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. 2021 Feb 10;16:77. doi: 10.1186/s13023-021-01699-3

Table 2.

Sex-, age- and period-specific estimates of prevalence and incidence of Huntington disease (HD) in Navarre, for the period 1991–2017

Number of HD cases Prevalence Incidence
Total Symptomatic Asymptomatic (n)a Total prevalent cases Point prevalence rate per 100,000 (95% CI) Total incident cases Annual incidence rate per 100,000 (95% CI)
Total 80 61 19 65 63 0.40 (0.31, 0.50)
Sex
Men 38 (47%) 26 12 (4) 15 (47%) b 4.68 (2.31, 7.05)b 29 (46%) 0.37 (0.25, 0.52)
Women 42 (53%) 35 7 17 (53%) b 5.20 (2.73, 7.67)b 34 (54%) 0.43 (0.30, 0.59)
Age (years)
 ≤ 20 4 1 3 (1) 0b 0b 1 0.03 (0, 0.16)
21–40 22 8 14 (2) 4b 2.64 (0.05, 5.22)b 10 0.19 (0.10, 0.35)
41–60 28 26 2 (1) 10b 5.11 (1.94, 8.27)b 26 0.63 (0.42, 0.91)
 > 60 26 26 0 18b 11.53 (6.20, 16.85)b 26 0.78 (0.52, 1.12)
Period
1991–1999 26 23 3 (2) 31c 5.70 (3.69, 7.71)c 22 0.46 (0.30, 0.69)
2000–2008 28 21 7 (1) 32d 5.07 (3.31, 6.83)d 21 0.39 (0.25, 0.59)
2009–2017 24 15 9 (1) 32b 4.94 (3.23, 6.65)b 20 0.35 (0.22, 0.53)

aNumber of HD presymptomatic cases that became symptomatic throughout the study period

Point prevalence dates: b31/12/2017, c31/12/1999, d31/12/2008