From: Global prevalence of Rett syndrome: systematic review and meta-analysis
Case definition criteria  Study | Country | Rett cases | Female population | Prevalence estimate | 95% LCL | 95% UCL |
---|---|---|---|---|---|---|
Clinical diagnosis onlya | ||||||
 Fombonne et al. (2003) [19] | UK | 2 | 5227 | 38.3 | 4.6 | 138.0 |
 Chakrabarti et al. (2005) [20] | UK | 1 | 13202 | 7.6 | 0.2 | 42.2 |
 Aguilera et al. (2007) [18] | Spain | 1 | 63675 | 1.6 | 0.0 | 8.7 |
 Isaksen et al. (2012) [16] | Norway | 3 | 15662 | 19.2 | 4.0 | 56.0 |
 Strømme et al. (2000) [15] | Norway | 1 | 14542 | 6.9 | 0.2 | 38.3 |
 Magnússon et al. (2001) [14] | Iceland | 0 | 41896 | 0.0 | 0.0 | 8.8 |
 Pooled prevalence |  | 8 | 154204 | 6.7 | 2.0 | 22.0 |
Clinical diagnosis + genetic testingb | ||||||
 Wong et al. (2007) [22] | China | 7 | 123968 | 5.6 | 2.3 | 11.6 |
 Sarajlija et al. (2015) [17] | Serbia | 102 | 857142 | 11.9 | 9.7 | 14.4 |
 Bienvenu et al. (2006) [13] | France | 251 | 4337627 | 5.8 | 5.1 | 6.5 |
 Fehr et al. (2011) [21] | Australia | 305 | 4094386 | 7.4 | 6.6 | 8.3 |
 Pooled prevalence |  | 665 | 9413123 | 7.6 | 5.4 | 10.8 |
All studiesc | Â | 673 | 9567327 | 7.1 | 4.8 | 10.5 |
 Pooled prevalence |  |  |  |  |  |  |