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The Sleep Disorder in Anti-lgLON5 Disease

  • Sleep (M Thorpy and M Billiard, Section Editors)
  • Published:
Current Neurology and Neuroscience Reports Aims and scope Submit manuscript

Abstract

Purpose of Review

To review the clinical and polysomnographic features of the sleep disorder occurring in the recently described anti-IgLON5 disease. The hallmark of the disease is the presence of antibodies against IgLON5, a neural cell adhesion molecule of unknown function. The disease presents a robust HLA association, and the neuropathological examination shows a novel neuronal tauopathy with predominant hypothalamic and brainstem involvement.

Recent Findings

Most patients (> 80%) present sleep-related vocalizations with movements and behaviors and sleep-disordered breathing. Polysomnographic studies show (1) a complex NREM sleep parasomnia at sleep initiation characterized by undifferentiated NREM or poorly structured N2 sleep with sleep-talking or mumbling, and simple or finalistic movements followed by normal periods of N3 or N2 NREM sleep, (2) REM sleep behavior disorder (RBD), and (3) obstructive sleep apnea with stridor. The last two features appear mainly in periods where NREM sleep normalizes.

Summary

Identification of the anti-IgLON5 sleep disorder is important to suspect the disease. The combination of abnormal NREM sleep initiation, followed by normal periods of NREM sleep and RBD, represents a novel parasomnia.

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Acknowledgments

We thank Dr. Ellen Gelpi, Institute of Neurology, Medical University of Vienna, for her help in the design of Fig. 2.

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Authors and Affiliations

  1. Neurology Service, Hospital Clínic of Barcelona, C/Villarroel 170, 08036, Barcelona, Spain

    Carles Gaig, Alex Iranzo, Joan Santamaria & Francesc Graus

  2. Institut d’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain

    Carles Gaig, Alex Iranzo, Joan Santamaria & Francesc Graus

  3. Multidisciplinary Sleep Unit, Hospital Clínic of Barcelona, Barcelona, Spain

    Carles Gaig, Alex Iranzo & Joan Santamaria

  4. Centro de Investigación Biomedica en Red Enfermedades Neurodegenerativas (CIBERNED), Madrid, Spain

    Carles Gaig, Alex Iranzo & Joan Santamaria

Authors
  1. Carles Gaig
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  2. Alex Iranzo
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  3. Joan Santamaria
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  4. Francesc Graus
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Corresponding author

Correspondence to Francesc Graus.

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Conflict of Interest

Carles Gaig, Alex Iranzo, and Joan Santamaria each declare no potential conflicts of interest. Francesc Graus received a licensing fee from Euroimmun for the use of IgLON5 as an autoantibody test.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

Additional information

This article is part of the Topical Collection on Sleep

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Gaig, C., Iranzo, A., Santamaria, J. et al. The Sleep Disorder in Anti-lgLON5 Disease. Curr Neurol Neurosci Rep 18, 41 (2018). https://doi.org/10.1007/s11910-018-0848-0

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  • DOI: https://doi.org/10.1007/s11910-018-0848-0

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