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三重阴性肌痛性肌痛症:临床和流行病学特征

Paula Raquel do Vale Pascoal Rodrigues^1,2, Cláudia Suemi Kamoi Kay¹, Renata Dal-Pra Ducci¹

¹Universidade Federal do Paraná, Hospital de Clínicas, Departamento de Clínica Médica, Serviço de Neurologia, Serviço de Doenças Neuromusculares, Curitiba PR, Brazil.

Arquivos de neuro-psiquiatria

|February 5, 2024

概括

三重阴性肌痛性肌痛性肌痛症 (MG) 在巴西很罕见. 这种状况主要影响女性,呈现出阴,双眼视和虚弱,并且通常对治疗反应良好.

科学领域:

神经学神经学
免疫学免疫学免疫学
自免疫性疾病自免疫性疾病

背景情况:

肌痛性肌痛症 (MG) 是一种自身免疫性疾病,通常与抗乙胆受体 (AChR-Abs),肌肉特异性氨酸激酶 (MuSK-Abs) 或低密度脂蛋白受体相关蛋白4 (LRP4-Abs) 的抗体有关.
一部分MG患者,称为三重阴性 (三重SN) MG,缺乏这些特定抗体,这对诊断和治疗提出了挑战.

研究的目的:

在巴西人口中确定三重阴性肌痛性肌痛病 (MG) 的频率.
描述三重SNMG患者的临床和流行病学特征.

主要方法:

一项回顾性横截面研究分析了93名MG患者的医疗记录.
患者根据他们的血清学概况 (AChR-Abs,MuSK-Abs,LRP4-Abs) 进行分类.
使用描述性和分析性统计方法来比较患者子组.

主要成果:

该研究确定了8.6%的MG患者是三重阴性 (n=8).
三重SN MG患者的中位发病年龄为30岁,常见的初始症状包括便死症,双眼视和全身虚弱.
大多数三重SNMG患者出现了轻微症状,表现出充分的治疗反应 (75%),并且与其他MG亚组没有显著差异.

结论:

三重阴性肌痛性肌痛性肌痛性肌痛性肌痛性肌痛性肌痛性肌痛性肌痛性肌痛性肌痛性肌痛性肌痛性肌痛性肌痛性肌痛性肌痛性肌痛性肌痛性肌痛性肌痛性肌痛性肌痛性肌痛性肌痛性肌痛性肌痛性肌痛
这一小组主要包括表现出特定神经症状的女性,并且通常对免疫抑制疗法反应良好.
三重SNMG的临床特征和治疗结果与抗体阳性MG亚组相似.

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