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Clinical features of neuromyelitis optica in a large Japanese cohort: comparison between phenotypes
  1. Akiko Nagaishi1,2,
  2. Mineo Takagi3,
  3. Atsushi Umemura4,
  4. Masami Tanaka4,
  5. Yoko Kitagawa1,
  6. Makoto Matsui1,
  7. Masatoyo Nishizawa5,
  8. Kenji Sakimura6,
  9. Keiko Tanaka1
  1. 1Department of Neurology, Kanazawa Medical University, Uchinadamachi, Kahokugun, Ishikawa, Japan
  2. 2Department of Neurology, Kawatana Medical Center, Nagasaki, Japan
  3. 3Department of Ophthalmology, Niigata University, Niigata, Japan
  4. 4Department of Neurology, Utano National Hospital, Kyoto, Japan
  5. 5Department of Neurology, Brain Research Institute, Niigata University, Niigata, Japan
  6. 6Department of Cellular and Neurobiology, Brain Research Institute, Niigata University, Niigata, Japan
  1. Correspondence to Professor Keiko Tanaka, Department of Neurology, Kanazawa Medical University, 11 Daigaku, Uchinadamachi, Kahokugun, Ishikawa, Japan; k-tana20{at}


Objective To analyse clinicoepidemiological features of neuromyelitis optica in a large cohort and to compare the differences between onset age, gender and clinical phenotypes.

Methods Antiaquaporin-4 antibody (AQP4-ab) levels were tested in 2366 serum samples of patients diagnosed as having central nervous system inflammatory demyelinating disorders by their referring physicians. AQP4-ab was measured by indirect immunofluorescence staining using human AQP4-transfected HEK 293 cells. A blinded analysis was performed and was combined with clinical information.

Results A total of 583 patients (91.4% women) were AQP4-ab-positive. The average onset age was 42.9±15.9 years. According to MRI studies, spinal-cord lesions were detected in 85.3% of the patients, longitudinally extensive transverse myelitis in 72.7% and cerebral lesions in 51.1%. Unilateral or bilateral blindness was observed in 16.2% of patients, 19.8% were associated with Sjögren syndrome, and 13.6% were associated with thyroid diseases. Myelin basic protein was detected in the cerebrospinal fluid of 57.5% patients. In addition, men presented with an older onset age, a greater number of brainstem MRI lesions and positive myelin basic protein in the cerebrospinal fluid. All child-onset patients (<15 years, n=9) presented with optic neuritis as the first symptom, while older-onset patients presented with myelitis. Twenty patients initially developed limited brain lesions, and seven of these patients did not develop optic or spinal lesions during the 1–5-year follow-up period.

Conclusions The clinical characteristics of AQP4-ab-positive patients were similar. However, optic neuritis was more common in paediatric patients, while myelitis was more common in older patients. A small number of patients exhibited only cerebral, brainstem, or cerebellar lesions during the initial several years and lower Extended Disability Status Scale scores.

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  • Funding This study was supported in part by Health and Labor Sciences Research Grants for Research on Psychiatry and Neurological Diseases and Mental Health (KT). Grant no H-021.

  • Competing interests None.

  • Patient consent Obtained.

  • Ethics approval Ethics approval was provided by the ethical committee of Kanazawa Medical University.

  • Provenance and peer review Not commissioned; externally peer reviewed.