Table 1.
Prevalence and incidence of progressive supranuclear palsy
| Study | Year of investigation | Phenotype | Population denominator | Prevalence (per 100,000) |
Incidence (per 100,000) |
Methods | Region | ||
|---|---|---|---|---|---|---|---|---|---|
| Crude prevalence/sex-specific prevalence | Age- adjusted prevalence | Crude incidence/sex-specific incidence | Age-adjusted incidence | ||||||
| Golbe et al. [8] | 1982–1987 | PSP-RS | 799,022 | 1.39 (95% CI 0.8–2.4) | 7.00 | N/A | N/A | Survey of neurologists and chronic care facilities: mail or telephone | US, New Jersey |
| Men: 1.53, women: 1.23 | |||||||||
| Radhakrishnan et al. [93] | 1983–1986 | PSP-RS | 519,000 | N/A | N/A | 0.3 | - | Part of a survey of different neurological diseases through polyclinics, university hospitals, rehabilitation centers and one neurology center | Libya, Benghazi |
| Bower et al. [12] | 1976–1990 | PSP-RS | 1,424,474 | N/A | N/A | 1.1 | 5.3 | Medical records-linkage system of the Rochester Epidemiology Project | US, Minnesota, Olmsted County |
| Men:1.3 | Men:6.9 | ||||||||
| Women: 0.9 | Women: 4.1 | ||||||||
| Wermuth et al. [94] | 1993–1995 | PSP-RS | 43,709 | 4.6 | - | N/A | N/A | Data from pharmacies, hospitals, general practices, and nursing homes | Faroe Islands |
| Chiò et al. [95] | 1991 | PSP-RS | 61,830 | 3.2 | - | N/A | N/A | Medical records and pharmacies | Northwestern Italy |
| Schrag et al. [32] | 1994–1997 | PSP-RS | 121,608 | 4.9 (95% CI 1.0–10.7) | 6.4 (95% CI 2.3–10.6) | N/A | N/A | Computerized medical records of 15 general practices | UK, London |
| Nath et al. [36] | 1999 | PSP-RS | 59,236,500 | 0.3 (95% CI 0.3–0.4) | 0.3 (95% CI 0.2–0.3) | N/A | N/A | ‘Russian doll’ design | UK, national |
| 2,589,240 | 3.1 (95% CI 2.4–3.8) | 2.4 (95% CI 1.9–3.0) | National study: passive referral mechanisms | UK, regional | |||||
| Men: 2.4 (95% CI 1.3–3.3) | Regional study: collaborative network of neurologists and nonneurologists | ||||||||
| Women: 3.7 (95% CI 2.7–4.8) | |||||||||
| 259,998 | 6.5 (95% CI 3.4–9.7) | 5.0 (95% CI 2.5–7.5) | Community study: computerized records | UK, community | |||||
| Men: 6.2 (95% CI 1.9–10.5) | |||||||||
| Women: 6.9 (95% CI 2.4–11.4) | |||||||||
| Caparros-Lefebvre et al. [96] | 1996–2001 | PSP-RS | 422,000 | 14 | - | N/A | N/A | Patients with parkinsonism referred to the Department of Neurology of the French West Indies University Hospital | France, Guadeloupe |
| Kawashima et al. [18] | 1999–2002 | PSP-RS | 137,420 | 5.82 (95% CI: 1.78–9.86) | 5.03 | N/A | N/A | Medical records of the university hospital, general hospitals, and nursing homes | Japan, Yonago |
| Men: 9.14 (95% CI: 1.82–16.47) | Men: 7.92 | ||||||||
| Women: 2.75 (95% CI: 1.08–6.65) | Women: 2.27 | ||||||||
| Savica et al. [10] | 1991–2005 | PSP-RS | 1,424,474 | N/A | N/A | 0.9 | - | Diagnostic codes and the review of medical records | US, Minnesota, Olmsted County |
| Men: 1.1 | |||||||||
| Women: 0.6 | |||||||||
| Caparros-Lefebvre et al. [15] | 2005–2014 | PSP-RS, PSP-P, PSP-PAGF, PSP-FTD, PSP-AOS | 51,551 | N/A | N/A | The ratio of observed to expected PSP incidence 12.3 (95% CI 4.7–35.9) | - | Patients with parkinsonism in the Centre Hospitalier de Wattrelos | France, Wattrelos and Leers |
| Coyle-Gilchrist et al. [97] | 2013–2014 | FTD, PSP-RS, and CBS | 1,690,000 | 10.8 for FTLDassociated syndrome (95% CI 9.3–12.4) | - | 1.61 for FTLDassociated syndromes (95% CI 1.1–1.9) | - | The PiPPIN (Pick’s Disease and Progressive Supranuclear Palsy: Prevalence and Incidence) | UK, Cambridgeshire and Norfolk |
| Takigawa et al. [9] | 2009–2014 | PSP-RS, PSP-P, PSP-PAGF | 148,271 | 17.9 (95% CI 12.12–26.42) | 17.26 (95% CI 17.03–17.48) | N/A | N/A | Six surveys and medical records | Japan, Yonago |
| Men: 18.05 (95% CI 10.33–31.55) | Men: 18.14 (95% CI 17.81–18.48) | ||||||||
| Women: 17.76 (95% CI 10.38–30.39) | Women: 16.63 (95% CI 16.32–16.95) | ||||||||
| PSP-RS | 14.32 (95% CI 9.27–22.12) | 13.80 (95% CI 13.60–14.01) | |||||||
| Men: 10.53 (95% CI 5.10–21.74) | Men: 10.62 (95% CI 10.37–10.88) | ||||||||
| Women: 17.76 (95% CI 10.38–30.39) | Women: 16.63 (95% CI 16.32–16.95) | ||||||||
| Fluery et al. [11] | 2003–2012 | PSP-RS | 470,512 | 8.3 (95% CI 5.9–11.3) | 8.8 (95% CI 6.1–11.6) | 1.9 (95% CI 1.3–2.6) | 2.0 (95% CI 0.7–3.4) | Medical records from public hospitals, private neurologists and nursing homes | Switzerland, Geneva |
| Men: 9.7 (95% CI 6.1–14.6) | Men: 12.1 (95% CI 7.0–17.2) | Men: 2.4 (95% CI 1.5–3.7) | Men: 3.9 (95% CI 1.0–6.7) | ||||||
| Women: 7.0 (95% CI 4.1–11.2) | Women: 6.7 (95% CI 3.5–9.9) | Women: 1.4 (95% CI 0.7–2.3) | Women: 0.8 (95% CI 0.0–1.8) | ||||||
N/A: not available, CI: confidence interval, FTD: frontotemporal dementia, FTLD: frontotemporal lobar degeneration, PSP: progressive supranuclear palsy, PSP-RS: PSP-Richardson’s syndrome, PSP-P: PSP-parkinsonism, PSP-PAGF: PSP-pure akinesia with gait freezing, PSP-FTD: PSP-frontotemporal dementia, PSP-AOS: PSP-apraxia of speech, CBS: corticobasal syndrome, US: United States, UK: United Kingdom.