Introduction: 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).
Methods: 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.
Results: 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.
Conclusion: 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.
Keywords: amyotrophic lateral sclerosis; autoimmunity; ganglionic acetylcholine receptor antibody; motor neuron disease; voltage-gated calcium channel antibody; voltage-gated potassium channel complex antibody.
© 2016 Wiley Periodicals, Inc.