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Case History: Here we present a case of Neuro-Behcet's disease that responded well to complement inhibition. The case involves a 27-year-old woman with recurrent optic neuritis (ON) and transverse myelitis (TM) as well as one episode suspicious for an area postrema syndrome since 2006. She carried a diagnosis of seronegative neuromyelitis optica spectrum disorder (NMOSD), as MOG and aquaporin 4 antibodies (AQP4-IgG) have been repeatedly negative through her disease course. Her past medical history is positive for psoriasis, and only in the past year for ulcers in the mouth. Given the recent mouth ulcers and Middle Eastern ancestry, she was tested for the HLA-B51 gene and tested positive, consistent with a diagnosis of Neuro-Behcet's disease. Intermittent treatment with steroids, IVIG and PLEX for recurrent attacks led to complications such as line infections, gastric ulcers and avascular necrosis of the hip. She had breakthrough disease on Avonex, rituximab and mycophenolate. However, while using eculizumab since 2018 and more recently ravulizumab, inhibitors of the C5 terminal complement, she remained in remission. Treatment interruption due to drug access issues resulted in recurrence of optic neuritis.

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We conclude that complement inhibitors could be potentially effective in Neuro-Behcet's disease.