Misdiagnosis and diagnostic pitfalls of chronic inflammatory demyelinating polyradiculoneuropathy

Merel C. Broers, Carina Bunschoten, Judith Drenthen, Tiago A.O. Beck, Esther Brusse, Hester F. Lingsma, Jeffrey A. Allen, Richard A. Lewis, Pieter A. van Doorn, Bart C. Jacobs

Research output: Contribution to journalArticlepeer-review

27 Scopus citations

Abstract

Background and purpose: The aim of this study was to determine the frequency of over- and underdiagnosis of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and to identify related diagnostic pitfalls. Methods: We conducted a retrospective study in Dutch patients referred to the Erasmus University Medical Centre Rotterdam between 2011 and 2017 with either a diagnosis of CIDP or another diagnosis that was revised to CIDP. We used the European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) 2010 diagnostic criteria for CIDP to classify patients into three groups: overdiagnosis, underdiagnosis, or confirmed diagnosis of CIDP. Clinical and laboratory features and treatment history were compared between groups. Results: A referral diagnosis of CIDP was revised in 32% of patients (31/96; overdiagnosis). Of 81 patients diagnosed with CIDP, 16 (20%) were referred with another diagnosis (underdiagnosis). In the overdiagnosed patients, 20% of muscle weakness was asymmetric, 48% lacked proximal muscle weakness, 29% only had distal muscle weakness, 65% did not fulfil the electrodiagnostic criteria for CIDP, 74% had an elevated cerebrospinal fluid (CSF) protein level, and 97% had another type of neuropathy. In the underdiagnosed patients, all had proximal muscle weakness, 50% had a clinically atypical CIDP, all fulfilled the electrodiagnostic criteria for CIDP, and 25% had an increased CSF protein level. Conclusion: Over- and underdiagnosis of CIDP is common. Diagnostic pitfalls include lack of attention to proximal muscle weakness as a diagnostic hallmark of CIDP, insufficient recognition of clinical atypical phenotypes, overreliance on CSF protein levels, misinterpretation of nerve conduction studies and poor adherence to electrodiagnostic criteria, and failure to exclude other causes of polyneuropathy.

Original languageEnglish (US)
Pages (from-to)2065-2073
Number of pages9
JournalEuropean Journal of Neurology
Volume28
Issue number6
Early online dateMar 3 2021
DOIs
StatePublished - Jun 2021

Bibliographical note

Publisher Copyright:
© 2021 The Authors. European Journal of Neurology published by John Wiley & Sons Ltd on behalf of European Academy of Neurology.

Keywords

  • CIDP
  • cerebrospinal fluid
  • chronic inflammatory demyelinating polyradiculoneuropathy
  • diagnostic errors
  • overdiagnosis
  • peripheral nervous system diseases
  • underdiagnosis

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