期刊
BRAIN
卷 135, 期 -, 页码 1834-1849出版社
OXFORD UNIV PRESS
DOI: 10.1093/brain/aws109
关键词
neuromyelitis optica; NMO; NMO spectrum disorder; aquaporin-4; AQP4; long-term disability; prognosis; outcomes
资金
- NHS National Specialised Commissioning Group for Neuromyelitis Optica
- Asahi Kasei Medical Co.
- Ministry of Education, Science and Technology [19209032, 20390241, 22229008]
- Ministry of Health, Labor and Welfare (Neuroimmunological Disease Research Committee) of Japan
- Mitsubishi Chemical Medience Corporation
- Ministry of Education, Culture, Sports, Science & Technology in Japan
- Bayer Schering Pharma
- Biogen Idec Japan
- Asahi Kasei Kuraray Medical Co.
- Chemo-Sero-Therapeutic Research Institute
- Teva Pharmaceutical K.K.
- Mitsubishi Tanabe Pharma Corporation
- Teijin Pharma
- Ministry of Health, Labor and Welfare of Japan
- Grants-in-Aid for Scientific Research [24591247, 22229008] Funding Source: KAKEN
Neuromyelitis optica and neuromyelitis optica spectrum disorders have been recently associated with the disease-specific autoantibody aquaporin-4, thought to be pathogenic. Identifying this antibody has allowed the clinical phenotype to be broadened. It is clear that some patients with similar clinical features do not have this antibody and may have a different condition with different outcomes and prognosis. Previous clinical neuromyelitis optica and neuromyelitis optica spectrum disorder studies have included such patients. We investigated clinical outcomes and prognostic characteristics of 106 aquaporin-4 antibody-seropositive patients from the UK and Japan. We looked at predictors of disability outcomes, namely visual disability (permanent bilateral visual loss with visual acuity of < 6/36 in the best eye), motor disability (permanent inability to walk further than 100 m unaided), wheelchair dependence and mortality. Data were collected largely retrospectively through review of case records. After median disease duration of 75 months, 18% had developed permanent bilateral visual disability, 34% permanent motor disability, 23% had become wheelchair dependent and 9% had died. Age at disease onset appeared to be an important predictor of disability type. Young-onset patients in the UK, but not the Japanese cohort, commonly presenting with optic neuritis, had a high risk of visual disability while older patients in both cohorts had a high risk of motor disability, regardless of their onset symptom. Genetic factors also appeared important. The UK cohort seemed to have more severe disease than the Japanese cohort, with more severe onset attacks, a higher relapse frequency and greater disability at follow-up, despite earlier immunosuppression. Moreover, within the UK cohort, there were important differences between ethnic groups, with Afro-Caribbean patients having a younger age at disease onset, more brain and multifocal attacks and higher likelihood of visual disability than Caucasian patients. Thus, age at disease onset and genetic factors are both likely to be important in determining clinical outcomes in aquaporin-4 disease. This has important implications for interpreting clinical neuromyelitis optica and neuromyelitis optica spectrum disorder studies, since clinical features and outcomes appear not to be generic across populations and may need to be tailored to individual groups. These factors need to be explored further in future prospective neuromyelitis optica and neuromyelitis optica spectrum disorder studies.
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