Muscle Nerve. 2022 Sep 23. doi: 10.1002/mus.27726. Online ahead of print.
ABSTRACT
INTRODUCTION/AIMS: Epidermal nerve fiber involvement in chronic inflammatory demyelinating neuropathy (CIDP) has been reported in a limited number of patients. We quantified small fiber involvement in a mixed cohort of patients with typical CIDP and CIDP variants to evaluate relationships with clinical outcome measures at different disease stages.
METHODS: Intraepidermal nerve fiber densities (IENFDs) were evaluated by skin punch biopsies of 23 patients with CIDP and 13 healthy controls at the forearm, thigh, and distal leg. Skin sections were optimally interpreted in all three regions in 16 CIDP patients and 10 age- and sex-matched healthy controls. Statistical analysis was performed in those subjects.
RESULTS: The IENFDs in forearm, thigh and distal leg were similar among 7 typical CIDP and 9 CIDP variants. IENFDs in those regions were significantly reduced in CIDP compared to healthy controls, and showed moderate negative correlation with International Neuropathy Cause and Treatment (INCAT) Upper Limb Functional Disability Scores. Reduction in IENFD compared to controls was more remarkable in the distal leg. In clinically unstable CIDP patients, the IENFDs of distal leg and forearm were significantly reduced compared to stable CIDP and controls. Stable CIDP patients had significantly reduced IENFDs in distal leg and forearm compared to controls.
DISCUSSION: In this exploratory study, we confirm that small fibers are also affected in CIDP. Larger studies are needed to explore longitudinal changes of IENFD in CIDP and its relation to disease stage. This article is protected by copyright. All rights reserved.
PMID:36151750 | DOI:10.1002/mus.27726
