TABLES
Relative frequency of autosomal dominant ataxias
(modified from Table 3. of Sequeiros J, Martins S, Silveira I: Epidemiology and population genetics of degenerative ataxias. Chapt. 14.
In: Subramony SH, Dürr A: Ataxic Disorders. Handbook of Clinical Neurology, 3rd series, vol. 103, pp.225-248. Elsevier, Edinburgh, 2011)
| Country | AD families described (n) | Relative frequency (% families) | References | ||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| SCA1 | DRPLA | SCA2 | MJD | SCA6 | SCA7 | SCA8 | SCA10 | SCA12 | SCA17 | Other | |||
| Australia | 88 | 16 | 0 | 6 | 12 | 17 | 2 | - | - | - | - | 47 | (Storey et al., 2000) |
| Brazil (Rio Grande Sul) | 114 | 1 | 0 | 4 | 84 | 2 | 1 | - | 2 | 0 | 0 | 6 | (Jardim et al., 2001; Trott et al., 2006) |
| China | 120 | 6 | 0 | 7 | 49 | 3 | 1 | - | - | - | - | 34 | (Jiang et al., 2005) |
| Finland | 49 | 4 | 0 | 2 | 0 | 2 | 12 | 18 | - | - | - | 62 | (Juvonen et al., 2005) |
| France | 146 | 15 | - | 10 | 32 | 1 | - | - | - | - | - | 42 | (Stevanin et al., 1997) |
| Germany | 77 | 9 | - | 10 | 42 | 22 | - | - | - | - | - | 17 | (Schöls et al., 1997) |
| India | 42/77 | 8/16 | 0/0 | 26/25 | 5/3 | 0/0 | 0/3 | - | - | -/6 | - | 61/47 | (Saleem et al., 2000; Srivastava et al., 2001) |
| Italy | 116/183 | 24/21 | <1/1 | 47/24 | 0/1 | 2/1 | 2/1 | -/1 | -/0 | -/0 | -/1 | 24/50 | (Filla et al., 2000; Brusco et al., 2004) |
| Japan | 330 | 6 | 7 | 2 | 28 | 26 | 1 | - | - | - | - | 30 | (Maruyama et al., 2002) |
| Korea | 87 | 0 | 3 | 13 | 5 | 7 | 0 | - | - | - | - | 72 | (Jin et al., 1999) |
| Mexico | 28 | 0 | 0 | 43 | 7 | 0 | 11 | - | 14 | - | - | 25 | (Rasmussen et al., 2000) |
| Netherlands | 145 | 6 | - | 7 | 28 | 15 | 8 | - | - | - | - | 36 | (van de Warrenburg et al., 2002) |
| Poland | (650 pts) | 58 fams | 0 | (26 pts) | 0 | 0 | 0 | 7 fams | - | 0 | 1 fam | ? | (Sulek et al., 2003) |
| Portugal | 269 | <1 | 5 | 2 | 52 | <1 | 1 | 2 | 0 | 0 | <1 | 36 | (Silveira et al., 2002 and personal communication) |
| Serbia | 38 | 34 | 0 | 13 | 0 | 0 | 0 | 0 | - | 0 | 0 | 53 | (Dragašević et al., 2006) |
| Singapore | 39 | 11 | 6 | 33 | 41 | 6 | 0 | 0 | 0 | 3 | - | 0 | (Zhao et al., 2002) |
| South Africa | 54 | 41 | - | 13 | 4 | 2 | 22 | - | - | - | - | 18 | (Bryer et al., 2003) |
| Spain | 72 | 6 | 1 | 15 | 15 | 1 | 3 | - | - | - | - | 59 | (Pujana et al., 1999) |
| Taiwan | 81 | 1 | - | 11 | 32 | 0 | 1 | - | - | - | - | 55 | (Tsai et al., 2004) |
| UK | 19 | 37 | - | 47 | 5 | - | - | - | - | - | - | 11 | (Giunti et al., 1998) |
| USA | 178 | 6 | - | 15 | 21 | 15 | 4 | 5 | - | - | - | 34 | (Moseley et al., 1998) |
Highlighted is the number of families of the SCA more frequent in each series.
Last Update:
June 6, 2016