title: Indications for an immune-mediated etiology of idiopathic sensory neuronopathy.
abstract: To investigate immune mechanisms in the etiology of idiopathic sensory neuronopathy (ISN), we studied neurite outgrowth inhibition and antibody binding to neuronal tissue of serum from 4 patients with ISN. Rat dorsal root ganglion (DRG) cells were cultured in the presence of serum from ISN patients and controls. After 48 h of incubation, neurite outgrowth was quantified with a neurofilament ELISA. Serum from ISN patients significantly inhibited DRG neurite outgrowth compared to controls. ISN serum also strongly immunostained fixed cultured and cryostat rat DRG neurons (at dilutions up to 1:10,240), whereas serum from controls did not. Western blots showed unique binding patterns to DRG proteins in 3 ISN patients compared with controls, but a single band corresponding in all ISN patients was not found. The inhibitory effect of ISN serum on neurite outgrowth and the presence of circulating anti-DRG antibodies in the acute phase of the disease supports an immune-mediated pathogenesis of ISN.
authors.affiliation: Department of Neuromuscular diseases, The Rudolf Magnus Institute of Neurosciences, University Hospital Utrecht, The Netherlands.
authors.forename: G W
authors.initials: GW
authors.lastname: van Dijk
authors.forename: J H
authors.initials: JH
authors.lastname: Wokke
authors.forename: N C
authors.initials: NC
authors.lastname: Notermans
authors.forename: L H
authors.initials: LH
authors.lastname: van den Berg
authors.forename: P R
authors.initials: PR
authors.lastname: Bär
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D000818:Animals; D000917:Antibody Formation; D015153:Blotting, Western; D002478:Cells, Cultured; D005260:Female; D019084:Fluorescent Antibody Technique, Indirect; D005727:Ganglia, Spinal / Q000166:cytology / Q000276:immunology; D006801:Humans; D007106:Immune Sera / Q000494:pharmacology; D008875:Middle Aged; D009422:Nervous System Diseases / Q000097:blood / Q000276:immunology*; D009433:Neural Inhibition; D016501:Neurites / Q000187:drug effects / Q000502:physiology; D051381:Rats / Q000196:embryology; D017208:Rats, Wistar; D012678:Sensation Disorders / Q000097:blood / Q000276:immunology*
chemical_list: D007106:Immune Sera