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Case Reports
. 2022 Nov;269(11):6139-6144.
doi: 10.1007/s00415-022-11262-0. Epub 2022 Jul 20.

Motor-neuron-disease-like phenotype associated with IgLON5 disease

Sri Raghav Sista  1 , Brian Crum  1 , Albert Aboseif  2 , Michelle F Devine  1 , Anastasia Zekeridou  1   3   4 , M Bakri Hammami  3 , Mohammed M Rezk  3 , André Truffert  5 , Patrice H Lalive  5 , Amy Kunchok  2 , Andrew McKeon  1   3   4 , Divyanshu Dubey  6   7   8
Affiliations
Case Reports

Motor-neuron-disease-like phenotype associated with IgLON5 disease

Sri Raghav Sista et al. J Neurol. 2022 Nov.

Abstract

A growing spectrum of neurological manifestations are being recognized in association with IgLON5 autoimmunity, including recent reports of motor-neuron-disease-like phenotype. Here we describe four cases of IgLON5 autoimmunity with motor neuron involvement and evaluate an additional 109 probable or definite amyotrophic lateral sclerosis cases seen in our neuromuscular clinic for IgLON5-IgG seropositivity. The presence of parasomnias, vocal cord dysfunction or hyperkinetic movements in a patient with motor-neuron-disease-like phenotype should prompt evaluation for IgLON5-IgG autoantibodies. Recognition and treatment of this autoimmune disease with immunosuppressive agents may bring about significant neurological improvement in a minority of cases.

Keywords: Amyotrophic lateral sclerosis; IgLON5 autoimmunity; Motor neuron disease.

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Conflict of interest statement

Conflicts of interest The authors declare that they have no conflict of interest related to the work submitted for publication.

References

    1. Landa J et al. (2020) Effects of IgLON5 antibodies on neuronal cytoskeleton: a link between autoimmunity and neurodegeneration. Ann Neurol 88(5):1023–1027 - PubMed
    1. Sabater L et al. (2014) A novel non-rapid-eye movement and rapideye-movement parasomnia with sleep breathing disorder associated with antibodies to IgLON5: a case series, characterisation of the antigen, and post-mortem study. Lancet Neurol 13(6):575–586 - PMC - PubMed
    1. Gaig C, Compta Y (2019) Neurological profiles beyond the sleep disorder in patients with anti-IgLON5 disease. Curr Opin Neurol 32(3):493–499 - PubMed
    1. Wenninger S (2017) Expanding the clinical spectrum of IgLON5-syndrome. J Neuromuscul Dis 4(4):337–339 - PubMed
    1. Honorat JA et al. (2017) IgLON5 antibody: neurological accompaniments and outcomes in 20 patients. Neurol-Neuroimmunol Neuroinflamm 4(5):e385. - PMC - PubMed

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