Carles Gaig 1,2,3 , Alex Iranzo 1,2,3 , Christian Cajochen 4 , Isabel Vilaseca 2,5 , Cristina Embid 2,6 , Josep Dalmau 7,8 , Francesc Graus 7 , Joan Santamaria 1,2,3 Show Affiliations »
Abstract
STUDY OBJECTIVE: To characterize the sleep disorder of anti-IgLON5 disease. METHODS: We reviewed 27 video-polysomnographies (V-PSG), 6 multiple sleep latency tests (MSLT), 2 videsomnoscopies with dexmedetomidine, and 10 actigraphies recorded during the disease course of five patients. Due to severe sleep architecture abnormalities, we used a novel modified sleep scoring system combining conventional stages with a descriptive approach in which two additional stages were identified: undifferentiated-NREM (UN-NREM) and poorly structured N2 (P-SN2) sleep that were characterized by abnormal motor activation and absence or sparse elements of conventional NREM sleep. RESULTS: Sleep-related vocalizations, movements, behaviors, and respiratory abnormalities were reported by bed-partners. In all patients, NREM sleep onset and sleep reentering after an awakening occurred as UN-NREM (median: 29.8% of total sleep time [TST]) and P-SN2 sleep (14.5% TST) associated with vocalizations and simple and quasi-purposeful movements. Sleep initiation was normalized in one patient with a high dose of steroids, but NREM sleep abnormalities reappeared in subsequent V-PSG. In all patients, if sleep continued uninterrupted, there was a progressive normalization with normal N2 (11.7% TST) and N3 (22.3% TST) sleep but stridor and obstructive apnea emerged. REM sleep behavior disorder (RBD) occurred in four patients. Sleep initiation was also altered in MSLT and dexmedetomidine-induced sleep. Actigraphy showed a 10-fold increase of nocturnal activity compared with controls. Sleep abnormalities remained stable during the disease. CONCLUSIONS: The sleep disorder of anti-IgLON5 disease presents as a complex sleep pattern characterized by abnormal sleep initiation with undifferentiated NREM sleep, RBD, periods of normal NREM sleep, stridor, and obstructive apnea. © Sleep Research Society 2019. Published by Oxford University Press on behalf of the Sleep Research Society. All rights reserved. For permissions, please email: journals.permissions@oup.com.
STUDY OBJECTIVE: To characterize the sleep disorder of anti-IgLON5 disease. METHODS: We reviewed 27 video-polysomnographies (V-PSG ), 6 multiple sleep latency tests (MSLT), 2 videsomnoscopies with dexmedetomidine , and 10 actigraphies recorded during the disease course of five patients . Due to severe sleep architecture abnormalities , we used a novel modified sleep scoring system combining conventional stages with a descriptive approach in which two additional stages were identified: undifferentiated-NREM (UN-NREM) and poorly structured N2 (P-SN2) sleep that were characterized by abnormal motor activation and absence or sparse elements of conventional NREM sleep. RESULTS: Sleep-related vocalizations, movements, behaviors, and respiratory abnormalities were reported by bed-partners. In all patients , NREM sleep onset and sleep reentering after an awakening occurred as UN-NREM (median: 29.8% of total sleep time [TST]) and P-SN2 sleep (14.5% TST) associated with vocalizations and simple and quasi-purposeful movements. Sleep initiation was normalized in one patient with a high dose of steroids , but NREM sleep abnormalities reappeared in subsequent V-PSG . In all patients , if sleep continued uninterrupted, there was a progressive normalization with normal N2 (11.7% TST) and N3 (22.3% TST) sleep but stridor and obstructive apnea emerged. REM sleep behavior disorder (RBD) occurred in four patients . Sleep initiation was also altered in MSLT and dexmedetomidine -induced sleep. Actigraphy showed a 10-fold increase of nocturnal activity compared with controls. Sleep abnormalities remained stable during the disease. CONCLUSIONS: The sleep disorder of anti-IgLON5 disease presents as a complex sleep pattern characterized by abnormal sleep initiation with undifferentiated NREM sleep, RBD, periods of normal NREM sleep, stridor, and obstructive apnea . © Sleep Research Society 2019. Published by Oxford University Press on behalf of the Sleep Research Society. All rights reserved. For permissions, please email: journals.permissions@oup.com.
Entities: Chemical
Disease
Gene
Species
Keywords:
anti-IgLON5 disease; NREM parasomnia; REM sleep behavior disorder; obstructive sleep apnea; stridor
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Year: 2019
PMID： 31198936 DOI： 10.1093/sleep/zsz133
Source DB: PubMed Journal: Sleep ISSN： 0161-8105 Impact factor: 5.849