Abstract
Background The European Academy of Neurology/Peripheral Nerve Society (EAN/PNS) 2021 revision of the diagnostic criteria for Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP) incorporated clinical phenotypes including absence or reduced reflexes before the application of electrodiagnostic criteria.1
Objective To identify any CIDP patients who do not meet clinical criteria but satisfy electrodiagnostic criteria.
Method We applied the 2021 EAN/PNS criteria to our cohort of 39 patients receiving intravenous immunoglobulin (IVIG) for CIDP. We compared clinical and electrodiagnostic criteria between those who fulfilled electrodiagnostic criteria but not clinical criteria.
Results Of our cohort 39-patients, 21 (53.8%) were classified as CIDP, 6 (15.3%) as possible CIDP and 12 patients did not meet criteria. Of the 10 patients who did not meet clinical criteria, 6/10 (60%) met electrodiagnostic criteria. These patients did not meet clinical criteria due to the presence of reflexes. There were no significant differences in the clinical phenotypes and demographics between these patients and CIDP or possible CIDP patients in our cohort. There was also no significant difference in the electrodiagnostic parameters between these patients and those with CIDP or possible CIDP. However the patients with preserved reflexes tended to have more frequent conduction velocity slowing (p = 0.08) than patients with CIDP or possible CIDP.
Conclusion The presence of reflexes should not exclude the diagnosis of CIDP if the patient meets electrodiagnostic criteria. This could be indicative of a subset of patients with CIDP who have more involvement of the intermediate segments of nerve rather than proximal segments.
Reference
Van den Bergh PYK, van Doorn PA, Hadden RDM, et al. European academy of neurology/peripheral nerve society guideline on diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy: report of a joint task force-second revision. J Peripher Nerv Syst 2021;26:242–68. doi:10.1111/jns.12455 pmid:http://www.ncbi.nlm.nih.gov/pubmed/34085743