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Neuropathy in sporadic inclusion body myositis: a multi-modality neurophysiological study
Clinical Neurophysiology ( IF 4.7 ) Pub Date : 2020-11-01 , DOI: 10.1016/j.clinph.2020.07.025
James H Lee 1 , Robert Boland-Freitas 1 , Christina Liang 1 , James Howells 2 , Karl Ng 3
Affiliation  

OBJECTIVE Sporadic inclusion body myositis (sIBM) has been associated with neuropathy. This study employs nerve excitability studies to re-examine this association and attempt to understand underlying pathophysiological mechanisms. METHODS Twenty patients with sIBM underwent median nerve motor and sensory excitability studies, clinical assessments, conventional nerve conduction testing (NCS) and quantitative thermal threshold studies. These results were compared to established normal controls, or results from a normal cohort of older control individuals. RESULTS Seven sIBM patients (35%) demonstrated abnormalities in conventional NCS, with ten patients (50%) demonstrating abnormalities in thermal thresholds. Median nerve motor and sensory excitability differed significantly in sIBM patients when compared to normal controls. None of these neurophysiological markers correlated significantly with clinical markers of sIBM severity. CONCLUSION A concurrent neuropathy exists in a significant proportion of sIBM patients, with nerve excitability studies revealing changes possibly consistent with axolemmal depolarization or concurrent neuronal adaptation to myopathy. Neuropathy in sIBM does not correlate with muscle disease severity and may reflect a differing tissue response to a common pathogenic factor. SIGNIFICANCE This study affirms the presence of a concurrent neuropathy in a large proportion of sIBM patients that appears independent of the severity of myopathy.

中文翻译:

散发性包涵体肌炎的神经病变:一项多模式神经生理学研究

目的 散发性包涵体肌炎 (sIBM) 与神经病变有关。本研究采用神经兴奋性研究来重新检查这种关联并试图了解潜在的病理生理机制。方法 20 名 sIBM 患者接受了正中神经运动和感觉兴奋性研究、临床评估、常规神经传导测试 (NCS) 和定量热阈值研究。将这些结果与建立的正常对照或来自老年对照个体的正常队列的结果进行比较。结果 七名 sIBM 患者 (35%) 表现出常规 NCS 异常,十名患者 (50%) 表现出热阈值异常。与正常对照相比,sIBM 患者的中位神经运动和感觉兴奋性显着不同。这些神经生理学标志物与 sIBM 严重程度的临床标志物均无显着相关性。结论 相当大比例的 sIBM 患者存在并发神经病变,神经兴奋性研究揭示的变化可能与轴突去极化或并发神经元对肌病的适应一致。sIBM 中的神经病变与肌肉疾病的严重程度无关,可能反映了对常见致病因素的不同组织反应。意义 本研究证实,大部分 sIBM 患者存在并发神经病变,且与肌病的严重程度无关。神经兴奋性研究揭示的变化可能与轴突去极化或并发神经元对肌病的适应一致。sIBM 中的神经病变与肌肉疾病的严重程度无关,可能反映了对常见致病因素的不同组织反应。意义 本研究证实,大部分 sIBM 患者存在并发神经病变,且与肌病的严重程度无关。神经兴奋性研究揭示的变化可能与轴突去极化或并发神经元对肌病的适应一致。sIBM 中的神经病变与肌肉疾病的严重程度无关,可能反映了对常见致病因素的不同组织反应。意义 本研究证实,大部分 sIBM 患者存在并发神经病变,且与肌病的严重程度无关。
更新日期:2020-11-01
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