Area, country | Period of incidence | Definition of NMOSD | New-onset NMOSD cases | Incidence (95% CI, per 100 000 person-years) | Mean age at onset, years | Female-to-male ratio | Latitude | Reference |
Olmsted County, USA* | 2003–2011 | 2006 criteria for NMO, AQP4-IgG seropositive IDD for NMOSD | 1 | 0.07 (0.00 to 0.21) | 37† | 9‡ | 44.1 | Flanagan et al 23 |
Martinique* | 2003–2011 | 2006 criteria for NMO, AQP4-IgG seropositive IDD for NMOSD | 27 | 0.73 (0.45 to 1.01) | 35† | 4.88‡ | 14.4 | Flanagan et al 23 |
Sweden | 2007–2013 | 2006 criteria for NMO, AQP4-IgG seropositive LETM or ON | 92 | 0.079 (0.055 to 0.103) | 41.5§ | 3.83‡ | 59.2 | Jonsson et al 6 |
Korea* | 2016 | 2006 criteria for 2010–2015, 2015 INPD criteria for 2016 | 387 | 0.73 (0.66 to 0.80) | 50–59¶ | 2.37** | 37.3 | Kim et al 7 |
The Netherlands | 2009–2015 | AQP4-IgG seropositive | 15 | 0.09 (CI was not provided in the article) | 47.6§ | 4.88‡ | 52.2 | Daniëlle van Pelt et al 14 |
China* | 2016–2018 | 2015 INPD criteria | 11 973 | 0.347 (0.34 to 0.353) in adults 0.075 (0.069 to 0.08) in children | 45–65¶ | 4.71§ | 20–46 | Tian et al 18 |
Hungary* | 2006–2015 | 2015 INPD criteria | 101 | 0.120 (0.098 to 0.146) | 39.5† | 7‡ | 47.3 | Papp et al 9 |
Malaysia†† | 2013–2017 | 2015 INPD criteria | 545 | 0.39 (0.35 to 0.47) | 33.4§ | 10‡ | 2.3 | Viswanathan and Wah16 |
Denmark | 2007–2013 | 2015 INPD criteria | 11 | 0.070 (0.046 to 0.102) | 35.5§ | 4.6‡ | 55.4 | Papp et al 10 |
Australia and New Zealand* | 2009–2012 | 2015 INPD criteria | 147 | 0.037 (0.035 to 0.039) | Female: 45–59¶ Male: 60–69¶ | 6‡ | 35.2 | Bukhari et al 11 |
Catalonia (Spain) | 2006–2016 | 2015 INPD criteria | 47 | 0.064 (0.045 to 0.082) | 42† | 3.1‡ | 41.6 | Sepulveda et al 4 |
*Age and sex adjusted.
†Median age of onset.
‡Number ratio.
§Mean age of onset.
¶Peak prevalence age.
**Incidence ratio.
††There may be some methodology and data issues in this study.12
AQP4-IgG, aquaporin-4 immunoglobulin G; IDD, inflammatory demyelinating disease; INPD, the International Panel for NMO Diagnosis; LETM, longitudinally extensive transverse myelitis; NMOSD, neuromyelitis optica spectrum disorder; ON, optic neuritis.