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

Sri Raghav Sista; Brian Crum; Albert Aboseif; Michelle F. Devine; Anastasia Zekeridou; M. Bakri Hammami; Mohammed M. Rezk; André Truffert; Patrice H. Lalive; Amy Kunchok; Andrew McKeon; Divyanshu Dubey

doi:10.1007/s00415-022-11262-0

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

Sri Raghav Sista, Brian Crum, Albert Aboseif, Michelle F. Devine, Anastasia Zekeridou, M. Bakri Hammami, Mohammed M. Rezk, André Truffert, Patrice H. Lalive, Amy Kunchok, Andrew McKeon, Divyanshu Dubey

Laboratory Medicine and Pathology

Research output: Contribution to journal › Article › peer-review

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.

Original language	English (US)
Pages (from-to)	6139-6144
Number of pages	6
Journal	Journal of Neurology
Volume	269
Issue number	11
DOIs	https://doi.org/10.1007/s00415-022-11262-0
State	Published - Nov 2022

Keywords

Amyotrophic lateral sclerosis
IgLON5 autoimmunity
Motor neuron disease

ASJC Scopus subject areas

Neurology
Clinical Neurology

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10.1007/s00415-022-11262-0

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title = "Motor-neuron-disease-like phenotype associated with IgLON5 disease",

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",

author = "Sista, {Sri Raghav} and Brian Crum and Albert Aboseif and Devine, {Michelle F.} and Anastasia Zekeridou and Hammami, {M. Bakri} and Rezk, {Mohammed M.} and Andr{\'e} Truffert and Lalive, {Patrice H.} and Amy Kunchok and Andrew McKeon and Divyanshu Dubey",

note = "Publisher Copyright: {\textcopyright} 2022, The Author(s), under exclusive licence to Springer-Verlag GmbH Germany.",

year = "2022",

month = nov,

doi = "10.1007/s00415-022-11262-0",

language = "English (US)",

volume = "269",

pages = "6139--6144",

journal = "Journal of Neurology",

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T1 - Motor-neuron-disease-like phenotype associated with IgLON5 disease

AU - Sista, Sri Raghav

AU - Crum, Brian

AU - Aboseif, Albert

AU - Devine, Michelle F.

AU - Zekeridou, Anastasia

AU - Hammami, M. Bakri

AU - Rezk, Mohammed M.

AU - Truffert, André

AU - Lalive, Patrice H.

AU - Kunchok, Amy

AU - McKeon, Andrew

AU - Dubey, Divyanshu

PY - 2022/11

Y1 - 2022/11

N2 - 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.

AB - 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.

KW - Amyotrophic lateral sclerosis

KW - IgLON5 autoimmunity

KW - Motor neuron disease

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AN - SCOPUS:85134544869

SN - 0340-5354

VL - 269

SP - 6139

EP - 6144

JO - Journal of Neurology

JF - Journal of Neurology

IS - 11

ER -

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

Abstract

Keywords

ASJC Scopus subject areas

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