bluuebunny/crossref_metadata_2025_split huggingface dataset:
0824 Can the interface utilized in continuous positive airway pressure therapy significantly alter the apnea-hypopnea index in certain patients with obstructive sleep apnea?
Authors: Dabaja Fauzieh , Skiba Virginia , Palmer William 
Published: May 2022

Abstract

Introduction
Multiple studies and analyses have demonstrated that use of an oronasal interface for delivery of continuous positive airway pressure (CPAP) therapy can lead not only to higher pressure requirements, but also a higher residual apnea hypopnea Index (AHI) in patients with obstructive sleep apnea (OSA).
Report of Cases: We record a 75-year-old man with severe OSA, AHI of 38, and treated with CPAP 16 cm water. On routine follow up, the residual AHI was 2.9, but he reported mouth-venting with nasal mask and stretched out the chinstrap. He was transitioned to an oronasal interface, and on subsequent follow-up his AHI was 19.6. The decision was made to change back to a nasal mask with a better-fitting chinstrap, which reduced his AHI to 4. To verify that the AHI recorded by the CPAP device was physiologic, the patient later used CPAP while wearing a home sleep apnea test (HSAT) utilizing peripheral arterial tonometry for 2 nights. On the night he used a nasal mask with a chinstrap, the AHI recorded on the CPAP was 1.4 and 3.9 on the HSAT. The following night, he wore the oronasal interface and the recorded AHI on CPAP was 31.6 and 18.5 on the HSAT.

Conclusion
Although oronasal masks are commonly used as an interface for CPAP, there is significant evidence that nasal delivery systems are more effective at controlling AHI in some individuals. Many studies show that oronasal masks not only correspond to an increase in AHI but also are associated with higher pressure requirements and a lower adherence compared to nasal interfaces. Imaging has shown greater retropalatal airway expansion when using a nasal interface compared to an oronasal device. Oronasal devices may also push the tongue posteriorly and cause increased occlusion of the airway. The consistent finding of worsening OSA control in the setting of oronasal masks has led many to consider CPAP with a nasal interface to be the gold standard for treating OSA. The clinician should therefore be mindful of these considerations when initiating or altering a CPAP interface.

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0838 Cheyne-Stokes Breathing in a Pediatric Patient with Dilated Cardiomyopathy and Muscular Dystrophy Prior to Heart Transplant
Authors: Dang LeQuan , Kaplan Kevin , Revana Amee 
Published: May 2022

Abstract

Introduction
Cheyne-Stokes breathing (CSB) has rarely been identified in the pediatric population. Neuromuscular diseases (NMD) such as Duchene Muscular Dystrophy (DMD) can predispose patients to sleep-disordered breathing including central sleep apnea (CSA) and CSB. Sleep-disordered breathing in children with NMD may not have symptoms; thus, treatment can be delayed. Currently, there is limited data to support resolution of CSB in DMD with dilated cardiomyopathy post-transplant.
Report of Cases: We present a 15-year old female with a significant history of both dilated cardiomyopathy and DMD who presented with acute on chronic heart failure. Due to her disease progression, she was listed for heart transplant. Prior to her transplant, she completed an inpatient polysomnography (PSG) to rule out sleep-disordered breathing due to concerns of snoring and dyspnea during sleep. Her Pediatric Daytime Sleepiness Scale score (PDSS) was 8. The polysomnogram recorded moderate obstructive sleep apnea (OSA) and central sleep apnea
(CSA) consistent with Cheyne-Stokes breathing along with rare premature ventricular
contractions (PVCs). Patient was started on BPAP of 13/8 cm H2O with a back-up rate of 12
breaths per minute after titration study. The patient subsequently received a heart transplant in
which the patient’s dyspnea and snoring resolved. Post-transplant PSG pending to reassess the
severity of sleep-disordered breathing.

Conclusion
Though CSA can be seen in children, CSB is rarely seen in children with either heart failure or muscular dystrophy. When CSB is observed, the cornerstone of treatment is correcting the underlying cause. This patient demonstrated CSB with symptoms that improved with BPAP and now post-heart transplant. When both heart failure and neuromuscular disease are involved, close monitoring for clinical symptoms along with screening for CSB is important and may affect overall quality of life and recovery.

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0840 Be Wary Of Adjustment Disorder with Anxiety In Patient With Insomnia And Discomfort With Positive Pressure Airway Therapy
Authors: Jamil Saad Bin , Kachechian Talar , Doghramji Karl , Fast Zhanna 
Published: May 2022

Abstract

Introduction
Insomnia is a common complaint. When it occurs in the context of treatment with continuous positive airway pressure (CPAP), it can complicate treatment and lead to dissatisfaction with CPAP therapy. Adjustment disorder with anxiety (ADWA) is a condition that develops following an identifiable stressor and can be associated with insomnia. We describe a case of ADWA causing disrupted sleep and dissatisfaction with CPAP therapy.
Report of Cases: The patient is an 80-year-old man with history of severe obstructive sleep apnea (OSA) and chronic obstructive lung disease, under stable management with CPAP. During a routine follow up visit, he reported the recent onset of frequent, and often prolonged, nocturnal awakenings associated with daytime sleepiness and fatigue. Insomnia was associated with discomfort with breathing, especially during exhalation, while using CPAP. His machine report indicated a significant decrement in compliance. Upon questioning, he reported that a few of his family members had died unexpectedly, following which he began to experience significant anxiety. His wife noted that he had become moody and irritable. On examination, the patient was visibly anxious; his speech was accelerated and animated, and he displayed psychomotor activation. Affect also displayed despondency. The patient was diagnosed with ADWA leading to insomnia and discomfort with CPAP use. He was prescribed buspirone 10 mg twice daily. On two week follow up patient reported no improvement in symptoms and was switched to escitalopram 10 mg daily. Following approximately three weeks, his clinical evaluation revealed significant improvement in mood; anxiety had dissipated, and sleep was more continuous with infrequent and brief awakenings. Daytime alertness had been restored. Of interest was the temporary return of anxiety and insomnia symptoms following the brief discontinuation of escitalopram because of hospitalization.

Conclusion
ADWA can occur in the setting of recent stressors and can result in sudden onset of insomnia, compromise CPAP adherence and decrease subjective benefit from PAP therapy. This case highlights the importance of questioning patients for the possibility of recent stressors and traumatic events when they develop difficulties with PAP use. These may indicate the presence of ADWA, whose management can restore CPAP compliance and improve sleep quality and daytime functioning.

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0844 Isolated, Idiopathic Periodic Breathing in a School Age Child
Authors: Isaacs Thomas , Valenzuela Andrew , Malhotra Sonal 
Published: May 2022

Abstract

Introduction
Periodic breathing (PB) is a well described physiologic phenomenon of infancy and usually resolves with age1. PB that persists outside of infancy has been described in association with various pathologies such as Chiari malformation, upper airway anomalies and genetic abnormalities2. Isolated PB that persists past infancy is less well described, and there is little data to guide subsequent management and clinical decision making.
Report of Cases: Here we present a case of a 12-year-old female with isolated PB on polysomnography (PSG). She initially presented to our sleep center clinic with complaints of snoring, gasping during sleep and daytime sleepiness. She had no significant past medical history and not on any sleep-modifying medications. PSG was ordered to evaluate for suspected obstructive sleep apnea. Total sleep time was 7.75 hours with a mildly reduced amount of REM (16% of total sleep time). She had a total of 4 minutes of central sleep apnea that met AASM criteria for periodic breathing. This occurred exclusively during transitions between N1 and N2 and was not associated with hypoxemia nor hypocapnia. The patient’s total apnea hypopnea index (AHI) was 2.97/hr, obstructive AHI 0.65/hr and central apnea index was 2.32/hr. Of note, this studywas not conducted at high altitude and was done in Houston (approximately 100 feet above sea level). Given the lack of significant sleep apnea, a conservative approach with nasal fluticasone and close follow-up was utilized.

Conclusion
Though rare, this case highlights the possibility of PB being an incidental finding of PSG in otherwise healthy, school aged children. The need for further research as to the significance of this incidental finding is also emphasized.

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1. MacLean, Joanna E., Dominic A. Fitzgerald, and Karen A. Waters. "Developmental changes in sleep and breathing across infancy and childhood." Paediatric respiratory reviews 16.4 (2015): 276-284.2. Ghirardo, Sergio, et al. "Central apnea and periodic breathing in children with underlying conditions." Journal of Sleep Research (2021): e13388.
0845 Large arachnoid cysts and hypersomnolence: symptomatic or not?
Authors: Shigeru Chiba , GoEun Han , Takashi Kambayashi 
Published: May 2022

Abstract

Introduction
Arachnoid cysts are the most common type of brain cyst. They are often congenital, and most of them are stable and do not require treatment. On the other hand, some of them present a symptomatic problem such as headache, nausea and seizures. This rare condition was seen in a female patient who also had hypersomnolence.
Report of Cases: A 37-year-old female presented to hospital for evaluation of excessive daytime sleepiness (EDS) for the past 20 years, with repeated attacks several times a day without any REM sleep related symptoms. She also showed irregular wake sleep pattern of sleep extending over 24 hours, disturbing nocturnal sleep after each episode. Her sleep log showed sudden onset hypersomnolence with sleep duration of 30-40 hours every week. Epworth sleep scale was 10/24. PSG demonstrated her sleep architecture of 8 hours duration was unusual with sleep onset REM period (SOREMP) and decreased N3 sleep stage. MSLT showed reduced sleep onset latency (average: 5 minutes) and sleep onset REM on 1 of 4 naps. Brain MRI revealed a large arachnoid cyst on her left cerebral hemisphere. She was diagnosed as narcolepsy type2 and referred to the department of neurosurgery, but due to unclear association between hypersomnolence and the cyst, surgical treatment was not carried out. She was started on Modafinil 50mg which progressively increased to 200mg, but little effect was seen in sudden onset hypersomnolence and caused side effects such as headache and nausea. After Modafinil was discontinued, Melatonin agonist was administered in order to control her irregular sleep-wake rhythm, and since then her sleep-wake cycle has changed relatively stable and regular. At present, the frequency of hypersomnolence is gradually getting low and Epworth is 4/24.

Conclusion
In the present case, there is no evidence that her symptoms concerning sleep were secondary to any other disorder. Also, to our knowledge, there have been only a few cases reporting coexistence of hypersomnolence and arachnoid cysts. We are carefully following up on this patient and will reconsider to refer her to neurosurgery if any other symptoms appear.

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0846 Severe OSA in a 17yr old patient with Prader Willi Syndrome and poorly controlled Diabetes Mellitus
Authors: Kochhar Rashi , Geer Jacqueline , Guandalini Cindy , Weiss Pnina 
Published: May 2022

Abstract

Introduction
Prader Willi Syndrome (PWS), while rare, is the most common genetic cause of obesity. It is caused by a functional loss of paternally expressed genes from chromosomal region 15q11-13. PWS is characterized by short stature, neurodevelopmental delay, obsessive-compulsive behaviors, hypothalamic dysfunction, and hyperphagia, associated with an aberrant satiety response and elevated levels of ghrelin. Excessive daytime sleepiness (EDS) and sleep-disordered breathing (SDB), including obstructive sleep apnea (OSA) and central sleep apnea, are common.
Report of Cases: A 17-year-old male, with PWS, morbid obesity, autism, and diabetes mellitus (DM) type-2 was referred for evaluation of snoring and EDS (Epworth Sleepiness Score 16). He had recently been admitted with diabetic ketoacidosis associated with binge eating. He had no prior sleep testing. He denied sleep paralysis, hypnagogic/hypnopompic hallucinations, and cataplexy. He had a body mass index of 54 kg/m2 with short stature (1.6m). On exam, he had a Mallampati score of 4 and 1+ tonsils. A recent echocardiogram was normal. Polysomnography showed sleep latency of 10 mins, apnea-hypopnea index (AHI) of 148/hr, nadir oxygen saturation of 73%, with 45% of total sleep time &lt; 90%. Hypoventilation was not present. He was prescribed auto-titrating positive airway pressure (PAP) therapy. His mother was instructed to start locking access to food again. He was treated with insulin and metformin, with a plan to start on a glucagon-like peptide-1 (GLP-1) agonist. He had been on growth hormone (GH) previously, but in the context of his other morbidities, it was not reinitiated.

Conclusion
It is critical to screen and monitor patients with PWS for OSA, as SDB occurs in almost 70%. While PAP therapy may improve OSA, there is often residual EDS (likely due to underlying hypothalamic dysfunction), and alerting agents may be needed. GH can be considered for short stature but is contraindicated in patients with morbid obesity, untreated severe OSA, and uncontrolled diabetes. Management of diabetes, obesity, and hyperphagia in patients with PWS is challenging. Supervision and strict limitation of food intake using physical barriers are important. While data are limited, GLP-1 agonists may provide potential benefits for weight, glycemic, and appetite control in patients with PWS.

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0847 Sleep Disordered Breathing in a case series of 4 children with Trisomy 18
Authors: Kochhar Rashi , Brumer Eliaz , Canapari Craig 
Published: May 2022

Abstract

Introduction
Trisomy 18 (Edwards syndrome) is the second-most-common viable autosomal trisomy syndrome after trisomy 21, occurring in 1 in 2500 pregnancies. Obstructive Sleep Apnea (OSA) in Trisomy 21 has been well described in the literature. Even though trisomy 18 patients have upper airway obstructive (UAO) features overlapping with trisomy 21 that could predispose them to develop SDB, there is a dearth of data published about it.
Report of Cases: We present a case series of four patients with trisomy 18 who were evaluated for SDB aged 17mo-3yrs at the time of the reported polysomnographies (PSGs). Two patients had multiple prior studies. Moderate OSA was noted in two patients, and one was noted to have severe OSA, while the fourth patient had resolution of their severe OSA post adenotonsillectomy (T&A). Reduced sleep efficiency was noted in 2 patients. All but one patient had abnormal EEGs, consistent with known underlying seizure disorders. While all patients desaturated during sleep, only two patients fulfilled the criteria for hypoxemia (SpO2 below 90% for more than 5
minutes). One of these had resolution of hypoxemia with a trial of positive airway pressure therapy (PAP) of 5 cmH2O on a titration study. While capnography showed hypoventilation in two patients, one of the patients was treated with supplemental oxygen and the recommendation for titration PSG study to evaluate the need for PAP.

Conclusion
With aggressive interventions, children with trisomy 18 have seen a higher survival over the recent years. These children often have micrognathia or retrognathia, midface hypoplasia, glossoptosis, and hypotonia, predisposing them to have UAO. Endoscopic assessments reveal laryngomalacia and/or tracheomalacia, tonsillar and adenoid hypertrophy. In a previously reported study by Kettler et al. (2020), a prevalence of SDB of 44.68% was noted compared to the 1-4% average prevalence in non-syndromic children, hence clinicians should have a low threshold to screen them. In our small case series, all 4 patients had moderate-to-severe OSA, to begin with. Our results show that both surgery and PAP therapy may be successful in the treatment of OSA. More longitudinal data is needed to understand the pathology and management of SDB in these children.

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0851 Longitudinal Management of Narcolepsy with Atypical presentation of Cataplexy (unilateral with symptoms only on the right side for the past 13 years)
Authors: Pant Alok , Ghildiyal Naomi , Khan Yousaf , Ho Minh , Monceaux Brittany , Liendo Cesar , Chernyshev Oleg 
Published: May 2022

Abstract

Introduction
Narcolepsy with atypical unilateral cataplexy is a rare phenotype. We would like to share single center, single patient experience over the last 13 years of managing this patient.
Report of Cases: 64-year-old female diagnosed with narcolepsy with unilateral cataplexy in 2008 with MSLT. Her cataplexy syndrome has been atypical, in that it has a strictly unilateral presentation for the past 13 years, with right face, arm, and leg weakness with strong emotions(anger/laughter). Her right-side extremities go limp for a few seconds to a minute.
Her cataplexy syndrome has never evolved to become bilateral or unilaterally on her left side.
Her physical examination has been largely unremarkable. For Narcolepsy, was on Modafinil (2008) which was up titrated to 600 mg with no side effects, eventually switched to Armodafinil 250 mg (2013). Eventually did not control EDS fully, methylphenidate (2014) was added. The patient reported cognitive impairment with gradual memory loss on stimulants (starting in 2012) and nocturnal insomnia on methylphenidate SR. Due to worsening cognition and memory, stimulants were discontinued in 2014 and patient referred to neurology and had neuropsychiatric evaluation for memory loss. Dementia lab workup and MRI with spectroscopy were unremarkable. Due to continuation of EDS was restarted on Modafinil until May 2021. In May 2021, she reported no longer fully controlled daytime sleepiness and Pitolisant was added with improvement in ESS from 17/24 to 9/24. For the management of unilateral cataplexy, patient was well controlled initially on venlafaxine (2008), eventually up titrated with an additional dose a few times. After cognitive impairment, patient stopped refilling it in 2014 and subsequently slept better without the venlafaxine. She reported that she would fall when she got very upset/mad so learnt not to get mad at people. She had many episodes of unilateral cataplexy mainly with laughing or excitement so was started on Fluoxetine 20 mg and eventually up titrated to 40 mg. Her unilateral cataplexy symptoms have been well controlled with the addition of Pitolisant.

Conclusion
Management of Narcolepsy with atypical unilateral cataplexy is challenging and requires use of multiple medications with different sites of action.

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Targeted muscle reinnervation prevents and reverses rat pain behaviors after nerve transection
Authors: Roth Elizabeth , Linehan Allison , Weihrauch Dorothee , Stucky Cheryl , Hogan Quinn , Hoben Gwendolyn 
Published: May 2022

Abstract
Targeted muscle reinnervation (TMR) is a clinical intervention that is rapidly becoming common in major limb amputation to prevent or reduce amputation-related pain. However, TMR is much less effective when applied long after injury compared with acute TMR. Since the mechanisms governing pain relief in TMR of amputated nerves are unknown, we developed a preclinical model as a platform for mechanistic examination. Following spared nerve injury (SNI), rats underwent either TMR, simple neuroma excision, or a sham manipulation of the injury site. These interventions were performed immediately or delayed (3 or 12 weeks) after SNI. Pain behavior was measured as sensitivity to mechanical stimuli (pin, von Frey, and dynamic brush) and thermal stimuli (acetone and radiant heat). Spared nerve injury produced hypersensitivity to all mechanical stimuli and cold, which persisted after sham surgery. Targeted muscle reinnervation at the time of SNI prevented the development of pain behaviors and performing TMR 3 weeks after SNI reversed pain behaviors to baseline. By contrast, TMR performed at 12 weeks after SNI had no effect on pain behaviors. Neuroma excision resulted in significantly less reduction in hyperalgesia compared with TMR when performed 3 weeks after SNI but had no effect at 12 weeks after SNI. In this model, the pain phenotype induced by nerve transection is reduced by TMR when performed within 3 weeks after injury. However, TMR delayed 12 weeks after injury fails to reduce pain behaviors. This replicates clinical experience with limb amputation, supporting validity of this model for examining the mechanisms of TMR analgesia.
Resurgent neuropathic discharge: an obstacle to the therapeutic use of neuroma resection?
Authors: Tal Michael , Minert Anne , Devor Marshall 
Published: May 2022

Abstract
Ectopic discharge (“ectopia”) in damaged afferent axons is a major contributor to chronic neuropathic pain. Clinical opinion discourages surgical resection of nerves proximal to the original injury site for fear of resurgence of ectopia and exacerbated pain. We tested this concept in a well-established animal neuroma model. Teased-fiber recordings were made of ectopic spontaneous discharge originating in the experimental nerve-end neuroma and associated dorsal root ganglia in rats that underwent either a single transection (with ligation) of the sciatic nerve or 2 consecutive transections separated by 7, 14, 21, or 30 days. Ectopia emerged in afferent A and C fibers after a single cut with kinetics anticipated from previous studies. When resection was performed during the early period of intense A-fiber activity, a brief period of resurgence was observed. However, resection of neuromas of more than 14 days was followed by low levels of activity with no indication of resurgence. This remained the case in trials out to 60 days after the first cut. Similarly, we saw no indication of resurgent ectopia originating in axotomized dorsal root ganglion neuronal somata and no behavioral reflection of resurgence. In summary, we failed to validate the concern that proximal resection of a problematic nerve would lead to intense resurgent ectopic discharge and pain. As the well-entrenched concept of resurgence is based more on case reports and anecdotes than on solid evidence, it may be justified to relax the stricture against resecting neuromas as a therapeutic strategy, at least within the framework of controlled clinical trials.