Chronic idiopathic axonal polyneuropathy: Electrophysiological progression and human leukocyte antigen associations.

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. [ABSTRACT FROM AUTHOR]