特集 神経疾患治療の進歩2023
脱髄疾患（中枢神経系）の治療の進歩
キーワード:
inebilizumab,
eculizumab,
satralizumab,
ravulizumab,
International MOGAD Panel proposed criteria
2024 年 41 巻 5 号 p. 725-729
詳細
2024 年 41 巻 5 号 p. 725-729
Recent advances and new knowledge in neuromyelitis optica spectrum disorder (NMOSD) and myelin oligodendrocyte glycoprotein antibody–associated disease (MOGAD) were reviewed.
In NMOSD, subgroup analyses of the N–MOmentum study in Asian participants reported similar efficacy and safety of inebilizumab between the Asian and non–Asian subgroups. An interim analysis of the post–marketing surveillance of eculizumab in Japan provided real–world evidence of the long–term safety and efficacy of eculizumab in patients with aquaporin–4 antibody (AQP4 Ab)–positive NMOSD, consistent with the results of the PREVENT study. Analyses based on the SAkuraSky and SAkuraStar studies, including the double–blind and open–label extension periods, demonstrated the long–term efficacy of satralizumab in AQP4 Ab–positive NMOSD. CHAMPION–NMOSD (NCT04201262), a Phase 3, open–label, externally controlled interventional study, reported on the efficacy and safety of ravulizumab in adult patients with AQP4 Ab–positive NMOSD.
Diagnostic criteria for MOGAD have been proposed by an international panel. In these criteria, diagnosis of MOGAD is required to fulfill 1) One or more core clinical demyelinating events, including optic neuritis, myelitis, acute disseminated encephalomyelitis (ADEM), cerebral monofocal or polyfocal deficits, brainstem or cerebellar deficits, and cerebral cortical encephalitis often with seizures 2) Positive MOG IgG test 3) Supporting clinical or MRI features including optic neuritis, myelitis, and brain, brainstem, or cerebral syndrome 4) Exclusion of better diagnoses including multiple sclerosis. The nationwide survey in Japan reported the estimated prevalence and incidence (1.34/100,000 and 0.39/100,000, respectively), the predominance of ADEM in younger patients and that of brainstem encephalitis, encephalitis, and myelitis in older patients at onset, and the high efficacy of immunotherapy at both onset and relapse. These findings were similar to those reported in other countries. Another study reported the association of early immunotherapy with longer corticosteroid treatment and lower risk of relapse in pediatric MOGAD.
