Sindhu Ramchandren1, Mamta Jaiswal, Eva Feldman, Michael Shy. 1. From the Department of Neurology (S.R., E.F.), University of Michigan Medical Health System, Ann Arbor, MI; and Department of Neurology (M.J., M.S.), University of Iowa, Iowa City.
Abstract
OBJECTIVE: Assess the prevalence and impact of pain in children with Charcot-Marie-Tooth (CMT) disease. METHODS: In this prospective cross-sectional study on children with CMT disease seen at study sites of the Inherited Neuropathy Consortium, we collected standardized assessments of pain (Wong-Baker FACES Pain Rating Scale) from 176 patients (140 children aged 8-18 years, and 36 children aged 2-7 years through parent proxies), along with standardized clinical assessments and quality-of-life (QOL) outcomes. We then developed a series of multivariate regression models to determine whether standardized measures of neuropathy severity, functional impact, or structural changes to the feet explained the observed pain scores. RESULTS: The mean score on the Wong-Baker FACES Pain Rating Scale was 2 (range 0-5). Increased pain strongly correlated with worse QOL scores but not with more severe neuropathy. Independent determinants of increased pain in children with CMT disease included measures of ankle inflexibility. CONCLUSION: Pain is present in children with CMT disease and negatively affects QOL. Pain scores do not positively correlate with neuropathy severity but do correlate in limited univariate analyses with measures of ankle inflexibility. Further studies to elucidate the mechanisms of pain may help identify treatments that can reduce pain and improve QOL in patients with CMT disease.
OBJECTIVE: Assess the prevalence and impact of pain in children with Charcot-Marie-Tooth (CMT) disease. METHODS: In this prospective cross-sectional study on children with CMT disease seen at study sites of the Inherited Neuropathy Consortium, we collected standardized assessments of pain (Wong-Baker FACES Pain Rating Scale) from 176 patients (140 children aged 8-18 years, and 36 children aged 2-7 years through parent proxies), along with standardized clinical assessments and quality-of-life (QOL) outcomes. We then developed a series of multivariate regression models to determine whether standardized measures of neuropathy severity, functional impact, or structural changes to the feet explained the observed pain scores. RESULTS: The mean score on the Wong-Baker FACES Pain Rating Scale was 2 (range 0-5). Increased pain strongly correlated with worse QOL scores but not with more severe neuropathy. Independent determinants of increased pain in children with CMT disease included measures of ankle inflexibility. CONCLUSION:Pain is present in children with CMT disease and negatively affects QOL. Pain scores do not positively correlate with neuropathy severity but do correlate in limited univariate analyses with measures of ankle inflexibility. Further studies to elucidate the mechanisms of pain may help identify treatments that can reduce pain and improve QOL in patients with CMT disease.
Authors: Vera Bril; John England; Gary M Franklin; Miroslav Backonja; Jeffrey Cohen; David Del Toro; Eva Feldman; Donald J Iverson; Bruce Perkins; James W Russell; Douglas Zochodne Journal: PM R Date: 2011-04 Impact factor: 2.298
Authors: C Ribiere; M Bernardin; S Sacconi; E Delmont; M Fournier-Mehouas; H Rauscent; M Benchortane; P Staccini; M Lantéri-Minet; C Desnuelle Journal: Ann Phys Rehabil Med Date: 2012-03-06
Authors: M E Shy; J Blake; K Krajewski; D R Fuerst; M Laura; A F Hahn; J Li; R A Lewis; M Reilly Journal: Neurology Date: 2005-04-12 Impact factor: 9.910
Authors: J Miró; E Castarlenas; R de la Vega; E Solé; C Tomé-Pires; M P Jensen; J M Engel; M Racine Journal: Eur J Pain Date: 2015-03-31 Impact factor: 3.931
Authors: Maria Nolano; Fiore Manganelli; Vincenzo Provitera; Chiara Pisciotta; Annamaria Stancanelli; Giuseppe Caporaso; Rosa Iodice; Michael E Shy; Lucio Santoro Journal: Neurology Date: 2014-12-24 Impact factor: 9.910
Authors: Anthony Tucker-Bartley; Jordan Lemme; Andrea Gomez-Morad; Nehal Shah; Miranda Veliu; Frank Birklein; Claudia Storz; Seward Rutkove; David Kronn; Alison M Boyce; Eduard Kraft; Jaymin Upadhyay Journal: Neurosci Biobehav Rev Date: 2021-02-10 Impact factor: 9.052
Authors: Sean Ekins; Nadia K Litterman; Renée J G Arnold; Robert W Burgess; Joel S Freundlich; Steven J Gray; Joseph J Higgins; Brett Langley; Dianna E Willis; Lucia Notterpek; David Pleasure; Michael W Sereda; Allison Moore Journal: F1000Res Date: 2015-02-26