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Abstract
Objective: We aimed to explore the diagnosis and misdiagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP) and to identify pitfalls that erroneously lead to a misdiagnosis.
Methods: A retrospective study of 59 consecutive patients referred with a diagnosis of CIDP was performed. Patients were classified as having or not having CIDP according to European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) criteria. Diagnostic and treatment data were compared in the 2 groups.
Results: Forty-seven percent of patients referred with a diagnosis of CIDP failed to meet minimal CIDP diagnostic requirements. All misdiagnosed patients who satisfied EFNS/PNS clinical criteria would be considered atypical as defined by the EFNS/PNS. CSF cytoalbuminologic dissociation was present in 50% of those without CIDP, although protein elevations were generally mild. Nerve conduction studies in patients without CIDP were heterogeneous, but generally showed demyelinating features better explained by a process other than CIDP. Patients frequently reported improvements after being treated with immunotherapy, even if the CIDP diagnosis was incorrect.
Conclusions: CIDP misdiagnosis is common. Over-reliance on subjective patient-reported perception of treatment benefit, liberal electrophysiologic interpretation of demyelination, and placing an overstated importance on mild or moderate cytoalbuminologic dissociation are common diagnostic errors. Utilization of clear and objective indicators of treatment efficacy might improve our ability to make informed treatment decisions.
GLOSSARY
- CIDP=
- chronic inflammatory demyelinating polyneuropathy;
- CMAP=
- compound motor action potential;
- CV=
- conduction velocity;
- EFNS/PNS=
- European Federation of Neurological Societies/Peripheral Nerve Society;
- IVIg=
- IV immunoglobulin;
- MAG=
- myelin-associated glycoprotein;
- MMN=
- multifocal motor neuropathy;
- MS=
- multiple sclerosis;
- R-ODS=
- Rasch-built Overall Disability Scale;
- SFN=
- small fiber neuropathy;
- SMA=
- spinal muscular atrophy;
- SPS=
- stiff-person syndrome
Footnotes
Go to Neurology.org for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
Editorial, page 488
- Received December 22, 2014.
- Accepted in final form March 27, 2015.
- © 2015 American Academy of Neurology
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