Treatment of NMOSD When Both AQP4-IgG and MOG-IgG Test Negative in Serum
This protocol covers neuromyelitis optica spectrum disorder (NMOSD) in patients whose serology returns negative for both aquaporin-4 immunoglobulin G (AQP4-IgG) and myelin-oligodendrocyte-glycoprotein immunoglobulin G (MOG-IgG) — a presentation known as double-negative NMOSD. The double-negative serostatus defines a specific subgroup and directly shapes long-term management decisions.
Clinical scenario: NMOSD diagnosis confirmed; serum testing negative for AQP4-IgG and negative for MOG-IgG. This double-negative profile distinguishes the patient from the more common AQP4-IgG-positive and MOG-IgG-positive subgroups and informs a tailored treatment strategy.
Treatment approach — partial overview
Long-term immunotherapy in double-negative NMOSD is initiated after a second attack or following a severe first attack. The first-line strategy centres on immunosuppressive treatment, with the specific agent chosen according to individual patient characteristics. The full protocol details which therapies are indicated, the decision criteria, and the complete management algorithm.
References
DOI: 10.1007/s00415-024-12288-2
- Patients with NMOSD who are negative for both AQP4-IgG and MOG-IgG in serum are described as "double-negative" throughout the manuscript.
- Long-term immunotherapy in double-negative NMOSD should be initiated after a second attack or after a severe first attack.
- First-line treatments in double-negative NMOSD are classical immunosuppressive therapies or rituximab, depending on the patient's characteristics.
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