[{"data":1,"prerenderedAt":-1},["ShallowReactive",2],{"detail-sidebar-cat-0-en-105":3,"doc-seo-435875-105":59,"doc-detail-435875-en":131},{"code":4,"msg":5,"data":6},0,"success",[7,13,18,23,28,33,38,43,48,51,55],{"id":8,"doc_module":4,"doc_module_name":9,"category_name":10,"show_sort_weight":11,"slug":12},1,"Document","Story & Novel",90,"story-novel",{"id":14,"doc_module":4,"doc_module_name":9,"category_name":15,"show_sort_weight":16,"slug":17},2,"Literature",80,"literature",{"id":19,"doc_module":4,"doc_module_name":9,"category_name":20,"show_sort_weight":21,"slug":22},4,"Exam",70,"exam",{"id":24,"doc_module":4,"doc_module_name":9,"category_name":25,"show_sort_weight":26,"slug":27},5,"Comic",60,"comic",{"id":29,"doc_module":4,"doc_module_name":9,"category_name":30,"show_sort_weight":31,"slug":32},6,"Technology",50,"technology",{"id":34,"doc_module":4,"doc_module_name":9,"category_name":35,"show_sort_weight":36,"slug":37},7,"Healthcare",40,"healthcare",{"id":39,"doc_module":4,"doc_module_name":9,"category_name":40,"show_sort_weight":41,"slug":42},8,"Research & Report",30,"research-report",{"id":44,"doc_module":4,"doc_module_name":9,"category_name":45,"show_sort_weight":46,"slug":47},9,"Religion & Spirituality",20,"religion-spirituality",{"id":46,"doc_module":4,"doc_module_name":9,"category_name":49,"show_sort_weight":46,"slug":50},"World Cup","world-cup",{"id":52,"doc_module":4,"doc_module_name":9,"category_name":53,"show_sort_weight":52,"slug":54},10,"Lifestyle","lifestyle",{"id":56,"doc_module":4,"doc_module_name":9,"category_name":57,"show_sort_weight":24,"slug":58},19,"General","general",{"code":4,"msg":60,"data":61},"ok",{"site_id":62,"language":63,"slug":64,"title":65,"keywords":66,"description":67,"schema_data":68,"social_meta":124,"head_meta":126,"extra_data":128,"updated_unix":130},105,"en","on-the-natural-history-of-charcot-marie-tooth-disease-in-children-and-adolescents-editorial","On the natural history of Charcot-Marie-Tooth disease in children and adolescents - Editorial","","Charcot-Marie-Tooth (CMT) disease, first independently described in 1886, shows marked heterogeneity in inheritance, onset, clinical course, and prognosis. Electromyography has enabled subdivision by nerve conduction velocities, distinguishing demyelinating (type 1) and axonal (type 2) forms, with intermediate and recessive variants later refined by genetic discoveries. The editorial highlights limited Brazilian epidemiology, summarizing longitudinal pediatric evaluations using CMTPedS and functional measures, contrasting observed progression with consortium cohorts, and emphasizing urgent need for multicenter genetic studies to clarify natural history and genotype-phenotype correlations.",{"@graph":69,"@context":123},[70,84,106],{"@type":71,"itemListElement":72},"BreadcrumbList",[73,77,79,82],{"item":74,"name":75,"@type":76,"position":8},"https://docshare.wps.com","Home","ListItem",{"item":78,"name":9,"@type":76,"position":14},"https://docshare.wps.com/document/",{"item":80,"name":35,"@type":76,"position":81},"https://docshare.wps.com/document/healthcare/",3,{"item":83,"name":65,"@type":76,"position":19},"https://docshare.wps.com/document/on-the-natural-history-of-charcot-marie-tooth-disease-in-children-and-adolescents-editorial/435875/",{"url":83,"name":65,"@type":85,"image":86,"author":91,"headline":65,"publisher":94,"fileFormat":97,"inLanguage":63,"description":67,"dateModified":98,"datePublished":99,"encodingFormat":97,"isAccessibleForFree":100,"interactionStatistic":101},"DigitalDocument",{"url":87,"@type":88,"width":89,"height":90},"https://docshare.wps.com/thumbnails/on-the-natural-history-of-charcot-marie-tooth-disease-in-children-and-adolescents-editorial/435875.png","ImageObject",300,407,{"name":92,"@type":93},"Himbo","Person",{"url":74,"name":95,"@type":96},"DocShare","Organization","application/pdf","2026-11-04","2026-09-29",true,{"@type":102,"interactionType":103,"userInteractionCount":105},"InteractionCounter",{"@type":104},"ViewAction",11,{"@type":107,"mainEntity":108},"FAQPage",[109,115,119],{"name":110,"@type":111,"acceptedAnswer":112},"What is Charcot-Marie-Tooth disease, and why is it considered heterogeneous?","Question",{"text":113,"@type":114},"CMT disease was first described in 1886 and is considered heterogeneous because it varies by inheritance patterns, onset, clinical course, prognosis, and can show distinct features such as tremor.","Answer",{"name":116,"@type":111,"acceptedAnswer":117},"How does electromyography classification relate to CMT types?",{"text":118,"@type":114},"Electromyography subdivides CMT by nerve conduction velocities into type 1 (slow/demyelinating) and type 2 (higher CV with axonal features), while later work also addressed intermediate-range cases (DI-CMT).",{"name":120,"@type":111,"acceptedAnswer":121},"What does the editorial emphasize about studies in Brazil and future research?",{"text":122,"@type":114},"It notes that Brazilian epidemiology and evolution data for different CMT subtypes remain scarce, calling for multicenter collaborative genetic studies to better define natural history and genotype-phenotype correlations and support upcoming genetic treatments.","https://schema.org",{"og:url":83,"og:type":125,"og:title":65,"og:site_name":95,"og:description":67},"article",{"robots":127,"canonical":83},"index,follow",{"doc_id":129,"site_id":62},435875,1790891480,{"code":4,"msg":5,"data":132},{"doc_id":129,"user_id":133,"nickname":92,"user_avatar":134,"doc_module":4,"category_id":34,"category_name":35,"doc_title":65,"doc_description":67,"doc_content":135,"file_id":136,"file_url":137,"file_type":138,"file_size":139,"view_count":105,"is_deleted":4,"is_public":8,"is_downloadable":8,"audit_status":8,"page_count":14,"language":140,"language_code":63,"site_id":62,"html_lang":63,"table_of_contents":141,"faqs":142,"seo_title":143,"seo_description":67,"update_tm":144,"read_time":24,"preview_page_count":4},687197100911,"https://ap-avatar.wpscdn.com/avatar/a000239b6f1da00475?x-image-process=image/resize,m_fixed,w_180,h_180&k=1785132997149421697","Editorial  \nTHIEME  \nEditorial  \n1  \nArticle published online: 2025-08-25  \nOn the natural history of Charcot-Marie-Tooth disease in children and adolescents  \nFrancisco de Assis Aquino Gondim 1  \n1 Universidade Federal do Ceará, Departamento de Medicina Clínica, Divisão de Neurologia, Fortaleza CE, Brazil  \nArq. Neuro-Psiquiatr. 2025;83(8):s00451810624 .  \nCharcot-Marie-Tooth (CMT) disease was independently described in 1886 by Charcot and Marie in France and by Toothin England. 1,2 Few years later, Déjerine and Sottas described severe infancy onset cases.3 Soon, it also became clear that the disease was rather heterogenous, with major variation in inheritance patterns, onset, clinical course, prognosis and could also include very distinct features, such as tremor.4  \nWith the development of electromyography, CMT disease was subdivided according to nerve conduction velocities (CV)s into 1 . CMT type 1: slow CVs or demyelinating form (median motor CV below 38 m/s); and 2. CMT type 2: median motor CVs above 38 m/s with decreased motor and sensory amplitudes or axonal form.5 In the 90s, the most common form of CMT, i.e. CMT1A, was linked to a duplication within the chromosome 17p11.2.6 In this new era of gene identifi cation, the term CMT4 (or ARCMT1) has been employed to describe demyelinating autosomal recessive phenotypes and ARCMT2 for autosomal recessive axonal variants. To date, more than 110 genes have been linked to different CMT subtypes. Genetic studies have also evidenced that the past subdivision of demyelinating and axonal forms is not adequate to describe families with CVs in the intermediate range (later named DI-CMT) .7  \nIn Brazil, studies about the epidemiology and evolution of the different subtypes of CMT are scarce, and important lacunes remain to be addressed by multicenter collaborative studies. Alvarenga Soares and colleagues8 reported in this issue of Arquivos de Neuropsiquiatria the longitudinal findings of 3 consecutive evaluations of 30 children/adolescents with CMT (80% with CMT1A) seen at the Faculdade de Medicina de Ribeirão Preto over a 2-year period.  \nIn this study, the participants were evaluated by the CMT Pediatric Scale (CMTPedS) and additional body indexes. In the CMT1A subgroup, the authors observed a 4-point increase in the CMTPedS score over2years(2point increase per year). The functional dexterity test also showed a significant difference between evaluation one and evaluation three (p ¼0.049) as  \nwell as the vibration sensation (p ¼0.043) and gait scores (p ¼0.048). Those results contrast with the 2.4 􀀂 4.9 point progression in the CMTPedS over 2-years observed in a large CMT Consortium study that included 206 participants.9 It also differs from the 1.8 􀀂 4.2 point progression among 111 participants with CMT1A from the same study.9 An increase in body mass index (BMI) was also observed in the results from Alvarenga Soares and colleagues,8 which together with a possible type II error due to sample size differences, may at least partially explain the diverse findings. Overall, the study from Alvarenga Soares and colleagues8 further emphasize the urgent need for additional multicenter genetic studies in Brazil and all over the world, including patients from different age groups and larger number of patients. Only with larger collaborative studies, we will be able to understand the clinical evolution/natural history and genotype-phenotype correlation of the different (and especially the more rare) CMT mutations. This subject is especially relevant, when one considers the ongoing prospects for the development of new different genetic treatments for CMT patients in the near future.  \nConflict of Interest  \nThere is no conflict of interest to declare.  \nReferences  \n1 Charcot JM. Sur une forme particulaire d’atrophie musculaire progressive souvent familial debutant par les pieds et les jambeset atteingnant plus tard les mains. Rev Med 1886;6:97–138  \n2 Tooth HH. The peroneal type of progressive mus","cbCaiegnUTyxI6hb","https://ap.wps.com/l/cbCaiegnUTyxI6hb","pdf",146706,"English","# Editorial\n## Background and classification of CMT\n## Pediatric longitudinal findings and comparisons\n## Research needs and future genetic treatments\n## Conflict of interest and references","[{\"question\":\"What is Charcot-Marie-Tooth disease, and why is it considered heterogeneous?\",\"answer\":\"CMT disease was first described in 1886 and is considered heterogeneous because it varies by inheritance patterns, onset, clinical course, prognosis, and can show distinct features such as tremor.\"},{\"question\":\"How does electromyography classification relate to CMT types?\",\"answer\":\"Electromyography subdivides CMT by nerve conduction velocities into type 1 (slow/demyelinating) and type 2 (higher CV with axonal features), while later work also addressed intermediate-range cases (DI-CMT).\"},{\"question\":\"What does the editorial emphasize about studies in Brazil and future research?\",\"answer\":\"It notes that Brazilian epidemiology and evolution data for different CMT subtypes remain scarce, calling for multicenter collaborative genetic studies to better define natural history and genotype-phenotype correlations and support upcoming genetic treatments.\"}]","On the natural history of Charcot-Marie-Tooth disease in children and adolescents - Editorial | PDF",1790675737]