著者

高井 良樹 三須 建郎 藤原 一男 青木 正志

出版者

日本神経治療学会

雑誌

神経治療学 (ISSN:09168443)

巻号頁・発行日

vol.39, no.3, pp.282-288, 2022 (Released:2022-11-22)

参考文献数

51

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MOG (Myelin oligodendrocyte glycoprotein) is a myelin protein expressed exclusively in the central nervous system (CNS). MOG has long been studied as a target antigen for autoimmune inflammatory demyelinating diseases of the CNS because of its distribution in the outermost layer of the myelin sheath and its structural features as a member of the immunoglobulin superfamily. Recently, the detection of conformation–sensitive MOG antibodies has led to the establishment of the clinical concept of MOG–antibody associated disease (MOGAD) as an independent autoimmune demyelinating disease. The clinical phenotypes include acute/multiphasic disseminated encephalomyelitis, optic neuritis, myelitis and brain stem and cerebral cortical encphalitis, whereas MOG antibodies are rarely detected in typical multiple sclerosis.The treatment of MOGAD is divided into acute and chronic phases. The acute treatment is mainly provided by high dose methylprednisolone. The response to the treatment is generally good, and most cases recover without severe sequelae. On the other hand, there is still no established treatment to relapse prevention. The currently accepted approach is to treat the acute phase of the disease followed by 3–6 months of oral steroid therapy, reassessment of MOG antibodies, and continued maintenance treatment in patients with persistent positive MOG antibodies. For patients who become negative for MOG antibodies, it may be appropriate to discontinue maintenance therapy and follow–up. However, the pathogenesis of MOGAD is complex, with variability in response to B–cell removal therapy between cases. We hoped that the pathophysiology of MOGAD will be further elucidated in the future.

著者

大城 あずさ 仲村 貞郎 玉城 邦人 藤原 一男

出版者

一般社団法人 日本小児神経学会

雑誌

脳と発達 (ISSN:00290831)

巻号頁・発行日

vol.48, no.3, pp.199-203, 2016

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症例は10歳男児. 3週間続く弛張熱, 虫垂炎罹患の後, 両眼の高度視力低下, 歩行障害が出現し, MRIで両側視神経・視交叉・小脳・皮質下白質・脊髄にT2高信号病変を認めた. 視神経脊髄炎 (neuromyelitis optica ; NMO) あるいはNMO spectrum disorders (NMOSD) と考え, ステロイドパルス療法を2クール施行した. 歩行障害は改善したが中心視力の改善が乏しいため, 血漿交換療法を追加し, 視力は両側1.0に改善した. 血清抗aquaporin (AQP) 4抗体は陰性で, 抗myelin oligodendrocyte glycoprotein (MOG) 抗体が1,024倍であった. 急性期以後はprednisoloneおよびazathioprineの内服を行ったが, 血清抗MOG抗体は経時的に低下し, 治療漸減にて症状再燃を認めないため発症11カ月で治療を中止した. 近年, 抗AQP4抗体陰性のNMO/NMOSDの中で, 抗MOG抗体陽性例の報告がみられる. 抗AQP4抗体陽性例と異なりステロイドパルス療法のみで軽快する例が多いが, 本例のようにステロイドの効果が不十分で血漿交換が有効な症例もある. 今後さらなる症例蓄積による検討が必要である.