Pathological Findings in Chronic Inflammatory Demyelinating Polyradiculoneuropathy: A Single-Center Experience.

Marco Luigetti,Giulia Bisogni,Angela Romano,Salvatore Rossi,Mario Sabatelli,Andrea Di Paolantonio,Amelia Conte,Francesca Madia

doi:10.3390/brainsci10060383

Abstract

Objective: Segmental demyelination is the pathological hallmark of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), but other elementary lesions are frequently observed, configuring a series of different pathological pictures. In this article, we review the pathological findings of a large series of sural nerve biopsies from our cohort of CIDP patients. Patients and Methods: Patients with CIDP who underwent nerve biopsy were retrospectively selected from those referred to the Institute of Neurology of the “Università Cattolica del Sacro Cuore” in Rome, Italy, from 1982 to February 2020. Sural nerve biopsy was performed according to standard protocols. Results: Sural nerve biopsy was performed in 43/130 CIDP patients. Demyelinating abnormalities and axonal loss were found in 67.4% and 83.7% of biopsies, respectively. Conversely, onion bulbs and inflammatory infiltrates were rare (18.6% and 4.7%, respectively). In three cases, we observed normal pathological findings. Conclusions: A pathognomonic pathological finding of CIDP cannot be established, but we confirm the utility of nerve biopsy in this setting to confirm the diagnosis (also in atypical phenotypes) and to elucidate pathogenic mechanisms.

Highlights

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a clinically heterogeneous, roughly symmetric, sensory and motor neuropathy of likely immune origin [1–3]
We review the pathological findings of a large series of sural nerve biopsies from our cohort of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) patients in order to underline the most frequent pathological alterations and to make a correlation with clinical findings
Obvious demyelinating abnormalities in both semithin sections and teased fiber analysis were found in 16 biopsies (37.2%)

Summary

Introduction

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a clinically heterogeneous, roughly symmetric, sensory and motor neuropathy of likely immune origin [1–3]. Originally coined by Dyck and co-workers [4], summarizes the main clinicopathological features of the disease, its hallmark being inflammation-mediated demyelination [1]. CIDP appears as a mainly motor neuropathy, affecting both distal and proximal muscles of the four limbs, along with sensory involvement and generalized areflexia, evolving as a monophasic, relapsing, or progressive disorder in more than two months. There is a remarkable heterogeneity in clinical presentation, and several variants of CIDP have far been described, all characterized by electrophysiological and/or histopathological features of segmental demyelination [5]. Brain Sci. 2020, 10, 383; doi:10.3390/brainsci10060383 www.mdpi.com/journal/brainsci.

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Conclusion