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
. 2018 Feb 7;8(3):e00932.
doi: 10.1002/brb3.932. eCollection 2018 Mar.

Challenges in the diagnosis of chronic inflammatory demyelinating polyneuropathy

Jeffrey A Allen  1 , Kenneth C Gorson  2 , Deborah Gelinas  3
Affiliations

Challenges in the diagnosis of chronic inflammatory demyelinating polyneuropathy

Jeffrey A Allen et al. Brain Behav. .

Abstract

Introduction: We explored adherence to the European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) guidelines for the diagnosis and treatment of chronic inflammatory demyelinating polyneuropathy (CIDP) by reviewing data from a specialty pharmacy database.

Materials and methods: Clinical and electrophysiologic data were reviewed for 65 consecutive patients treated with intravenous immunoglobulin (IVIG) for CIDP. Three neuromuscular neurologists independently classified cases according to EFNS/PNS criteria as (1) fulfilling CIDP criteria; (2) non-CIDP (neither clinical nor electrophysiologic criteria met); or (3) unknown (insufficient information).

Results: Patients were treated by 31 different community neurologists in 14 states. Only seven patients (11%) met clinical and electrodiagnostic CIDP criteria. The remainder (89%) did not have CIDP (49%) or were unknown (40%). IVIG mean induction dose was 1.25 g/kg, mean maintenance dose 0.79 g/kg, and mean interval between infusions was 23 days.

Conclusions: Adherence to EFNS/PNS CIDP diagnostic and treatment guidelines in the general neurologic community was poor. Improved education and awareness of widely available CIDP guidelines are recommended.

Keywords: chronic inflammatory demyelinating polyneuropathy; clinical practice; diagnosis; guidelines; intravenous immunoglobulin.

PubMed Disclaimer

Figures

Figure 1
Figure 1
Diagnostic prevalence of CIDP. CIDP, chronic inflammatory demyelinating polyneuropathy; MAG, myelin‐associated glycoprotein; MMN, multifocal motor neuropathy
See this image and copyright information in PMC

References

    1. Allen, J. A. , & Lewis, R. A. (2015). CIDP diagnostic pitfalls and perception of treatment benefit. Neurology, 85, 498–504. https://doi.org/10.1212/WNL.0000000000001833 - DOI - PubMed
    1. Breiner, A. , & Brannagan, T. H. 3rd (2014). Comparison of sensitivity and specificity among 15 criteria for chronic inflammatory demyelinating polyneuropathy. Muscle and Nerve, 50, 40–46. https://doi.org/10.1002/mus.24088 - DOI - PubMed
    1. Cornblath, D. R. , Gorson, K. C. , Hughes, R. A. , & Merkies, I. S. (2013). Observations on chronic inflammatory demyelinating polyneuropathy: A plea for a rigorous approach to diagnosis and treatment. Journal of the Neurological Sciences, 330, 2–3. https://doi.org/10.1016/j.jns.2013.04.015 - DOI - PubMed
    1. Hughes, R. A. , Donofrio, P. , Bril, V. , Dalakas, M. C. , Deng, C. , Hanna, K. , … van Doorn, P. A. (2008). Intravenous immune globulin (10% caprylate‐chromatography purified) for the treatment of chronic inflammatory demyelinating polyradiculoneuropathy (ICE study): A randomised placebo‐controlled trial. The Lancet Neurology, 7, 136–144. https://doi.org/10.1016/S1474-4422(07)70329-0 - DOI - PubMed
    1. Laughlin, R. S. , Dyck, P. J. , Melton, L. J. 3rd , Leibson, C. , Ransom, J. , & Dyck, P. J. (2009). Incidence and prevalence of CIDP and the association of diabetes mellitus. Neurology, 73, 39–45. https://doi.org/10.1212/WNL.0b013e3181aaea47 - DOI - PMC - PubMed

Publication types

MeSH terms

Substances

LinkOut - more resources