Table 5 Inherited muscle disease prevalence in Navarre by January 1, 2016
Type of myopathy | Cases, n | PRx105 (CI 95%) | PR male | PR female | p | Mean age (SD) |
|---|---|---|---|---|---|---|
Muscular dystrophy | 312 | 48.70 (43.59–54.41) | 53.80 | 43.67 | 0.066 | 46.43 (17.14) |
Myotonic dystrophy 1 | 230 | 35.90 (31.55–40.85) | 36.18 | 35.63 | 0.906 | 47.06 (15.39) |
FSHD | 33 | 5.15 (3.67–7.23) | 6.92 | 3.41 | 0.052 | 55.51 (14.44) |
FSHD1 | 27 | 4.21 (2.90–6.13) | 5.98 | 2.48 | 0.320 | 58.15 (13.06) |
FSHD2 | 6 | 0.94 (0.43–2.04) | 0.94 | 0.93 | 0.985 | 43.67 (15.54) |
LGMD2 | 27 | 4.21 (0.90–6.13) | 5.03 | 3.41 | 0.325 | 45.04 (17.67) |
LGMD2A | 16 | 2.50 (1.54–4.05) | 2.52 | 2.48 | 0.975 | 43.81 (14.63) |
LGMD2B | 1 | 0.16 (0.03–0.88) | 0.31 | 0.00 | 0.496 | 56 (−) |
LGMD2C | 3 | 0.47 (0.16–1.38) | 0.94 | 0.00 | 0.122 | 21.33 (24.45) |
LGMD2D | 1 | 0.16 (0.03–0.88) | 0.00 | 0.31 | 0.503 | 46 (−) |
LGMD2I | 1 | 0.16 (0.03–0.88) | 0.31 | 0.00 | 0.496 | 53 (−) |
LGMD2L | 5 | 0.78 (0.33–1.83) | 0.94 | 0.62 | 0.675 | 59.20 (16.51) |
OPMD | 2 | 0.31 (0.08–1.14) | 0.63 | 0.00 | 0.246 | 72 (15,56) |
EDMD | 5 | 0.78 (0.33–1.83) | 0.94 | 0.62 | 0.675 | 41.80 (19.32) |
Emerin EDMD | 1 | 0.16 (0.03–0.88) | 0.31 | 0.00 | 0.496 | 19 (−) |
Lamin EDMD | 4 | 0.62 (0.24–1.60) | 0.63 | 0.62 | 0.988 | 47.5 (16.76) |
Dystrophinopathy | 11 | 1.71 (0.95–3.07) | 3.46 | 0.00 | 0.000 | 18.73 (18.26) |
DMD | 6 | 0.94 (0.43–2.04) | 1.89 | 0.00 | 0.015 | 8.33 (4.80) |
BMD | 5 | 0.78 (0.33–1.83) | 1.57 | 0.00 | 0.030 | 31.2 (21.18) |
CMD | 4 | 0.62 (0.24–1.60) | 0.63 | 0.62 | 0.988 | 13.25 (6.34) |
Glycosylation disorder | 3 | 0.47 (0.16–1.38) | 0.63 | 0.31 | 0.616 | 10.67 (4.51) |
Unclassified CMD | 1 | 0.16 (0.03–0.88) | 0.00 | 0.31 | 0.503 | 21 (−) |
Metabolic myopathies | 11 | 1.71 (0.95–3.07) | 2.52 | 0.93 | 0.139 | 45.91 (19.13) |
Glycogen storage | 10 | 1.56 (0.85–2.87) | 2.52 | 0.62 | 0.062 | 49.10 (16.80) |
GSD-II | 2 | 0.31 (0.08–1.14) | 0.63 | 0.00 | 0.246 | 43 (12.73) |
GSD-V | 6 | 0.94 (0.43–2.04) | 1.26 | 0.62 | 0.442 | 5.15 (20.83) |
Unclassified | 2 | 0.31 (0.08–1.14) | 0.63 | 0.00 | 0.246 | 48 (9.90) |
Lipid storage disease | 1 | 0.16 (0.03–0.88) | 0.00 | 0.31 | 0.503 | 14 (−) |
Congenital myopathy | 8 | 1.25 (0.63–2.46) | 2.20 | 0.31 | 0.037 | 36.12 (14.20) |
Central Core | 2 | 0.31 (0.08–1.14) | 0.63 | 0.00 | 0.246 | 22.50 (24.75) |
Myosin storage myopathy | 3 | 0.47 (0.16–1.38) | 0.94 | 0.00 | 0.122 | 44.67 (9.07) |
Fibre type disproportion | 3 | 0.47 (0.16–1.38) | 0.63 | 0.31 | 0.616 | 36.67 (4.62) |
Distal myopathy | 3 | 0.47 (0.16–1.38) | 0.63 | 0.31 | 0.616 | 65.67 (17.78) |
Zaspopathy | 5 | 0.78 (0.33–1.83) | 1.26 | 0.31 | 0.212 | 63.40 (4.88) |
Unclassified myopathy | 39 | 6.09 (4.45–8.32) | 6.92 | 5.27 | 0.402 | 59.92 (21.86) |
Total | 378 | 59.00 (53.35–65.26) | 67.33 | 50.80 | 0.006 | 46.93 (17.77) |