用医学研究委员会量表对特发性炎性肌病不同亚组的肌肉测试进行详细分析

IF 2.8 4区医学 Q2 CLINICAL NEUROLOGY

Neuromuscular Disorders Pub Date : 2025-09-01 DOI:10.1016/j.nmd.2025.105518

F. Hakim , M. Snoussi , S. Sakka , S. Daoud , N. Bouattour , K. Moalla , N. Charfi , S. Marzouk , M. Damak

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引用次数: 0

摘要

特发性炎症性肌病（IIM）是罕见的自身免疫性疾病，在不同亚群中有不同的肌肉和器官受累。不同亚组之间肌肉损伤的可变性仍然知之甚少。本研究采用医学研究理事会（MRC）量表分析IIM亚群的肌肉受累情况。在突尼斯Sfax成人神经内科进行了一项回顾性研究，涉及根据2017年ACR-EULAR标准或2018年ENMC皮肌炎标准诊断为特发性炎症性肌病（IIM）的患者。患者分为五组：皮肌炎（DM）、多发性肌炎（PM）、重叠肌炎（OM）、包涵体肌炎（IBM）和免疫介导的坏死性肌病（IMNM）。收集并分析流行病学和临床资料。使用MRC量表对每块肌肉进行肌肉力量的详细评估，然后按区域（近端或远端）和分布（前、后、内、外侧）分组。然后在前面提到的分类组中比较每个地区和分布。共纳入101例患者：DM 46例，OM 34例，PM 14例，IBM 6例，IMNM 1例。平均年龄44.91岁（SD = 19.15，范围3 ~ 91岁），性别比0.4。不对称弱点与IBM诊断相关（50%,p=0.04）。PM表现出最严重的上肢和下肢近端无力（近端MRC中位数评分：3.35,p = 0.03）。DM患者下肢近端无力最轻（近端MRC中位数评分：4.09,p < 0.01），而IBM患者上肢近端无力最轻（平均MRC评分：4.85,p=0.089）。IBM患者的上肢和下肢远端无力最为明显（MRC平均评分：3.75,p=0.048）。亚组之间关于前、后、内、外侧缺陷的分布没有明显的相关性。本研究强调了IIM亚组中肌肉受累程度的显著差异，强调了精确的肌肉测试在诊断和分类中的重要性。

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55VPDetailed analysis of muscle testing using the medical research council scale in different subgroups of idiopathic inflammatory myopathies

Idiopathic inflammatory myopathies (IIM) are rare autoimmune disorders with variable muscle and organ involvement across subgroups. The variability in muscle impairment between different subgroups remains poorly understood. This study utilizes the Medical Research Council (MRC) scale to analyze muscle involvement among IIM subgroups. A retrospective study was conducted in the adult neurology Dept Sfax, Tunisia, involving patients diagnosed with idiopathic inflammatory myopathies (IIM) based on the 2017 ACR-EULAR criteria or the 2018 ENMC criteria for dermatomyositis. Patients were classified into five groups: dermatomyositis (DM), polymyositis (PM), overlap myositis (OM), inclusion body myositis (IBM), and immune-mediated necrotizing myopathy (IMNM). Epidemiological and clinical data were collected and analyzed. Muscle strength was assessed in detail using the MRC scale for each individual muscle, then grouped by region (proximal or distal) and by distribution (anterior, posterior, medial, and lateral). Each region and distribution was then compared across the previously mentioned classification groups. A total of 101 patients were included: 46 DM, 34 OM, 14 PM, 6 IBM, and 1 IMNM. The mean age was 44.91 years (SD = 19.15, range 3–91 years), with a sex ratio of 0.4. Asymmetric weakness correlated with IBM diagnosis (50%, p=0.04). PM exhibited the most severe proximal weakness in both upper and lower limbs (Median proximal MRC score: 3.35, p = 0.03). DM had the least severe proximal weakness in the lower limbs (Median proximal MRC score: 4.09, p < 0.01), while IBM displayed the least severe proximal weakness in the upper limbs (mean MRC score: 4.85, p=0.089). IBM had the most pronounced distal weakness in both upper and lower limbs (mean MRC score: 3.75, p=0.048). No significant correlation was observed between the subgroups regarding the distribution of anterior, posterior, medial, or lateral deficits. This study highlights significant differences in muscle involvement severity among IIM subgroups, emphasizing the importance of precise muscle testing in diagnosis and classification.

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来源期刊

Neuromuscular Disorders 医学-临床神经学

CiteScore

4.60

自引率

3.60%

发文量

543

审稿时长

53 days

期刊介绍： This international, multidisciplinary journal covers all aspects of neuromuscular disorders in childhood and adult life (including the muscular dystrophies, spinal muscular atrophies, hereditary neuropathies, congenital myopathies, myasthenias, myotonic syndromes, metabolic myopathies and inflammatory myopathies). The Editors welcome original articles from all areas of the field: • Clinical aspects, such as new clinical entities, case studies of interest, treatment, management and rehabilitation (including biomechanics, orthotic design and surgery). • Basic scientific studies of relevance to the clinical syndromes, including advances in the fields of molecular biology and genetics. • Studies of animal models relevant to the human diseases. The journal is aimed at a wide range of clinicians, pathologists, associated paramedical professionals and clinical and basic scientists with an interest in the study of neuromuscular disorders.