Acute Vogt-Koyanagi-Harada: a case report

Abstract

Context: Vogt-Koyanagi-Harada disease is an inflammatory disorder, which presents with intraocular, auditory and central nervous system involvement. It has two distinct courses: acute onset and chronic recurrence, whose differential diagnoses are, respectively, diseases of the optic neuromyelitis spectrum, and chronic meningitis. The diagnostic criteria developed by the international disease committee in 2001 classify patients into: probable disease (ocular findings only), incomplete (ocular plus cutaneous system or neurological manifestations) and complete (when the three forms occur together). Methods: Report the case of a patient seen at the emergency room of Santa Casa de São Paulo, diagnosed with Vogt-Koyanagi-Harada disease. Case report: 43-year-old woman, reporting occipital headache, with irradiation to the retro-orbital region, progressed to sudden bilateral amaurosis, in addition to conjunctival hyperemia. Neurological physical examination presented bilateral visual acuity (Snellen)> 20/200, poorly delimited optical discs. Uveitis and scleritis were also found. Brain and orbit MRI showed: bilateral retinal detachment, with small subretinal collections; regular thickening and impregnation of the choroid; tenuous episcleral impregnation; alteration of the sign of the inner ears, more evident in the cochleae; tenuous linear leptomeningeal impregnation at the level of the cerebellobulbar cistern. Liquor: 21 cells (99% lymphocytes), 31 proteins and 47 glucose, negative culture for bacteria. We proceeded to infusion 1000mg of methylprednisolone for 3 days, with daily use, subsequently, of prednisone 60mg, with significant improvement of the condition. Conclusions: It is a rare disease, but it must be recognized by every neurologist, since it is treatable and can leave serious visual sequelae.