Table 1.
Population-based prevalence and incidence studies of NMOSD.
| Population-based study | Geographical location | Prevalence of NMOSD (per 100,000 population), as according to racial groups | Incidence (per million population) | AQP4-ab testing methods | AQP4-ab positivity | Female-to-male ratio | |||
|---|---|---|---|---|---|---|---|---|---|
| Whites/Caucasians | Blacks | East Asians | Other Asians/Other Races | ||||||
| Cabrera-Gomez et al. (2009) (24) | Cuba | 0.43 | 0.80 | 0.53 | Not tested | Not tested | 7.3:1 | ||
| Asgari et al. (2011) (25) (re-analyzed 2019) (27) | South Denmark | 1.68* | 1.5 | CBA | 62% | 5.3:1 | |||
| Cossburn et al. (2012) (28) | South East Wales | 1.96 | NR | NR | 71% | 6:1 | |||
| Jacob et al. (2013) (29) | Merseyside, England | 0.66* | 1.8* | 0.8 | Oxford CBA | 88% | 3:1 | ||
| Aboul-Enein et al. (2013) (30) | Austria | 0.77 | 0.54 | Innsbruck CBA | 100% | 7:1 | |||
| Pandit and Kundapur (2014) (31) | Mangalore, India | South Indians: 2.6 (0.72 if using 2015 IPND criteria) |
NR | NR | 27% | 1.2:1 | |||
| Etemadifar et al. (2014) (32) | Isfahan, Iran | 1.9 | NR | NR | 66% | 2.3:1 | |||
| Kashipazha et al. (2015) (33) | Khuzestan, Iran | 1.1 | NR | NR | 54% | 7.5:1 | |||
| Flanagan et al. (2016) (34) | Olmsted county, USA | 4.0 | 13.0 | 0.7 | Mayo CBA | 83% | 5:1 | ||
| French Martinique Island | 6.1 (single case, AQP4-ab negative) |
11.5 | 7.3 | Mayo CBA | 79% | 8.8:1 | |||
| van Pelt et al. (2016) (35) | Netherlands | — | 0.9 | CBA | NA | 4.9:1 | |||
| Houzen et al. (2017) (36) | Tokachi, Hokkaido, Japan | Japanese: 4.1 | NR | Sendai CBA | 79% | 6:1 | |||
| Eskandarieh et al. (2017) (37) | Tehran, Iran | 0.86 | NR | ELISA | 47% | 5.1:1 | |||
| Sepúlveda et al. (2018) (38) | Catalonia | 0.89 | 0.63 | Mainly CBA (96%) | 73% | 3.1:1 | |||
| Hor et al. (2018) (39) | Penang Island, Malaysia | Chinese: 3.31 | Malays: 0.80 (revised) |
NR | Euroimmun CBA | 100% | 14:1 | ||
| Miyamoto et al. (2018) (40) | Japan (nationwide estimate) | Japanese: 3.42 | NR | NA | NA | 6.4:1 | |||
| Holroyd et al. (2018) (41) | Abu Dhabi, UAE | Arabs: 1.09 | 1.16 | NR | 83% | All females | |||
| Papp et al. (2018) (42) | Denmark | 1.09* | 0.70 | Various, incl. CBA | 70% | 4.5:1 | |||
| Jonsson et al. (2019) (43) | Sweden | 1.04 | 0.79 | Immunoblot and CBA | NR | 2.8:1 | |||
| Kim et al. (2019) (44) | South Korea | Koreans: 2.56 | 7.3 | CBA | NA | 2.37:1 | |||
| Papp et al. (2020) (45) | Hungary | Hungarians: 1.91* | 1.32 | CBA | 83% | 8.8:1 | |||
| Bukhari et al. | Australia and | 0.55 | 1.84 | Asians: 1.57 | 0.37 | IF tissue | >90% | 6:1 | |
| (PACTRIMS | New Zealand | Māoris: 1.50 | assay, | ||||||
| 2019) (46) (updated from 2017 study) (47) | Australian Aborigines: 0.38 | some also ELISA and CBAs | |||||||
| Lee et al. (2020) (48) | South Korea | Koreans: 3.56 | 4.1–6.5 | NA | NA | 4.7:1 | |||
*
Only consider adult population. (As AQP4-antibody-positive NMOSD is rare in children, thus, if full population is considered, the prevalence will be slightly lower).
NMOSD, neuromyelitis optica spectrum disorder; AQP4-ab, aquaporin 4-antibody; CBA, cell-based assay; IF, immunofluorescence; NA, not applicable; NR, not reported.