Neuro-Behçet's Syndrome Developing During Follow-Up for Behçet Uveitis: A Tertiary Uveitis Center Experience

To evaluate the clinical, neurological, and neuropsychiatric characteristics, subtype distribution, and multidisciplinary management in patients developing Neuro-Behçet's syndrome (NBS) during follow-up for Behçet uveitis (BU).

This retrospective study included 12 patients with panuveitis who developed NBS during follow-up for uveitis. Demographic data, ophthalmic findings, neurological and neuropsychiatric manifestations, neuroimaging results, cerebrospinal fluid (CSF) analyses, and treatments were reviewed. Neurological involvement was classified into parenchymal, vascular, neuropsychiatric, and peripheral types.

Among 300 consecutive patients with BU followed in our tertiary uveitis clinic, 12 patients (4%) developed NBS during follow-up. Parenchymal involvement was the most frequent subtype (50%), primarily affecting the brainstem, while neuropsychiatric symptoms occurred in 41.7% of patients. Headache and behavioral changes often preceded neurological deficits, and some patients showed subclinical imaging findings. Neuropsychiatric cases demonstrated higher pathergy positivity and skin lesions, whereas genital ulcers were more common in parenchymal and mixed involvement. Notably, 41.7% of patients had active uveitis at the time of NBS onset, suggesting that neurological involvement can occur independently of ocular inflammatory activity. Management required escalation of systemic therapy, including pulse steroids and biologics. At final follow-up, visual acuity remained stable (p > 0.05) with no permanent neurological sequelae.

In this uveitis cohort, NBS developed in 4% of patients with BU. Because neurological involvement may occur subclinically or independently of ocular activity, clinicians should maintain a high index of suspicion for neurological and neuropsychiatric manifestations. A prompt multidisciplinary approach and timely treatment escalation are essential to prevent permanent neurological damage in this patient population.