bluuebunny/crossref_metadata_2025_split huggingface dataset:
1228 A CASE OF PULMONARY HYPERTENSION AND SEVERE OBSTRUCTIVE SLEEP APNEA IN A PEDIATRIC PATIENT WITH DOWN SYNDROME
Authors: Asis Aristotle , Kaplan Kevin 
Published: May 2020

Abstract

Introduction
Children with Down syndrome (DS) are at risk for obstructive sleep apnea (OSA) and related health consequences. Adherence to treatment plans, especially positive airway pressure (PAP), can be difficult for people with DS. We present a case of a pediatric patient with DS who developed pulmonary arterial hypertension (PAH) due to poor adherence of OSA therapy.

Report of Case
A 4-year-old male with a past medical history of DS was diagnosed with severe OSA and hypoventilation at 18 months of age – AHI 27, (oAHI 27), CO2 above 50 mmHg for 53.2% of total sleep time (TST)). A titration study showed that on a bi-level pressure (BPAP) of 11/6 cm H2O his OSA was improved – AHI 5.9 (oAHI 3.7), O2 nadir of 93%. The family attempted PAP at home but discontinued after 2 weeks. He underwent an adenoidectomy at 22 months of age and tonsillectomy and repeat adenoidectomy at 40 months of age. Patient presents to the emergency center with respiratory distress. Chest films showed cardiomegaly. Echocardiogram revealed evidence of worsening PAH (tricuspid regurgitation of 3.5-3.8 m/s and flattening of the interventricular septum). He was admitted to the pediatric intensive care unit for further management of his PAH attributed to his underlying uncorrected OSA and hypoventilation. Repeat polysomnogram showed continue severe OSA and hypoventilation – AHI 30.3 (oAHI 30.3) CO2 above 50 mmHg for 89% of TST. Repeat titration study showed that a pressure of 11/7 cm H2O improved his OSA– AHI 6.4 (oAHI 6.4) CO2 max of 52 mmHg. Treatment with BPAP was initiated prior to discharge. Repeat echocardiogram 2 months after admission showed improvement of the PAH while on BPAP without additional vasoactive drug therapy.

Conclusion
Children with DS are at high risk for OSA. Although treatment can be difficult, proper management of the OSA essential to preventing potential serious health consequences.
1232 Improvement in Excessive Daytime Sleepiness in Two Narcoleptic Patients on Oral Contraceptives
Authors: Macias Maria , Singh Suprya , Patel Amee 
Published: May 2020

Abstract

Introduction
Narcolepsy is a chronic disorder characterized by excessive daytime sleepiness (EDS). We present two patients with narcolepsy with improvement in symptoms with the use of oral contraceptives.

Report of Case
Patient A is a 15-year-old female with a family history of narcolepsy who presents with excessive daytime sleepiness and cataplexic episodes (eye twitching, abnormal sensations of the face, drop attacks). Her pediatric daytime sleepiness scale score (PDSS) was 29. The polysomnography (PSG) did not record sleep disordered breathing. Multiple sleep latency test (MSLT) recorded 4 out 4 sleep onset rapid eye movement period (SOREMPS) and mean sleep onset latency (SOL) of 6.2 minutes. She was diagnosed with Narcolepsy Type 1 and started on stimulants as family was not interested in Xyrem. Over two years, her PDSS the doses of the stimulants were increased due to significant daytime sleepiness. Caregiver was not interested in Xyrem. Patient demonstrated increased sleepiness during menstrual cycles. She was treated with OCPs for menorrhagia. Her EDS improved, PDSS decreased from an average of 24.5 to 17.5 and the dose of the stimulants was decreased.
Patient B is an 11-year-old female with excessive daytime sleepiness and fragmented sleep with multiple awakenings. She took frequent naps during the day. Her PDSS was 29. The PSG showed mild OSA and the MSLT recorded 3 out of 4 SOREMPS and SOL was less than 1 minute. She was diagnosed with Narcolepsy Type 2. During her treatment course, several medication regimens were trialed but were not effective including stimulants, Modafinil, and Armodafinil. Caregiver was not interested in Xyrem. At the age of 13 years, she started OCPs for dysmenorrhea. Her EDS improved and PDSS decreased from an average of 29 to 16.75.

Conclusion
We present two patients with narcolepsy who continued to have EDS in spite of treatment with wake promoting agents and daytime naps. Their EDS and PDSS improved after initiating OCP therapy Pubertal changes may have a significant influence on narcolepsy patients. The use of OCPs may be beneficial in conjunction to wake promoting agents.
1239 Mind Blown: Exploding Head Syndrome as a Side Effect of Marijuana
Authors: Missak Christopher , George Jenie , Gurubhagavatula Indira 
Published: May 2020

Abstract

Introduction
Marijuana use is increasing the United States and has been associated with increased sensory perception, euphoria, and altered cognition. Exploding head syndrome is a parasomnia characterized by loud explosion-like noises occurring prior to or during sleep. This the first report to link marijuana use with the occurrence of EHS.

Report of Case
A 45-year-old man with depression, anxiety and multisubstance abuse reported two years of neuropsychiatric symptoms including: “fireworks going off in my brain,” visual hallucinations, and sudden arousals characterized by panic and vertigo. He reported bouts of occupational stress and severe anxiety previously treated with clonazepam for six months, but discontinued it due to poor clinical response. Normal findings were reported on a previous work-up including: MRI, EEG, and head CT Scan.
During his visit, he reported that he had been smoking marijuana three times a day for 24 years. One month prior to his visit he discontinued marijuana, stopped clonazepam, and started using mirtazapine with improvement in all aforementioned symptoms. During the same month, he relapsed and smoked marijuana once with a sudden return of all previously described symptoms on the night following its use. These symptoms then resolved and did not recur again until 9 months later, after another episode of weekend marijuana use. An in-lab polysomnogram revealed an apnea-hypopnea index 2.2 per hour, increased alpha intrusion throughout the study, and no epileptiform activity. No parasomnias were observed during rapid-eye-movement (REM) or non-REM sleep.

Conclusion
The etiology of EHS remains elusive. A review of the research has proposed five major theories and case reports suggest a complex etiology. Given the temporal correlation with marijuana use and symptom resolution with its cessation, we presume that EHS resulted from a complex interplay between neurons susceptible to cannabinoids and their derivatives.
1242 An Adult with ROHHAD Disease
Authors: Ahmad Hamna , Gohar Ashraf 
Published: May 2020

Abstract

Introduction
Rapid-onset obesity with hypothalamic dysfunction, hypoventilation and autonomic dysregulation (ROHHAD) is an extremely rare and fatal disease presenting in early childhood.

Report of Case
A 25-year-old male, seen in our adult sleep clinic to establish care at the age of 21. He was previously followed by pediatric sleep department. He was diagnosed with ROHHAD syndrome in early childhood after presenting at the age of 18 months. At the time, he was diagnosed with central hypoventilation with multiple endocrinopathies. He was noted to have mild mental retardation and behavioral problems. He was initially worked up for multiple genetic disorders and was eventually diagnosed with ROHHAD syndrome. As a pediatric patient, he had a CPAP titration study that was unsuccessful with persistent central apneas. Eventually, he was treated with auto-SV (with minimum and maximum pressure support of 1 and +15 respectively, an EPAP max of +20, and EPAP min of +5). He has since been doing well with ASV. He also had insomnia and hypersomnia and is being treated with Modafinil for excessive daytime sleepiness and Zolpidem for the insomnia.

Conclusion
The mortality related to cardiorespiratory arrest in ROHHAD disease has been estimated to be 40-50%. To our knowledge, case reports of adult patients with ROHHAD in literature, are few and far between.
1253 Multiple sleep onset REM episodes in middle age woman with excessive daytime sleepiness – Is this automatically assumed narcolepsy?
Authors: Patel Kamal , Lang Bianca J 
Published: May 2020

Abstract

Introduction
Presence of sleep onset REM episodes often raises concerns of narcolepsy. However other conditions have shown to have presence of sleep on REM episodes which include but not limited to obstructive sleep apnea, sleep wake schedule disturbance, alcoholism, neurodegenerative disorders, depression and anxiety

Report of Case
Here we present a case of 30 year old female with history of asthma, patent foraman ovale, migraine headache, and anxiety who presented with daytime sleepiness, falling asleep while at work, occasional scheduled naps, non-restorative sleep, sleep paralysis, and hypnopompic hallucination. Pertinent physical exam included; mallampati score of 4/4, retrognathia, high arched hard palate, crowded posterior oropharynx. She had a score of 16 on Epworth sleepiness scale. Patient previously had multiple sleep latency test at outside facility which revealed 4/5 SOREM, with mean sleep onset latency of 11.5 minutes. She however was diagnosed with narcolepsy and tried on modafinil which she failed to tolerate. She was tried on sertraline as well which was discontinued due to lack of benefit. She had repeat multiple sleep latency test work up which revealed 2/5 SOREM, with mean sleep onset latency was 13.1 minutes. Her overnight polysomnogram prior to repeat MSLT showed SOREM with sleep onset latency of 10 minutes. Actigraphy showed consistent sleep pattern overall with sufficient sleep time but was taking hydroxyzine and herbal medication. Patient did not meet criteria for hypersomnolence disorder and sleep disordered breathing.

Conclusion
There is possibility her medication may have played pivotal role with her daytime symptoms. We also emphasize SOREMs can be present in other disorders such as anxiety in this case and not solely in narcolepsy
1258 No Laughing Matter - Laughter Induced Syncope (Gelastic Syncope) Mimicking Cataplexy
Authors: Belani Dinesh , Simon Edwin 
Published: May 2020

Abstract

Introduction
Laughter is a common emotion and may rarely be a manifestation of neurological illnesses. It has been associated with cataplexy as well. Cataplexy is usually triggered by strong emotions. Gelastic syncope is an uncommon phenomenon which may be mistaken for cataplexy. We summarize 3 cases referred to the Sleep Medicine clinic for evaluation for Narcolepsy.

Report of Case
55 yo male comes with 2 episodes of blacking out and falling down relating to episodes of laughter in 3 months. Patient describes loss of consciousness and no episodes of freezing. Reported 15 years of snoring and witnessed apneas along with grinding his teeth while sleeping. Polysomnogram revealed Obstructive Sleep Apnea (OSA) with an AHI of 20.
60 yo male comes with episodes of loss of consciousness over the past 6 months, including sitting in a chair, laughing, urinating, washing dishes while standing, expressing strong emotions (father’s funeral), etc. Also reports bugs crawling over his legs when trying to sleep, loud snoring and waking up choking while sleeping. Polysomnogram revealed OSA with an AHI of 20.
43 yo male comes 3 episodes of loss of consciousness, 2 of them related to laughing and the last one related to stretching his arms out. He passes out for 5-10 seconds at a time and a period of 20-30 seconds before passing out where he feels dizzy when he is unable to respond at this time, no post episode confusion. Positive on the Cataplexy Emotional Trigger Questionairre. Reported witnessed apneas, snoring and sleep talking. Polysomnogram revealed OSA, hence the Multiple Sleep Latency Testing ordered was not completed.

Conclusion
While the first two episodes point towards Gelastic Syncope based on symptoms, the third did warrant MSLT if there was no OSA on PSG. It is important to recognize gelastic syncope as an entity and differentiate it from cataplexy.
0131 Decreased Habitual Sleep Efficiency is Associated with Increased Insulin Resistance in Healthy Adult Men
Authors: Kelly M R , O’Byrne N , Iranmanesh A , Martin J L , Liu P Y 
Published: May 2020

Abstract

Introduction
Partial sleep deprivation is associated with increased insulin resistance (IR), a metabolic disease risk marker. Little is known about habitual sleep patterns and IR in the absence of acute sleep restriction. We anticipated greater change in habitual sleep over one month would be associated with increased IR.

Methods
24 males (age=33.6±6.4 years; BMI=25.7±2.5kg/m2) completed baseline (T1) and follow-up (T2; ≥4 weeks post-T1) study procedures: actigraphy (one week) followed by polysomnography (PSG; one 10h sleep opportunity) and a next morning oral glucose tolerance test (OGTT; homeostatic model assessment insulin resistance [HOMA-IR], β-cell function [HOMA-β], and Matsuda Index). Weekly average actigraphy total sleep time (aTST; 291-511min) and sleep efficiency (aSE; 72–93%) were computed at T1 and T2, as well as across the 1, 2, and 3 days prior to PSG/OGTT. Pearson and Spearman correlations assessed the change (T1-T2) in actigraphy (aSEΔ, aTSTΔ, PSGΔ) or PSG sleep (PSG-TSTΔ, PSG-SEΔ, sleep stages) versus change in metabolic risk (HOMA-IRΔ, HOMA-βΔ, MatsudaΔ).

Results
There were significant correlations between HOMA-IRΔ and aSEΔ [r(22)=-0.42, p=0.01; rs=-0.45, p=0.03], PSG TSTΔ [r(22)=0.50, p=0.012; rs=0.41, p=.045], and PSG-SEΔ [r(22)=0.49, p=0.015; rs=0.43, p=.037]. No significant associations emerged between change in metabolic risk versus aTSTΔ one week prior to PSG/OGTT, aSEΔ or aTSTΔ across 1–3 days prior to PSG/OGTT, or PSG sleep stages.

Conclusion
Within-subject T1-T2 decrease in habitual sleep quality, but not TST, was associated with increased IR. T1-T2 PSG TST and SE were associated with following day IR. At home sleep 1–3 days beforehand were not correlated with IR. Although preceding night sleep quality and TST are associated with IR, habitual sleep quality, rather than TST, may be a more important determinant of metabolic risk in community dwelling middle-aged men.

Support
This work was supported by NIH/NHLBI R01HL124211, NIH/NHLBI K24HL138632, NIH National Center for Advancing Translational Sciences (NCATS) UCLA CTSI Grant UL1TR001881 (PI: Liu); and NIH/NHLBI K24HL143055 (PI: Martin). Dr. Kelly is supported by the VA Office of Academic Affiliations through the Advanced Fellowship Programs in Geriatrics.
0141 A Novel, Orally Available Orexin 2 Receptor-Selective Agonist, TAK-994, Shows Wake-Promoting Effects Following Chronic Dosing in an Orexin-Deficient Narcolepsy Mouse Model
Authors: Ishikawa T , Suzuki M , Kimura H 
Published: May 2020

Abstract

Introduction
The use of an orexin 2 receptor (OX2R) agonist may be a promising approach for the treatment of narcolepsy type 1. TAK-994 is a novel, orally available OX2R-selective agonist with &gt;700-fold selectivity against orexin 1 receptor. Single administration of TAK-994 ameliorates narcolepsy-like symptoms such as fragmentation of wakefulness and cataplexy-like episodes in orexin/ataxin-3 mice, a narcolepsy mouse model with orexin deficiency. In this study, we evaluated the effect of chronic dosing with TAK-994 on sleep/wakefulness states in orexin/ataxin-3 mice.

Methods
Orexin/ataxin-3 mice were grouped into two cohorts: a control group and a 14-day treatment group. In the control group, vehicle was administered orally to mice three times a day: zeitgeber time 12 (ZT12), ZT15 and ZT18, for 14 days. In the 14-day treatment group, TAK-994 was administered orally to mice at ZT12, ZT15 and ZT18 for 14 days. Electroencephalogram/electromyogram analysis was performed on day 1 and day 14 (ZT12-ZT21), and the subsequent sleep phase (ZT0-ZT10).

Results
On day 1, TAK-994 significantly increased wakefulness time, accompanied by a decrease in non-rapid eye movement (NREM) sleep time and rapid eye movement (REM) sleep time, in orexin/ataxin-3 mice compared with the control group. On day 14, TAK-994 also significantly increased wakefulness time, and decreased NREM sleep time and REM sleep time in orexin/ataxin-3 mice. There were no changes in the time spent in wakefulness, NREM sleep and REM sleep during the subsequent sleep phase after chronic dosing with TAK-994.

Conclusion
Wake-promoting effects of TAK-994 were observed following chronic dosing for up to 14 days in orexin/ataxin-3 mice with no rebound of sleep. Overall, there was no clear difference in efficacy between the single and repeated administration of TAK-994 in orexin/ataxin-3 mice.

Support
This work was conducted by Takeda Pharmaceutical Company Limited.
0163 Investigating the Role of Vasoactive Intestinal Peptide-Containing Neurons of the Ventromedal Preoptic Area in Sleep-Wake Control
Authors: Venner A , Fuller P M 
Published: May 2020

Abstract

Introduction
A role for vasoactive intestinal peptide (VIP) in promoting rapid eye movement (REM) sleep has been suggested, but the anatomical location of the neurons that release VIP to promote REM sleep has not been identified. Here, we investigated the role of VIP-containing cell groups in the ventromedial preoptic area (VMPOVIP) in sleep-wake regulation. The VMPO has also previously been implicated in thermoregulation and the febrile response.

Methods
We first investigated the native firing activity of VMPOVIP neurons, over repeated sleep-wake cycles, using in vivo fiber photometry in VIP-ires-Cre mice. We next examined the afferent and efferent profile of this cell group using conditional retrograde (pseudotyped modified rabies) and anterograde (adeno-associated viral vector-based) tracers. We finally utilized a chemogenetic strategy to selectively activate VMPOVIP neurons cells while monitoring electroencephalogram/electromyogram activity and core body temperature, in order to determine their role in sleep-wake and thermoregulatory control.

Results
We found that VMPOVIP cells were predominantly and strikingly REM-active, that they received many synaptic inputs from surrounding hypothalamic regions (including the ventromedial hypothalamus, dorsomedial hypothalamus and the arcuate nucleus), and that they targeted established sleep-wake nodes, such as the ventrolateral preoptic nucleus, tuberomammillary nucleus, lateral hypothalamus and ventrolateral periaqueductal gray area. To our surprise, chemogenetic activation of the VMPOVIP cell population had little effect upon all measures of sleep-wake analysed and no effect upon core body temperature.

Conclusion
We conclude that VMPOVIP neurons do not promote REM sleep per se. However, their REM-active profile and anatomical connectivity suggest that these neurons may play a functional role in generating certain cardinal features of REM sleep, which is an active focus of on-going research in our laboratory.

Support
SRSF CDA #016-JP-17 to A.V. and NS073613, NS092652 and NS103161 to P.M.
0169 Disruption of Sleep Architecture and Circadian Rhythms Following High Frequency Head Impacts
Authors: Korthas H T , Main B S , Harvey A C , Wicker E W , Sloley S S , Burns M P 
Published: May 2020

Abstract

Introduction
Mild Traumatic Brain Injury (mTBI) can cause a broad array of behavioral problems including cognitive and emotional deficits. Sleep disturbances including disrupted sleep latency and efficiency are common amongst human mTBI patients. Crucially, sleep plays a key role in hippocampal learning and memory consolidation, yet the contribution of single and repetitive mTBIs influencing sleep related cognitive outcomes remains unclear.

Methods
To study the effect of repetitive mTBI on sleep and circadian rhythms, C57Bl/6 mice underwent sham or High Frequency-Head Impact (HF-HI, 30 closed head impacts, 5/per day for 6-days) procedures before brains were assessed at 1d, 1m and 2m using a combination of molecular neurobiology (RNA/protein), EEG/ EMG recordings and behavioral analysis.

Results
HF-HI induces learning and memory deficits in the Barnes and T-Maze at both 1d and 1m post injury, in the absence of axonal injury, inflammation, or protein deposition. Disruptions in circadian mRNA expression was identified at multiple time points post HF-HI. RNA analysis of mouse cortex, hippocampus, and hypothalamus for core circadian rhythm genes (Bmal1, Clock, Cryptochrome 2, Period1 and Period 2) was conducted at 1d and 1m post HF-HI. We found dysregulated expression of these core biological clock genes in these regions at both time points. Furthermore, we find distinct changes to sleep architecture chronically post injury. Animals were implanted with EEG and EMG for monitoring at 2m post injury. EEG and EMG signals were coded for wake, NREM, and REM. One-month post injury, HFHI injured mice showed dysregulated sleep architecture compared to sham mice, while both groups had the same total sleep time. We also demonstrate that HF-HI alters EEG activity in awake animals.

Conclusion
Overall, our data shows disruptions in both sleep architecture and expression of circadian rhythm genes following HF-HI. This opens up an important avenue of potential therapeutic intervention following injury.

Support
If deficits in sleep and circadian rhythms can be rescued after mTBI, it may assist in improving symptoms and chronic outcomes after injury.
0176 Short Sleep Duration and Poor Sleep Quality Predict Blunted Weight Loss in a Behavioral Weight Loss Intervention
Authors: Kline C E , Lambiase M J , Conroy M B , Brooks M M , Kriska A M , Barinas-Mitchell E J 
Published: May 2020

Abstract

Introduction
Short sleep duration and poor sleep quality have each been associated with obesity and weight gain. However, less is known regarding how sleep may impact attempted weight loss. The purpose of this study was to investigate the associations between sleep duration and sleep quality, both independently and in combination, with weight loss in a 12-month behavioral weight loss intervention.

Methods
Young to middle-aged adults who were overweight or obese (N=296) completed a 12-month behavioral weight loss intervention, with weight assessed at baseline, 6 and 12 months. Sleep duration and quality were derived from the Pittsburgh Sleep Quality Index. Analyses examined the change in sleep over time and the association between baseline sleep and changes in sleep with 6- and 12-month weight loss following adjustment for relevant covariates including age, gender, race, education, baseline body mass index, and baseline risk for sleep apnea.

Results
Participants (with an average baseline weight of 97.0±1.0 kg) lost 6.6±1.1 kg (6.8%) and 6.7±1.2 kg (6.9%) at 6 and 12 months relative to baseline, respectively. Global sleep quality significantly improved over the 12-month intervention (P=.03), but average sleep duration and the prevalence of short sleep duration (&lt;6 h) or poor sleep quality did not change significantly (each P≥.45). Adults with short sleep duration at baseline lost 3.3±0.9% less weight than those with ≥6 h sleep duration (P&lt;.001). Adults with poor sleep quality at baseline lost 1.6±0.8% less weight than those with good sleep quality (P=.04). When considered together, adults with both short sleep duration and poor sleep quality lost at least 5.0% less weight compared with all other sleep duration/quality group combinations (P&lt;.001).

Conclusion
Our findings highlight the importance of both sleep duration and sleep quality as predictors of behavioral weight loss and suggest that screening for sleep disturbance may be useful to determine who may benefit from additional counseling and resources.

Support
R01HL077525, K23HL118318