypical fundoscopic findings and lack of evidence of increased intracranial pressure, we considered a diagnosis of Vogt-Koyanagi-Harada (VKH) syndrome and started an empiric course of high dose methylprednisolone followed by an oral taper.  At follow up examination 2 weeks post-discharge, there was significant improvement from the initial fundoscopic findings.  

VKH is classically described as a multi-system autoimmune granulomatous disorder, with ocular, auditory, dermatologic and neurologic manifestations.  Patients may present with headache and swollen optic discs, and the characteristic ophthalmologic and systemic findings may appear later in the disease course, leading to misdiagnosis in approximately 9% of cases.  Although our patient lacked extra-ocular manifestations of VKH, the acute bilateral ocular involvement, the findings of macular detachment and the lack of evidence of other intracranial or autoimmune processes were supportive of the diagnosis.  Our patient had isolated ocular involvement, and an absence of ocular trauma with alternative diagnoses (particularly infective causes) ruled out, therefore meeting the criteria for "probable" VKH.  A 2007 study found that about half of patients diagnosed with acute VKH have ocular manifestations only.  Delay in diagnosis and subsequent treatment can result in significant disability.  In our patient, there was a prompt response to immunosuppressive therapy, supporting that this diagnosis should be considered in patients with atypical presentations of ocular disease, in which alternative conditions are effectively ruled out.