Vogt-Koyanagi-Harada Disease Mimicking Idiopathic Intracranial Hypertension

Vogt-Koyanagi-Harada (VKH) disease is a multisystem autoimmune disorder characterized by an inflammatory response targeting melanocyte-associated antigens in tissues such as the uveal tract, inner ear, meninges, and the skin. The classic presentation is bilateral pan-uveitis (choroiditis and multifocal serous retinal detachment) in association with extra-ocular features such signs of meningeal irritation (headache, neck stiffens), auditory signs (tinnitus, hearing loss), and skin changes (poliosis, vitiligo). This is a case of an incomplete form of VKH presenting predominantly with bilateral disc edema. The patient was erroneously diagnosed as having idiopathic intracranial hypertension at first based on a high CSF opening pressure on lumbar puncture and normal MRI/MRV of the brain. However, atypical features in the case such as mild anterior uveitis and the persistence of headache and auditory signs led to further work up and to the diagnosis of incomplete VKH with bilateral disc edema.