Universidade Federal do Paraná, Hospital de Clínicas, Departamento de Medicina Interna, Divisão de Neurologia, Serviço de Doenças Neuromusculares, Curitiba PR, Brazil.
Universidade Federal do Paraná, Curitiba PR, Brazil.
Arq Neuropsiquiatr. 2022 Mar;80(3):270-279. doi: 10.1590/0004-282X-ANP-2020-0561.
Polyneuropathies are characterized by a symmetrical impairment of the peripheral nervous system, resulting in sensory, motor and/or autonomic deficits. Due to the heterogeneity of causes, an etiological diagnosis for polyneuropathy is challenging.
The aim of this study was to determine the main causes of polyneuropathy confirmed by electrodiagnostic (EDX) tests in a tertiary service and its neurophysiological aspects.
This observational cross-sectional study from a neuromuscular disorders center included individuals whose electrodiagnostic tests performed between 2008 and 2017 confirmed a diagnosis of polyneuropathy. Through analysis of medical records, polyneuropathies were classified according to etiology and neurophysiological aspect.
Of the 380 included patients, 59.5% were male, with a median age of 43 years. The main etiologies were: inflammatory (23.7%), hereditary (18.9%), idiopathic (13.7%), multifactorial (11.1%), and diabetes (10.8%). The main electrophysiological patterns were axonal sensorimotor polyneuropathy (36.1%) and "demyelinating and axonal" sensorimotor polyneuropathy (27.9%). Axonal patterns showed greater etiological heterogeneity, with a predominance of idiopathic and multifactorial polyneuropathy, while demyelinating and "demyelinating and axonal" polyneuropathies had a significantly fewer etiologies, with a predominance of hereditary and inflammatory polyneuropathies.
The main causes of polyneuropathy confirmed by EDX test in this study were those that presented a severe, atypical and/or rapidly progressing pattern. Other causes were hereditary and those that defy clinical reasoning, such as multiple risk factors; some polyneuropathies did not have a specific etiology. EDX tests are useful for etiological diagnosis of rare polyneuropathies, because neurophysiological patterns are correlated with specific etiologies.
多发性神经病的特征是周围神经系统的对称性损害,导致感觉、运动和/或自主神经缺陷。由于病因的异质性,多发性神经病的病因诊断具有挑战性。
本研究旨在确定三级服务中经电诊断(EDX)检查证实的多发性神经病的主要原因及其神经生理学特征。
本研究为肌病神经病学中心的观察性横断面研究,纳入了 2008 年至 2017 年间电诊断检查证实为多发性神经病的患者。通过病历分析,根据病因和神经生理学特征对多发性神经病进行分类。
380 例患者中,59.5%为男性,中位年龄为 43 岁。主要病因包括:炎症性(23.7%)、遗传性(18.9%)、特发性(13.7%)、多因素(11.1%)和糖尿病(10.8%)。主要的电生理模式为轴索性感觉运动多发性神经病(36.1%)和“脱髓鞘和轴索性”感觉运动多发性神经病(27.9%)。轴索性表现出更大的病因异质性,特发性和多因素多发性神经病更为常见,而脱髓鞘和“脱髓鞘和轴索性”多发性神经病的病因明显较少,以遗传性和炎症性多发性神经病为主。
本研究中经 EDX 检查证实的多发性神经病的主要原因是那些表现出严重、非典型和/或快速进展的模式。其他原因包括遗传性和那些无法用临床推理解释的原因,如多种危险因素;一些多发性神经病没有特定的病因。EDX 检查有助于罕见多发性神经病的病因诊断,因为神经生理模式与特定病因相关。