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Table 5 Inherited muscle disease prevalence in Navarre by January 1, 2016

From: Epidemiological study and genetic characterization of inherited muscle diseases in a northern Spanish region

Type of myopathyCases, nPRx105 (CI 95%)PR malePR femalepMean age (SD)
Muscular dystrophy31248.70 (43.59–54.41)53.8043.670.06646.43 (17.14)
 Myotonic dystrophy 123035.90 (31.55–40.85)36.1835.630.90647.06 (15.39)
 FSHD335.15 (3.67–7.23)6.923.410.05255.51 (14.44)
 FSHD1274.21 (2.90–6.13)5.982.480.32058.15 (13.06)
 FSHD260.94 (0.43–2.04)0.940.930.98543.67 (15.54)
 LGMD2274.21 (0.90–6.13)5.033.410.32545.04 (17.67)
 LGMD2A162.50 (1.54–4.05)2.522.480.97543.81 (14.63)
 LGMD2B10.16 (0.03–0.88)0.310.000.49656 (−)
 LGMD2C30.47 (0.16–1.38)0.940.000.12221.33 (24.45)
 LGMD2D10.16 (0.03–0.88)0.000.310.50346 (−)
 LGMD2I10.16 (0.03–0.88)0.310.000.49653 (−)
 LGMD2L50.78 (0.33–1.83)0.940.620.67559.20 (16.51)
 OPMD20.31 (0.08–1.14)0.630.000.24672 (15,56)
 EDMD50.78 (0.33–1.83)0.940.620.67541.80 (19.32)
 Emerin EDMD10.16 (0.03–0.88)0.310.000.49619 (−)
 Lamin EDMD40.62 (0.24–1.60)0.630.620.98847.5 (16.76)
 Dystrophinopathy111.71 (0.95–3.07)3.460.000.00018.73 (18.26)
 DMD60.94 (0.43–2.04)1.890.000.0158.33 (4.80)
 BMD50.78 (0.33–1.83)1.570.000.03031.2 (21.18)
 CMD40.62 (0.24–1.60)0.630.620.98813.25 (6.34)
 Glycosylation disorder30.47 (0.16–1.38)0.630.310.61610.67 (4.51)
 Unclassified CMD10.16 (0.03–0.88)0.000.310.50321 (−)
Metabolic myopathies111.71 (0.95–3.07)2.520.930.13945.91 (19.13)
 Glycogen storage101.56 (0.85–2.87)2.520.620.06249.10 (16.80)
 GSD-II20.31 (0.08–1.14)0.630.000.24643 (12.73)
 GSD-V60.94 (0.43–2.04)1.260.620.4425.15 (20.83)
 Unclassified20.31 (0.08–1.14)0.630.000.24648 (9.90)
 Lipid storage disease10.16 (0.03–0.88)0.000.310.50314 (−)
Congenital myopathy81.25 (0.63–2.46)2.200.310.03736.12 (14.20)
 Central Core20.31 (0.08–1.14)0.630.000.24622.50 (24.75)
 Myosin storage myopathy30.47 (0.16–1.38)0.940.000.12244.67 (9.07)
 Fibre type disproportion30.47 (0.16–1.38)0.630.310.61636.67 (4.62)
Distal myopathy30.47 (0.16–1.38)0.630.310.61665.67 (17.78)
Zaspopathy50.78 (0.33–1.83)1.260.310.21263.40 (4.88)
Unclassified myopathy396.09 (4.45–8.32)6.925.270.40259.92 (21.86)
Total37859.00 (53.35–65.26)67.3350.800.00646.93 (17.77)
  1. SD Standard deviation.
  2. OPMD Oculopharyngeal muscular dystrophy, EDMD Emery-Dreifuss muscular dystrophy, DMD Duchenne muscular dystrophinopathy, CMD Congenital muscular dystrophy, BMD Becker muscular dystrophinopathy, CMD congenital muscle dystrophy, GSD glycogen storage disease.