bluuebunny/crossref_metadata_2025 huggingface dataset:
0790 Racial Differences in Self-Efficacy for Positive Airway Pressure Therapy Among Individuals Newly Diagnosed with Obstructive Sleep Apnea
Authors: Byrne Sean , Sharma Akanksha , Deng Annan , Chu Jen-Hwa , Sands Scott , Wellman Andrew , Redeker Nancy , Yaggi Henry , Zinchuk Andrey
Published: May 2022

Introduction

Continuous positive airway pressure (CPAP) therapy is the first-line treatment for OSA, yet its effectiveness is limited by poor adherence. Self-efficacy is an individual’s belief that they can successfully execute a behavior to achieve a desired outcome. Evidence shows that self-efficacy predicts adherence to and outcomes of CPAP therapy, as do race and ethnicity. Little, however, is known about how self-efficacy may differ between racial and ethnic groups. Accordingly, we aimed to determine whether self-efficacy for CPAP differs by race and ethnicity among individuals newly diagnosed with OSA.

Methods

Adults newly diagnosed with OSA and prescribed CPAP who were enrolled in the NICEPAP Study (n=267, NCT05067088), a prospective, observational cohort study investigating predictors of CPAP adherence were assessed. Those with need for non-CPAP therapy or with unstable medical conditions (e.g., cancer receiving chemotherapy, severe lung, heart or mental health disorders) were excluded. Exposures were race and ethnicity. Outcomes were subscale scores of the Self-Efficacy Measure for Sleep Apnea (SEMSA) completed prior to CPAP initiation: perceived risk, outcome expectancy, and self-efficacy. SEMSA sub-scale scores for race/ethnicity were compared using Kruskal-Wallis test. Medians (Q1, Q3) are reported.

Results

We analyzed data for 52 participants (33 women) enrolled to date who identified as White (n=29), Black (n=14), More than one race (n=5) and Other (N=5). Participants were 51.0 (36.8, 58.8) years old with an apnea-hypopnea index of 17.0/hour (11.1, 26.0) and body-mass-index of 35.0 (31.6, 43.5) kg/m2. Baseline characteristics did not differ by race, except higher poverty (p=0.005) and less completed years of education (p=0.010) for Black participants. The SEMSA scores were not statistically different between each race. However, self-efficacy was significantly lower for Black participants vs. rest of the cohort combined (2.4 (1.9, 3.1) vs. 3.3 (2.7, 3.8) p=0.020). Poverty, but not education, may be a potential mediator of this relationship (mediation analysis p=0.052). There were no differences in SEMSA scores between Hispanic and Non-Hispanic participants.

Conclusion

In our cohort, self-efficacy for OSA therapy was lower for Black participants compared to those of other races. Targeting early interventions to improve CPAP self-efficacy in Black patients may improve OSA therapy outcomes.

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0795 Association of Nasal and Sleep Apnea Clinical Measures
Authors: Ikeda Allison , Weaver Edward
Published: May 2022

Introduction

The association between nasal measures and polygsomnography measures is modest. However, nasal treatment can improve sleep quality in people without sleep apnea. The association of nasal and sleep apnea clinical measures in patients with sleep apnea is unknown. Our primary hypothesis is that the subjective nasal obstruction is associated with reduced sleep apnea-specific quality of life at baseline and with sleep apnea treatment after 6 months.

Methods

This prospective cohort study included patients with newly diagnosed sleep apnea who were prescribed CPAP. Data were collected before CPAP and 6 months later, including the validated Nasal Obstruction Symptom Evaluation (NOSE, range 0-100, higher worse) scale and validated Symptoms of Nocturnal Obstruction & Related Events (SNORE-25, range 0-5, higher worse) quality of life instrument. Additional nasal measures, sleep apnea clinical measures, and covariates were collected. We tested the association between nasal and sleep apnea clinical measures with multivariate linear regression, adjusting for important confounding variables.

Results

The cohort (N=242) was middle-aged (47+/-12 years), included both sexes (65% men), and had severe sleep apnea (apnea-hypopnea index 33+/-24 events/hour). The baseline NOSE was strongly associated with SNORE-25, independent of demographics, comorbidity, and sleep apnea severity: beta 18, 95% CI 15-22, p<0.001, meaning for every unit of SNORE-25 increase there was an adjusted increase in NOSE score of 18. The 6-month changes in NOSE and SNORE-25 were strongly associated, independent of demographics, comorbidity, sleep apnea severity, and CPAP use: beta 17, 95%CI 13-22, p<0.001. Secondary nasal and sleep apnea clinical measures were associated, but nasal and polysomnography measures were not associated.

Conclusion

Nasal clinical measures are associated with sleep apnea clinical measures at baseline and with sleep apnea treatment, while the nasal measures are not associated with polysomnography measure. These data support the importance of the nasal airway on sleep apnea clinical outcomes.

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R01 HL084139 (Weaver), T32 DC000018 (Stone), Seattle Veterans Affairs Medical Center
0802 Central Sleep Apnea as a Result of Cerebral Cavernous Malformation Hemorrhage in the Pediatric Population
Authors: Bowling Joshua , Hassan Fauziya
Published: May 2022

Introduction

Cerebral Cavernous malformations (CCMs) consist of a collection of capillaries in the central nervous system (CNS) that are enlarged and irregular in structure. Patients with CCMs are at increased risk of hemorrhage into the brain or spinal cord, resulting in seizures, focal neurologic deficits, hydrocephalus, and death. Given the importance of the brainstem and central chemoreceptors in regulating respiratory function, rupture of CCMs can also lead to dysregulation of breathing. We present a series of 3 pediatric patients who have a diagnosis of central sleep apnea (CSA) due to rupture of CCMs managed with home ventilators with significant differences in clinical presentation from mild CSA to night time respiratory support for severe CSA and chronic respiratory failure requiring continuous mechanical ventilation.

Report of Cases: Subject 1 is a 13 yo M with a history of ruptured posterior fossa

arteriovenous malformation (AVM) complicated by post-hemorrhagic hydrocephalus, right hemiparesis and severe central apnea requiring nighttime mechanical ventilation via

tracheostomy. Subject 2 is a 19 yo M with a history of mid-pontine cavernoma with multiple episodes of hemorrhage failing surgical resection resulting in right hemiparesis, severe central apnea and hypoventilation requiring continuous mechanical ventilator support via tracheostomy. Subject 3 is a 6 yo F with a history of thoracic cavernous hemangioma resulting in spinal cord injury and mild central sleep apnea requiring mask ventilation via home ventilator during sleep. All of these patients experienced loss of respiratory drive as a result of complications from brainstem or spinal cord lesions with varying degrees of ventilator support requirement and clinical presentation.

Conclusion

Due to the propensity for CNS bleeds among patients with CCM they are at increased risk of respiratory compromise. Home ventilator support can be used effectively to treat central apneas and chronic respiratory failure but this is a moving paradigm as subsequent bleeds worsen respiratory compromise.

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0803 Extreme upper extremity movements in patient with narcolepsy type 1 and REM sleep behavior disorder affecting actigraphy results
Authors: Galagan Jack , York Carla , Soca Rodolfo
Published: May 2022

Introduction

Narcolepsy with Cataplexy is a central disorder of hypersomnia that is characterized by excessive daytime sleepiness and Rapid eye movement (REM) dissociation phenomena. A common manifestation of narcolepsy is REM behavioral disorder (RBD), a parasomnia characterized by loss of muscle atonia during REM sleep (RSWA). While RBD is relatively common in patients with narcolepsy, very extreme movements are considered rare.

Report of Cases: A 20-year-old male with no significant past medical history presented with 8 months of new onset daytime sleepiness, sleep paralysis, and auditory hallucinations at sleep onset. After initial evaluation at our sleep center, he was scheduled for actigraphy testing and sleep logs, followed by video polysomnogram and mean sleep latency testing (MSLT). Initial PSG results were notable for a total sleep time of 498 minutes, a normal apnea-hypopnea index of 0.7/hr., sleep onset latency of 0 minutes, and REM latency of 0 minutes. Based upon military medical standards, the MSLT scheduled for the following morning was cancelled due to limited sleep during the preceding 2 weeks as measured via actigraphy. However, the patient’s sleep logs

reported over 7.5 hours of sleep per night. The patient’s video PSG was reviewed, which

showed evidence of frank RSWA, as well as episodes of dream enactment behavior during REM

sleep, all including very violent movement in his upper extremities. Repeat trial of MSLT 2

weeks later showed mean sleep latency of 1.7 minutes, with 5 sleep onset REM periods

(SOREMPS), and evidence of dream enactment involving the upper extremities during 3 of

these SOREMPS.

Conclusion

We present the case of a patient with narcolepsy and RBD with significant upper extremity movements to the point of affecting the actigraphy sleep/wake detection algorithm. Actigraphy has been validated as a diagnostic tool in assessing sleep and wake patterns in individuals without significant REM sleep dissociation phenomena; however, our case highlights the necessity of further research of the validity of actigraphy in patients with Narcolepsy and/or REM behavioral disorder.

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0806 REM sleep without atonia in the Setting of Duchenne muscular dystrophy: Can it Serve a Protective Role?
Authors: Sarrami Kayvon , Patel Jay , Sisan Mahtab Moshtagh , Avidan Alon
Published: May 2022

Introduction

Duchenne muscular dystrophy (DMD) has well-known associations with sleep-related breathing disorders such as sleep apnea and nocturnal hypoventilation. These disorders naturally follow from the progressive muscle weakness that is the hallmark of DMD. New data is emerging on the prevalence of other polysomnographic features affecting patients with DMD, particularly as they relate to the phenotype of the sleep disorders unique to DMD. We report on a patient with REM sleep without atonia (RWA) in DMD.

Report of Cases: A 13-year-old male with DMD was referred for a polysomnogram (PSG) due to new-onset snoring, but there was no history to suggest dream enactment behavior. He was not taking any CNS-acting medication such as serotonergic or sedative-hypnotic medications. Neurologic examination was notable for diffuse symmetric muscle weakness requiring the use of a wheelchair. PSG revealed obstructive sleep apnea syndrome with an apnea-hypopnea index of 3/hr overall and 5/hr during REM sleep, a respiratory disturbance index of 9/hr with minimum O saturation of 84%, and evidence of RWA in three 30-second epochs meeting AASM scoring criteria. Dream-enactment behavior was absent during the PSG and historically.

Conclusion

We report on a previously unrecognized finding of RWA the setting of DMD. While sleep studies are routinely performed in DMD due to the significant association with sleep-disordered breathing and REM-related hypoventilation, the presence of RWA was unusual. The finding of RWA in the setting of a dystrophinopathy prompts important questions. While it is unlikely that RWA is an emerging sign of an ⍺-synucleinopathy in the setting of DMD, its presence in a young teenager raises the possibility of hypocretin deficiency in the setting of DMD, as is the case with myotonic dystrophy type 1 reflecting impaired hypocretin neurotransmission. However, a more attractive explanation is that RWA may promote a protective effect against sleep apnea in patients with vulnerability to REM-related hypoventilation. Our data suggest a potential protective role conferred by the preservation of muscle tone in the setting of sleep apnea in neuromuscular conditions.

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None
0807 Sleep-related hiccups: A case report of antidepressant associated hypnic jerks
Authors: Mah Cheri , West Leslie , Hekmat Anahid
Published: May 2022

Introduction

A hiccup is a sudden activation of inspiratory muscles, followed by paradoxical glottic closure, producing a familiar sound [1]. Antidepressants are commonly prescribed and hypnic jerks and hiccups have been reported as a rare adverse drug reaction [2]. Few reports have described pronounced hypnic hiccups with subsequent audio/video polysomnogram confirmation.

Report of Cases: A 44-year old man with a history of depression, anxiety, and alcohol use disorder presented to clinic with a long history of violent hypnic jerks associated with a loud hiccup. These episodes started 30 minutes before he fell asleep and continued throughout the night every 30 minutes with forceful hiccups that were so loud they wake others in his house. We believe that these episodes were likely induced by venlafaxine since they were noted initially 8 years ago when he started taking venlafaxine and there was complete remission after discontinuation of the medication. He was later prescribed escitalopram 20 mg daily, restarted on venlafaxine XR 150 mg daily, and later sertraline 100 mg daily due to refractory depression and anxiety recurrence. Episodes progressively became more frequent and pronounced that he avoided sleep and they impacted his ability to hold a relationship. Gabapentin and clonazepam did not previously improve symptoms. He took trazodone 50 mg every other night to aid falling asleep and reported to have less frequent hypnic jerks. Upon presentation to our clinic, subsequent PSG demonstrated severe obstructive sleep apnea (AHI 63, HI 52, CI 11) with significant sleep fragmentation (sleep efficiency 46%) with very frequent sudden axial myoclonic contractions with head and neck movements along with vocalizations likely representing hiccups. Video and audio demonstrated the loud hiccups described. These events occurred while the patient was resting awake and transitioning to sleep and was less frequent but persisted in N2. The patient was prescribed CPAP for sleep apnea and recommended to follow up with psychiatry to consider other medications.

Conclusion

Pronounced hypnic jerks and sleep-related hiccups can significantly impact total sleep time, sleep quality, and quality of life. Clinicians should be aware of these potential side effects in patients on antidepressants.

Support (If Any)

1. Askenasy JJ. About the mechanism of hiccup. Eur Neurol. 1992;32:159-63.2. Bagheri H, Cismondo S, Montastruc JL. Hoquet d'origine médicamenteuse: enquête à partir de la Banque Nationale de Pharmacovigilance [Drug-induced hiccup: a review of the France pharmacologic vigilance database]. Therapie. 1999 Jan-Feb;54(1):35-9. French. PMID: 10216420.
0809 Late Onset Narcolepsy With Cataplexy
Authors: Matlub Gulraiz , Greenough Glen
Published: May 2022

Introduction

NT1 is characterized by EDS, cataplexy, sleep related paralysis, and hallucinations. Cataplexy is defined as weakness precipitated by emotions, more commonly with positive emotions and is associated with narcolepsy.

Report of Cases: 70 yo with history of OSA on APAP referred with year history of worsening EDS, dream enactment, and cataplexy. Initially CPAP titration followed by MSLT was performed. Unfortunately sleep logs/actigraphy were not performed. CPAP was titrated 7-10 cm demonstrating suboptimal control (AHI 30) and presence of RSWA. MSLT demonstrated MSL of 2.9 minutes with 5 SOREMPS, however was confounded by THC use, suboptimal OSA control, and Trazodone. Considering above, patient was started on 11-16cm, and reevaluation was recommended. Patient was seen by neurology and started on Fluoxetine 20mg with some relief in symptoms (4-5episodes/day to 1-2/day). Imaging was unremarkable. Reevaluation was planned. He stopped Fluoxetine and THC 3 weeks prior. Actigraphy demonstrated significant movement. PSG demonstrated SOREMP with optimal OSA control (15-17cm), but there was recurrent evidence of vocalization with RSWA. MSLT was significant for MSL of 2.5 minutes, and 4 SOREMPs. Patient was restarted on Fluoxetine 40mg and melatonin 15mg for RBD. With optimal CPAP pressure daytime symptoms improved, however cataplexy worsened (3-4/day) triggered by laughter. Fluoxetine dose was increased with some relief the following days, but cataplexy returned leading to fall. He was transitioned to venlafaxine. With that patient noted complete resolution for 3 weeks. Cataplexy later recurred but resolved with an increase to venlafaxine 150mg. Three weeks later episodes recurred 3-4/week, with few causing collapse. Pitolisant was prescribed and currently pending prior authorization.

Conclusion

This case illustrates an unusual case given late onset of NT1 and refractory cataplexy that is disproportionately severe than the sleepiness. Cataplexy in geriatric populations is associated with falls and fractures. Medication for treatment of cataplexy comes with its own risks. Considering age and frequent falls, Pitolisant was added to the venlafaxine. It is noteworthy that daytime sleepiness improved with CPAP. There have been case reports with delayed onset NT1, however EDS was the prominent factor which responded well to stimulants, in our case cataplexy was refractory and prominent.

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0811 Patient with Pierre Robin Sequence and Moebius Syndrome - Diagnostic and Treatment Challenges
Authors: Gallo Michael , Hassan Fauziya
Published: May 2022

Introduction

Pierre Robin sequence includes craniofacial anomalies - micrognathia, mandibular hypoplasia, glossoptosis, and cleft palate resulting in obstructive sleep apnea (OSA), respiratory dysfunction, and feeding difficulties. Moebius syndrome is a brainstem anomaly that causes cranial nerve VI and VII palsy, causing oculofacial paralysis, palatal weakness with feeding difficulties, and aspiration. We present a case of an infant with Pierre Robin and Moebius syndrome with sequalae of both - OSA and aspiration diagnosed after the neonatal period.

Report of Cases: A 4-month-old male infant was referred for suspected sleep-disordered breathing (SDB) in context of Pierre Robin sequence, Moebius syndrome, and a premature birth at 32 weeks gestation. Shortly after birth, the patient experienced respiratory distress requiring continuous positive airway pressure (CPAP) support in the neonatal intensive care unit. Also, he required a pediatric intensive care admission at 2 months of age for acute respiratory failure with hypoxemia requiring high-flow nasal cannula, and difficulty feeding. Parents reported snoring, apneas with cyanosis, and gasping respirations at night. Physical exam demonstrated micrognathia, glossoptosis, high-arched narrow palate, and the inability to fully close his eyes or mouth. Care at an outside facility did not include a laryngoscopy, swallow study, or sleep study. The patient had inadequate weight gain, eventually developed failure to thrive (FTT), and was admitted for an upper respiratory tract infection and dehydration that required a nasogastric tube. An urgent split-night polysomnogram was performed, which demonstrated OSA with an apnea-hypopnea index (AHI) of 22/hour and an oxygen saturation nadir of 83%. Use of 0.5 L/min of supplemental oxygen via nasal cannula, lowered his AHI to 1/hour. Urgent referrals to otolaryngology, oral and maxillofacial surgery were placed and both specialties recommended delay in surgical intervention. A swallow study was performed and demonstrated aspiration with all consistencies. He was discharged with supplemental oxygen for OSA with gastrostomy tube placement pending.

Conclusion

This case highlights a delayed diagnosis of OSA and aspiration in an infant with Pierre Robin and Moebius syndrome with FTT. The presentation emphasizes the importance of early recognition of SDB to provide appropriate treatment during the essential developmental stages of life.

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0813 Post-Pinealectomy Insomnia and Melatonin Therapy
Authors: Johal Arminder , Manchanda Shalini , Bodkin Cynthia , Stahl Stephanie
Published: May 2022

Introduction

Melatonin is a hormone produced in the pineal gland that has an important role in sleep; immune, neurologic, psychiatric, metabolic, and endocrinologic function; cardiac-autonomic regulation and even cancer risk. We present a case of insomnia, somnambulism, dream enactment, and periodic limb movements of sleep (PLMS) after a pinealectomy.

Report of Cases: A 40-year-old woman with a history of a complete pinealectomy due to a pineal cyst presented to the sleep medicine clinic. Shortly after the pinealectomy, she developed sleep onset and maintenance insomnia. Two years later she developed somnambulism, and four years later she developed dream enactment and PLMS. She reported no prior treatments for her sleep issues, including no history of melatonin use. On average, her total sleep time (TST) was 2-8 hours/night with awakenings every 2 hours. Sleep latency was 10-45 minutes. Polysomnograghy demonstrated an apnea-hypopnea index of 0.6/hr, PLM index of 68.1/hr,

normal REM atonia, and no complex behaviors. The patient started 1mg immediate release (IR)

melatonin, which did not help her insomnia, but parasomnias resolved. She had improvement

in her PLMS with iron supplementation and melatonin. The melatonin dose was increased to

3mg IR which helped increase her TST to 4-8 hours. She was switched to 3mg extended

release (ER) melatonin, and then increased to 4mg ER. She obtained the most benefit for her

insomnia with 1mg IR plus 4mg ER with sleep latency reduced to 5-10 minutes and TST

improved up to 7.5 hours with rare awakenings.

Conclusion

Pinealectomy in humans is rarely reported. Most data about the consequences of pinealectomy and pathophysiology of melatonin come from animal research. Melatonin level after pinealectomy is often undetectable or severely diminished. Current limited literature on patients with pinealectomy consists of case reports about patients who experienced insomnia, non-24-hour sleep-wake rhythm disorder [SSM1] and mood disorders. Melatonin doses ranging from 0.5mg to 14mg IR and up to 5mg ER have been trialed with most patients having symptomatic improvement with doses above 3mg. We found that a combination of 1mg immediate and 4mg extended release melatonin was the most beneficial for our patient.

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0815 Treatment Success in a Resistant Case of Insomnia with an Irregular Circadian Rhythm Disorder
Authors: Culp Marilyn , Wani Anna
Published: May 2022

Introduction

A 19-year-old non-verbal male with history of CHARGE syndrome, severe autism, intellectual disability, coloboma with blindness OD and severely imparied vision OS, deafness, self-injurious and aggressive behavior, Tetralogy of Fallot status post repair, pulmonary valve replacement, hypertension, hypothyroidism, megacolon, gastrostomy tube dependence, eosinophilic esophagitis and chronic kidney disease with an irregular sleep cycle who has failed multiple medications for insomnia has shown treatment success with suvorexant.

Report of Cases: This patient’s sleep schedule ranges from 1.5 to 5 hour segments at various times of day or night including naps at school with occasional longer periods of sleep up to 10 hours and longer periods of wakefulness up to 22 hours who has been treated with the following medications: trazodone, clonidine, hydroxyzine, diphenhydramine, quetiapine, gabapentin, mirtazapine, eszopiclone, melatonin and ramelteon. His behavioral problems have been treated with olanzapine. He continued to be aggressive and difficult to direct. His parents reported exhaustion. Then, suvorexant 5mg was added at bedtime while the following sleep medications were continued: gabapentin total daily dose of 1500mg (300mg in morning and 3pm; 900mg at bedtime, 300mg one hour later if still awake), ramelteon 8mg, mirtazapine 7.5mg and olanzapine 10mg at bedtime and bid prn aggressive behavior. He also takes the following daily medications: bisacodyl, polyethylene glycol, simethicone, hyoscyamine, cholecalciferol, aspirin, levothyroxine, hypoallergenic nutritional formula, starch and albuterol prn. With the addition of suvorexant 5mg, he had been able to get 9.5 hours of consolidated sleep at night with improvement in his behavior until he contracted Covid-19 and regressed. The suvorexant dose was increased to 10mg which again improved his insomnia and behavior.

Conclusion

Various medications have either not worked at all or have worked suboptimally for insomnia in this medically complex patient who has an irregular Circadian rhythm disorder. Adding an orexin receptor antagonist as a novel mechanism to his regimen has shown promise. At this time, this patient has been stable for one month with suvorexant 10mg at bedtime after regression on the 5mg dose that coincided with a Covid-19 infection. We are proceeding with cautious optimism.

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0819 Restless Sleep, Snoring, Excessive Daytime Sleepiness, And Ataxia: A Pediatric Quandary
Authors: Francis Natalie , Stanton Crystal , Freire Amado , Bashir Ahsan
Published: May 2022

Introduction

Central sleep apnea in pediatrics is inherently complex, often going undiagnosed and untreated. Signs and symptoms of central nervous system dysfunction may be attributed to more common childhood conditions. We aim to educate on the diagnosis and treatment of central sleep apnea in this vulnerable patient population.

Report of Cases: A 12-year-old girl presents for initial sleep visit with global developmental delay, borderline obesity, chronic headaches, early morning tremors, and insulin resistance. She has history of snoring and restless sleep for years with severe daytime sleepiness for the past three months with worsening over two to four weeks. She is unable to stay awake in school. Epworth sleepiness scale (ESS) score is twenty-one. There is history of recurrent Strep but no history of infectious mononucleosis or influenza infection. Primary sleep disorders or CNS hypersomnia are suspected, and polysomnogram with MSLT is ordered. MRI of the brain is pending. The patient presents to the emergency department ten days later with frontal headaches, ataxia, altered speech, and oral aversion. CT head is normal. Hematology, neurology, and neurosurgery are consulted. MRI of the brain shows thrombosis of the superior sagittal sinus, the right transverse sinus, the right sigmoid sinus, the right jugular bulb, as well as cortical veins overlying both cerebral hemispheres, and she is started on anticoagulation for suspected stroke. A polysomnogram shows a severe degree of central sleep apnea with a central apnea index of 49.9 events per hour. There is significant periodic breathing present. There is no nonapneic hypoxemia or sleep hypoventilation present, and the patient is scheduled for an oxygen titration study.

Conclusion

Central sleep apnea is more prevalent in older male individuals and may affect patients with heart failure1.Pediatricians and subspecialists must remain vigilant as children are also susceptible to medical conditions which result in central nervous system dysfunction.

Support (If Any)

1.) Donovan LM, Kapur VK. Prevalence and Characteristics of Central Compared to Obstructive Sleep Apnea: Analyses from the Sleep Heart Health Study Cohort. Sleep. 2016;39(7):1353-1359. Published 2016 Jul 1.doi:10.5665/sleep.5962
0820 Sleep and Oculopharyngeal Muscular Dystrophy: disease progression affecting ventilatory needs and treatment of sleep-disordered breathing
Authors: Muraida Susan , Cutrufello Nicholas , Begay Melissa , Grigg-Damberger Madeleine , Kern Joseph
Published: May 2022

Introduction

Oculopharyngeal muscular dystrophy (OPMD) is an autosomal-dominant, late-onset, and progressive disease characterized by ptosis and dysphagia, sometimes proximal limb weakness and gait abnormalities. It often presents in patients in their 50s. The progressive functional decline of the pharyngeal muscles results in feeding difficulties and aspiration; however, patients may also have risk of nocturnal hypoventilation and sleep apnea, complicated by variable airway obstruction and compliance.

Report of Cases: Using retrospective chart review, we identified patients with a known diagnosis of OPMD treated at the Raymond G. Murphy VA Sleep Center. We present a case where OPMD progression necessitated increased ventilatory support and affected positive airway pressure (PAP) compliance. A 58-year-old male with OPMD, DMT2, depression, and memory impairment underwent home sleep apnea testing showing severe OSA (REI 32.7, SpO2 nadir 72%). He started Auto-PAP 6-16 cwp and presented to discuss issues tolerating PAP. Pressures were lowered, but he continued to require maximum pressures without increased utilization. An in-lab CPAP titration showed treatment-emergent centrals but did not find optimal pressures due to limited sleep time. Having failed CPAP, he returned for an ASV titration which controlled his apnea in lateral position. Patient was switched to auto ASV to increase efficacy and comfort. Two months later he discontinued ASV due to frustration with disease progression and feeling unable to breathe deeply with the machine. Nocturnal oxygen at 1L was ordered while he awaited Neurology consult for OPMD. Later, concerned about progressive dyspnea, he resumed ASV, now with 3L O2 bleed. Given suspicion of hypoventilation (bicarbs 27-30), and that ASV could not adjust to his continually changing airway tone with his OPMD, he was switched to iVAPS. This resulted in good control of his sleep apnea, tidal volumes and minute ventilation. However, he reported pressures felt too high, returned to ASV for a period, then discontinued PAP altogether.

Conclusion

Patients with OPMD and sleep apnea require close follow-up as their disease progression may affect their ventilatory support needs. These patients may require more complex PAP modalities, such as AVAPs, and routine PFTs to help determine timing of ENT involvement for surgical airway planning.

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0823 ACDF - A hidden etiology of OSA
Authors: Manahil Naila , Wasey Waiz , Mohammed Asiya
Published: May 2022

Introduction

OSA is characterized by recurrent episodes of upper airway collapse and obstruction during sleep, leading to oxygen desaturations. Risk factors include obesity, age, sex, family history, craniofacial abnormalities, stroke, diabetes, polycystic ovarian syndrome. This case illustrates symptomatic and clinically proven worsening of OSA after anterior cervical discectomy and fusion (ACDF), which can lead to narrowing of the airspace and dysfunction of the pharyngeal plexus leading to upper airway collapse.

Report of Cases: A 49-year-old female with cervical myelopathy was evaluated in the sleep clinic for snoring, witnessed apnea, and daytime somnolence. Her Epworth score was 11, and Mallampati score was 4, suggestive of underlying sleep-disordered breathing (SDB). A home diagnostic sleep test revealed mild OSA with AHI of 6.8/hr and was started on an Auto Continuous Positive Airway Pressure (APAP) device. She then reported worsening of her cervical pain leading to decreased use of APAP and total sleep time and underwent ACDF approximately 6 months after her diagnosis of OSA was made. Post-surgery she reported mild dysphagia, frequent nighttime awakening due to a choking sensation, and an increase in snoring. Following hospital discharge, the patient was re-evaluated in the sleep clinic for these complaints. Upon review, her BMI had decreased to 33.99 kg/m2, from 37.35 kg/m2, and the only medication change was the addition of hydrocodone-acetaminophen 5-325, 1-2 times daily as needed for pain. A repeat diagnostic home sleep study showed progression of OSA to moderate severity with an AHI of 24.5/hr, with no evidence of central sleep apnea or hypoventilation. Therefore, it was unlikely that the worsening of AHI was due to opioids. Thus, it was concluded that ACDF most likely led to the worsening of OSA.

Conclusion

Although there is no strong association in medical or sleep literature, this case demonstrates a strong association between ACDF and OSA. This highlights the importance of a timely diagnosis of new-onset or reassessment for worsening OSA in patients post-ACDF to improve sleep quality and prevent morbidity.

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0826 A Case of Propriospinal Myoclonus in a patient with Multiple Sclerosis
Authors: Maynard Rebeca
Published: May 2022

Introduction

Described first in 1991, by Brown et al, Propriospinal Myoclonus is a rare movement disorder of rhythmic, typically flexor, jerking motions most pronounced when lying supine. Although most cases are thought to be idiopathic, identified causes have been infection, spinal lesions, and psychogenic.

Report of Cases: A 38-year-old female with medical history of multiple sclerosis, anxiety and PCOS was referred for jerking body movements with sleep onset. Symptoms of nightly, full body jerking, beginning in her back, were described as “legs being pulled up towards the chest” and present only while lying supine in bed with symptom onset of 1 month. Difficulty initiating sleep and daytime sleepiness ensued as a result of these jerks. Work-up with in-lab polysomnogram failed to demonstrate obstructive sleep apnea (AHI 2.8/hr), however, did demonstrate periodic limb movements of sleep. MRI of the brain, cervical spine, and thoracic spine were negative for new or enhancing brain or spinal cord lesions. CBC, CMP, TSH and ceruloplasmin were ordered for a complete myoclonus evaluation and found to be within normal limits. She was started on Klonopin 0.5 mg at night. Three month follow-up revealed improvement of symptoms, with the frequency of her myoclonus decreased to 1-2 episodes per week with reported less intensity of each event.

Conclusion

While proper work-ups are always important, this case reminds us of the diligence required when certain conditions exist. Although our patient had a negative myoclonus work-up, and is thought to have idiopathic myoclonus, the presence of multiple sclerosis made the investigation increasingly relevant. Work-up should include an MRI to rule-out spinal cord lesions.

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None
0830 A Pediatric Case of Myoclonic Seizures Precipitating Central Sleep Apnea And Sequences of Periodic Breathing During Sleep
Authors: Wisniewski Benjamin , Witt Rochelle , Dye Thomas , Arya Ravindra , Simakajornboon Narong
Published: May 2022

Introduction

Myoclonic seizures are known to be influenced by the sleep-wake cycle. Although myoclonic seizures have been shown to alter sleep architecture, little is known about their influence on sleep-disordered breathing.

Report of Cases: A 7-year-old male with a history of generalized epilepsy of unknown etiology with absence as well as myoclonic seizures, poor impulse control, and fine motor delay was referred for restless sleep and frequent nocturnal awakenings. A routine electroencephalography (EEG) performed 33 months prior to presentation, at an outside institution, reportedly showed 3.0 Hz generalized frontally dominant spike and wave discharges lasting less than two seconds. Video EEG (vEEG) revealed diffuse bilateral poly-spike and slow wave discharges, predominantly during sleep, and myoclonic seizures with abrupt whole-body jerks. Polysomnography, at this time, was characterized by mild central sleep apnea (AHI: 3.7; OAHI: 0.0; CAI: 2.8; REM AHI: 14.4). His lamotrigine dosing was increased to from 1.8 mg/kg/day to 3.6 mg/kg/day, at presentation, with seizure control for three months but he developed a recurrence of myoclonic seizures in the setting of sleep deprivation. A repeat vEEG was similar to previous findings and his lamotrigine was increased further and clobazam was added. Due to worsening behaviors, clobazam was discontinued and a weaning plan for lamotrigine was formulated. Repeat polysomnography demonstrated worsening central sleep apnea (AHI: 18.3, OAHI: 2.3), without hypoventilation, characterized by sequences of periodic breathing that occurred in conjunction with myoclonic seizures. The majority of seizure-induced periodic breathing episodes were observed during a one-hour period of NREM sleep near the end of the study. Stereotyped epileptiform activity occurred in conjunction with a resumption of hyperventilatory respirations following apneic events. The increased respiratory drive, however, did not appear to be clinically epileptic in nature as the epileptiform activity either preceded or trailed the respirations. A variable REM and then NREM predominance of central events on successive polysomnograms further suggests that his central events are not intrinsic but that they are precipitated by epileptiform activity. Zonisamide, an anticonvulsant with carbonic anhydrase

inhibitory properties was initiated and is being titrated to therapeutic goal.

Conclusion

This case illustrates an unusual presentation of myoclonic seizure-induced periodic breathing during sleep.

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Cincinnati Children’s Research Foundation.
0836 Atypical presentation of Biot’s breathing in a patient with Arnold Chiari Malformation II with complex sleep apnea/Sleep hypoventilation/hypoxemia not on opioid and with no history of meningitis
Authors: Pant Alok , Ghildiyal Naomi , Khan Yousaf , Baldridge Scott , Ho Minh , Monceaux Brittany , Koothrezhi Rupa , Liendo Cesar , Chernyshev Oleg
Published: May 2022

Introduction

Sleep disordered breathing is very common in Arnold Chiari malformation but Biots’s breathing has not been reported in these cases.

Report of Cases: 3-year-old with history of Arnold Chiari II malformation with hydrocephalus (s/p VP shunt and surgical decompression), spina bifida myelomeningocele (s/p in utero repair), subglottic stenosis with prior tracheostomy and eventual decannulation presented for evaluation of sleep disordered breathing. Presenting symptoms included witnessed apneas, cyanosis, daytime sleepiness and frequent awakenings. Physical examination was largely unremarkable.

Initial PSG demonstrated complex sleep apnea, with an AHI 20.6, REM AHI 57.8 per hour of

sleep, ETCO2 peak of 69 cm H20 and an O2 saturation nadir of 34%, with sleep related

hypoventilation/hypoxemia and Biot's breathing in the absence of opioid use and CNS infection.

During titration study, Biot’s breathing, complex apnea and sleep related hypoventilation/hypoxemia responded well to BiPAP ST of IPAP 18 cm H20 and EPAP 14 cm H20 BUR of 12 and oxygen of 1 L/min. She later developed intolerance to BiPAP due to high

pressures and was decreased to BIPAP 13/11 cm H2O. Later the patient discontinued the use of

BIPAP due to intolerance and was switched to night time O2 at 3-4 L/min. Per the parents, the

patient has been maintaining her oxygen saturation in the absence of BIPAP therapy with

oxygen use. Due to COVID, patient was unable to follow up but will be scheduled for a repeat

PSG in the near future. She followed with Neurosurgery for Arnold Chiari II and they

recommended no surgical intervention at this time due to functional VP shunt.

Conclusion

This is an atypical presentation of Biot’s breathing in the absence of CNS infections and opioid use in a patient with Arnold Chiari malformation II. Patient has complex sleep apnea, initially well controlled with BiPAP ST, but developed BiPAP intolerance. She is on oxygen with good control of hypoxemia in the absence of BiPAP therapy.

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0841 Case Report: Can LSD be Associated with Chronic Exploding Head Syndrome?
Authors: Manikat Feby Puravath , Kushida Clete
Published: May 2022

Introduction

Exploding head syndrome is a rare phenomenon characterized by a loud imagined noise at sleep onset. Less commonly, it is associated with a simultaneous stab of pain in the forehead. It may result in recurring arousals and anxiety regarding the events. The underlying pathophysiology remains unknown.

Report of Cases: A 27 year old male with no past medical history presented to the sleep medicine clinic with a 2-year history of sleep-onset pain between his eyes, like “a flash of

lightning,” associated with moderate to severe pain. He experiences “brain zaps” or “a jolt of electricity between his eyes every night.” He would yell and jolt out of bed due to this sensation. These episodes occur approximately 60 to 90 minutes after sleep onset almost every night, jolting him from sleep and lasting approximately 1 minute. He also has difficulty seeing in the dark and also eye twitching. He experiences palpitations, diaphoresis, and a sense of fear often with these episodes. He denies any neurological deficits or muscle jerks. He is not on any daily

medications. He does not regularly use any sleep aids but has tried melatonin 3 mg in

the past. Evaluation by ophthalmology has ruled out ophthalmologic and neuro ophthalmic

causes. He does report use of illicit substances including lysergic acid diethylamide (LSD),

marijuana, methylenedioxymethamphetamine (MDMA), and cocaine. He did not experience any unpleasant effects after his first use of LSD, however within minutes of his second ingestion, he felt a sharp pain on his nasal bridge and forehead 10/10 in intensity. He states that since then, this pain has recurred almost every night. He does not have a family history of any neurologic disorders or migraines. The current plan for workup includes an in lab polysomnography with seizure montage and, if negative, a trial of a tricyclic antidepressant is planned.

Conclusion

This case illustrates the potential issues after LSD use causing severe dopaminergic and serotonin surge with drug use and the value of a complete history. Awareness of a possible correlation is clinically useful and may serve as a caution in the use of recreational drugs.

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0842 Narcolepsy or Sarcoidosis?
Authors: Alam Ariful , Pham Christopher , Stansbury Robert
Published: May 2022

Introduction

Narcolepsy is a common cause of chronic sleepiness, affecting 1 in 2000 people. Despite the frequency of narcolepsy, the average time from the onset of symptoms to diagnosis is 5 to 15 years, and may remain undiagnosed in as many as half of all affected people. The etiology of this problem is largely unknown and usually multifactorial. The prevalence of these sleep disorders is increased in sarcoidosis compared to the general population. We present a case of narcolepsy in a patient with biopsy proven extrapulmonary sarcoidosis.

Report of Cases: 42-year-old female has a history of Sjogren’s syndrome (SSA/SSB positive, ANA positive 1:160), antiphospholipid syndrome with past left lower extremity deep venous thrombosis and positive LAC and anticardiolipin antibody, and Raynaud’s syndrome for more than 15 years, asthma, pleurisy, irritable bowel syndrome, GERD, depression, migraine and fibromyalgia. Her primary sleep complaint is excessive daytime sleepiness and nonrestorative sleep requiring frequent napping. She exhibited intermittent hypnagogic hallucinations but denies cataplexy. She has no history of smoking and denies drinking caffeine/alcohol. Has 3-5 isolated awakenings at night otherwise denies insomnia. She had a score of 17 on the Epworth scale. Her in-lab polysomnography demonstrated an AHI of 0/hr, sleep latency of 4.5 minutes and REM latency of 31.5 minutes. No other abnormalities noted. She then underwent a multiple

sleep latency test. Sleep was achieved in all the nap trials with a mean sleep latency of 1.2 minutes with three Sudden Onset REM Sleep recorded meeting criteria for narcolepsy. A subsequent PET/CT scan was obtained due to elevated liver enzymes and positive MRI findings, which incidentally showed a 2 mm enhancing focus involving the medial right cerebellar peduncle. She underwent biopsy which showed granulomatous disease consistent with sarcoidosis. Patient is currently successfully treated with methylphenidate for her underlying narcolepsy.

Conclusion

We present a patient with narcolepsy and multiple underlying autoimmune diseases including biopsy indicating extrapulmonary sarcoidosis. We, furthermore, present radiographic evidence in her brain that may have contributed to her narcolepsy.

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0843 Sexsomnia in a female patient with nightmares and obstructive sleep apnea: a case report
Authors: Bigman Daniel , Greenough Glen
Published: May 2022

Introduction

Sexsomnia has been described as various sleep-related abnormal sexual behaviors primarily associated with confusional arousals and more rarely linked to rapid eye movement (REM) sleep behavior disorder (RBD). Clinical descriptions are based on case reports and review series, which leads to diagnostic ambiguity, challenges with appropriate classification, and forensic and legal ramifications

Report of Cases: We report a 34 year old female with a past medical history of polycystic ovarian syndrome and childhood post-traumatic stress disorder. Family history significant for parent with lifelong parasomnias. The patient was referred to Sleep Medicine with concern for snoring, sleepiness, and sleep behaviors. The patient reports sleep talking since childhood which predate trauma, traumatic themed nightmares, and ex-husband and current spouse reporting sleep-related masturbation, sexual advances described as coital-like pelvic movements which often lead to intercourse and subsequent amnesia. In addition, spouse describes bruxism and ongoing night terrors. Physical examination notable for body mass index 44 kg/m2, high arched palate, Mallampati class III, and 18 inch neck circumference. The patient underwent a polysomnagram which demonstrated fragmented sleep architecture, total arousal index up to 20.3/hr, and totalsleep time in stage N3 20%, Four awakenings fromN3, and findings of N3 partial arousal. The patient was observed in v-PSG to have periods of nocturnal lagophthalmos, finger movements, and vocalizations during NREM sleep. However, many of these observations were in the setting of respiratory events. The apnea-hypopnea index was 16.9/hr and a minimum oxygen saturation of 81% and mean saturation of 95%. A total of 3.1 minutes were spent less than or equal to 90%. TCO2 was without concern for hypoventilation. The patient was started on Auto CPAP 5-15cmH2O with resolution of snoring and some improvement to sleepiness. However, spouse reports ongoing sexsomnia in the same frequency despite treatment of obstructive sleep apnea.

Conclusion

This case reports a female adult with persistent sexsomnia despite early treatment of obstructive sleep apnea which highlights the possibility of co-occurring sleep disorder. The case does support current limited literature around sexsomnias having features of partial arousals from NREM sleep given the co-occuring history of night terrors, elevated N3 awakenings, and evidence of cortico-cortical dissociation.

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None