633 Dysregulated REM Sleep in Children with Moebius syndrome

SLEEP ◽  
2021 ◽  
Vol 44 (Supplement_2) ◽  
pp. A248-A248
Author(s):  
Wendy Edlund ◽  
Suresh Kotagal
Keyword(s):  
Rem Sleep ◽  
Rem Sleep Behavior Disorder ◽  
Behavior Disorder ◽  
Moebius Syndrome ◽  
Sleep Behavior ◽  
Mri Brain ◽  
Pontine Tegmentum ◽  
Rem Sleep Without Atonia ◽  
Brain Scan ◽  
Extensive Involvement

Abstract Introduction Moebius syndrome (MBS) is a rare disorder characterized by failure of development of the 6th and 7th cranial nerve nuclei located in the pons. Though the congenital lesion abuts the pontine tegmentum, sleep architecture has not been evaluated in MBS. Methods We report the clinical features of three children with MBS, and discuss implications for “developmental” RBD. Results Three clinically and MRI brain scan-confirmed MBS children, age 0.5 to 16 years, mean 8.5 ± 6.3 years, were evaluated for disrupted sleep, with repetitive crying (1/3), yelling (2/3), agitation in sleep (2/3), and injuring a co-sleeping sibling (1/3); 3/3 had REM sleep without atonia while 2/3 had RBD. REM sleep was reduced in all three: 21.7% in the infant (reference REM percentage value in infancy is about 40%), 11.4% in subject 2 and 3% in subject 3. Treatment with clonazepam (patients 2 and 3) or melatonin (patient 1) had variable results. Conclusion Children with Moebius syndrome have both quantitative (reduced percentage) and qualitative abnormalities of REM sleep (RSWA/RBD). The association of MBS with RSWA/RBD suggests more extensive involvement than published in literature, with extension into the sub-laterodorsal pontine tegmentum and “REM-off” neurons. Moebius syndrome might serve as a model for study of “developmental” REM sleep behavior disorder. Support (if any):

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.

scholarly journals REM Sleep Behavior Disorder and REM Sleep Without Atonia in Probable Alzheimer Disease

SLEEP ◽  
2006 ◽  
Vol 29 (10) ◽  
pp. 1321-1325 ◽  
Author(s):  
Jean-François Gagnon ◽  
Dominique Petit ◽  
Maria Livia Fantini ◽  
Sylvie Rompré ◽  
Serge Gauthier ◽  
...  
Keyword(s):  
Alzheimer Disease ◽  
Rem Sleep ◽  
Rem Sleep Behavior Disorder ◽  
Behavior Disorder ◽  
Sleep Behavior ◽  
Rem Sleep Without Atonia
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