bluuebunny/crossref_metadata_2025_split huggingface dataset:
0807 Sleep-related hiccups: A case report of antidepressant associated hypnic jerks
Authors: Mah Cheri , West Leslie , Hekmat Anahid 
Published: May 2022

Abstract

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

Abstract

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

Abstract

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

Abstract

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

Abstract

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

Abstract

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

Abstract

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

Abstract

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

Abstract

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