Proposed revised electrophysiological criteria for chronic inflammatory demyelinating polyradiculoneuropathy

Abstract

Electrophysiological criteria for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) were proposed by an Ad Hoc Subcommittee of the American Academy of Neurology (AAN) in 1991. Only 60% of CIDP patients fulfilled these criteria, which therefore appear poorly sensitive. We therefore sought to revise the electrophysiological criteria. We selected 40 CIDP patients and compared them with 35 patients with axonal polyneuropathy, 116 patients with Charcot-Marie-Tooth type 1A (CMT1A) disease, and 66 patients with immunoglobulin M (IgM) monoclonal gammopathy. The proposed electrophysiological criteria identified 90% of the CIDP patients, although 3% of patients with axonal polyneuropathy were falsely identified. For the CIDP patients, sensitivity and specificity were 90% and 97%, respectively. Of the patients with IgM monoclonal gammaglobulin of undetermined significance (MGUS) and CMT1A, 100% fulfilled these new criteria, whereas 90% and 97%, respectively, fulfilled the AAN criteria. These results suggest that the AAN criteria are more appropriate for IgM MGUS and CMT1A patients than for CIDP patients. We therefore propose new electrophysiological criteria for CIDP that appear to have better sensitivity.

Incidence and prevalence of CIDP and the association of diabetes mellitus

“…In identifying potential CIDP cases, we emphasized the clinical presentation, as was done in the original large series of 1975 describing CIDP, 4 and then used other characteristics, including electrophysiologic data, as confirmatory features for the diagnosis. We, like others, [21][22][23][24] believed that rigorous electrophysiologic criteria as the initial inclusion tool may be appropriate for therapeutic research trials but may miss clinical cases of CIDP that would skew an epidemiologic study such as ours. The observation that the AAN electrophysiologic criteria 5 only identified 54% of our cases validates our concern and is in keeping with the experience of other authors.…”

A Comparison of Clinically Atypical with Typical Chronic Inflammatory Demyelinating Polyradiculoneuropathy

“…Only 60% of CIDP patients fulfill the electrodiagnostic criteria of the American Academy of Neurology. A higher sensitivity of 90% and specificity of 97% have been reported in later criteria [13]. In clinically atypical CIDP patients, if the electrodiagnostic criteria are not met initially, a repeat nerve conduction study in more nerves, MRI of nerves, roots and plexus and nerve biopsy may be helpful.…”

Chronic inflammatory demyelinating polyneuropathy presenting with autonomic symptoms: a case report

“…Nerve conduction performed at this time revealed classic features of chronic inflammatory demyelinating polyneuropathy. The neurophysiological criteria for diagnosis of CIDP was proposed by Nicolas et al 8 The nerve conduction studies must show one of the following abnormalities in at least three different nerves: conduction block or temporal dispersion in at least 3 different nerves with demyelination evidence in at least one nerve or conduction block or temporal dispersion in at least two different nerves and abnormal conduction values in at least one nerves or conduction block or temporal dispersion in one nerve and abnormal nerve conduction values in at least two other nerves or abnormal conduction values in three different nerves. Our patient had nerve conduction abnormalities in three nerves with conduction block three nerves.…”