Objective Sleep Profile in LGI1/CASPR2 Autoimmunity

SLEEP ◽  
2021 ◽  
Author(s):  
Michelle F Devine ◽  
John C Feemster ◽  
Elizabeth A Lieske ◽  
Stuart J McCarter ◽  
David J Sandness ◽  
...  
Keyword(s):  
Sleep Apnea ◽  
Rem Sleep ◽  
Sleep Disturbances ◽  
Rem Sleep Behavior Disorder ◽  
Behavior Disorder ◽  
Apnea Hypopnea Index ◽  
Chi Square ◽  
Sleep Behavior ◽  
Rem Sleep Without Atonia ◽  
Anterior Tibialis

Abstract Study Objectives Rapid eye movement (REM) sleep behavior disorder (RBD) and other sleep disturbances are frequent in leucine-rich, glioma inactivated protein 1-IgG (LGI1) and contactin-associated protein 2-IgG (CASPR2) autoimmunity, yet polysomnographic analyses of these disorders remain limited. We aimed to characterize clinical presentations and analyze polysomnographic manifestations, especially quantitative REM sleep without atonia (RSWA) in LGI1/CASPR2-IgG seropositive (LGI/CASPR2+) patients. Methods We retrospectively analyzed clinical and polysomnographic features and quantitative RSWA between LGI1+/CASPR2+ patients and age-sex matched controls. Groups were compared with Wilcoxon rank-sum and chi-square tests. Combined submentalis and anterior tibialis (SM+AT) RSWA was the primary outcome Results Among 11 (LGI1+, n=9; CASPR2+, n=2) patients, Morvan syndrome sleep features were present in 7 (63.6%) LGI1+/CASPR2+ patients, with simultaneous insomnia and DEB in 3 (27.3%), and the most common presenting sleep disturbances were dream enactment behavior (DEB, n=5), insomnia (n=5), and sleep apnea (n=8; median apnea hypopnea index=15/hour). Median Epworth Sleepiness Scale (ESS) was 9 (range 3-24; n=10), with hypersomnia in 4 (36.4%). LGI1+/CASPR2+ patients had increased N1 sleep (p=0.02), decreased REM sleep (p=0.001), and higher levels of SM+AT any RSWA (p < 0.001). Eight of 9 (89%) LGI1+ exceeded RBD RSWA thresholds (DEB, n=5; isolated RSWA, n=3). RSWA was greater in anterior tibialis than submentalis. All 10 LGI1+/CASPR2+ patients treated with immunotherapy benefitted, and 5/10 had improved sleep disturbances. Conclusion LGI1/CASPR2-IgG autoimmunity is associated with prominent dream enactment, insomnia, RSWA, sleep apnea, and shallower sleep. Polysomnography provides objective disease markers in LGI1+/CASPR2+ autoimmunity and immunotherapy may benefit associated sleep disturbances.

scholarly journals Normative and isolated rapid eye movement sleep without atonia in adults without REM sleep behavior disorder

SLEEP ◽  
2019 ◽  
Vol 42 (10) ◽  
Author(s):  
John C Feemster ◽  
Youngsin Jung ◽  
Paul C Timm ◽  
Sarah M Westerland ◽  
Thomas R Gossard ◽  
...  
Keyword(s):  
Rem Sleep ◽  
Rem Sleep Behavior Disorder ◽  
Behavior Disorder ◽  
Older Men ◽  
Sleep Behavior ◽  
Rem Sleep Without Atonia ◽  
Rem Atonia ◽  
Male Sex ◽  
Anterior Tibialis

Abstract Study Objectives Values for normative REM sleep without atonia (RSWA) remain unclear. Older age and male sex are associated with greater RSWA, and isolated elevated RSWA has been reported. We aimed to describe normative RSWA and characterize isolated RSWA frequency in adults without REM sleep behavior disorder (RBD). Methods We visually quantified phasic, “any,” and tonic RSWA in the submentalis (SM) and anterior tibialis (AT) muscles, and the automated Ferri REM Atonia Index during polysomnography in adults without RBD aged 21–88. We calculated RSWA percentiles across age and sex deciles and compared RSWA in older (≥ 65) versus younger (<65) men and women. Isolated RSWA (exceeding diagnostic RBD cutoffs, or >95th percentile) frequency was also determined. Results Overall, 95th percentile RSWA percentages were SM phasic, any, tonic = 8.6%, 9.1%, 0.99%; AT phasic and “any” = 17.0%; combined SM/AT phasic, “any” = 22.3%, 25.5%; and RAI = 0.85. Most phasic RSWA burst durations were ≤1.0 s (85th percentiles: SM = 1.07, AT = 0.86 seconds). Older men had significantly higher AT RSWA than older women and younger patients (all p < 0.04). Twenty-nine (25%, 18 men) had RSWA exceeding the cohort 95th percentile, while 17 (14%, 12 men) fulfilled diagnostic cutoffs for phasic or automated RBD RSWA thresholds. Conclusions RSWA levels are highest in older men, mirroring the demographic characteristics of RBD, suggesting that older men frequently have altered REM sleep atonia control. These data establish normative adult RSWA values and thresholds for determination of isolated RSWA elevation, potentially aiding RBD diagnosis and discussions concerning incidental RSWA in clinical sleep medicine practice.

scholarly journals P077 Reliability of the Clinical Characterization of Isolated REM Sleep Behavior Disorder

2021 ◽  
Vol 2 (Supplement_1) ◽  
pp. A46-A46
Author(s):  
D Levendowski ◽  
J Lee-Iannotti ◽  
D Shprecher ◽  
C Guevarra ◽  
P Timm ◽  
...  
Keyword(s):  
Risk Assessment ◽  
Rem Sleep ◽  
False Positive ◽  
False Negative ◽  
Rem Sleep Behavior Disorder ◽  
Behavior Disorder ◽  
Sleep Behavior ◽  
Negative Case ◽  
Rem Sleep Without Atonia

Abstract Purpose Compare agreements between polysomnography-based (PSG) diagnosis of isolated REM-sleep-behavior-disorder (iRBD) and Non-REM-Hypertonia (NRH), a novel biomarker independently associated with synucleinopathy-related neurodegenerative diseases. Methods Sixteen patients with histories of dream-enactment-behavior (DEB)(women=38%; age:64.6±13.0) underwent PSG with simultaneously-recorded Sleep Profiler (SP). Two boarded sleep neurologists independently characterized iRBD. Physician1 combined abnormal qualitative REM-sleep-without-atonia (RSWA) by submental electromyography, with video-confirmation of probably DEB. Physician2 relied solely on qualitative RSWA. SP was auto-staged, technically reviewed, and reprocessed for automated abnormal NRH detection. Kappa scores measured physician and NRH agreements. Results In the 14 records with REM sleep, iRBD was characterized in: Physician1=64%, Physician2=79%, NRH=71% of the records. Across the three methods, unanimous iRBD agreement occurred in 57% of the records (positive=7, negative=1). The between-physician agreement in iRBD classifications was fair (kappa=0.32). The agreement between NRH and Physician1 was moderate (kappa=0.52) versus slight with Physician2 (kappa=0.05). NRH comparisons to consensus physician agreement yielded one false-positive and one false-negative iRBD finding. Physician2 classified: a) iRBD in two cases that were negative by Physician1 and NRH, and b) one negative case that Physician1 and NRH characterized as iRBD. Physician1 identified one negative case that was classified iRBD by Physician2 and NRH. Additionally, NRH was abnormal in one of the two records with no REM sleep. Discussion NRH may assist in iRBD risk assessment, given it agreed with at least one physician in 86% of the cases and the between-physician iRBD agreement was only fair. NRH also characterized iRBD-risk in patients with insufficient REM sleep for RSWA assessment.

scholarly journals REM sleep behavior disorder in children with type 1 Narcolepsy treated with Sodium Oxybate

Neurology ◽  
2020 ◽  
pp. 10.1212/WNL.0000000000011157
Author(s):  
Elena Antelmi ◽  
Marco Filardi ◽  
Fabio Pizza ◽  
Stefano Vandi ◽  
Monica Moresco ◽  
...  
Keyword(s):  
Rem Sleep ◽  
Sleep Stage ◽  
Rem Sleep Behavior Disorder ◽  
Behavior Disorder ◽  
Sodium Oxybate ◽  
Direct Role ◽  
Sleep Behavior ◽  
Rem Sleep Without Atonia ◽  
Nighttime Sleep

Objective:The aim was to study the effect of stable treatment with Sodium Oxybate (SO) on nocturnal REM sleep behavior disorder (RBD) and REM sleep without atonia (RSWA) that severely affected children with type 1 narcolepsy (NT1.Methods:Nineteen NT1 children and adolescents (nine females; mean age 12.5±2.7, mean disease duration: 3.4±1.6 years) underwent neurological investigations and video-polysomnography (v-PSG) at baseline and after three months of stable treatment with SO.v-PSG was independently analysed by two sleep experts, in order to rate RBD episodes. RSWA was automatically computed by means of the validated REM sleep atonia index (RAI).Results:Compared to baseline, RAI significantly improved (p< 0.05) and complex movements during REM sleep were remarkably reduced after stable treatment with SO. Compared to baseline, children also reported improvement in clinical complaints and showed a different nighttime sleep stage architecture.Conclusions:RBD and RSWA improved after treatment with SO, pointing to a direct role of the drug in modulating motor control during REM sleep.

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