Sturm Dietrich, Güttsches Anne-Katrin, Vorgerd Matthias, Schwenkreis Peter, Westermann Andrea, Greiner Tineke, Vollert Jan, Eitner Lynn, Maier Christoph, Forsting Johannes, Enax-Krumova Elena
Department of Neurology, Agaplesion Bethesda Krankenhaus, Wuppertal, Germany.
Department of Neurology, BG-University Hospital Bergmannsheil, Ruhr University Bochum, Bochum, Germany.
Eur J Neurol. 2025 Aug;32(8):e70294. doi: 10.1111/ene.70294.
The extent of peripheral nerve involvement in inclusion body myositis (IBM) remains a topic of ongoing scientific discussion. In this study, we aimed to explore the involvement of small nerve fibers in IBM using a range of diagnostic methods, including, for the first time, corneal confocal microscopy (CCM).
Nineteen clinic-pathologically or clinically defined IBM patients underwent comprehensive clinical and electrophysiological examinations. A multimodal small fiber examination was performed, including a skin biopsy of the thigh and lower leg, quantitative sensory testing of the feet, and CCM. Pain levels and quality of life were also assessed using standardized questionnaires (Small Fiber Neuropathy Screening List, PainDETECT, Brief Pain Inventory, Short Form-36).
The motor and sensory neurography identified large fiber neuropathy in 67% of cases. Regarding small fibers evaluation, skin biopsy revealed pathological findings in 72% of cases and CCM in 32% of cases. Quantitative sensory testing results predominantly indicated large fiber damage (61%), with small fiber pathology identified in only 12% of cases. Questionnaire responses suggested a slight pain-related impact on patients' quality of life.
Small nerve fiber pathology is a frequent finding in skin biopsies of IBM patients. However, noninvasive methods like CCM appear less sensitive than skin biopsy for diagnosing small fiber pathology in IBM patients. Further studies are needed to refine diagnostic approaches and better understand the role of small fiber involvement in IBM.
包涵体肌炎（IBM）中周围神经受累的程度仍是一个持续进行科学讨论的话题。在本研究中，我们旨在使用一系列诊断方法，首次包括角膜共焦显微镜检查（CCM），来探究IBM中小神经纤维的受累情况。
19例经临床病理或临床确诊的IBM患者接受了全面的临床和电生理检查。进行了多模式小纤维检查，包括大腿和小腿的皮肤活检、足部的定量感觉测试以及CCM。还使用标准化问卷（小纤维神经病变筛查清单、疼痛检测量表、简明疼痛问卷、简短健康调查量表-36）评估了疼痛程度和生活质量。
运动和感觉神经电图检查在67%的病例中发现了大纤维神经病变。关于小纤维评估，皮肤活检在72%的病例中发现了病理结果，CCM在32%的病例中发现了病理结果。定量感觉测试结果主要表明大纤维损伤（61%），仅在12%的病例中发现了小纤维病变。问卷回答表明疼痛对患者生活质量有轻微影响。
小神经纤维病变在IBM患者的皮肤活检中是常见发现。然而，像CCM这样的非侵入性方法在诊断IBM患者的小纤维病变方面似乎不如皮肤活检敏感。需要进一步研究以完善诊断方法，并更好地理解小纤维受累在IBM中的作用。
