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Published on: July 16, 2014
Effect of pain in pediatric inherited neuropathies
Sindhu Ramchandren1, Mamta Jaiswal, Eva Feldman
1From 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.
Insights
Pain is common in children with Charcot-Marie-Tooth (CMT) disease, significantly impacting their quality of life. Ankle inflexibility, not neuropathy severity, appears to be a key factor associated with increased pain in pediatric CMT patients.
Area of Science:
- Neurology
- Pediatrics
- Pain Management
Background:
- Charcot-Marie-Tooth (CMT) disease is a group of inherited neuropathies affecting peripheral nerves.
- Pain is a frequently reported symptom in CMT, but its prevalence and impact in children are not well understood.
Purpose of the Study:
- To assess the prevalence and impact of pain in children diagnosed with Charcot-Marie-Tooth disease.
- To identify factors associated with pain in pediatric CMT patients.
Main Methods:
- A prospective cross-sectional study was conducted with 176 children (aged 2-18 years) with CMT disease.
- Standardized pain assessments (Wong-Baker FACES Pain Rating Scale), clinical evaluations, and quality-of-life (QOL) outcomes were collected.
- Multivariate regression models were used to analyze the relationship between pain scores and neuropathy severity, functional impact, and foot structure.
Main Results:
- The mean pain score was 2 (on a 0-5 scale), indicating a notable presence of pain.
- Increased pain strongly correlated with worse QOL scores.
- Ankle inflexibility was identified as an independent determinant of increased pain, while neuropathy severity did not show a significant correlation.
Conclusions:
- Pain is a significant issue for children with CMT disease, negatively affecting their QOL.
- Pain levels in pediatric CMT are not directly correlated with neuropathy severity.
- Further research into pain mechanisms is needed to develop effective treatments for pain and improve QOL in children with CMT.
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.
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