Metabolic Syndrome, Neurotoxic 1-Deoxysphingolipids and Nervous Tissue Inflammation in Chronic Idiopathic Axonal Polyneuropathy (CIAP)

• Published in: PloS one 2017-01, Vol.12 (1), p.e0170583
• Main Authors: Hube, Larissa, Dohrn, Maike F, Karsai, Gergely, Hirshman, Sarah, Van Damme, Philip, Schulz, Jörg B, Weis, Joachim, Hornemann, Thorsten, Claeys, Kristl G
• Format: Article
• Language: English
• Subjects: Aged; Axons - pathology; Basal lamina; Biology and Life Sciences; Biopsy; Case-Control Studies; CD8 antigen; Chronic Disease; Complications and side effects; Diabetes; Diabetes mellitus; Diabetic Neuropathies - complications; Diabetic Neuropathies - metabolism; Diabetic Neuropathies - pathology; Diabetic neuropathy; Enzymes; Female; Hospitals; Humans; Hypercholesterolemia; Inflammation; Laboratories; Male; Medicine and Health Sciences; Metabolic syndrome; Metabolic Syndrome - complications; Metabolic Syndrome - metabolism; Metabolic Syndrome - pathology; Metabolic syndrome X; Metabolites; Middle Aged; Neurology; Neuropathology; Neurosciences; Neurotoxicity; Pain; Patients; Peripheral neuropathy; Physiology; Polyneuropathies; Polyneuropathies - complications; Polyneuropathies - metabolism; Polyneuropathy; Risk factors; Sphingolipids; Sphingolipids - metabolism; Studies; Sural nerve
• ISSN: 1932-6203; 1932-6203
• DOI: 10.1371/journal.pone.0170583

Chronic idiopathic axonal polyneuropathy (CIAP) is a slowly progressive, predominantly sensory, axonal polyneuropathy, with no aetiology being identified despite extensive investigations. We studied the potential role of the metabolic syndrome, neurotoxic 1-deoxysphingolipids (1-deoxySLs), microangiopathy and inflammation in sural nerve biopsies. We included 30 CIAP-patients, 28 with diabetic distal symmetrical polyneuropathy (DSPN) and 31 healthy controls. We assessed standardised scales, tested for the metabolic syndrome, measured 1-deoxySLs in plasma, performed electroneurography and studied 17 sural nerve biopsies (10 CIAP; 7 DSPN). One third of the CIAP-patients had a metabolic syndrome, significantly less frequent than DSPN-patients (89%). Although the metabolic syndrome was not significantly more prevalent in CIAP compared to healthy controls, hypercholesterolemia did occur significantly more frequent. 1-deoxySLs were significantly and equally elevated in both patient groups compared to healthy controls. Mean basal lamina thickness of small endoneurial vessels and the number of CD68- or CD8-positive cells in biopsies of CIAP- and DSPN-patients did not differ significantly. However, the number of leucocyte-common-antigen positive cells was significantly increased in CIAP. A non-significant trend towards a higher occurrence of the metabolic syndrome in CIAP-patients compared to healthy controls was found. 1-deoxySLs were significantly increased in plasma of CIAP-patients. Microangiopathy and an inflammatory component were present in CIAP-biopsies.