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BACKGROUND: The spectrum of autoimmune neurological diseases associated with voltage-gated potassium channel (VGKC)-complex antibodies (Abs) ranges from peripheral nerve disorders to limbic encephalitis. Recently, low titers of VGKC-complex Abs have also been reported in neurodegenerative disorders, but their clinical relevance is unknown. OBJECTIVE: The aim of the study was to explore the prevalence of VGKC-complex Abs in slow-progression motor neuron disease (MND). METHODS: We compared 11 patients affected by slow-progression MND with 9 patients presenting typical progression illness. Sera were tested for VGKC-complex Abs by radioimmunoassay. The distribution of VGKC-complex Abs was analyzed with the Mann-Whitney U test. RESULTS: The statistical analysis showed a significant difference between the mean values in the study and control groups. A case with long-survival MND harboring VGKC-complex Abs and treated with intravenous immunoglobulins is described. CONCLUSION: Although VGKC-complex Abs are not likely to be pathogenic, these results could reflect the coexistence of an immunological activation in patients with slow disease progression.

Original publication

DOI

10.1159/000447715

Type

Journal article

Journal

Neurodegener Dis

Publication Date

2017

Volume

17

Pages

59 - 62

Keywords

Adult, Aged, Aged, 80 and over, Autoantibodies, Biomarkers, Disease Progression, Female, Humans, Immunoglobulins, Intravenous, Immunologic Factors, Male, Middle Aged, Motor Neuron Disease, Potassium Channels, Voltage-Gated, Retrospective Studies