Neuropatia de fibras finas na doença de Wilson: revisão de literatura / Small fiber neuropathy in Wilson's disease: literature review

Autores

  • Jéssica Queiroz Sobrinho Universidade Federal do Ceará. Faculdade de Medicina. Fortaleza – CE - Brasil
  • José Lopes Tabatinga Neto Universidade Federal do Ceará. Faculdade de Medicina. Fortaleza – CE - Brasil
  • Francisco de Assis Aquino Gondim Universidade Federal do Ceará. Faculdade de Medicina. Fortaleza – CE - Brasil

DOI:

https://doi.org/10.26432/1809-3019.2021.66.036

Resumo

Introdução: A neuropatia periférica é um distúrbio neurológico bastante comum e vem aumentando sua prevalência devido ao processo de envelhecimento da população e aumento de comorbidades como diabetes e obesidade. A neuropatia periférica de fibras finas (NFF) é considerada uma síndrome que apresenta manifestações sensitivas isoladas, ou a combinação de manifestações sensitivas e autonômicas. A doença de Wilson (DW) é um transtorno autossômico recessivo causado por deficiência na metabolização do cobre decorrente de mutações no gene ATP7B. Entre as alterações neurológicas apresentadas pelos pacientes com DW está a neuropatia periférica. Objetivo: Revisar a literatura acerca da neuropatia de fibras finas na Doença de Wilson aumentando a compreensão dessa disfunção sobre suas causas e tipos de abordagens apresentadas na literatura. Métodos: Foi realizada uma revisão bibliográfica a partir do levantamento de dados presente nas bases de dados Scielo e PUBMED no período de janeiro a junho de 2021, utilizando os descritores: “Small Fiber Neuropathy”, “Wilson’s Disease”, “Peripheral Neuropathy". Resultados: Foram selecionados 29 artigos, cujos originais foram utilizados para composição deste estudo. Para melhor compreensão, os resultados encontrados no levantamento bibliográfico foram divididos em tópicos. Dos 29 artigos recrutados 15 foram excluídos, pois apresentavam outras doenças de base que pudessem justificar a presença de neuropatia além da doença de Wilson. Dos 14 artigos incluídos na pesquisa: 3 descreveram a presença de neuropatia de fibras finas na DW, 4 artigos destacaram a presença de leve polineuropatia sensitivo motora axonal na DW, 7 artigos mostraram evidência de neuropatia autonômica, sendo 2 com disfunção predominantemente simpática e 1 parassimpática.  Conclusão: Neuropatia de fibras finas permanece como um diagnóstico desafiador na literatura, podendo estar presente em formas leves, mesmo em doenças com envolvimento predominante do SNC. Na DW também há relatos desse acometimento neurológico periférico, com predomínio de envolvimento de fibras finas autonômicas de repercussões ainda não completamente compreendidas. A compreensão dessa disfunção ainda não está totalmente esclarecida e ainda há muitas coisas a serem compreendidas sobre a doença de Wilson, portanto faz-se necessário mais estudo sobre essa temática uma vez que ainda é escasso na literatura estudos que abordem esse assunto mesmo havendo aumento do número de casos diagnosticados com doença de Wilson e que apresentam queixas sensitivas associadas.

Palavras chave: Neuropatia de fibras finas, Doença de Wilson, Doenças do sistema nervoso periférico, Neuropatias periféricas

 

Abstract

Introduction: Peripheral neuropathy is a very common neurological disorder and its prevalence is increasing due to the aging process of the population and the increase in comorbidities such as diabetes and obesity. Fine fiber peripheral neuropathy (NFF) is considered a syndrome that presents isolated sensory manifestations, or a combination of sensory and autonomic manifestations. Wilson's disease (WD) is an autosomal recessive disorder caused by impaired copper metabolism due to mutations in the ATP7B gene. Among the neurological alterations presented by patients with WD is peripheral neuropathy. Objective: To review the literature about thin fiber neuropathy in Wilson's Disease, increasing the understanding of this dysfunction about its causes and types of approaches presented in the literature. Methods: A literature review was carried out based on the data survey present in the Scielo and PUBMED databases from January to June 2021, using the descriptors: "Small Fiber Neuropathy", "Wilson's Disease", "Peripheral Neuropathy". Results: 29 articles were selected, whose For a better understanding, the results found in the literature review were divided into topics.From 29 recruited articles, 15 were excluded, as they had other underlying diseases that could justify the presence of neuropathy in addition to Wilson's disease. Of the 14 articles included in the research: 3 described the presence of fine fiber neuropathy in WD, 4 articles highlighted the presence of mild axonal sensory motor polyneuropathy in WD, 7 articles showed evidence of autonomic neuropathy, 2 with predominantly sympathetic dysfunction and 1 parasympathetic. Conclusion: Fine fiber neuropathy remains a challenging diagnosis in the field. and may be present in mild forms, even in diseases with predominant involvement of the CNS. In WD there are also reports of this peripheral neurological involvement, with a predominance of involvement of thin autonomic fibers with repercussions that are not yet completely understood. The understanding of this dysfunction is still not fully clarified and there are still many things to be understood about Wilson's disease, so further study on this topic is necessary since studies addressing this issue are still scarce in the literature, even with an increase in number of cases diagnosed with Wilson's disease and presenting associated sensory complaints.

Keywords: Small fiber neuropathy, Wilson disease, Peripheral nervous system diseases, Peripheral neuropathies

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2021-11-29

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