Beyond the Brain-Vogt-Koyanagi-Harada Syndrome (VKH): A Rare Brain Eye and Ear (BEE) Syndrome Presenting initially as Aseptic Meningitis- a Neurologist Perspective.

Abstract

Background: The triad of central nervous system, hearing, and visual disturbances is an often encountered scenario. Vogt Koyanagi-Harada (VKH) is a rare syndrome affecting tissues with melanocytes and characterized by bilateral diffuse granulomatous uveitis, meningeal involvement, and hearing impairment. VKH is considered a rare cause of Brain Eye and Ear (BEE) syndrome.

Case: We describe a case of a 32-year-old healthy lady who was admitted to neurology with the initial impression of aseptic meningitis. She had subacute onset of headache and fever, associated with blurring of vision and painful eye movements. Visual acuity 20/250 of the right eye and 20/80 of the left eye. Intra-ocular pressure measured 12 for the right eye and 14 for the left eye, and extraocular muscle movements were full Slit lamp examination showed a quite conjunctiva and clear cornea; however, there was an anterior chamber reaction of 2+ cells. The fundus exam showed mild vitritis with hyperemic disc swelling of both eyes and exudative retinal detachment bilaterally. Macular optical coherence tomography (OCT) demonstrated the presence of vitritis, pockets of subretinal fluids with bacillary layer detachment, and choroidal thickening. She was treated with steroids and mycophenolate mofetil with an excellent outcome.

Conclusion: Early diagnosis has good outcomes and of crucial to prevent damage to the photoreceptors and subsequent poor visual outcomes. The presence of a distinctive expression in one BEE organ should prompt the appropriate investigations and multidisciplinary team involvement to avoid permanent vision loss.