title: Clinical significance of cation channel antibodies in motor neuron disease.
abstract: Antibodies against cation channels, including voltage-gated potassium channel (VGKC) complex, voltage-gated calcium channel (VGCC), and ganglionic acetylcholine receptor (gAChR), are detected in subgroups of autoimmune disorders, and rarely occur in motor neuron disease (MND).
  
  
  
  This investigation was a case-control study of 28 MND patients positive for cation channel antibodies in comparison with 56 age/gender/onset/diagnostic-category-matched MND patients without such antibodies.
  
  
  
  One or more cation channel antibodies were detected in 6.9% of MND patients, mostly at low titers. The rate of MND progression determined by the revised ALS Functional Rating Scale-revised (ALSFRS-R) and Kaplan-Meier survival analysis was statistically indistinguishable between the antibody-positive and control groups. Incidence rates of cancer and coexisting autoimmune disorders were similar between both groups, based on non-comprehensive screening.
  
  
  
  Cation channel antibodies in MND patients do not appear to affect disease progression. Routine testing for paraneoplastic antibodies is probably of limited usefulness in most MND patients. Muscle Nerve 54: 228-231, 2016.
authors.affiliation: Department of Neurology, Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, Ohio, 44195, USA.
authors.forename: Rachel
authors.initials: R
authors.lastname: Donaldson
authors.affiliation: Department of Quantitative Health Sciences, Learner Research Institute, Cleveland Clinic Foundation, Cleveland, Ohio, USA.
authors.forename: Jianbo
authors.initials: J
authors.lastname: Li
authors.affiliation: Department of Neurology, Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, Ohio, 44195, USA.
authors.forename: Yuebing
authors.initials: Y
authors.lastname: Li
mesh_terms: D001323:Autoantibodies / Q000097:blood*; D016022:Case-Control Studies; D005260:Female; D006801:Humans; D007473:Ion Channels / Q000276:immunology*; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D016472:Motor Neuron Disease / Q000097:blood* / Q000276:immunology / Q000401:mortality; D012189:Retrospective Studies
chemical_list: D001323:Autoantibodies; D007473:Ion Channels
keywords: amyotrophic lateral sclerosis; autoimmunity; ganglionic acetylcholine receptor antibody; motor neuron disease; voltage-gated calcium channel antibody; voltage-gated potassium channel complex antibody