CC BY-NC-ND 4.0 · Arq Neuropsiquiatr 2021; 79(05): 415-419
DOI: 10.1590/0004-282X-ANP-2020-0132
Articles

Prevalence and characterization of pain in patients with Charcot-Marie-Tooth disease type 1A

Prevalência e caracterização da dor na doença de Charcot-Marie-Tooth tipo 1A
Helen AZEVEDO 
1   Universidade Federal Fluminense, Hospital Universitário Antônio Pedro, Departamento de Neurologia, Niterói RJ, Brasil.
,
Henrique COSTA 
1   Universidade Federal Fluminense, Hospital Universitário Antônio Pedro, Departamento de Neurologia, Niterói RJ, Brasil.
,
Eduardo DAVIDOVICH 
1   Universidade Federal Fluminense, Hospital Universitário Antônio Pedro, Departamento de Neurologia, Niterói RJ, Brasil.
,
Camila PUPE 
1   Universidade Federal Fluminense, Hospital Universitário Antônio Pedro, Departamento de Neurologia, Niterói RJ, Brasil.
,
Osvaldo José Moreira NASCIMENTO 
1   Universidade Federal Fluminense, Hospital Universitário Antônio Pedro, Departamento de Neurologia, Niterói RJ, Brasil.
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ABSTRACT

Background: Charcot-Marie-Tooth disease type 1A (CMT1A) is the most common form of hereditary neuropathy. Objective: To investigate the prevalence and characteristics of pain in patients with CMT1A. Methods: Nineteen patients with a diagnosis of CMT1A were evaluated between September 2018 and October 2019, and other causes of neuropathy were ruled out. The following tools were used for the pain assessment: neurological assessment, LANSS, DN4, clinical evaluation, VAS, CMTNS2 and SF-36. Statistical analysis was performed using prevalence analysis, t test, chi-square test and Spearman's rho. Results: The prevalence of pain was 84.2% in the sample of this study, with moderate intensity and nociceptive characteristics according to the LANSS scale (75%) and clinical evaluation (50%), but differing from DN4, which found neuropathic pain in the majority of the patients (56.2%). Mixed pain was also observed in 43.7% of the patients, according to clinical criteria. There was a statistically significant correlation between pain intensity and SF-36, thus demonstrating that the lower the pain was, the lower the impairment was, in all domains. Conclusion: Pain is a prevalent and important symptom in CMT1A, with moderate intensity and nociceptive characteristics according to two tools, but neuropathic pain is also present, and there may even be a mixed pattern of pain. The correlation of the pain with SF-36 suggests that pain relief could provide improvements to the quality of life of these individuals.

RESUMO

Introdução: A doença de Charcot-Marie-Tooth tipo 1 A (CMT1A) é a forma mais comum de neuropatia hereditária. Objetivo: Investigar a prevalência e as características de dor nos pacientes com a doença de CMT1A. Métodos: Dezenove pacientes com diagnóstico de CMT1A foram avaliados de setembro 2018 a outubro de 2019, e outras causas de neuropatia foram excluídas. As seguintes ferramentas foram utilizadas para avaliar a dor: avaliação neurológica, LANSS, DN4, avaliação clínica, EVA, CMTNS2 e SF-36. A análise estatística foi realizada pelo teste de análise de prevalência, bem como pelos testes T, do qui-quadrado e rô de Sperman. Resultados: A prevalência de dor foi de 84,2% na amostra do estudo, com intensidade moderada e características nociceptivas de acordo com a escala LANSS (75%) e a avaliação clínica (50%), diferentemente da escala DN4, que encontrou dor neuropática na maioria dos pacientes (56,2%). Dor mista também foi verificada em 43,7% dos pacientes, de acordo com os critérios clínicos. Houve significância estatística da correlação entre a intensidade da dor e o SF-36, demonstrando que quanto menor a dor, menor o comprometimento em todos os domínios. Conclusão: A dor é um sintoma prevalente e relevante na CMT1A, com intensidade moderada e características nociceptivas de acordo com duas ferramentas, mas dor neuropática também está presente, e ainda pode haver padrão misto de dor. A correlação da dor com SF-36 sugere que o alívio da dor pode proporcionar melhorias na qualidade de vida desses indivíduos.

Keywords:

Charcot-Marie-Tooth Disease - Pain

Palavras-chave:

Doença de Charcot-Marie-Tooth - Dor

Authors’ contributions:

HMA: performed literature search review, physical therapy evaluation, data collection and extraction, data interpretation and draft of the manuscript. HVMC: performed neurological evaluation. ED: performed nerve conduction study. CPCB: reviewed the concept and design of the study .and reviewed the manuscript. OJMN: reviewed the concept and design of the study and reviewed the manuscript.




Publication History

Received: 29 May 2020

Accepted: 11 September 2020

Article published online:
07 June 2023

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