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視神経脊髄炎

著者: 毛塚剛司 毛塚眼科医院

監修: 沖波聡 倉敷中央病院眼科

著者校正/監修レビュー済:2020/04/22
参考ガイドライン:
  1. 日本神経学会多発性硬化症治療ガイドライン委員会編:多発性硬化症治療ガイドライン2017
  1. 日本神経眼科学会抗アクアポリン4抗体陽性視神経炎診療ガイドライン2014
患者向け説明資料

概要・推奨   

  1. 視神経炎の急性期治療において、まずステロイド大量点滴療法が行われるが、ステロイド治療に抵抗性の場合は免疫グロブリン(IVIg)療法が推奨される(推奨度2、乾燥スルホ化人免疫グロブリンとして保険収載された)。原則として、免疫グロブリン製剤は血中抗アクアポリン4抗体陽性例で投与されるが、他の免疫制御療法で治療改善が認められない視神経炎に対して投与を検討する。
薬剤監修について:
オーダー内の薬剤用量は日本医科大学付属病院 薬剤部 部長 伊勢雄也 以下、林太祐、渡邉裕次、井ノ口岳洋、梅田将光による疑義照会のプロセスを実施、疑義照会の対象については著者の方による再確認を実施しております。
※薬剤中分類、用法、同効薬、診療報酬は、エルゼビアが独自に作成した薬剤情報であり、
著者により作成された情報ではありません。
尚、用法は添付文書より、同効薬は、薬剤師監修のもとで作成しております。
※薬剤情報の(適外/適内/⽤量内/⽤量外/㊜)等の表記は、エルゼビアジャパン編集部によって記載日時にレセプトチェックソフトなどで確認し作成しております。ただし、これらの記載は、実際の保険適用の査定において保険適用及び保険適用外と判断されることを保証するものではありません。また、検査薬、輸液、血液製剤、全身麻酔薬、抗癌剤等の薬剤は保険適用の記載の一部を割愛させていただいています。
(詳細はこちらを参照)
著者のCOI(Conflicts of Interest)開示:
毛塚剛司 : 特に申告事項無し[2021年]
監修:沖波聡 : 特に申告事項無し[2021年]

改訂のポイント:
  1. 視神経炎の急性期治療に対する新たな治療薬が保険収載となった。

病態・疫学・診察

疾患情報(疫学・病態)  
  1. 視神経脊髄炎(Neuromyelitis optica: NMO)は、長大な範囲にわたる視神経障害および同じく3椎体以上の範囲にわたる脊髄炎が併発した神経炎症性疾患である。視神経脊髄炎はDevic病とも呼ばれる。視神経障害は視神経のみに留まらず、視交叉や視索にまで及ぶことも多く、非可逆的な視力障害、視野障害などの視機能異常を来す。脊髄病変も視神経病変と同様、非可逆的な経過をたどることが多い。
  1. NMOは長らく多発性硬化症(multiple sclerosis: MS)の視神経脊髄型との鑑別が難しく、議論されてきた。2004年にLennonらによりNMOに特異的なNMO-IgGが発見され、そのIgGはアストロサイト上のアクアポリン4(aquapolin 4: AQP4)に結合し、補体を介してアストロサイトを傷害することが判明した[1][2]
  1. NMOでは、血中の抗AQP4抗体が陽性となるばかりではなく、脊髄障害に伴い、髄液中のアストロサイトの器質であるグリア細胞線維性酸性タンパク質(glial fibllary acid protein: GFAP)が脱落し、髄液中GFAPが陽性となる[3]。最近、視神経炎と脊髄炎を来す典型的なNMOではないが、再発性で長大な視神経傷害のみに留まる症例や脊髄炎のみに留まる症例も報告され始め、NMO関連疾患(NMO spectrum disorder: NMOSD)と分類されるようになった[4]
  1. 血清中抗AQP4抗体陽性NMOSDは、視神経炎全体の10%程度である。NMOSDの中には抗AQP4抗体陰性例も存在し、アストロサイトを標的細胞としない抗ミエリンオリゴデンドロサイトグリコプロテイン(Myelin Oligodendrocyte glycoprotein: MOG)抗体陽性例が最近注目されている[5][6]。抗MOG抗体陽性例は、特に視神経障害を来しやすく、再発しやすいことが知られている[7]
 
EBMに基づいた情報
  1. NMOSDにおいて、NMO-IgGが陽性の場合には再発が多い:エビデンスランクO, J, G
  1. 解説:視神経炎ないし脊髄炎を来している患者で、血清中NMO-IgGが陽性の場合は再発しやすい。脊髄炎では、エビデンスレベルIVa(分析学的研究、コホート研究)、視神経炎ではエビデンスレベルIVb(分析疫学的研究:症例対照研究、横断研究)である。
  1. 多発性硬化症(MS)に対するステロイドパルス療法:エビデンスランクO, J, G
  1. 解説:NMOやNMOSDの急性増悪は重症であることが多く、失明につながる視機能障害や四肢麻痺や対麻痺、呼吸障害などの脊髄・脳幹障害に至ることがある。このため、NMOやNMOSDの急性期にはステロイドパルス療法が第一選択と言われているが、MSに対するエビデンスレベルはIVaからV(記述的研究:症例報告など)とあまり高くない。この研究では、MSとNMOを混同させている可能性があり、NMOやNMOSDでも効果的である可能性が高い。
  1. NMOに対する単純血漿交換療法:エビデンスランクC, J, G
  1. 解説:ステロイドパルス療法が無効例における血液浄化療法であるが、単純血漿交換療法のみでエビデンスレベルIVb(分析疫学的研究:症例対照研究、横断研究)に留まる。
  1. NMOに対する免疫グロブリン大量静注療法:エビデンスランクR
  1. 解説:NMOSDの一分症である急性期視神経炎(特に抗アクアポリン4抗体陽性視神経炎)に対する免疫グロブリン大量静注療法が、2019年に保険収載された。
 
  1. NMOに対する後療法としての低濃度(10mg/日)プレドニゾロン療法:エビデンスランクO, J, G
  1. 解説:低濃度(10mg/日)プレドニゾロンの後療法は寛解期の再発予防に有効であるという報告は多いが、エビデンスレベルIVb~Vに留まる。ただし、プレドニゾロン10mg/日を長期間内服すると、ステロイド薬の合併症が必発なので、その管理には十分注意する必要がある。
  1. NMOに対する後療法としてのアザチオプリン:エビデンスランクO, J, G
  1. 解説:我が国ではNMOにおけるアザチオプリンの投与量は50~100mg/日が多く、エビデンスレベルIVbである。アザチオプリンは、プレドニゾロンと併用していることが多く、抗AQP4抗体も低下させ、再発を予防することができる(エビデンスレベルIVb)。
問診・診察のポイント  
  1. NMOは、重篤な視神経炎に脊髄炎が併発した典型例NMOだけではなく、再発性で長大な範囲に及ぶ視神経障害のみのNMOSD、3椎体以上にわたる脊髄炎のみのNMOSDも多い。このような重症な症例では必ず血清中の抗AQP4抗体を測定して陽性判定を確認しなければならない。

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文献 

著者: Vanda A Lennon, Dean M Wingerchuk, Thomas J Kryzer, Sean J Pittock, Claudia F Lucchinetti, Kazuo Fujihara, Ichiro Nakashima, Brian G Weinshenker
雑誌名: Lancet. 2004 Dec 11-17;364(9451):2106-12. doi: 10.1016/S0140-6736(04)17551-X.
Abstract/Text BACKGROUND: Neuromyelitis optica is an inflammatory demyelinating disease with generally poor prognosis that selectively targets optic nerves and spinal cord. It is commonly misdiagnosed as multiple sclerosis. Neither disease has a distinguishing biomarker, but optimum treatments differ. The relation of neuromyelitis optica to optic-spinal multiple sclerosis in Asia is uncertain. We assessed the capacity of a putative marker for neuromyelitis optica (NMO-IgG) to distinguish neuromyelitis optica and related disorders from multiple sclerosis.
METHODS: Indirect immunofluorescence with a composite substrate of mouse tissues identified a distinctive NMO-IgG staining pattern, which we characterised further by dual immunostaining. We tested masked serum samples from 102 North American patients with neuromyelitis optica or with syndromes that suggest high risk of the disorder, and 12 Japanese patients with optic-spinal multiple sclerosis. Control patients had multiple sclerosis, other myelopathies, optic neuropathies, and miscellaneous disorders. We also established clinical diagnoses for 14 patients incidentally shown to have NMO-IgG among 85000 tested for suspected paraneoplastic autoimmunity.
FINDINGS: NMO-IgG outlines CNS microvessels, pia, subpia, and Virchow-Robin space. It partly colocalises with laminin. Sensitivity and specificity were 73% (95% CI 60-86) and 91% (79-100) for neuromyelitis optica and 58% (30-86) and 100% (66-100) for optic-spinal multiple sclerosis. NMO-IgG was detected in half of patients with high-risk syndromes. Of 14 seropositive cases identified incidentally, 12 had neuromyelitis optica or a high-risk syndrome for the disease.
INTERPRETATION: NMO-IgG is a specific marker autoantibody of neuromyelitis optica and binds at or near the blood-brain barrier. It distinguishes neuromyelitis optica from multiple sclerosis. Asian optic-spinal multiple sclerosis seems to be the same as neuromyelitis optica.

PMID 15589308  Lancet. 2004 Dec 11-17;364(9451):2106-12. doi: 10.1016/・・・
著者: Vanda A Lennon, Thomas J Kryzer, Sean J Pittock, A S Verkman, Shannon R Hinson
雑誌名: J Exp Med. 2005 Aug 15;202(4):473-7. doi: 10.1084/jem.20050304. Epub 2005 Aug 8.
Abstract/Text Neuromyelitis optica (NMO) is an inflammatory demyelinating disease that selectively affects optic nerves and spinal cord. It is considered a severe variant of multiple sclerosis (MS), and frequently is misdiagnosed as MS, but prognosis and optimal treatments differ. A serum immunoglobulin G autoantibody (NMO-IgG) serves as a specific marker for NMO. Here we show that NMO-IgG binds selectively to the aquaporin-4 water channel, a component of the dystroglycan protein complex located in astrocytic foot processes at the blood-brain barrier. NMO may represent the first example of a novel class of autoimmune channelopathy.

PMID 16087714  J Exp Med. 2005 Aug 15;202(4):473-7. doi: 10.1084/jem.2・・・
著者: T Misu, K Fujihara, A Kakita, H Konno, M Nakamura, S Watanabe, T Takahashi, I Nakashima, H Takahashi, Y Itoyama
雑誌名: Brain. 2007 May;130(Pt 5):1224-34. doi: 10.1093/brain/awm047. Epub 2007 Apr 2.
Abstract/Text Neuromyelitis optica (NMO) is an inflammatory and necrotizing disease clinically characterized by selective involvement of the optic nerves and spinal cord. There has been a long controversy as to whether NMO is a variant of multiple sclerosis (MS) or a distinct disease. Recently, an NMO-specific antibody (NMO-IgG) was found in the sera from patients with NMO, and its target antigen was identified as aquaporin 4 (AQP4) water channel protein, mainly expressed in astroglial foot processes. However, the pathogenetic role of the AQP4 in NMO remains unknown. We did an immunohistopathological study on the distribution of AQP4, glial fibrillary acidic protein (GFAP), myelin basic protein (MBP), activated complement C9neo and immunoglobulins in the spinal cord lesions and medulla oblongata of NMO (n = 12), MS (n = 6), brain and spinal infarction (n = 7) and normal control (n = 8). The most striking finding was that AQP4 immunoreactivity was lost in 60 out of a total of 67 acute and chronic NMO lesions (90%), but not in MS plaques. The extensive loss of AQP4 accompanied by decreased GFAP staining was evident, especially in the active perivascular lesions, where immunoglobulins and activated complements were deposited. Interestingly, in those NMO lesions, MBP-stained myelinated fibres were relatively preserved despite the loss of AQP4 and GFAP staining. The areas surrounding the lesions in NMO had enhanced expression of AQP4 and GFAP, which reflected reactive gliosis. In contrast, AQP4 immunoreactivity was well preserved and rather strongly stained in the demyelinating MS plaques, and infarcts were also stained for AQP4 from the very acute phase of necrosis to the chronic stage of astrogliosis. In normal controls, AQP4 was diffusely expressed in the entire tissue sections, but the staining in the spinal cord was stronger in the central grey matter than in the white matter. The present study demonstrated that the immunoreactivities of AQP4 and GFAP were consistently lost from the early stage of the lesions in NMO, notably in the perivascular regions with complement and immunoglobulin deposition. These features in NMO were distinct from those of MS and infarction as well as normal controls, and suggest that astrocytic impairment associated with the loss of AQP4 and humoral immunity may be important in the pathogenesis of NMO lesions.

PMID 17405762  Brain. 2007 May;130(Pt 5):1224-34. doi: 10.1093/brain/a・・・
著者: Dean M Wingerchuk, Vanda A Lennon, Claudia F Lucchinetti, Sean J Pittock, Brian G Weinshenker
雑誌名: Lancet Neurol. 2007 Sep;6(9):805-15. doi: 10.1016/S1474-4422(07)70216-8.
Abstract/Text Neuromyelitis optica (also known as Devic's disease) is an idiopathic, severe, demyelinating disease of the central nervous system that preferentially affects the optic nerve and spinal cord. Neuromyelitis optica has a worldwide distribution, poor prognosis, and has long been thought of as a variant of multiple sclerosis; however, clinical, laboratory, immunological, and pathological characteristics that distinguish it from multiple sclerosis are now recognised. The presence of a highly specific serum autoantibody marker (NMO-IgG) further differentiates neuromyelitis optica from multiple sclerosis and has helped to define a neuromyelitis optica spectrum of disorders. NMO-IgG reacts with the water channel aquaporin 4. Data suggest that autoantibodies to aquaporin 4 derived from peripheral B cells cause the activation of complement, inflammatory demyelination, and necrosis that is seen in neuromyelitis optica. The knowledge gained from further assessment of the exact role of NMO-IgG in the pathogenesis of neuromyelitis optica will provide a foundation for rational therapeutic trials for this rapidly disabling disease.

PMID 17706564  Lancet Neurol. 2007 Sep;6(9):805-15. doi: 10.1016/S1474・・・
著者: Takeshi Kezuka, Yoshihiko Usui, Naoyuki Yamakawa, Yoshimichi Matsunaga, Ryusaku Matsuda, Masayuki Masuda, Hiroya Utsumi, Keiko Tanaka, Hiroshi Goto
雑誌名: J Neuroophthalmol. 2012 Jun;32(2):107-10. doi: 10.1097/WNO.0b013e31823c9b6c.
Abstract/Text BACKGROUND: Damage to astrocytes by anti-aquaporin-4 antibody (AQP4-Ab), also known as NMO antibody, has been implicated as the cause of neuromyelitis optica. Myelin oligodendrocyte glycoprotein (MOG) is well known as the causative protein of multiple sclerosis (MS). MOG antigen is currently considered as a cause of optic neuritis (ON) associated with MS because immunization with MOG antigen derived from oligodendrocytes induces murine ON with myelitis. We investigated the relationship between NMO antibody (NMO-Ab) and anti-MOG antibody (MOG-Ab) and potential in patients with ON for recovery of vision.
METHODS: Thirty-three eyes of 23 patients with ON were studied. At presentation, serum NMO-Ab was measured by immunofluorescence using HEK 293 cells transfected with AQP4-GFP, and anti-MOG1-125 antibody was measured by enzyme-linked immunosorbent assay. MOG-Ab seropositivity was defined by comparing with MOG-Ab level obtained from 8 healthy normal subjects.
RESULTS: Eleven (47%) of 23 ON patients were NMO-Ab seropositive, while 8 (34%) of the 23 patients were MOG-Ab seropositive. Six (26%) of 23 patients were seropositive for both NMO-Ab and MOG-Ab. Ten (43%) of 23 patients were seronegative for both antibodies. Three (50%) of 6 eyes of patients seropositive for both antibodies did not respond to corticosteroid pulse therapy and plasmapheresis, and visual acuity remained unchanged. In the NMO-Ab/MOG-Ab group, visual acuity improved significantly (P < 0.0001). In the other 3 groups (NMO-Ab/MOG-Ab, NMO-Ab/MOG-Ab, and NMO-Ab/MOG-Ab), visual acuity did not change significantly (P = 0.53, 0.42, and 0.45, respectively).
CONCLUSION: NMO-Ab and MOG-Ab could be potential biomarkers to determine visual prognosis in patients with ON.

PMID 22157536  J Neuroophthalmol. 2012 Jun;32(2):107-10. doi: 10.1097/・・・
著者: Douglas Kazutoshi Sato, Dagoberto Callegaro, Marco Aurelio Lana-Peixoto, Patrick J Waters, Frederico M de Haidar Jorge, Toshiyuki Takahashi, Ichiro Nakashima, Samira Luisa Apostolos-Pereira, Natalia Talim, Renata Faria Simm, Angelina Maria Martins Lino, Tatsuro Misu, Maria Isabel Leite, Masashi Aoki, Kazuo Fujihara
雑誌名: Neurology. 2014 Feb 11;82(6):474-81. doi: 10.1212/WNL.0000000000000101. Epub 2014 Jan 10.
Abstract/Text OBJECTIVE: To evaluate clinical features among patients with neuromyelitis optica spectrum disorders (NMOSD) who have myelin oligodendrocyte glycoprotein (MOG) antibodies, aquaporin-4 (AQP4) antibodies, or seronegativity for both antibodies.
METHODS: Sera from patients diagnosed with NMOSD in 1 of 3 centers (2 sites in Brazil and 1 site in Japan) were tested for MOG and AQP4 antibodies using cell-based assays with live transfected cells.
RESULTS: Among the 215 patients with NMOSD, 7.4% (16/215) were positive for MOG antibodies and 64.7% (139/215) were positive for AQP4 antibodies. No patients were positive for both antibodies. Patients with MOG antibodies represented 21.1% (16/76) of the patients negative for AQP4 antibodies. Compared with patients with AQP4 antibodies or patients who were seronegative, patients with MOG antibodies were more frequently male, had a more restricted phenotype (optic nerve more than spinal cord), more frequently had bilateral simultaneous optic neuritis, more often had a single attack, had spinal cord lesions distributed in the lower portion of the spinal cord, and usually demonstrated better functional recovery after an attack.
CONCLUSIONS: Patients with NMOSD with MOG antibodies have distinct clinical features, fewer attacks, and better recovery than patients with AQP4 antibodies or patients seronegative for both antibodies.

PMID 24415568  Neurology. 2014 Feb 11;82(6):474-81. doi: 10.1212/WNL.0・・・
著者: Ryusaku Matsuda, Takeshi Kezuka, Akihiko Umazume, Yoko Okunuki, Hiroshi Goto, Keiko Tanaka
雑誌名: Neuroophthalmology. 2015 Oct;39(5):213-219. doi: 10.3109/01658107.2015.1072726. Epub 2015 Aug 25.
Abstract/Text We have studied the clinical picture of anti-aquaporin antibody (AQP4-Ab)- and anti-myelin oligodendrocyte glycoprotein antibody (MOG-Ab)-positive optic neuritis. However, optic neuritis associated with MOG-Abs has not been elucidated using new methods such as cell-based assay. Hence, we conducted a comprehensive investigation on its clinical profile. Serum samples from 70 patients (17 males and 53 females, mean age 43.1 years) with optic neuritis were tested for MOG-Abs by cell-based assay. In MOG-Ab seropositive patients, the disease type, recurrence status, and visual function outcome were analysed. Among 70 patients, 18 were MOG-Ab seropositive. The 18 patients comprised 2 with chronic relapsing inflammatory optic neuropathy, 2 with AQP4-Ab seropositive optic neuritis (neuromyelitis optica), 12 with idiopathic optic neuritis, and 2 with optic neuritis associated with multiple sclerosis. Excluding two cases that were also AQP4-Ab seropositive, MOG-Ab seropositive cases had relatively favourable visual acuity outcome (although not significantly different from seronegative cases) but had significant residual visual field deficit (p = 0.0015). Furthermore, the number of relapses of optic neuritis per year was significantly greater in MOG-Ab seropositive cases than in seronegative cases (0.82 vs. 0.40; p = 0.0005). MOG-Abs may contribute to the heterogeneous clinical picture of optic neuritis, and although visual acuity outcome is favourable, there is a tendency of residual visual field deficit and a possibility of repeated relapses.

PMID 27928358  Neuroophthalmology. 2015 Oct;39(5):213-219. doi: 10.310・・・
著者: D M Wingerchuk, V A Lennon, S J Pittock, C F Lucchinetti, B G Weinshenker
雑誌名: Neurology. 2006 May 23;66(10):1485-9. doi: 10.1212/01.wnl.0000216139.44259.74.
Abstract/Text BACKGROUND: The authors previously proposed diagnostic criteria for neuromyelitis optica (NMO) that facilitate its distinction from prototypic multiple sclerosis (MS). However, some patients with otherwise typical NMO have additional symptoms not attributable to optic nerve or spinal cord inflammation or have MS-like brain MRI lesions. Furthermore, some patients are misclassified as NMO by the authors' earlier proposed criteria despite having a subsequent course indistinguishable from prototypic MS. A serum autoantibody marker, NMO-IgG, is highly specific for NMO. The authors propose revised NMO diagnostic criteria that incorporate NMO-IgG status.
METHODS: Using final clinical diagnosis (NMO or MS) as the reference standard, the authors calculated sensitivity and specificity for each criterion and various combinations using a sample of 96 patients with NMO and 33 with MS. The authors used likelihood ratios and logistic regression analysis to develop the most practical and informative diagnostic model.
RESULTS: Fourteen patients with NMO (14.6%) had extra-optic-spinal CNS symptoms. NMO-IgG seropositivity was 76% sensitive and 94% specific for NMO. The best diagnostic combination was 99% sensitive and 90% specific for NMO and consisted of at least two of three elements: longitudinally extensive cord lesion, onset brain MRI nondiagnostic for MS, or NMO-IgG seropositivity.
CONCLUSIONS: The authors propose revised diagnostic criteria for definite neuromyelitis optica (NMO) that require optic neuritis, myelitis, and at least two of three supportive criteria: MRI evidence of a contiguous spinal cord lesion 3 or more segments in length, onset brain MRI nondiagnostic for multiple sclerosis, or NMO-IgG seropositivity. CNS involvement beyond the optic nerves and spinal cord is compatible with NMO.

PMID 16717206  Neurology. 2006 May 23;66(10):1485-9. doi: 10.1212/01.w・・・
著者: Dean M Wingerchuk, Brenda Banwell, Jeffrey L Bennett, Philippe Cabre, William Carroll, Tanuja Chitnis, Jérôme de Seze, Kazuo Fujihara, Benjamin Greenberg, Anu Jacob, Sven Jarius, Marco Lana-Peixoto, Michael Levy, Jack H Simon, Silvia Tenembaum, Anthony L Traboulsee, Patrick Waters, Kay E Wellik, Brian G Weinshenker, International Panel for NMO Diagnosis
雑誌名: Neurology. 2015 Jul 14;85(2):177-89. doi: 10.1212/WNL.0000000000001729. Epub 2015 Jun 19.
Abstract/Text Neuromyelitis optica (NMO) is an inflammatory CNS syndrome distinct from multiple sclerosis (MS) that is associated with serum aquaporin-4 immunoglobulin G antibodies (AQP4-IgG). Prior NMO diagnostic criteria required optic nerve and spinal cord involvement but more restricted or more extensive CNS involvement may occur. The International Panel for NMO Diagnosis (IPND) was convened to develop revised diagnostic criteria using systematic literature reviews and electronic surveys to facilitate consensus. The new nomenclature defines the unifying term NMO spectrum disorders (NMOSD), which is stratified further by serologic testing (NMOSD with or without AQP4-IgG). The core clinical characteristics required for patients with NMOSD with AQP4-IgG include clinical syndromes or MRI findings related to optic nerve, spinal cord, area postrema, other brainstem, diencephalic, or cerebral presentations. More stringent clinical criteria, with additional neuroimaging findings, are required for diagnosis of NMOSD without AQP4-IgG or when serologic testing is unavailable. The IPND also proposed validation strategies and achieved consensus on pediatric NMOSD diagnosis and the concepts of monophasic NMOSD and opticospinal MS.

© 2015 American Academy of Neurology.
PMID 26092914  Neurology. 2015 Jul 14;85(2):177-89. doi: 10.1212/WNL.0・・・
著者: Hitoshi Ishikawa, Takeshi Kezuka, Keigo Shikishima, Akiko Yamagami, Miki Hiraoka, Hideki Chuman, Makoto Nakamura, Keika Hoshi, Toshiaki Goseki, Kimiyo Mashimo, Osamu Mimura, Takeshi Yoshitomi, Keiko Tanaka, Working Group on Diagnostic Criteria for Refractory Optic Neuritis Based on Neuroimmunological Perspective
雑誌名: Ophthalmology. 2019 Oct;126(10):1385-1398. doi: 10.1016/j.ophtha.2019.04.042. Epub 2019 May 6.
Abstract/Text PURPOSE: To elucidate the clinical and epidemiologic characteristics of optic neuritis in Japan.
DESIGN: Multicenter cross-sectional, observational cohort study.
PARTICIPANTS: A total of 531 cases of unilateral or bilateral noninfectious optic neuritis identified in 33 institutions nationwide in Japan.
METHODS: Serum samples from patients with optic neuritis were tested for anti-aquaporin-4 antibodies (AQP4-Abs) and anti-myelin oligodendrocyte glycoprotein antibodies (MOG-Abs) using a cell-based assay and were correlated with the clinical findings.
MAIN OUTCOME MEASURES: Antibody positivity, clinical and radiologic characteristics, and visual outcome.
RESULTS: Among 531 cases of optic neuritis, 12% were AQP4-Ab positive, 10% were MOG-Ab positive, 77% were negative for both antibodies (double-negative), and 1 case was positive for both antibodies. Pretreatment visual acuity (VA) worsened to more than a median 1.0 logarithm of the minimum angle of resolution (logMAR) in all groups. After steroid pulse therapy (combined with plasmapheresis in 32% of patients in AQP4-Ab-positive group), median VA improved to 0.4 logMAR in the AQP4-Ab-positive group, 0 logMAR in the MOG-Ab-positive group, and 0.1 logMAR in the double-negative group. The AQP4-Ab-positive group showed a high proportion of females, exhibited diverse visual field abnormalities, and demonstrated concurrent spinal cord lesions on magnetic resonance imaging (MRI) in 22% of the patients. In the MOG-Ab-positive group, although posttreatment visual outcome was good, the rates of optic disc swelling and pain with eye movement were significantly higher than those in the AQP4-Ab-positive and double-negative groups. However, most cases showed isolated optic neuritis lesions on MRI. In the double-negative group, 4% of the patients had multiple sclerosis. Multivariate logistic regression analysis of all participants identified age and presence of antibodies (MOG-Ab and AQP4-Ab) as significant factors affecting visual outcome.
CONCLUSIONS: The present large-scale cohort study revealed the clinicoepidemiologic features of noninfectious optic neuritis in Japan. Anti-aquaporin-4 antibody-positive optic neuritis has poor visual outcome. In contrast, MOG-Ab positive cases manifested severe clinical findings of optic neuritis before treatment, but few showed concurrent lesions in sites other than the optic nerve and generally showed good treatment response with favorable visual outcome. These findings indicate that autoantibody measurement is useful for prompt diagnosis and proper management of optic neuritis that tends to become refractory.

Copyright © 2019 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.
PMID 31196727  Ophthalmology. 2019 Oct;126(10):1385-1398. doi: 10.1016・・・

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