To highlight the importance of considering VKH syndrome in patients presenting with subacute headaches, vision changes, and signs of increased intracranial pressure, such as papilledema.

VKH syndrome is a rare, T-cell mediated autoimmune disorder targeting melanocyte-containing tissues, primarily affecting the eyes, central nervous system (CNS), inner ear, and skin. The neurological phase often manifests with aseptic meningitis, headaches, and cranial nerve symptoms.

A 47-year-old female presented after a one-month history of a pressure headache involving her entire head, blurry vision, and recent vision loss in the top right corner of her right eye. She had light sensitivity and bilateral facial numbness and tingling. Ophthalmology found swelling of optic discs bilaterally. MRI brain with and without contrast was unremarkable. However, an MRI of the orbits demonstrated nonspecific thickening and enhancement of the choroid bilaterally. Analysis of the CSF showed significant pleocytosis (195 nucleated cells) with lymphocytic predominance (91%) and elevated total protein (68 mg/dL). Based on the clinical presentation and investigative findings, the patient was diagnosed with VKH syndrome.

It is critical for neurologists to include VKH syndrome in the differential diagnosis for patients presenting with chronic headaches, visual disturbance, and facial paresthesia. This case demonstrates that patients with VKH may present initially with primarily neurological complaints, such as persistent headaches and facial tingling, potentially leading to initial misdiagnosis (e.g., sinusitis). Early recognition of signs like headaches, vision changes, and papilledema, combined with evidence of CNS inflammation (CSF pleocytosis and choroid enhancement), necessitates a prompt neuro-ophthalmic workup to confirm the diagnosis and initiate timely treatment. The diagnosis of VKH requires consideration of the constellation of neurological and ophthalmological findings.