PMID- 26849231 OWN - NLM STAT- MEDLINE DCOM- 20161219 LR - 20220331 IS - 1423-0208 (Electronic) IS - 0251-5350 (Linking) VI - 46 IP - 3 DP - 2016 TI - Epidemiologic Study of Charcot-Marie-Tooth Disease: A Systematic Review. PG - 157-65 LID - 10.1159/000443706 [doi] AB - BACKGROUND: Charcot-Marie-Tooth disease (CMT) is the most common inherited neuropathy. CMT is classified into 2 main subgroups: CMT type 1 (CMT1; demyelinating form) and CMT type 2 (CMT2; axonal form). The objectives of this study were to systematically review and assess the quality of studies reporting the incidence and/or prevalence of CMT worldwide. SUMMARY: A total of 802 studies were initially identified, with only 12 meeting the inclusion criteria. CMT prevalence was reported in 10 studies and ranged from 9.7/100,000 in Serbia to 82.3/100,000 in Norway. The frequency of the main subtypes varied from 37.6 to 84% for CMT1 and from 12 to 35.9% for CMT2; the country with the lowest prevalence of CMT1 was Norway, and the country with the highest prevalence of CMT1 was Iceland; on the other hand, CMT2 was least prevalent in the United Kingdom and most prevalent in Norway. KEY MESSAGES: This review reveals the gaps that still exist in the epidemiological knowledge of CMT around the world. Published studies are of varying quality and utilise different methodologies, thus precluding a robust conclusion. Additional research focusing on epidemiological features of CMT in different nations and different ethnic groups is needed. CI - (c) 2016 S. Karger AG, Basel. FAU - Barreto, Lidiane Carine Lima Santos AU - Barreto LC AD - Nx00FA;cleo de Px00F3;s-Graduax00E7;x00E3;o em Medicina da Universidade Federal de Sergipe (UFS), Rua Clx00E1;udio Batista S/N Bairro Sanatx00F3;rio, Aracaju, SE, Brazil. FAU - Oliveira, Fernanda Santos AU - Oliveira FS FAU - Nunes, Paula Santos AU - Nunes PS FAU - de Franca Costa, Iandra Maria Pinheiro AU - de Franca Costa IM FAU - Garcez, Catarina Andrade AU - Garcez CA FAU - Goes, Gabriel Mattos AU - Goes GM FAU - Neves, Eduardo Luis Aquino AU - Neves EL FAU - de Souza Siqueira Quintans, Jullyana AU - de Souza Siqueira Quintans J FAU - de Souza Araujo, Adriano Antunes AU - de Souza Araujo AA LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review PT - Systematic Review DEP - 20160206 PL - Switzerland TA - Neuroepidemiology JT - Neuroepidemiology JID - 8218700 SB - IM MH - Charcot-Marie-Tooth Disease/*diagnosis/*epidemiology MH - Epidemiologic Studies MH - Humans MH - *Population Surveillance/methods EDAT- 2016/02/06 06:00 MHDA- 2016/12/20 06:00 CRDT- 2016/02/06 06:00 PHST- 2015/07/05 00:00 [received] PHST- 2015/12/27 00:00 [accepted] PHST- 2016/02/06 06:00 [entrez] PHST- 2016/02/06 06:00 [pubmed] PHST- 2016/12/20 06:00 [medline] AID - 000443706 [pii] AID - 10.1159/000443706 [doi] PST - ppublish SO - Neuroepidemiology. 2016;46(3):157-65. doi: 10.1159/000443706. Epub 2016 Feb 6.