Effect of Pain in Pediatric Inherited Neuropathies

Overview

Journal Neurology

Specialty Neurology

Date 2014 Jan 31

PMID 24477108

Citations 10

Authors

Sindhu Ramchandren

Mamta Jaiswal

Eva Feldman

Michael Shy

Affiliations

Soon will be listed here.

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.

Citing Articles

Charcot-Marie-Tooth disease: from historical landmarks in Brazil to current care perspectives.

Boiteux Uchoa Cavalcanti E, Leal R, Junior W, Nascimento O Arq Neuropsiquiatr. 2023; 81(10):913-921.

PMID: 37611635 PMC: 10631856. DOI: 10.1055/s-0043-1770348.

Challenges in Treating Charcot-Marie-Tooth Disease and Related Neuropathies: Current Management and Future Perspectives.

Pisciotta C, Saveri P, Pareyson D Brain Sci. 2021; 11(11).

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Pain Phenotypes in Rare Musculoskeletal and Neuromuscular Diseases.

Tucker-Bartley A, Lemme J, Gomez-Morad A, Shah N, Veliu M, Birklein F Neurosci Biobehav Rev. 2021; 124:267-290.

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Neuropathic pain in patients with Charcot-Marie-Tooth type 1A.

Bjelica B, Peric S, Basta I, Bozovic I, Kacar A, Marjanovic A Neurol Sci. 2019; 41(3):625-630.

PMID: 31729581 DOI: 10.1007/s10072-019-04142-5.

Do Commonly Used Measures of Pain Intensity Only Reflect Pain Intensity in Youths With Bothersome Pain and a Physical Disability?.

Miro J, Vega R, Gertz K, Thong I, Jensen M, Engel J Front Pediatr. 2019; 7:229.

PMID: 31281805 PMC: 6595400. DOI: 10.3389/fped.2019.00229.

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