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

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2024年12月12日

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

摘要

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.