Abstract
AimChronic 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.MethodsWe 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).ResultsOne 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.ConclusionsA 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.
Highlights
Chronic idiopathic axonal polyneuropathy (CIAP) is defined as a slowly progressive, predominantly sensory polyneuropathy with an axonal pattern in nerve conduction studies (NCS), in which all known causes were excluded [1]
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 elevated in both patient groups compared to healthy controls
A non-significant trend towards a higher occurrence of the metabolic syndrome in CIAPpatients compared to healthy controls was found. 1-deoxySLs were significantly increased in plasma of CIAP-patients
Summary
Chronic idiopathic axonal polyneuropathy (CIAP) is defined as a slowly progressive, predominantly sensory polyneuropathy with an axonal pattern in nerve conduction studies (NCS), in which all known causes were excluded [1]. Further arguing for a metabolic source is the clinical similarity of CIAP to diabetic distal symmetrical polyneuropathy (DSPN) [3]. A recent study links DSPN with elevated 1-deoxysphingolipids (1-deoxySLs) in blood [10]. Sphingolipids play an important role in the formation of plasma membranes and lipoproteins; they are usually formed by the precursors L-serine and palmitoyl-CoA. Their condensation is catalysed by the enzyme serine palmitoyltransferase (SPT) leading to the formation of sphinganine. Considering the similarities with DSPN, the presence of 1-deoxySLs might play a role in the pathomechanism of CIAP
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