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Current Neurology and Neuroscience Reports
Published in: Current Neurology and Neuroscience Reports 7/2018

01-07-2018 | Sleep (M Thorpy and M Billiard, Section Editors)

The Sleep Disorder in Anti-lgLON5 Disease

Authors: Carles Gaig, Alex Iranzo, Joan Santamaria, Francesc Graus

Published in: Current Neurology and Neuroscience Reports | Issue 7/2018

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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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Metadata
Title
The Sleep Disorder in Anti-lgLON5 Disease
Authors
Carles Gaig
Alex Iranzo
Joan Santamaria
Francesc Graus
Publication date
01-07-2018
Publisher
Springer US
Published in
Current Neurology and Neuroscience Reports / Issue 7/2018
Print ISSN: 1528-4042
Electronic ISSN: 1534-6293
DOI
https://doi.org/10.1007/s11910-018-0848-0

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