Chronic idiopathic axonal polyneuropathy: Electrophysiological progression and human leukocyte antigen associations. Issue 4 (16th January 2021)

• Record Type: Journal Article
• Authors: Zis, Panagiotis; Sarrigiannis, Ptolemaios G.; Artemiadis, Artemios; Skarlatou, Vasiliki; Hadjivassiliou, Marios
• Is Part Of: Muscle & nerve. Volume 63:Issue 4(2021)
• Page Start: 567
• Page End: 571
• Publication Date: 2021-01-16
• Subjects: CIAP -- HLA genotyping -- idiopathic -- natural history -- peripheral neuropathy; Neuromuscular diseases -- Periodicals; Muscles -- Periodicals; Nerves -- Periodicals; 616.74
• DOI: 10.1002/mus.27164
• Languages: English
• ISSNs: 0148-639X

Abstract

Abstract: Background: We aimed to describe the electrophysiological progression rate of chronic idiopathic axonal polyneuropathy (CIAP) and look into the potential role of human leukocyte antigen (HLA) genetic susceptibility in its development. Methods: We recruited 57 patients with CIAP (mean age at diagnosis 67, mean follow‐up 7 years). The assessments included clinical and electrophysiological data and HLA‐DQ genotyping. Results: The DQA1*05 allele was found more frequently in patients than in healthy controls (odds ratio, 1.96, P = .011). In patients with length‐dependent CIAP, a linear effect of time on the electrophysiological findings was found in the superficial radial (3.2% mean annual decrement, P < .001), sural (4.7% mean annual decrement, P = .002) and tibial nerve (6.1% mean annual decrement, P = .007) amplitudes, independently from age or gender. Conclusions: Patients with length‐dependent CIAP, show a linear progression over time. Interesting associations of HLA‐DQA1*05 allele with length‐dependent CIAP and non‐DQ2/DQ8 with idiopathic sensory ganglionopathy were found. These merit further investigation in larger cohorts and may suggest a role of the immune system in the pathogenesis of CIAP.