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使用角膜共聚焦显微镜评估慢性炎症性脱髓鞘性多发性神经病中的轴突病理学和疾病进展。

Assessing axonal pathology and disease progression in chronic inflammatory demyelinating polyneuropathy using corneal confocal microscopy.

作者信息

Klimas Rafael, Sturm Dietrich, Altenborg Annika, Stylianou Nayia, Huckemann Sophie, Gasz Zornitsa, Grüter Thomas, Philipps Jörg, Greiner Tineke, Maier Christoph, Eitner Lynn, Enax-Krumova Elena, Vorgerd Matthias, Schwenkreis Peter, Gold Ralf, Fisse Anna Lena, Motte Jeremias, Pitarokoili Kalliopi

机构信息

Department of Neurology, St. Josef-Hospital, Ruhr-University, Gudrunstrasse 56, 44791, Bochum, Germany.

Immunmediated Neuropathies Biobank (INHIBIT), Ruhr-University, Bochum, Germany.

出版信息

J Neurol. 2024 Dec 12;272(1):51. doi: 10.1007/s00415-024-12812-4.

Abstract

OBJECTIVE

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an autoimmune neuropathy characterized by progressive or relapsing-remitting weakness and sensory deficits. This study aims to evaluate the utility of corneal confocal microscopy (CCM) in diagnosing and monitoring CIDP.

METHODS

We analysed 100 CIDP patients and 31 healthy controls using CCM to measure corneal nerve fiber density (CNFD), length (CNFL), and branch density (CNBD). Standardized clinical and electroneurographic evaluation were conducted, and statistical analyses were performed to compare CCM parameters between groups and across disease stages.

RESULTS

CIDP patients and subgroups exhibited significant reduction in CNFD, CNFL, and CNBD compared to controls. This reduction was observed in late disease stages and severe overall disability sum score (ODSS), and Inflammatory Neuropathy Cause and Treatment Sensory Sum Score (ISS). CCM parameters correlated with axonal pathology in electroneurography of sensory, but not motor nerves. Despite the significant differences, the diagnostic sensitivity (41%) and specificity (77%) of CCM parameters were limited.

CONCLUSION

While CCM effectively differentiates CIDP patients from healthy controls and was associated with disease severity, its diagnostic accuracy for routine clinical use is a posteriori. However, CCM shows promise as a non-invasive tool for monitoring sensory axonal pathology in CIDP.

摘要

目的

慢性炎症性脱髓鞘性多发性神经根神经病(CIDP)是一种自身免疫性神经病,其特征为进行性或复发-缓解性肌无力和感觉障碍。本研究旨在评估角膜共焦显微镜检查(CCM)在CIDP诊断和监测中的应用价值。

方法

我们使用CCM分析了100例CIDP患者和31名健康对照者,以测量角膜神经纤维密度(CNFD)、长度(CNFL)和分支密度(CNBD)。进行了标准化的临床和神经电生理评估,并进行统计分析以比较各组之间以及不同疾病阶段的CCM参数。

结果

与对照组相比,CIDP患者及其亚组的CNFD、CNFL和CNBD均显著降低。在疾病晚期以及严重总体残疾总分(ODSS)和炎性神经病病因与治疗感觉总分(ISS)中均观察到这种降低。CCM参数与感觉神经而非运动神经的神经电生理检查中的轴索性病理改变相关。尽管存在显著差异,但CCM参数的诊断敏感性(41%)和特异性(77%)有限。

结论

虽然CCM能有效区分CIDP患者与健康对照者,并与疾病严重程度相关,但其在常规临床应用中的诊断准确性是事后的。然而,CCM有望成为监测CIDP感觉轴索性病理改变的非侵入性工具。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1969/11638281/1b9373cec1a8/415_2024_12812_Fig1_HTML.jpg

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