Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2023 Oct 1;29(5):1357-1377.
doi: 10.1212/CON.0000000000001290.

Chronic Immune-Mediated Demyelinating Neuropathies

Karissa Gable

Chronic Immune-Mediated Demyelinating Neuropathies

Karissa Gable. Continuum (Minneap Minn). .

Abstract

Objective: This article is an overview of chronic demyelinating neuropathies and highlights the phenotypic categorization, diagnosis, and treatment of chronic immune-mediated neuropathies. The clinical and diagnostic characteristics of other chronic demyelinating neuropathies that are common mimics of immune-mediated neuropathies are also discussed.

Latest developments: The underlying pathophysiology of chronic demyelinating neuropathies is heterogeneous, and components of both humoral and cellular immune responses are thought to play a role in the immune-mediated types of chronic demyelinating neuropathy. The role of the humoral response is highlighted with a specific focus on the relatively recent discovery of antibody-mediated antinodal and paranodal demyelinating neuropathies. Additionally, new diagnostic criteria for some of the chronic demyelinating neuropathies, as well as ways to differentiate chronic inflammatory demyelinating polyradiculoneuropathy from other chronic demyelinating polyneuropathies, are discussed.

Essential points: Chronic demyelinating neuropathies can present with overlapping clinical characteristics with seemingly subtle variations. It is clinically important to differentiate these types of neuropathies because the treatment and management can vary and affect prognosis.

PubMed Disclaimer

References

    1. Lunn MP, Manji H, Choudhary PP, Hughes RA, Thomas PK. Chronic inflammatory demyelinating polyradiculoneuropathy: a prevalence study in south east England. J Neurol Neurosurg Psychiatry 1999;66(5):677–680. doi:10.1136/jnnp.66.5.677 - DOI
    1. Laughlin RS, Dyck PJ, Melton LJ, et al. Incidence and prevalence of CIDP and the association of diabetes mellitus. Neurology 2009;73(1):39–45. doi:10.1212/WNL.0b013e3181aaea47 - DOI
    1. Bunschoten C, Jacobs BC, Van den Bergh PYK, Cornblath DR, van Doorn PA. Progress in diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy. Lancet Neurol 2019;18(8):784–794. doi:10.1016/S1474-4422(19)30144-9 - DOI
    1. Doneddu PE, Cocito D, Manganelli F, et al. Atypical CIDP: diagnostic criteria, progression and treatment response. Data from the Italian CIDP Database. J Neurol Neurosurg Psychiatry 2019;90(2):125–132. doi:10.1136/jnnp-2018-318714 - DOI
    1. Van den Bergh PYK, van Doorn PA, Hadden RDM, et al. European Academy of Neurology/Peripheral Nerve Society guideline on diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy: report of a joint task force—second revision. J Peripher Nerv Syst 2021;26(3):242–268. doi:10.1111/jns.12455 - DOI

MeSH terms

LinkOut - more resources