Tips in navigating the diagnostic complexities of chronic inflammatory demyelinating polyradiculoneuropathy

Abstract

The 2021 guideline of the European Academy of Neurology/Peripheral Nerve Society on chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) includes important revisions to the previous 2010 guideline. This article highlights the new criteria and recommendations for the differential diagnosis of CIDP. In the revised guideline, the CIDP spectrum has been modified to include typical CIDP and four well-characterized CIDP variants, namely distal, multifocal/focal, motor and sensory CIDP, replacing the term 'atypical' CIDP. To improve the diagnosis of CIDP, the revised guideline attempts to improve the specificity of the diagnostic criteria for typical CIDP and the four CIDP variants. Specific clinical and electrodiagnostic (including both motor and sensory conduction) criteria are provided for typical CIDP and each of the CIDP variants. The levels of diagnostic certainty have been changed to CIDP and possible CIDP, with the removal of probable CIDP (due to the lack of difference in the accuracy of the electrodiagnostic criteria for probable CIDP) and definite CIDP (due to the lack of a gold standard for diagnosis). If the clinical and electrodiagnostic criteria allow only for a diagnosis of possible CIDP, cerebrospinal fluid analysis, nerve ultrasound, nerve magnetic resonance imaging, objective treatment response, and nerve biopsy can be used as supportive criteria to upgrade the diagnosis to CIDP. Although the revised guideline needs to be validated and its strengths and weaknesses assessed, using the guideline will likely improve the accuracy of diagnosis of CIDP and variants of CIDP, and aid in distinguishing CIDP from conditions with similar features.

Keywords: Biomarkers; Chronic inflammatory demyelinating polyradiculoneuropathy; Clinical outcome measures; Diagnosis; Disease activity; Impairment.