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

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...

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Main Authors: Helen AZEVEDO, Henrique COSTA, Eduardo DAVIDOVICH, Camila PUPE, Osvaldo José Moreira NASCIMENTO
Format: Article
Language:English
Published: Thieme Revinter Publicações 2021-05-01
Series:Arquivos de Neuro-Psiquiatria
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Online Access:http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2021000500415&tlng=en
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author Helen AZEVEDO
Henrique COSTA
Eduardo DAVIDOVICH
Camila PUPE
Osvaldo José Moreira NASCIMENTO
author_facet Helen AZEVEDO
Henrique COSTA
Eduardo DAVIDOVICH
Camila PUPE
Osvaldo José Moreira NASCIMENTO
author_sort Helen AZEVEDO
collection DOAJ
description 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.
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spelling doaj-art-4c9e65936aef4045a1ef03239b53ba4f2025-08-20T01:56:46ZengThieme Revinter PublicaçõesArquivos de Neuro-Psiquiatria1678-42272021-05-0179541541910.1590/0004-282x-anp-2020-0132Prevalence and characterization of pain in patients with Charcot-Marie-Tooth disease type 1AHelen AZEVEDOhttps://orcid.org/0000-0002-6600-5221Henrique COSTAhttps://orcid.org/0000-0001-8541-7494Eduardo DAVIDOVICHhttps://orcid.org/0000-0003-2484-3574Camila PUPEhttps://orcid.org/0000-0001-9590-435XOsvaldo José Moreira NASCIMENTOhttps://orcid.org/0000-0003-3516-485XABSTRACT 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.http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2021000500415&tlng=enCharcot-Marie-Tooth DiseasePain
spellingShingle Helen AZEVEDO
Henrique COSTA
Eduardo DAVIDOVICH
Camila PUPE
Osvaldo José Moreira NASCIMENTO
Prevalence and characterization of pain in patients with Charcot-Marie-Tooth disease type 1A
Arquivos de Neuro-Psiquiatria
Charcot-Marie-Tooth Disease
Pain
title Prevalence and characterization of pain in patients with Charcot-Marie-Tooth disease type 1A
title_full Prevalence and characterization of pain in patients with Charcot-Marie-Tooth disease type 1A
title_fullStr Prevalence and characterization of pain in patients with Charcot-Marie-Tooth disease type 1A
title_full_unstemmed Prevalence and characterization of pain in patients with Charcot-Marie-Tooth disease type 1A
title_short Prevalence and characterization of pain in patients with Charcot-Marie-Tooth disease type 1A
title_sort prevalence and characterization of pain in patients with charcot marie tooth disease type 1a
topic Charcot-Marie-Tooth Disease
Pain
url http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2021000500415&tlng=en
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