title: Metabolic Syndrome, Neurotoxic 1-Deoxysphingolipids and Nervous Tissue Inflammation in Chronic Idiopathic Axonal Polyneuropathy (CIAP).
abstract: OBJECTIVE
  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.
  
  
  METHODS
  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).
  
  
  RESULTS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Neurology, RWTH Aachen University, Aachen, Germany.|Institute of Neuropathology, RWTH Aachen University, Aachen, Germany.
authors.forename: Larissa
authors.initials: L
authors.lastname: Hube
authors.affiliation: Department of Neurology, RWTH Aachen University, Aachen, Germany.
authors.forename: Maike F
authors.initials: MF
authors.lastname: Dohrn
authors.affiliation: Center for Integrative Human Physiology, University of Zurich, Zurich, Switzerland.|Institute for Clinical Chemistry, University Hospital, Zurich, Switzerland.
authors.forename: Gergely
authors.initials: G
authors.lastname: Karsai
authors.affiliation: Department of Neurology, RWTH Aachen University, Aachen, Germany.
authors.forename: Sarah
authors.initials: S
authors.lastname: Hirshman
authors.affiliation: Department of Neurology, University Hospitals Leuven, Leuven, Belgium.|Department of Neurosciences, Experimental Neurology, University of Leuven (KU Leuven), Leuven, Belgium.|VIB, Vesalius Research Center, Laboratory of Neurobiology, Leuven, Belgium.
authors.forename: Philip
authors.initials: P
authors.lastname: Van Damme
authors.affiliation: Department of Neurology, RWTH Aachen University, Aachen, Germany.|JARA-Institute Molecular Neuroscience and Neuroimaging, Forschungszentrum Jülich GmbH and RWTH Aachen University, Aachen, Germany.
authors.forename: Jörg B
authors.initials: JB
authors.lastname: Schulz
authors.affiliation: Institute of Neuropathology, RWTH Aachen University, Aachen, Germany.
authors.forename: Joachim
authors.initials: J
authors.lastname: Weis
authors.affiliation: Center for Integrative Human Physiology, University of Zurich, Zurich, Switzerland.|Institute for Clinical Chemistry, University Hospital, Zurich, Switzerland.
authors.forename: Thorsten
authors.initials: T
authors.lastname: Hornemann
authors.affiliation: Department of Neurology, RWTH Aachen University, Aachen, Germany.|Institute of Neuropathology, RWTH Aachen University, Aachen, Germany.|Department of Neurology, University Hospitals Leuven, Leuven, Belgium.|Department of Neurosciences, Experimental Neurology, Laboratory for Muscle Diseases and Neuropathies, University of Leuven (KU Leuven), Leuven, Belgium.
authors.forename: Kristl G
authors.initials: KG
authors.lastname: Claeys
mesh_terms: D000368:Aged; D001369:Axons / Q000473:pathology*; D016022:Case-Control Studies; D002908:Chronic Disease; D003929:Diabetic Neuropathies / Q000150:complications / Q000378:metabolism / Q000473:pathology; D005260:Female; D006801:Humans; D008297:Male; D024821:Metabolic Syndrome / Q000150:complications / Q000378:metabolism / Q000473:pathology*; D008875:Middle Aged; D011115:Polyneuropathies / Q000150:complications / Q000378:metabolism*; D013107:Sphingolipids / Q000378:metabolism*
chemical_list: D013107:Sphingolipids