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

Research output: Contribution to journalArticlepeer-review


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 languageEnglish (US)
Pages (from-to)6139-6144
Number of pages6
JournalJournal of Neurology
Issue number11
StatePublished - Nov 2022


  • Amyotrophic lateral sclerosis
  • IgLON5 autoimmunity
  • Motor neuron disease

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology


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