Epidemiologic Study of Charcot-Marie-Tooth Disease: A Systematic Review
Top Cited Papers
- 6 February 2016
- journal article
- review article
- Published by S. Karger AG in Neuroepidemiology
- Vol. 46 (3), 157-165
- https://doi.org/10.1159/000443706
Abstract
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.Keywords
This publication has 23 references indexed in Scilit:
- Charcot-Marie-Tooth disease: Frequency of genetic subtypes in a German neuromuscular center populationNeuromuscular Disorders, 2013
- Charcot–Marie–Tooth disease in Northern England: Figure 1Journal of Neurology, Neurosurgery & Psychiatry, 2011
- Genetic epidemiology of Charcot–Marie–Tooth in the general populationEuropean Journal of Neurology, 2010
- Charcot-Marie-Tooth Disease in Cyprus: Epidemiological, Clinical and Genetic CharacteristicsNeuroepidemiology, 2010
- Revisão dos estudos de avaliação da ocorrência de eventos adversos em hospitaisRevista Brasileira de Epidemiologia, 2005
- An Epidemiological Genetic Study of Charcot-Marie-Tooth Disease in Western JapanNeuroepidemiology, 2002
- Charcot-Marie-Tooth Disease in Molise, a Central-Southern Region of Italy: An Epidemiological StudyNeuroepidemiology, 2002
- Alguns pilares para a apreciação da validade de estudos epidemiológicosRevista Brasileira de Epidemiologia, 1998
- The Charcot‐Marie‐Tooth syndrome: clinical aspects from a population study in South Wales, UKClinical Genetics, 1994
- Bias in analytic researchJournal of Chronic Diseases, 1979