{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,8,6]],"date-time":"2025-08-06T13:24:03Z","timestamp":1754486643392,"version":"3.37.3"},"reference-count":39,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2017,12,14]],"date-time":"2017-12-14T00:00:00Z","timestamp":1513209600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Euro J of Neurology"],"published-print":{"date-parts":[[2018,2]]},"abstract":"<jats:sec><jats:title>Background and purpose<\/jats:title><jats:p>The aim of our study was to describe, by a case\u2010control and cross\u2010sectional design, the correlation between clinical impairment and age in Charcot\u2010Marie\u2010Tooth type 1A (CMT1A) patients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Seventy CMT1A patients and 70 sex\u2010 and age\u2010matched healthy controls were enrolled. Motor performance was assessed through the 10\u2010m walk test, the 6\u2010min walk test and the 9\u2010hole peg test of the dominant and non\u2010dominant side, and muscle strength was measured by using the Medical Research Council score. In the CMT1A group, disability and quality of life were evaluated using the Charcot\u2010Marie\u2010Tooth Neuropathy Score (<jats:styled-content style=\"fixed-case\">CMTNS<\/jats:styled-content>) and the Short Form 36 (<jats:styled-content style=\"fixed-case\">SF<\/jats:styled-content>\u201036) questionnaire. Cross\u2010sectional relationships between age and all clinical measures were analyzed and differences in the slopes between cases and controls were calculated. The occurrence of a structural change in the age\u2010related progression of clinical measures was explored.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The deterioration of motor performance correlated with age in both groups with a greater slope in <jats:styled-content style=\"fixed-case\">CMT<\/jats:styled-content>1A patients than controls. The deterioration of <jats:styled-content style=\"fixed-case\">CMTNS<\/jats:styled-content> and <jats:styled-content style=\"fixed-case\">SF<\/jats:styled-content>\u201036 correlated with age in the <jats:styled-content style=\"fixed-case\">CMT<\/jats:styled-content>1A group. The deterioration of all clinical measures with the exception of the <jats:styled-content style=\"fixed-case\">SF<\/jats:styled-content>\u201036 questionnaire showed a structural change at the 50th year of age. The rate of deterioration was no different between patients and controls until 50 years of age, whereupon it became significantly greater in <jats:styled-content style=\"fixed-case\">CMT<\/jats:styled-content>1A patients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Our study supports that the disease progression in <jats:styled-content style=\"fixed-case\">CMT<\/jats:styled-content>1A patients is an age\u2010related process and the 50th year of age represents a critical moment after which the clinical decline becomes faster.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ene.13494","type":"journal-article","created":{"date-parts":[[2017,10,20]],"date-time":"2017-10-20T21:37:31Z","timestamp":1508535451000},"page":"301-306","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":14,"title":["Motor performance deterioration accelerates after 50 years of age in Charcot\u2010Marie\u2010Tooth type 1A patients"],"prefix":"10.1111","volume":"25","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9672-4577","authenticated-orcid":false,"given":"S.","family":"Tozza","sequence":"first","affiliation":[{"name":"Department of Neuroscience, Reproductive Sciences and Odontostomatology University of Naples \u2018Federico II\u2019 Naples Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"D.","family":"Bruzzese","sequence":"additional","affiliation":[{"name":"Department of Public Health University of Naples \u2018Federico II\u2019 Naples Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"C.","family":"Pisciotta","sequence":"additional","affiliation":[{"name":"Department of Neuroscience, Reproductive Sciences and Odontostomatology University of Naples \u2018Federico II\u2019 Naples Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"R.","family":"Iodice","sequence":"additional","affiliation":[{"name":"Department of Neuroscience, Reproductive Sciences and Odontostomatology University of Naples \u2018Federico II\u2019 Naples Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M.","family":"Esposito","sequence":"additional","affiliation":[{"name":"Department of Neuroscience, Reproductive Sciences and Odontostomatology University of Naples \u2018Federico II\u2019 Naples Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"R.","family":"Dubbioso","sequence":"additional","affiliation":[{"name":"Department of Neuroscience, Reproductive Sciences and Odontostomatology University of Naples \u2018Federico II\u2019 Naples Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"L.","family":"Ruggiero","sequence":"additional","affiliation":[{"name":"Department of Neuroscience, Reproductive Sciences and Odontostomatology University of Naples \u2018Federico II\u2019 Naples Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"A.","family":"Topa","sequence":"additional","affiliation":[{"name":"Department of Neuroscience, Reproductive Sciences and Odontostomatology University of Naples \u2018Federico II\u2019 Naples Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5123-2459","authenticated-orcid":false,"given":"E.","family":"Spina","sequence":"additional","affiliation":[{"name":"Department of Neuroscience, Reproductive Sciences and Odontostomatology University of Naples \u2018Federico II\u2019 Naples Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"L.","family":"Santoro","sequence":"additional","affiliation":[{"name":"Department of Neuroscience, Reproductive Sciences and Odontostomatology University of Naples \u2018Federico II\u2019 Naples Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1442-9604","authenticated-orcid":false,"given":"F.","family":"Manganelli","sequence":"additional","affiliation":[{"name":"Department of Neuroscience, Reproductive Sciences and Odontostomatology University of Naples \u2018Federico II\u2019 Naples Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2017,12,14]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1111\/jns.12092"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(09)70110-3"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000297553.36441.ce"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/123.7.1516"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00415-004-0578-x"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000327643.05073.eb"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1468-1331.2010.03027.x"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1111\/ene.13079"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2007.11.008"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awp251"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.39.10.1302"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.53.7.1458"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0038830"},{"key":"e_1_2_6_15_1","doi-asserted-by":"publisher","DOI":"10.1093\/gerona\/glw043"},{"key":"e_1_2_6_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2007.09.006"},{"key":"e_1_2_6_17_1","doi-asserted-by":"publisher","DOI":"10.1111\/ene.13033"},{"key":"e_1_2_6_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.phrs.2006.09.001"},{"key":"e_1_2_6_19_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp-2014-307598"},{"key":"e_1_2_6_20_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1529-8027.2011.00350.x"},{"key":"e_1_2_6_21_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1529-8027.2008.00159.x"},{"key":"e_1_2_6_22_1","doi-asserted-by":"publisher","DOI":"10.1002\/sim.1545"},{"key":"e_1_2_6_23_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000181027.21574.df"},{"key":"e_1_2_6_24_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000176062.44360.49"},{"key":"e_1_2_6_25_1","first-page":"22","article-title":"Overwork weakness in partially denervated skeletal muscle","volume":"12","author":"Bennett RL","year":"1958","journal-title":"Clin Orthop"},{"key":"e_1_2_6_26_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.880111206"},{"key":"e_1_2_6_27_1","first-page":"348","article-title":"Overwork weakness with evidence of muscle damage in a patient with residual paralysis from polio","volume":"71","author":"Peach FE","year":"1990","journal-title":"Arch Phys Med Rehabil"},{"key":"e_1_2_6_28_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.13.9.728"},{"key":"e_1_2_6_29_1","first-page":"333","article-title":"Overwork weakness in facioscapulohumeral muscular dystrophy","volume":"52","author":"Johnson EW","year":"1971","journal-title":"Arch Phys Med Rehabil"},{"key":"e_1_2_6_30_1","first-page":"304","article-title":"Amyotrophic lateral sclerosis: a comprehensive rehabilitation approach","volume":"64","author":"Janiszewski DW","year":"1983","journal-title":"Arch Phys Med Rehabil"},{"key":"e_1_2_6_31_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0003-9993(02)04949-3"},{"key":"e_1_2_6_32_1","doi-asserted-by":"publisher","DOI":"10.2340\/16501977-0552"},{"key":"e_1_2_6_33_1","doi-asserted-by":"publisher","DOI":"10.2340\/16501977-0274"},{"key":"e_1_2_6_34_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2012.02.008"},{"key":"e_1_2_6_35_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2016.06.002"},{"key":"e_1_2_6_36_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1529-8027.2000.00026.x"},{"key":"e_1_2_6_37_1","doi-asserted-by":"publisher","DOI":"10.1016\/0022-510X(92)90282-P"},{"key":"e_1_2_6_38_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0960-8966(99)00114-5"},{"key":"e_1_2_6_39_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.10419"},{"key":"e_1_2_6_40_1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1097-4598(199807)21:7<869::AID-MUS4>3.0.CO;2-4"}],"container-title":["European Journal of Neurology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fene.13494","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/ene.13494","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/ene.13494","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/ene.13494","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,8,30]],"date-time":"2023-08-30T00:50:57Z","timestamp":1693356657000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/ene.13494"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,12,14]]},"references-count":39,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2018,2]]}},"alternative-id":["10.1111\/ene.13494"],"URL":"https:\/\/doi.org\/10.1111\/ene.13494","archive":["Portico"],"relation":{},"ISSN":["1351-5101","1468-1331"],"issn-type":[{"type":"print","value":"1351-5101"},{"type":"electronic","value":"1468-1331"}],"subject":[],"published":{"date-parts":[[2017,12,14]]},"assertion":[{"value":"2017-06-25","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2017-10-16","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2017-12-14","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}