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
. 2021 Feb;28(2):620-629.
doi: 10.1111/ene.14545. Epub 2020 Oct 15.

Chronic inflammatory demyelinating polyradiculoneuropathy: can a diagnosis be made in patients not fulfilling electrodiagnostic criteria?

G Liberatore  1 , F Manganelli  2 , P E Doneddu  1 , D Cocito  3 , R Fazio  4 , C Briani  5 , M Filosto  6 , L Benedetti  7   8 , A Mazzeo  9 , G Antonini  10 , G Cosentino  11   12   13 , S Jann  14 , A Cortese  12   13   15 , G A Marfia  16 , A M Clerici  17 , G Siciliano  18 , M Carpo  19 , M Luigetti  20 , G Lauria  21   22 , T Rosso  23 , G Cavaletti  24 , L Santoro  2 , E Peci  3 , S Tronci  4 , M Ruiz  5 , S Cotti Piccinelli  6 , A Schenone  7 , L Leonardi  10 , A Toscano  9 , G Mataluni  16 , E Spina  2 , L Gentile  9 , E Nobile-Orazio  1   25 ; Italian CIDP Database Study Group
Collaborators, Affiliations
Free article

Chronic inflammatory demyelinating polyradiculoneuropathy: can a diagnosis be made in patients not fulfilling electrodiagnostic criteria?

G Liberatore et al. Eur J Neurol. 2021 Feb.
Free article

Abstract

Background and purpose: The aim was to identify the clinical and diagnostic investigations that may help to support a diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) in patients not fulfilling the European Federation of Neurological Societies and Peripheral Nerve Society (EFNS/PNS) electrodiagnostic criteria.

Methods: The data from patients with a clinical diagnosis of CIDP included in a national database were retrospectively reviewed.

Results: In all, 535 patients with a diagnosis of CIDP were included. This diagnosis fulfilled the EFNS/PNS criteria in 468 patients (87.2%) (definite in 430, probable in 33, possible in three, while two had chronic immune sensory polyradiculopathy). Sixty-seven patients had a medical history and clinical signs compatible with CIDP but electrodiagnostic studies did not fulfill the EFNS/PNS criteria for CIDP. These patients had similar clinical features and frequency of abnormal supportive criteria for the diagnosis of CIDP compared to patients fulfilling EFNS/PNS criteria. Two or more abnormal supportive criteria were present in 40 (61.2%) patients rising to 54 (80.6%) if a history of a relapsing course as a possible supportive criterion was also included. Increased cerebrospinal fluid proteins and response to immune therapy most frequently helped in supporting the diagnosis of CIDP. Response to therapy was similarly frequent in patients fulfilling or not EFNS/PNS criteria (87.3% vs. 85.9%).

Conclusions: Patients with a clinical diagnosis of CIDP had similar clinical findings, frequency of abnormal supportive criteria and response to therapy compared to patients fulfilling EFNS/PNS criteria. The presence of abnormal supportive criteria may help in supporting the diagnosis of CIDP in patients with a medical history and clinical signs compatible with this diagnosis but non-diagnostic nerve conduction studies.

Keywords: chronic inflammatory demyelinating polyradiculoneuropathy; diagnostic criteria; electrophysiology.

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: 677-680.
    1. Laughlin RS, Dyck PJ, Melton LJ III, Leibson C, Ransom J, Dyck PJ. Incidence and prevalence of CIDP and the associations with diabetes mellitus. Neurology 2009; 73: 39-45.
    1. Koller H, Kieseier BC, Jander S, Hartung HP. Chronic inflammatory demyelinating polyneuropathy. N Engl J Med 2005; 352: 1343-1356.
    1. Vallat JM, Sommer C, Magy L. Chronic inflammatory demyelinating polyradiculoneuropathy: diagnostic and therapeutic challenges for a treatable condition. Lancet Neurol 2010; 9: 402-412.
    1. Nobile-Orazio E. Chronic inflammatory demyelinating polyradiculoneuropathy and variants: where we are and where we should go. J Peripher Nerv Syst 2014; 19: 2-13.

Publication types

MeSH terms