Figure 1
Diagram of literature sorting to identify articles that fit the search criteria.
Table 1
Prevalence Studies on Primary Adult-onset CD
| First Author, Year | Study Design | Source of Cases | Type of Dystonia Included | Geographic Region | Population Size | Age of Study Population | Prevalence per 1,000,000 | F:M ratio | Age of onset (mean ± SD, years) |
|---|---|---|---|---|---|---|---|---|---|
| Li 198519 | Door-to- door survey | General population | Focal/segmental dystonia | China | 63,195 | All ages | 30, age-adjusted to the 1960 U.S. population: 30 | NR | NR |
| Nutt 198820 | Record-linkage system | Neurological and non-neurological services | Focal/segmental dystonia | U.S. | 56,433 | All ages | 89 | 4.5:1 | 45 |
| Kandil 199421 | Door-to- door survey | General population | Focal/segmental dystonia | Egypt | 42,000 | All ages | 100 | 0.3:1 | 34.6 ± 4.9 |
| Nakashima 199522 | Service-based | Neurological services | Focal dystonia | Japan | 244,935 | All ages | 28.5 | 1.3:1 | NR |
| ESDE 200023 | Service-based | Neurological services | Focal/segmental dystonia | 8 European countries | 5,792,937 | >20 years | 57 | 1.3:1 | NR |
| Castelon Konkiewitz 200224 | Service-based | Neurological services | Focal/segmental dystonia | Germany | 1,322,883 | All ages | 54 | 1.3:1 | 41.6 ± 14.1 |
| Matsumoto 200325 | Service-based | Neurological services | Focal/segmental dystonia | Japan | 1,459,130 | All ages | 23 | 0.5:1 | 42.7 ± 16.7 F: 47.0 ± 18.7 M: 40.3 ± 15.3 |
| Pekmezovic 200326 | Service-based | Neurological and non-neurological services | Focal/segmental dystonia | Yugoslavia | 1,602,226 | ≥20 years | 59 | 1.7:1 | 43.3 ± 12.1 F: 46.4 ± 10.4 M: 37.4 ± 13.0 |
| Le 200327 | Service-based | Neurological and non-neurological services | Focal/segmental dystonia | Norway | 508,726 | All ages | 130 | 1.9:1 | 41.2 |
| Butler 200428 | Record-linkage system | Neurological and non-neurological services | Focal/segmental dystonia | England | 2,605,100 | All ages | 183.1 | 2.1:1 | 42.1 |
| Asgeirsson 200629 | Service-based | Neurological and non-neurological services | Focal/segmental dystonia | Iceland | 288,201 | All ages | 115 | 2.3:1 | 41.7 ± 14.4 |
| Fukuda 200630 | Service-based | Neurological and non-neurological services | Focal dystonia | Japan | 247,973 | All ages | 20 | 0.7:1 | 36.6 ± 8.2 F: 36.0 ± 15.6 M: 36.0 ± 3.6 |
| Sugawara 200631 | Service-based | Neurological and non-neurological services | Focal/segmental dystonia | Japan | 1,166,967 | All ages | 28 | NR | NR |
| Jankovic 20071 | Online questionnaire | General population | Focal/segmental dystonia | U.S. | 60,062 | >18 years | 4,100 Census weighted: 3,900 | NR | NR |
| Das 200732 | Door-to- door survey | General population | Focal dystonia | India | 52,377 | All ages | 38.1, age-standardized to the world population: 39.6 | 1:1 | 49 |
| Papantonio 200933 | Service-based | Neurological and non-neurological services | Focal/segmental dystonia | Italy | 541,653 | >17 years | 44.3, age- and sex-adjusted to the 2001 Italian population: 44.8 | 1.4:1 | NR |