Electrodiagnostic criteria for acute and chronic inflammatory demyelinating polyradiculoneuropathy

TLDR

Electrodiagnosis is crucial for early detection of inflammatory demyelinating polyradiculoneuropathies, as timely treatment reduces morbidity and disability. The study aims to define electrodiagnostic criteria that are highly specific for primary demyelination yet sufficiently sensitive for clinical use. The authors compared ten published criterion sets in 53 Guillain–Barré syndrome and 28 chronic inflammatory demyelinating polyradiculoneuropathy patients, and tested specificity in 40 amyotrophic lateral sclerosis and 32 diabetic polyneuropathy patients. Sensitivity of the criteria ranged from 24–83% (mean 54.3%) in GBS and 39–89% (mean 64.9%) in CIDP; nine sets achieved 100% specificity for ALS and 97% for one set, while 3–66% of DPN patients met criteria in eight sets; the authors propose a new set with 72% GBS and 75% CIDP sensitivity and 100% specificity for ALS and DPN, showing that most but not all patients can be electrodiagnostically identified. Published in Muscle Nerve 29: 565–574, 2004.

Abstract

Abstract Electrodiagnosis plays an important role in the early detection and characterization of inflammatory demyelinating polyradiculoneuropathies, because timely treatment reduces morbidity and disability. The challenge consists of defining electrodiagnostic criteria that are highly specific for primary demyelination but sufficiently sensitive to be useful in clinical practice. We compared 10 published sets of criteria in 53 patients with demyelinating Guillain–Barré syndrome (GBS) and 28 with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Specificity of criteria sets was tested in 40 patients with amyotrophic lateral sclerosis (ALS) and 32 with diabetic polyneuropathy (DPN). Sensitivity ranged from 24 to 83% (mean, 54.3%) in GBS and 39 to 89% (mean, 64.9%) in CIDP. With regard to ALS, specificity was 100% for nine sets but was 97% in one. In contrast, 3–66% of DPN patients fulfilled criteria in eight of ten sets. We propose a set of criteria with 72% and 75% sensitivity in our GBS and CIDP patient series, respectively, and 100% specificity with regard to ALS and DPN. Our data illustrate that most, but not all, patients can be electrodiagnostically ascertained. Muscle Nerve 29: 565–574, 2004