bluuebunny/crossref_metadata_2025_split huggingface dataset:
1190 Periodic Limb Movement Disorder Masked by Concurrent Focal Aware Seizures
Authors: Rasul Ammar , Khan Anees , Peters Jon , Motamedi Gholam 
Published: April 2024

Abstract

Introduction
Sleep disorders such as sleep apnea and sleep-related movement disorders are more common in patients with epilepsy compared to the general population. Complex rhythmic motor events related to periodic limb movement disorder (PLMD) can overlap with the hypermotor phenomenon of nocturnal seizures, making it a challenging diagnosis solely based on clinical description. The recognition of these repetitive stereotypical movements from seizures would be essential to guide targeted therapy.

Report of case(s)
We report a patient with focal aware seizures who concurrently experienced sensory symptoms typical of restless leg syndrome (RLS) as her ictal symptoms, as well as paroxysmal nocturnal motor symptoms meeting the diagnostic criteria of PLMD. A 51-year-old right-handed female presented with two years of seizures and nocturnal episodes primarily characterized by creepy crawly feeling in both her upper and lower extremities as well as her trunk with intact awareness. The patient also endorsed non-restorative sleep and daytime sleepiness. During an admission to the epilepsy monitoring unit, several habitual events were recorded that were consistent with fronto-temporal seizures. Interestingly, the majority of her ictal events started with the same creepy crawly sensation. Blood count, serum chemistry, and liver and renal function tests were unremarkable, except for very low serum ferritin levels (4.5 ng/ml) which, in combination with her sensory symptoms raised suspicion for PLMD. Subsequent polysomnography (PSG) showed mild OSA (AHI 9.8/h) and moderate to severe PLMD (43 events/h). Symptoms improved after iron supplementation along with better seizure control (adding lamotrigine to her levetiracetam). A repeat ferritin improved to normal range (46 ng/ml).

Conclusion
Our case demonstrates that clinical presentation of RLS/PMLD can be indistinguishable from the primary seizure semiology. RLS/PLMD can be a consideration in patients with epilepsy complaining of sensory ictal semiology as seen in this case and may warrant further work-up.

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1191 A Pediatric Case of Treatment Emergent Central Sleep Apnea During High Flow Nasal Cannula Polysomnography
Authors: Giles Cristina , Wisniewski Benjamin , Hawkins Stephen 
Published: April 2024

Abstract

Introduction
High flow nasal cannula (HFNC) is an alternative therapy to continuous positive airway pressure (CPAP) in pediatric populations with behavioral intolerance or contraindications to CPAP. Studies evaluating HFNC as a treatment modality in the pediatric literature have been limited.

Report of case(s)
A previously healthy 12-year-old male with a history of obesity presented with partial and full thickness burns to his face, neck, chest, and bilateral upper-extremities following a bonfire accident. His burns covered 15% of his total body surface area. He required hospitalization for acute burn management and skin grafting. During his hospitalization, he was observed to have snoring, witnessed apneas, and nocturnal hypoxemia concerning for underlying obstructive sleep apnea. He was unable to utilize a non-invasive positive pressure mask interface due to his extensive facial dressings and pressure sensitive skin grafts. He was discharged home on high flow nasal cannula at 10-15 Liters/minute. The patient experienced weight gain following hospitalization, possibly related to Cyproheptadine use for burn-related itching. Approximately 10 months after discharge, the patient underwent split-night HFNC polysomnography. The diagnostic portion of his study revealed severe obstructive sleep apnea with an OAHI of 28.8 events/hour. There were no observed central or mixed respiratory events and no REM stage sleep during the diagnostic portion of the study. After initiating HFNC at 40 L/minute and 34 degrees Celsius per sleep laboratory protocol, the OAHI decreased to 0.0 events/hour despite REM rebound, and the CAHI increased to 16.8 events/hour. This study was conducted at approximately the same altitude as the patient’s home.

Conclusion
This patient experienced treatment emergent central sleep apnea during HFNC polysomnography. Treatment emergent central sleep apnea with HFNC has previously only been described in a limited number of pediatric patients with underlying chronic lung disease, developmental delay, or genetic syndromes. This case highlights HFNC as an alternative treatment modality to CPAP as well as the need for further understanding of HFNC titration in pediatric sleep laboratories.

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The Breathing Institute at Children’s Hospital Colorado
1194 A Case of Unsuccessful Positive Airway Pressure Titration
Authors: Walia Neal , Chang Melisa , Zeidler Michelle 
Published: April 2024

Abstract

Introduction
Oronasal masks are commonly used interfaces when treating obstructive sleep apnea (OSA). Masks modalities are often chosen based on patient preference and comfort however, oronasal masks may cause worsening of OSA in some individuals.

Report of case(s)
An 83 year old male presented for snoring, witnessed apneas, and daytime somnolence. A split-night PSG was ordered for evaluation. During the diagnostic portion of the study, the patient exhibited moderate OSA with an apnea hypopnea index (AHI) of 27/hr. The titration portion of the study was initiated with an oronasal (full face) interface. Continuous positive airway pressure (CPAP) pressure was titrated from 5cm H2O to 15 cm H20 with worsening of obstructive events and a residual AHI of 21 to 40/hr. Bilevel positive airway pressure (BiPAP) was then initiated and titrated from 19/15 cm H2O to 21/17 cm H20 with a continued residual AHI of 40/hr. No central apneas were noted. Upon review, suspicion arose whether the oronasal mask exacerbated the patient’s OSA. A repeat titration PSG was conducted with a nasal mask. With this mask a pressure of 8cm H20 resulted in a residual AHI of 0/hr. The patient was prescribed APAP 6-10 cm H20 with a nasal mask which resulted in excellent compliance and a residual AHI of 3.6/hr.

Conclusion
This case report presents a patient with worsening OSA due to use of an oronasal interface. Prior studies have suggested that oronasal masks produce less retropalatal airway opening than nasal masks. There may also be an effect on anterior-posterior distance of the mandible in the setting of exhalation. When possible, oronasal masks should be avoided in lab or home titration studies as they may lead to worsening OSA leading to escalation of PAP pressures and/or adjustment in titration modality.

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1195 Sleep-related Hallucinations
Authors: Deshpande Pushkar , Vyata Vishruth , Diaz-Abad Montserrat , Konikkara John 
Published: April 2024

Abstract

Introduction
Sleep-related hallucinations, encompassing visual and auditory experiences pose a complex clinical challenge. Here, we illustrate 2 cases with diverse underlying etiologies.

Report of case(s)
Case 1: 76-year-old male with 3 months of nocturnal visual hallucinations, occurring 3-5 nights per week. These ranged from familiar and unfamiliar faces and characters reminiscent of movies. Symptoms occurred when waking up in the middle of the night, with difficulty returning to sleep. Polysomnography (PSG) unveiled moderate obstructive sleep apnea (OSA) without findings indicative of parasomnias and no symptoms on the night of PSG. Brain MRI revealed chronic infarcts involving right basal ganglia and bilateral optic radiations. Lesions of structures that commonly cause visual hallucinations (optic lens, retina, thalamus, midbrain) were absent. The frequency of episodes decreased to 1-2 per month after initiating CPAP therapy. Potential mechanism of improvement could be PAP mediated optimization of perfusion to the ischemic penumbra. Case 2: 93-year-old male with an eight-year history of insomnia due to musical hallucinations (MH). Auditory experiences included church and semi-classical music, with 80% being familiar tunes. The patient, a former church choir member, has a history of progressive hearing loss and tinnitus. Brain MRI/MRA revealed chronic periventricular microvascular ischemic changes. Extended EEG showed no evidence of seizures. Audiometry confirmed moderate-severe bilateral sensorineural hearing loss. No response was seen to Levetiracetam, Gabapentin, Donepezil and behavioral therapy. Home sleep study revealed severe OSA. CPAP therapy did not result in improvement of MH. MH improved significantly with Lorazepam. Hypoacusis, psychiatric disorders, focal brain lesions, epilepsy and intoxication are common etiologies for MH. The etiology in this case is very likely due to hypoacusis. The hypothesis is deafferentation resulting in release phenomenon or spontaneous activity in the auditory pathway, manifesting as MH.

Conclusion
Sleep-related and nocturnal hallucinations warrant a comprehensive evaluation. Differential diagnoses include neurological causes, including stroke, seizure, Multiple Sclerosis, tumor, vascular lesions, and neurodegenerative disorders. Other etiologies include psychiatric disorders, medications, alcohol, otologic and ophthalmic pathologies. It is also reasonable to evaluate for comorbid OSA as appropriate treatment may decrease the frequency of episodes and improve overall sleep in some patients.

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1196 Use of Home Sleep Apnea Testing to Investigate Persistent Hypoxia in the Remote Setting
Authors: Epstein Sam , Eng Kevin 
Published: April 2024

Abstract

Introduction
Nocturnal oxygen titrations can be resource intensive, and often require the patient to undergo an attended polysomnogram (PSG). Veterans who live in remote areas are often reluctant to undergo PSG, particularly in those with multiple medical comorbidities that prohibit prolonged travel. We present a case of a veteran with severe OSA and persistent nocturnal hypoxia who underwent a remote oxygen titration using home sleep apnea testing (HSAT) equipment.

Report of case(s)
79-year-old male with history of hypertension and diabetes diagnosed with severe OSA (AHI 38 events/hour), with significant oxygen nadir of 65%. He was prescribed automatic positive airway pressure (APAP) of 6-16 cmH2O and a follow up 3-night oximetry was ordered which showed persistent nocturnal hypoxia. Remote monitoring showed regular PAP use with AHI &lt; 5/hr during the nights of oximetry. Unfortunately, the patient was unable to travel for attended PAP and oxygen titration study for further diagnostic assessment. It was instead decided to repeat 3-night oximetry with use of supplemental oxygen (1, 2, 3 liters/minute for nights one, two, and three respectively). Results again showed persistent nocturnal hypoxia with AHI &lt; 5/hr on remote data monitoring. 3-night HSAT connected to the patient’s APAP device was ordered, with bleed in oxygen at 2, 3, and then 4 liters/minute over the consecutive nights of the study. Results included the C-flow channel, which surprisingly showed persistent snoring and obstructive events (average AHI of 8/h) despite APAP, a discordant finding to that on remote data monitoring. The decision was made to transition patient to an automatic bilevel positive airway pressure mode with two-liter oxygen bleed in. This resulted in adequate treatment of OSA and nocturnal hypoxia as confirmed by follow up HSAT. It is notable that ongoing diagnostic evaluation of hypoxia is underway.

Conclusion
Aside from its promise in diagnosing moderate to severe OSA, HSAT over oximetry alone may provide additional diagnostic information (C-flow channel) in patients with residual nocturnal hypoxia despite PAP. In patients who are not able to undergo formal attended titration studies such as those who live in remote settings, this is a potential alternative.

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1197 Cataplexy - Management Conundrums
Authors: Buntak Vesna , Masroor Kiran , Geer Jacqueline 
Published: April 2024

Abstract

Introduction
Creating a guideline for the management of patients with narcolepsy is complex due to the number of potential mechanisms that could account for the increased risk of perioperative complications: aggravation of narcolepsy itself, dysautonomia, narcolepsy-related medications, anesthesia interactions, etc.

Report of case(s)
An 80-year-old woman with a history of narcolepsy type 1, depression, restless leg syndrome, obstructive sleep apnea, and atrial fibrillation presented to our sleep clinic to establish care. Her narcolepsy was managed with strategic naps, but no medications. She had previously experienced rare episodes of cataplexy, described as the inability to talk, and syncope. While on anticoagulation for her atrial fibrillation, she sustained a fall during an episode of cataplexy resulting in brain bleed and eye loss, which prompted her to start pitolisant. Subsequent polysomnography showed an apnea-hypopnea index of 72/hr with nadir oxygen of 74%, requiring Adaptive Servo-Ventilation (ASV), which she did not tolerate. She noted cognitive decline and extreme weakness resulting in hospitalization, during which neurology reported frequent myoclonic jerks and excessive sleepiness. Given her untreated sleep apnea, we recommended re-initiation of ASV during the inpatient hospital stay, which improved her symptoms significantly. She subsequently developed an unrelated need for urgent bowel surgery, necessitating cessation of oral intake, which was temporally related to debilitating and refractory cataplexy. Following recovery from this acute hospitalization, she recovered and was again seen in the clinic, with no further episodes of cataplexy and improved sleepiness in the setting of ASV compliance and resumption of her pitolisant.

Conclusion
This case emphasizes the importance of controlling even rare cataplexy in the setting of potentially catastrophic outcomes. It also raises concerns about the management of narcolepsy with cataplexy in the perioperative period, which currently lacks quality data and guidelines.

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1200 Overstimulated: A Case of Hypoglossal Nerve Stimulator Over-titration
Authors: Seibert Katherine , Ryden Armand , Chang Melisa 
Published: April 2024

Abstract

Introduction
Hypoglossal nerve stimulation (HNS) is an effective treatment for obstructive sleep apnea (OSA) that stimulates nerves that protrude the tongue and move the hyoid bone anteriorly to restore airway patency during sleep. After implantation, the stimulator voltage is titrated to an optimal therapeutic level. We describe the case of a patient whose OSA was effectively treated at a lower voltage and then demonstrated worsening sleep apnea after over-titration to a higher voltage.

Report of case(s)
A 37-year-old healthy man with a body mass index of 32 kg/m2 presented with snoring. A polysomnogram revealed an apnea hypopnea index (AHI) of 53.9 events per an hour consistent with severe OSA. He was intolerant of continuous positive airway pressure (CPAP) and trial of a mandibular advancement device (MAD) was ineffective. The patient subsequently underwent hypoglossal nerve stimulator implantation without complications. After an appropriate healing period, his device was activated with a noted functional amplitude of 0.8V and an allowed range of 0.8V to 1.8V. During self-titration, the patient noted improvement of snoring and apneas at an amplitude of 1.6V. However when he increased the amplitude further he noted recurrence of his OSA symptoms. He underwent an attend polysomnography titration study that revealed an estimated AHI of less than 10 with his HNS set to 1.6V representing an appropriate treatment response. However, when his HNS was titrated up to 1.7V, his estimated AHI increased to 26.4 consistent with moderate OSA. The patient’s device was set to a voltage of 1.6V.

Conclusion
HNS is an important treatment option for patients with OSA who are intolerant of CPAP. After implantation, attention must be given to careful titration of the stimulation voltage during an attended polysomnogram to avoid over titration which can lead to an increased number of obstructive events in some patients. While the mechanism of this phenomenon is currently unknown, theoretical mechanisms include stimulation of the lateral division of the hypoglossal nerve triggering tongue retraction at higher voltages and genioglossus muscle fatigue.

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1203 When the Options Run Out… Less Is More… a Case of Pediatric Narcolepsy with Limited Treatment Options
Authors: Khan Yousaf , Revana Amee 
Published: April 2024

Abstract

Introduction
We report a pediatric patient with Narcolepsy Type 1 with significant daytime impairment who faced challenges with the standard treatment options of pediatric narcolepsy.

Report of case(s)
The patient is a 16 year old female with confirmed diagnosis of narcolepsy type 1 (mean sleep latency &lt; 5 minutes, 4 sleep onset REM periods). Her symptoms (excessive daytime sleepiness (PDSS 26), cataplexy, hypnagogic/hypnopompic hallucinations and depression) gradually started at the age of 12 years of age and worsened overtime. Cataplexy initially involved eye and facial twitching that were triggered by laughter or anger which gradually progressed to generalized loss of tone. As the initial management of narcolepsy, she was started on atomoxetine with no change in symptoms. She transitioned to methylphenidate which improved her daytime sleepiness minimally but with no improvement of the cataplexy. Due to further adverse effects with stimulants, she started sodium oxybate with low dose dextroamphetamine. Initially, the combination improved wakefulness but then with dose titration, the patient developed severe depression and suicidal ideation after 8 months of use. She also demonstrated manic episodes and anorexia. With each treatment regimen, her depression and anxiety vacillated in severity. Lastly, a trial of pitolisant was titrated to 13.35 mg. She developed heart palpitations with the higher doses, so low dose Pitolisant 8.9 mg daily was continued. At this dose sleepiness improved, with some improvement in functionality. Venlafaxine 225 mg daily was added to the regimen to control cataplexy and depression with good benefit. With the low dose pitolisant and venlafaxine combination, she has been able to participate in extracurricular activities at school.

Conclusion
Pediatric narcolepsy can be challenging to treat as there are limited treatment options. We presented a case of a teen who had multiple symptoms of narcolepsy with coinciding depression and anxiety. Despite multiple drug regimens, her symptoms improved with off label use of Pitlosant , interestingly at low dose. Although clinical manifestations of pediatric narcolepsy may be life altering for the patient, close clinical follow up is imperative to screen for adverse effects after implementing a treatment plan.

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1207 Intercostal Monitoring as a Novel Approach to Polysomnography During Hypoglossal Nerve Stimulator Titration
Authors: Sedar Cole , Andre Aguillon 
Published: April 2024

Abstract

Introduction
Hypoglossal nerve stimulation is an alternative treatment modality for patients with obstructive sleep apnea who are not able to achieve ideal compliance rates with PAP therapy, and is becoming more widespread, with growing numbers of patients considering and undergoing implantation. Protocol for the only commercially available hypoglossal nerve stimulator (HNS) in the US includes a follow up titration polysomnogram (PSG) after implantation for device adjustment. This is typically performed using a standard PSG setup, which does not include leads for monitoring of the intercostals. Following implantation of HNS in our patient, we added intercostal electromyography to the standard PSG during HNS titration study, allowing us to directly monitor activation of the intercostal muscle group responsible for the sensing lead on the HNS. A 2016 study comparing intercostal EMG activity to respiratory inductance plethysmography (RIP) monitoring for differentiating obstructive from central events showed an increase in sensitivity for chest wall EMG in detecting respiratory effort, with 10.4% of events initially detected as central by RIP monitoring being reclassified as obstructive by chest well EMG, and 2.8% noted to be mixed rather than central4

Report of case(s)
Our patient is a 46-year-old female with a past medical history May-Thurner syndrome and nutcracker syndrome s/p stenting, Ehlers-Danlos syndrome, mitral valve regurgitation, Barrett’s esophagus, chronic migraines, thyroid cancer s/p radical resection and resultant hypothyroidism, Insomnia, gastroparesis s/p total colectomy, melanoma in situ s/p resection, cholecystectomy, and spinal nerve stimulator implantation. She was initially diagnosed with OSA in 2022 with an AHI 21.8 events per hour, nadir SpO2 87%. She trialed CPAP, but was intolerant to therapy. She underwent HNS implantation on 3/9/23. The device was activated in the clinic and she underwent polysomnography on 7/31/23 for voltage and setting titration. During the study, we monitored electromyography of the intercostals along with a standard PSG montage.

Conclusion
Intercostal electromyography, in addition to standard polysomnographic montage allowed for direct visualization of the intercostal contraction and subsequent activation of the hypoglossal nerve stimulator. This expanded montage provides more information for the titration study, and may allow for more precise HNS device settings given the increased sensitivity in detecting respiratory effort.

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1209 A Case of Morquio Syndrome Presenting as Respiratory Failure Due to Severe OSA and Successful Long-term Treatment
Authors: Vyata Vishruth , Deshpande Pushkar , Guntupalli Vineeta , Diaz-Abad Montserrat , Glick Danielle , Lasso-Pirot Anayansi 
Published: April 2024

Abstract

Introduction
Morquio Syndrome is a lysosomal storage disease (mucopolysaccharidosis type 4) characterized by the accumulation of glycosaminoglycans (GAG) and causes craniofacial, skeletal, and soft-tissue abnormalities. The pathophysiology of obstructive sleep apnea (OSA) is multifactorial due to GAG accumulation in upper airways, adenoids, trachea, and bronchi causing narrowing and collapsibility of the airways.

Report of case(s)
A nine-year-old male with Morquio Syndrome presented to the emergency department with difficulty staying awake for 5 days and an upper respiratory tract infection. He would doze off and wake up every few minutes several times a day. He had no prior history of somnolence. He was admitted to the ICU given the severity of the presentation and was clinically started on BiPAP. There was also a history of loud snoring but no witnessed apneas. An in-laboratory polysomnogram was performed to confirm his diagnosis of severe OSA and sleep-related hypoxemia, with an apnea-hypopnea index of 34.1, minimum oxygen saturation 78%, and time &lt; 90% of 74.8 minutes. After a titration study, he was started on noninvasive ventilation (NIV) with bilevel PAP. He remained compliant with outpatient NIV therapy and did not have any hospital admission for respiratory illness. Seven years later, he had a repeat titration study and was switched to NIV with AVAPS. He had one emergency visit for dyspnea and tested positive for influenza. A CT at that time showed severe tracheal stenosis of 8 mm at the thoracic inlet. On direct visualization with laryngoscopy, the stenosis was noted to be around 3 mm, probably due to dynamic airway obstruction. He improved with symptomatic treatment and was discharged the next day. Despite having significant airway abnormalities, he has never required intubation for respiratory failure, likely attributable to consistent use of NIV therapy.

Conclusion
Symptoms of OSA can be subtle, and if unrecognized, can potentially progress to respiratory failure. It is crucial, particularly in children with airway abnormalities, to actively screen for symptoms of OSA and initiate appropriate treatment. Our case highlights successful long-term treatment spanning more than a decade in an individual with Morquio Syndrome and avoidance of tracheostomy using NIV with bilevel PAP and AVAPS therapy.

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