bluuebunny/crossref_metadata_2025 huggingface dataset:
1181 Rock Around the Clock: Non-24 Sleep-Wake Disorder Successfully Treated with Circadian Entrainment, a Case Series
Authors: Benavides David , Benge Elizabeth , Epstein Lawrence
Published: April 2024

Introduction

Non-24-hour sleep-wake rhythm disorder (N24SWD) is a circadian rhythm disorder characterized by an inability to entrain to the 24-hour environment. It can occur in both blind and sighted individuals- usually with a longer sleep period, advanced temperature rhythm, and higher sensitivity to light than normal controls. Current therapies, derived from treating N24SWD in the blind and the human phase response curve, typically involve timed melatonin administration. It is more complex in sighted individuals, requiring multiple time cues such as light, melatonin, social interactions, feeding, and activity. We present two cases of non-24 sleep-wake disorder in sighted individuals who were successfully treated with circadian entrainment.

Report of case(s)

Report of Cases: Case 1: Sighted 31-year-old unemployed male with ADHD, presented with an irregular sleep pattern. He reported a lack of routine, prolonged dim basement computer use, and a circadian rhythm appearing longer than 24 hours. Actigraphy and sleep diaries confirmed N24SWD. Comprehensive measures, including timed light and melatonin therapy, blue light blocking glasses, and activity scheduling, led to a subjective "miraculous" improvement, with follow-up actigraphy confirming resolution. Case 2: Sighted 23-year-old female with autism and affective disorders presented with shifting sleep times alternating with prolonged video-game use. Actigraphy and sleep diaries were consistent with N24SWD. Following a prescribed schedule with light therapy, timed melatonin, and activity scheduling led to circadian alignment, confirmed by sleep diaries and patient report.

Conclusion

Effectively managing N24SWD in sighted individuals is challenging, as evidenced by limited treatment success and total cases documented in the literature, including challenges in long-term patient adherence. This case series highlights two successful instances of entrainment using a combination of timed melatonin, bright and low light therapy and activity scheduling, supported by actigraphy and diaries reflecting adaptation. These cases provide insights into contributors to the development of N24SWD and methods for resolving them.

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1182 A Case of Hypersomnolence in a Solid-Organ Transplant Recipient
Authors: Mukharesh Lana , Revana Amee
Published: April 2024

Introduction

Epstein-Barr virus (EBV) infects more than 90% of humans worldwide, with no evidence of active infection and persists for life. In pediatric renal transplant recipients, EBV primary infection or reactivation occur more frequently (63% and 44%, respectively). There is limited data to suggest duration of symptoms secondary to EBV and treatment options for pediatric hypersomnia secondary to EBV infection.

Report of case(s)

We describe a case of hypersomnia in a 7-year-old male with ADHD. He had received a renal transplant three years prior, secondary to renal dysplasia from a deceased donor who was EBV positive. He had a baseline polysomnogram (PSG) completed for symptoms of snoring, witnessed apneas and nocturnal enuresis, and was diagnosed with severe obstructive sleep apnea. He was started on CPAP and demonstrated excellent adherence to positive airway pressure (PAP). At age 9, he developed hypersomnolence, where he would sleep for 12-16 hours a day, fall asleep in school, during meals and vacation trips. The parents reported he was diagnosed with EBV a few months prior (EBV IgG and EBV nuclear antigen positive). Completed diagnostic work up included PAP device interrogation with adherence data, an actigraphy, a diagnostic PSG while on PAP and a multiple sleep latency test (MSLT). All the work up was reassuring, except for the MSLT where the patient had a sleep onset latency of 11 minutes and 3 out of 5 naps with sleep onset REM periods. Over the course of his evaluation, his pediatric daytime sleepiness scale ranged between 20-28 (>15 is significant). He was started on atomoxetine, with some reported improvement in hypersomnolence. Repeat PSG revealed primary snoring; therefore, PAP was discontinued. At age 15, his hypersomnolence had resolved, but conversely, exhibited difficulty initiating and maintaining sleep with higher doses of atomoxetine, therefore it was discontinued after 5 years of treatment.

Conclusion

In pediatric solid-organ transplant recipients with EBV infection, it is imperative to screen for excessive daytime sleepiness in order to facilitate therapies to improve overall quality of life, as well as recognize that the excessive daytime sleepiness may be a short-term symptom.

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1183 Chasing Sleep: Circadian Rhythm Sleep Disorder - Free-Running Type
Authors: Truong Khanh , Patel Abha
Published: April 2024

Introduction

Circadian rhythm sleep disorder, free-running type (CRSD-FRT) is a disorder caused by a circadian pacemaker that is out of sync with the 24-hour light-dark cycle. CRSD-FRT is rare in sighted persons but prevalent in totally blind individuals. In sighted people, CRSD-FRT can be associated with psychiatric conditions such as depression, but the etiology remains unknown. It is postulated that sighted persons may have reduced light sensitivity for phase-resetting effects. Symptoms of CRSD-FRT include insomnia, excessive daytime sleepiness and functional impairment. Here we present a unique case of CRSD-FRT in a sighted female with a history of depression.

Report of case(s)

A 27-year-old sighted female with history of depression previously treated with an SSRI presented to the sleep clinic with a chief complaint of chronic insomnia. Historically, she went to bed at 3 A.M. and awoke at 2 P.M. Sleep onset latency was normal. Estimated total sleep time was 10 hours. She denied history of any other sleep disorders. She experienced a similar delayed sleep routine as a teenager and assumed it was due to late-night gaming. She tried multiple sleep aids including trazodone, melatonin, and diphenhydramine, unsuccessfully. She described a “normal” sleeping schedule once per month, but then her sleep schedule began to move later and later, until it was normal again. She worked from home and was self-employed, giving her flexibility over her schedule. Her Epworth Sleepiness Score was 13 at the initial visit. Interestingly, her sibling had a similar sleeping pattern. Actigraphy was done which confirmed patient’s diagnosis of CRSD-FRT. She was advised to start Tasimelteon.

Conclusion

The patient was previously misdiagnosed with insomnia. CRSD-FRT is rare in sighted persons and may be underdiagnosed. Our patient’s CRSD-FRT may be linked to her history of depression and SSRI use. According to one study, a clinical dose of SSRI resulted in 47% increase in melatonin suppression, which increases the human circadian system’s responsiveness to light. Her flexible work from home schedule may also be a contributing factor. When changing or eliminating social time cues could precipitate the development of CRSD-FRT due to variations in the length of circadian rhythm.

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1187 Non-24-hour Sleep-Wake Disorder in Transgender Male Receiving Exogenous Testosterone
Authors: Collins Joseph , Espiritu Joseph
Published: April 2024

Introduction

Little is known about the effects of hormonal therapy for adolescents undergoing transgender care. We present a case of a biologic female being treated with exogenous testosterone for transgender care who develops non-24-hour circadian rhythm disorder (N24SWD), a rare sleep-wake disorder, most commonly seen in individuals who are totally blind.

Report of case(s)

A 19-year-old transgender man (female at birth) presented to our clinic with complaints of inconsistent sleep schedule and daytime sleepiness. They have a past medical history of anxiety, obsessive-compulsive disorder (OCD), and social phobia. As a younger teenager, the patient reported having a delayed sleep phase pattern. A year ago, the patient started exogenous testosterone injections every 2 weeks. Over the past year, the patient noticed a change in sleep pattern, described as going to bed and waking up 1-2 hours later, with each passing day. This was verified with 4 weeks of sleep diaries. The patient was diagnosed with N24SWD and was prescribed melatonin 0.5 mg 6 hours before bedtime without benefit. Other behavioral modifications with set wake times, bright light therapy, and morning exercises were not helpful. We prescribed tasimelteon, a melatonin receptor agonist, but it was denied by the health insurance. The current management plan is to optimize sleep behaviors and continue psychiatric follow-up for mood disorders while appealing the denial of tasimelteon.

Conclusion

Transgender individuals are particularly vulnerable to sleep disorders. Testosterone replacement therapy has been linked to sleep disordered breathing, insomnia, and sleep disruption. It has not been well established what role hormone therapy, and specifically testosterone in this case, plays on an individual’s circadian rhythm. Previous studies have demonstrated that melatonin production decreases more abruptly for women going through menopause and that delayed sleep phase in teenagers may be due to hormonal changes during puberty, although pathophysiology is not fully understood5. A similar case of a transgender male receiving exogenous testosterone was recently reported. More research is needed to elucidate the potential adverse effect of testosterone replacement on the circadian rhythm of the growing population of patients undergoing transgender care.

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1189 Intriguing Correlation Between Melatonin and Restless Legs Syndrome
Authors: Buntak Vesna , Shukla Anant , Tobias Lauren
Published: April 2024

Introduction

A temporal relationship between the onset of melatonin secretion at night and the worsening of restless legs syndrome (RLS) symptoms has been described in the literature. As a frequently used adjunctive to help combat insomnia, melatonin continues to be widely used.

Report of case(s)

A 65-year-old man with RLS, migraines, and onset and maintenance insomnia presented to our clinic with severe insomnia. He takes melatonin 3 mg and falls asleep in his chair while watching television. Then, he moves to the bed and has difficulty falling asleep. He will occasionally take extra doses of melatonin. He wakes up multiple times for a variety of reasons. His wake time is 4.30 AM for work. He had a home sleep apnea test that was negative. He describes crawling and achy feelings in his legs throughout the day. Sensation is associated with an urge to move or walk. These achy feelings are worse at nighttime. He currently takes 1200 mg of gabapentin three times a day. In the last year, his dose tripled despite adequate ferritin levels. Our patient did not recognize any temporal correlation between starting melatonin and worsening of his RLS but was agreeable with stopping melatonin. Within a week of stopping melatonin, his RLS symptoms became controlled and were no longer interrupting his sleep. We continued to work on his sleeping habits.

Conclusion

This case adds to increasing evidence of a correlation between melatonin and RLS. No substantial evidence was found during our literature search. One theory suggests that melatonin can inhibit dopamine release by suppressing calcium influx into stimulated nerve endings. Considering the widespread use of melatonin and the detrimental effect of RLS on quality of life, more studies are needed to evaluate this hypothesis.

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1192 A Case of Worsening Exploding Head Syndrome When Using Positive Airway Pressure
Authors: Nguyen Thien , Chang Melisa
Published: April 2024

Introduction

Exploding head syndrome (EHS) is a rare parasomnia with unclear etiology. There are limited research studies to confirm the exact predisposing and precipitating factors for its onset and frequency. Some published case reports suggest symptomatic improvement with the treatment of comorbid obstructive sleep apnea (OSA). This case presents a patient who received automatic positive airway pressure (APAP) treatment for OSA with worsening EHS symptoms.

Report of case(s)

A 41-year-old male with obesity (BMI of 33) and chronic back pain complained of visual and audio sensations of explosion, vibration, and light when falling asleep and waking up in the middle of the night without any associated physical pain. These events would occur twice a month and were accompanied by waking up three to four times nightly due to loud snoring. A home sleep study (HSAT) was completed and consistent with mild obstructive sleep apnea, demonstrating a respiratory event index (REI) of 7.4 events per hour. The patient was diagnosed with exploding head syndrome and OSA. He was started on APAP therapy. At the six-week follow-up, he noted worsening frequency of EHS events to twice a week. During this time, he lost his job. At the three-month follow-up since starting APAP, he reported further worsening of his EHS episodes to three times a week. The patient was not compliant with APAP usage. The compliance data revealed average usage of 2-3 hours a night with residual AHI of 1.2/hour.

Conclusion

EHS is a rare disorder with unknown predisposing and precipitating factors. This patient noted worsening of his EHS after the initiation of APAP therapy for his obstructive sleep apnea. However, the patient was not fully compliant with PAP therapy. He also underwent further stressors during this period. This case raises consideration regarding the side effects of APAP therapy on the severity of EHS when treating OSA.

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1193 Oxycardiorespirogram? More Than a Fancy Word, an Alternate Way to Determine Respiratory Support in a Complex Infant
Authors: Wang Victoria , Teran Jose , Revana Amee
Published: April 2024

Introduction

Obtaining diagnostic cardiorespiratory data via a polysomnogram in extremely young infants can be challenging. A multi-channel oxycardiorespirogram (OCRG) can be used to evaluate cardiorespiratory status in infants during wake and sleep states. The study records heart rate and rhythm, respiratory rate, oxygen saturation, and chest wall movement over a course of 4-8 hours. During the study, supplemental oxygen may be provided and adjusted according to the patient’s needs. We present a case of an infant in which the OCRG was able to guide the amount of respiratory support as an alternate diagnostic tool.

Report of case(s)

Patient is a 2-month-old female born at 35 weeks gestation with prenatally diagnosed myelomeningocele, hydrocephalus (s/p ventriculoperitoneal shunt placement), absent cerebellum, and marked hypoplasia of the brainstem. She was admitted to NICU on room air and then developed frequent episodes of apnea associated with desaturations requiring escalation of respiratory support to intubation with mechanical ventilation. She was gradually weaned to high flow nasal cannula of 2 liters per minute (LPM). Capillary blood gas (CBG) at baseline was normal. A multi-channel OCRG to better characterize her apneic events was ordered. Apneas occurred most frequently during sleep and were associated with oxygen desaturations. Independent episodes of desaturations occurred during feeds and during sleep with an oxygen nadir or 86% and 75%, respectively. The frequency of the apneic/desaturation events improved with 0.5LPM supplemental oxygen given via conventional nasal cannula (NC). With these results, we recommended that she be discharged home on 24 hour supplemental oxygen support of 0.5LPM NC. At subsequent outpatient pulmonary clinic visits at 1-2 month intervals, the OCRG study recorded a decrease in frequency of the apneic episodes associated with desaturations over time. We have been able to wean her daytime supplemental oxygen use to 0.25LPM NC and keep the nighttime supplemental oxygen the same.

Conclusion

Our case highlights the importance of considering alternative diagnostic and monitoring techniques such as a multi-channel OCRG to determine supplemental oxygen respiratory support needs for patients who have frequent apneic episodes in both awake and sleep states in the neonatal and infant period.

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1198 Acute Onset Vision Loss in a Patient with NAION and OSA
Authors: Andersen Karl , Zacharias Rajesh , Grover Aarti , Schumaker Greg , Oster Joel , Ostrow Peter , He Wei
Published: April 2024

Introduction

Non-arteritic anterior ischemic optic neuropathy (NAION) is the most common optic neuropathy in middle-aged and older-aged adult. There are an estimated 6000 new cases per year and is most commonly diagnosed in patients between 61-72 years of age. It affects men and women equally. There is a slightly higher incidence in white patients. 55-59% of patients with NAION also have OSA.

Report of case(s)

A 51-year-old man with a past medical history of hypertension and erectile dysfunction developed painless left inferior altitudinal visual field loss and blurriness in April 2023. He was seen in the Ophthalmology Department and was found to have left unilateral optic disc swelling on fundoscopic examination. An MRI of his brain, orbits, and face were normal. He was referred to Neuro-Ophthalmology where a slit lamp and fundus exam was performed and was significant for 360 degrees of disc swelling with heme seen inferiorly and normal appearing vessels and macula. A follow up fundoscopic examination one month later was significant for a hyperemic nerve and nasal blurriness in the left eye. No changes were seen in the right eye. Based on these findings, he was diagnosed with NAION. A HST was ordered by his Ophthalmologist that showed moderate OSA with an AHI of 24.3/h, an O2 nadir of 81%, and an ODI of 32.3. He was then seen in the Sleep Medicine clinic and prescribed CPAP. At his two month follow up appointment, he had an improvement in his daytime sleepiness and concentration but still had residual visual deficits in his left eye, including blurriness, light sensitivity, and difficulty focusing.

Conclusion

NAION is a rare condition with untreated OSA as a known risk factor due to nocturnal hypoxia decreasing perfusion to the optic nerve. This case reinforces the notion that the hypoxic burden of untreated OSA is a systemic problem and can lead to damage throughout the body. In this case, the benefit of treating this patient’s OSA is that his contralateral eye, the right eye, is also vulnerable to developing the same ischemic optic neuropathy that the left eye suffered from.

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1201 A Drowsy Deployment Dilemma: Case Report of a Military Deployment-Induced Kleine-Levin Syndrome Episode
Authors: Sickbert Lydia , Cocozza Victoria , Tchopev Zahari , Brock Matthew , Frey William
Published: April 2024

Introduction

Kleine-Levin syndrome (KLS) is a rare central disorder of hypersomnolence that typically develops in adolescent males and is accompanied by behavioral and cognitive disturbances such as altered perception or disinhibited behavior. The underlying etiology of this disease is unknown. It has a benign clinical course that often resolves after a median of fourteen years.

Report of case(s)

A 24-year-old male with an unremarkable past medical history was deployed by the United States (U.S.) Air Force to Africa. Patient had an episode of extreme hypersomnolence during his deployment associated with hearing abnormalities, anterograde amnesia, and irritability. He endorsed sleeping 20 hours straight – only waking to eat and use the restroom. He ultimately required medical evacuation to Germany. In Germany, he had an unremarkable neurology evaluation, laboratory studies, electroencephalogram (EEG), and brain magnetic resonance imaging (MRI). Upon further questioning, the patient’s first episode occurred three years ago without an identifiable trigger. Subsequent episodes lasted 1-4 weeks and recurred every 3-4 months. He was asymptomatic between episodes. Upon returning to the U.S. the patient was evaluated by sleep medicine, and a clinical diagnosis of KLS was made. The following day, the patient returned to the sleep center escorted by his work supervisor with an acute onset of hypersomnia. A multiple sleep latency test (MSLT) was performed with modifications (no polysomnography (PSG) or actigraphy prior and four naps). The patient demonstrated a mean sleep latency of 12 minutes and 37 seconds, which does not meet criteria for hypersomnolence. The patient was sent home after the MSLT with actigraphy for 2 weeks. He did not wear the device enough to allow for interpretation.

Conclusion

This is the first case report describing a military deployment-induced KLS episode. KLS is a clinical diagnosis of exclusion that does not require objective confirmation. The majority of available KLS literature states that objective measurements, such as MSLT and PSG, are not useful for diagnosis. However, KLS studies have limitations due to rarity of disease and lack of testing during episodes. We propose that the diagnostic criteria for KLS be re-evaluated to include objective data such as MSLT.

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1202 Worsening of Obstructive Sleep Apnea Following Resection of a Vagal Nerve Schwannoma
Authors: Chohan Moeed , Avidan Alon
Published: April 2024

Introduction

Schwannomas (SN) arising from the vagus cranial nerve (CN X) are uncommon (2–5%) benign nerve tumors. SN may promote obstructive sleep apnea (OSA) when parapharyngeal schwannoma narrows the airspace or as a direct result of injury to the recurrent laryngeal nerve which leads to vocal cord paralysis. We present an unusual presentation of worsening OSA in a patient who presented with CNX SN, underwent resection of the tumor, and developed unilateral vocal cord paralysis.

Report of case(s)

A 47-y/o male with a history of mild OSA s/p UPPP, presented with a new neck mass, worsening snoring, apneic episodes, fragmented nocturnal sleep, and worsening hypersomnolence. He was diagnosed with CN X SN and underwent left modified radical neck dissection for removal of a left-sided carotid mass which extended to the distal branches of the recurrent laryngeal nerve (RLN). Postoperatively, the patient exhibited dysphonia, dysphagia, and severe gasping at night attributed to left-sided vocal cord paralysis (VCP). Baseline Polysomnography (PSG) at age 32 before UPPP: AHI: 9 (REM:15, Supine: 18), SaO2: 89%. PSG following SN resection: AHI: 42 (REM: 21, Supine: 42), SaO2: 77%. The patient was prescribed autoPAP which resolved his symptoms.

Conclusion

The literature provides evidence of iatrogenic VCP, as a significant contributor to OSA. To our knowledge, this is the first report of VCP linked to SN of CN X. Multiple mechanisms may contribute to the OSA in our patient. (1) Resection of the CN X SN complex, including the distal branches CN X which lies near the RLN and may have been severed resulting in unilateral VCP and ultimately airflow limitations. (2) Resection of the CN X SN complex may have also altered neuronal pathways originating in the respiratory control centers in the brainstem. This mechanism, while somewhat elusive, highlights the potential role of vagal sensory innervation of the respiratory tract and provides fundamental insights into how derangement of afferent neuronal control modulated through CNX contributes to disorders of respiratory control.

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1204 Importance of Arm Electromyography for REM Without Atonia Detection
Authors: Das John , Johal Arminder , Stahl Stephanie
Published: April 2024

Introduction

Dream enactment raises concern for rapid-eye movement (REM) sleep behavior disorder (RBD). RBD is a parasomnia during which the patient has loss of normal atonia during REM sleep. RBD is highly associated with α-synucleinopathies (i.e., Parkinson disease, dementia with Lewy bodies, and multiple system atrophy) and can precede additional neurodegenerative signs and symptoms. REM sleep without atonia (RWA) is demonstrated through electromyography (EMG) during polysomnography. RWA requires the presence of tonic/sustained activity with ≥50% of the epoch containing excessive chin activity or phasic/transient muscle activity in ≥5 mini-epochs of 3-second duration in a 30-second epoch. We present a case that highlights the necessity of ordering polysomnography with arm EMG when evaluating for RBD.

Report of case(s)

A 73-year-old man presented to the clinic for concern of obstructive sleep apnea. He was also evaluated by neurology and given a diagnosis of essential tremor. During the initial visit, he also reported frequent “night terrors,” which he later described as dream enactment. His medications included metoprolol but no antidepressants; however, the dream enactment started prior to beta-blocker use. His physical exam was notable for right cogwheel rigidity, mild decrease in right serial finger tapping and rapid alternating movements, and mild dysphonia. A polysomnogram with arm EMG was ordered. On polysomnography, RWA was present in multiple epochs that was seen predominantly with arm (flexor digitorum superficialis) EMG. The RWA index (% of REM sleep meeting criteria for RWA) using the leg (anterior tibialis) and chin EMG was only 5%; however, the RWA index with the addition of arm EMG increased the RWA index to 64%.

Conclusion

The presence of RWA on PSG with dream enactment behavior gives this patient the diagnosis of RBD. While leg EMG is a part of routine PSG, arm EMG is not and needs to be specifically requested. Although research supports the use of arm EMG leads, we commonly find the addition of arm EMG leads is not ordered by providers when assessing for RBD. Arm EMG increases the chance of observing RWA and can be of strong importance to solidify an RBD diagnosis.

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1205 Haunted Slumbers: Decoding a Trauma-Induced Sleep Disorder in a Teen Patient
Authors: Iriana Sidney , Ibrahim Sally , Peth Dustin , Mistry Shikha , Lisio Catherine Di , Strohl Kingman
Published: April 2024

Introduction

This case examines a complex inpatient sleep presentation of a teenager with a history of foster care, depression, anxiety, PTSD, and ADHD, presenting with features of parasomnia. Key symptoms included excessive sleepiness, waking hallucinations, partial awareness, and significant uncontrolled movements reaching a level of severity requiring hospitalization.

Report of case(s)

A 14-year-old female presented with severe nocturnal episodes arising from sleep characterized by hallucinations of a dark figure, observed limb thrashing, screaming, and impaired arousal that gradually intensified over a 5 month period. Despite treatment for PTSD and an SSRI, her condition worsened - escalating to a severe and prolonged episode requiring EMS and hospitalization for her intense, potentially harmful movements. Concerns of parasomnia prompted inpatient sleep medicine consultation. Detailed history indicated these spells occurred primarily in early morning hours during sleep-wake transitions, with the patient having only partial recall of her movements. Each episode involved fear and visual hallucinations of threatening figures that she could recall later. Significant sleep disruption and daytime sleepiness were noted. Her psychiatric history revealed trauma from foster care. She also reported a fascination with horror media which seemed to influence these disturbances, reflecting in recurrent nightmares and similar traumatic experiences. Video EEG was negative for seizure disorder; PSG showed very mild OSA, REM with appropriate atonia, and sudden brief awakenings in both NREM and REM sleep. No spells were observed. Due her parents safety concerns, she stayed in a residential facility for intensive outpatient psychiatric therapy. Her episodes gradually improved with sleep scheduling, optimization of medications, and counseling. In outpatient therapy, she reported a swift cessation of the episodes, marked by her intentional disregard of the hallucinatory figure from her experiences.

Conclusion

This case highlights trauma-induced sleep disturbances in a young patient requiring hospitalization and inpatient sleep medicine consultation. The resolution of symptoms with changes in medication, improved sleep duration and hygiene in a residential setting underscores the importance of a comprehensive approach to managing complex sleep spells in adolescents with mood disturbance.

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1208 A Case of Complex Sleep Apnea Improved with Cardiac Resynchronization Therapy
Authors: Bhardwaj Puja , Naik Sreelatha , Suryanarayanana Manoj
Published: April 2024

Introduction

Congestive heart failure (CHF) is associated with higher risk of both obstructive (OSA) and central sleep apnea (CSA). While PAP therapy remains the gold standard in the treatment of obstructive sleep apnea, management of CSA needs to be tailored to the underlying pathophysiology. Optimal medical therapy for CHF is essential in the management of sleep-disordered breathing (SDB) among patients with CHF and reduced left ventricular ejection fraction (HFrEF). Cardiac resynchronization therapy (CRT) may have an important role.

Report of case(s)

We present the case of an 80 year old male, BMI 29 g/m2 who was evaluated at our sleep disorders center for the management of sleep apnea treated with CPAP 6 cm H2O and comorbid with HFrEF (ejection fraction 35% at the time of presentation) Therapy data from his CPAP device showed elevated residual AHI due to a combination of obstructive and central apneas as well as hypopneas. Significant leak and Cheyne-Stokes respiration were not detected. An empiric pressure increase was made as he was reluctant to return for PAP re-titration. He was also being treated with medical therapy for CHF and underwent CRT several months after presentation to the sleep clinic. Upon follow up he was found to have a dramatic improvement in residual events (AHI 31.2/hr- 6.7/hr) without further changes to CPAP. HFrEF increases risk of CSA through a vicious cycle stemming from significantly lower arousal threshold, high loop gain resulting in an exaggerated ventilatory response to arousals, and prolonged circulatory time leading to mismatched communication of arterial gas concentrations with the chemoreceptors. CHF may independently increase risk of OSA due to vascular engorgement of neck vessels that might affect upper airway patency especially among patients with hypervolemia. The terminal manifestation of a central apnea might be an occlusion of the pharyngeal airway requiring positive pressure to overcome the obstruction. This would explain why CPAP therapy might be effective among patients with HFrEF.

Conclusion

We conclude that the improvement in SDB in our patient is attributable to optimal medical therapy leading to euvolemia, CRT and medical therapy potentially improving cardiac systolic function and PAP therapy maintaining upper airway patency.

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1210 Initiation of Methadone for Refractory Restless Leg Syndrome with Significant Improvement of Symptoms
Authors: Damia Carla , Monceaux Brittany , Hall Catherine , Bhamrah Paul , Koothirezhi Rupa , Liendo Cesar , Ho Minh Tam , Ghildiyal Naomi , Chernyshev Oleg
Published: April 2024

Introduction

Restless legs syndrome (RLS) is a common sleep-related movement disorder. We are reporting a case of progressive and refractory RLS in which a patient had a long history of poorly controlled and worsening RLS with intolerance to first line medications and incomplete management of symptoms.

Report of case(s)

Patient is a 62-year-old Caucasian female with a past medical history of RLS, mild obstructive sleep apnea (diagnosed 2021), insomnia (managed with zolpidem), atrial fibrillation, coronary artery disease, obesity, diabetes, hypertension and osteoarthritis. Initial presentation to our clinic was 2 years ago. She reported RLS symptoms 20 years prior with treatment initiated 15 years ago. Symptoms started to worsen 5-6 years ago. She reported sensations in the upper extremities and symptoms starting earlier in the day. She noted intolerance, side effects to and ineffectiveness of first-line medications. Pregabalin produced oral blisters and gabapentin sedated her with minimal benefit to leg sensations. She was on ropinirole 0.25mg in the AM and 2mg in the evening. Dosage was increased but patient found that increased dosage during the day caused sedation and did not alleviate symptoms. Ferritin was low and patient had been on oral iron without change in symptoms. Iron infusion was recommended but deemed not feasible. After the first visit, patient presented 3 months later. She had required an emergency cholecystectomy and was given hydromorphone and morphine while hospitalized. These medications completely alleviated symptoms for several days. Patient had taken other opioid medications including tramadol, hydrocodone and oxycodone in the past without any effect on symptoms. Patient was continued on ropinirole 2mg at night and started on methadone 5mg daily. She has noted a significant improvement in symptoms with almost complete alleviation. She denies any side effects from this regimen and has been stable on this regimen for almost 2 years without worsening symptoms.

Conclusion

This case focuses on the benefit of specific opioids for management of refractory RLS. Initial selection of an opioid may not produce benefit but evaluating response to different medications and tailoring a treatment plan for each individual patient could produce notable alleviation of debilitating symptoms that occur with progressive and refractory RLS.

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1211 Pressure Injury of the Face After an Overnight Titration Study
Authors: Yaqoob Zaid , Jaffery Syed , Bazan Luisa
Published: April 2024

Introduction

We present a case of facial pressure injury related to a CPAP mask occurring within 24 hours of an overnight titration study, an uncommon finding.

Report of case(s)

Patient is a 69-year-old man with prior history of OSA who presented to the Henry Ford Sleep Medicine Clinic for reevaluation. His past medical history included class III obesity, diabetes mellitus, hypertension, coronary artery calcification, dyslipidemia and 88 pack-year history of smoking, he quit in 2010. When he was first diagnosed with OSA, he was treated with PAP therapy for 4 years without any reported skin adverse effects. He stopped using PAP therapy for 5 years prior to presentation to our clinic. A home sleep apnea test showed severe OSA with sleep related hypoxia. He underwent a titration study with the use of F&P Evora full face. CPAP was transitioned to BiPAP during the titration due to persistent hypoxia. He felt as though the plastic edge of the mask had cut into his skin during the study. Within 24 hours he noticed facial puffiness, tenderness, redness and facial wounds with increasing purulent discharge. He was seen in the sleep clinic the following day and was prescribed sulfamethoxazole-trimethoprim for a 7-day course for unstageable pressure injury to the face, which soon resolved. Patient was seen by allergy and an allergic reaction to the mask was ruled out. Further work up of hypoxia showed restrictive lung disease on pulmonary function testing and sniff test showed right diaphragmatic weakness.

Conclusion

Pressure injury to the face within 24 hours of using a mask is an uncommon complication of overnight titration studies. To our knowledge, this is the first reported case after using the F&P Evora full face mask which could have been the triggering factor for this type of injury. We suggest proper mask fitting during titration to minimize facial pressure injuries in the sleep lab.

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1212 Higher Is Not Always Better
Authors: Chang Stephanie , Seo Jiyeon , Rojanapairat Oragun
Published: April 2024

Introduction

Positive airway pressure (PAP) is the recommended first line treatment for obstructive sleep apnea (OSA). Overnight titration studies can be performed to find the optimal PAP settings for patients. However, manual titration studies can lead to higher PAP settings.

Report of case(s)

Cases: A 69-year-old man with a body mass index (BMI) of 33 and severe OSA on continuous PAP (CPAP) 10 cm of H2O was referred to sleep clinic for worsening daytime fatigue. He had an in-lab titration study with a full-face mask and was successfully titrated to bilevel PAP (BPAP) with a final inspiratory PAP (IPAP) of 20 cm of H2O and expiratory PAP (EPAP) of 16 cm H2O. Notably during his sleep study, CPAP improved the AHI up to 14 cm H2O, but further increase in CPAP and switching to BPAP worsened the AHI until a final setting of BPAP 20/16 cm H2O was reached. A 75-year-old man with a BMI of 20 and moderate OSA was referred to sleep clinic for persistent daytime sleepiness despite compliant auto-CPAP use with nasal pillows. He underwent an in-lab titration study with a full-face mask and was titrated to BPAP settings of 22/17 cm of H2O. Review of his sleep study showed effective therapy at CPAP of 6 cm of H2O, but further titration on CPAP and BPAP worsened the AHI until a final setting of BPAP 22/17 cm of H2O was reached. Discussion: PAP settings are an important determinant for PAP compliance as patients often do not tolerate higher settings. Manual titration studies have shown to overestimate CPAP requirements compared to the use of auto-titrating PAP in uncomplicated OSA. Furthermore, full-face masks can paradoxically worsen upper airway obstruction and increase treatment pressures compared to nasal masks. It is thought that full-face masks can cause worsening of upper airway obstruction and lead to higher pressure requirements to pneumatically stent the airways.

Conclusion

In conclusion, manual PAP titration studies may exaggerate PAP requirements, especially with the use of full-face masks. Careful review of the titration and choice of mask should be an important consideration in treating OSA.

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1213 Vagal-nerve Stimulation Induced Refractory Central Sleep Apnea
Authors: Shetty Amit , Thomas Robert , Heckman Eric
Published: April 2024

Introduction

Vagus nerve stimulation (VNS) is an effective therapy for epilepsy that has previously been suggested to worsen obstructive and central sleep apnea. Management of medically complex patients in the traditional sleep laboratory can present logistical barriers given the minimal medical oversight. Innovative forms of sleep monitoring offer new options to evaluate sleep serially.

Report of case(s)

A 69-year-old female with a history of medically refractory generalized epilepsy, on a vagal nerve stimulator, presented to sleep clinic in September of 2022 after undergoing a polysomnogram significant for severe sleep apnea with AHI 3% of 56, AHI 4% of 48, and oxygen nadir of 83%. A November 2022 titration was unsuccessful (residual AHI of 48 and oxygen desaturation nadir of 88%). Periodic breathing proved refractory to CPAP, BiPAP, BiPAP S/T, adaptive servo-ventilation, and attempts to mitigate high loop gain physiology with CO2 modulation techniques, acetazolamide, and supplemental oxygen. VNS setting could not be manipulated in the sleep laboratory safely. She was placed on CPAP setting of 5-10 cm H2O with compliance data showing average residual AHI of 24.3 and 29% of nights being in Hunter-Cheyne-Stokes breathing (HCSB). A planned hospital admission in July 2023 was arranged to monitor sleep with the SleepImage Ring system, which offered measurement of AHI and periodicity without the need for manual scoring. The VNS battery proved depleted during the stay but 5 nights on the same CPAP settings with the VNS off showed a residual AHI 4% of 1-3 with minimal periodic breathing. Patient underwent replacement of VNS generator with reduction of output current to 1/3 of prior value with gradual up-titration. Subsequent CPAP compliance data showed gradual increase in AHI and periodic breathing with rising VNS setting. Currently, the patient is undergoing VNS adjustments to optimize seizure control with plans to restudy sleep breathing at lower nocturnal settings when stabilized.

Conclusion

Sleep apnea induced by VNS can be refractory to therapy. Tracking sleep with the SleepImage system and PAP compliance data allowed understanding of the role of neurostimulation in inducing sleep dysfunction.

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Institute for Personalized Sleep Health, Beth Israel Deaconess Medical Center
1214 A Case of Mixed Obstructive and Central Sleep Apnea in Jacobsen Syndrome
Authors: Dlewati Mohammad , Leu Roberta
Published: April 2024

Introduction

Jacobsen syndrome is a rare genetic disorder due to variable deletion lengths on chromosome 11q. The estimated prevalence is only 1/100,000 births in the U.S. Clinical features are multi-system with characteristic facial features, growth and motor retardation, neurocognitive disability, cytopenias, and possible malformations in the CNS, heart, kidneys, and skeleton. Little information is available regarding their sleep and respiratory features.

Report of case(s)

A 3 month old girl with a history of Jacobsen syndrome presented to an outside sleep lab due to oxygen desaturations on overnight pulse oximetry. She was diagnosed prenatally due to intrauterine growth retardation. Whole genome sequencing revealed a 15.6 megabase deletion of 11q23.3q25. On polysomnography, she was found to have severe mixed obstructive and central sleep apnea (obstructive AHI 13.4 events/hour, central AHI 8.6 events/hour). She was treated with up to ½ Lpm of O2 via nasal cannula during sleep. CT imaging of the head and MRI of the brain and spine were obtained. These demonstrated absent olfactory nerves, pituitary interruption syndrome, periventricular white matter hypomyelination, lacunar skull, brachycephaly, mild congenital cervical spinal stenosis, and a “split atlas” with incomplete anterior and posterior arches of the C1 vertebra. There was no Chiari malformation and no radiographic abnormalities of the spinal cord. At 6 months of age she was started and maintained on levothyroxine and hydrocortisone for central hypothyroidism and adrenal insufficiency. Repeat polysomnogram at 15 months of age showed an obstructive AHI of 3.2 events/hour, and a central AHI of 5.6 events/hour. While most central apneas were brief (mean duration of 10.4 seconds), there were prolonged central apneas lasting as long as 20.9 seconds. Due to oxygen desaturations to the 70’s and 80’s following central apneas, oxygen supplementation in sleep was continued.

Conclusion

Multiple features of Jacobsen syndrome (e.g. hypotonia, craniofacial and skeletal abnormalities) increase risk for obstructive and central sleep apnea. We present a girl with Jacobsen syndrome demonstrating both obstructive and central sleep apnea from infancy into toddlerhood. This report highlights the importance of screening for sleep disordered breathing in patients with Jacobsen syndrome, and the need for further research on sleep disturbances in this population.

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1215 Adjunctive Use of Solriamfetol in Pediatric Excessive Daytime Sleepiness: A Single Center Experience
Authors: Varughese Robin , Palladino Christine , Kothare Sanjeev
Published: April 2024

Introduction

Solriamfetol (Sunosi) has been FDA-approved for adults aged 18 years or older particularly for excessive daytime sleepiness (EDS) associated with obstructive sleep apnea or narcolepsy. It works as a selective norepinephrine and dopamine reuptake inhibitor to promote wakefulness in this particular population. The benefit of this medication, compared to other medications to promote wakefulness, is that it is a non-stimulant medication. It has also been noted to have significant long-term efficacy rates for adults with EDS. Given the success of solriamfetol in the adult population, it has been trialed on EDS patients within the pediatric population. The purpose of this study is to better summarize the safety, efficacy, dosing, and side effect profile of pediatric patients using solriamfetol.

Report of case(s)

We performed a retrospective analysis of 11 pediatric patients who have been placed on solriamfetol and studied the improvement of daytime sleepiness with this intervention. Typical up-titration of the medication and monitoring for failure of improvement of sleepiness required a follow up period of at least 3 months. We collected the following data points: gender, weight, age, diagnosis (OSA or narcolepsy), medications, sleep study findings, and subjective endorsement of improvement based on Likert scale with increments of 0-25%, 26-50%, 51-75%, and 76-100% improvement. Our results demonstrated >50% responsiveness in 18% (2/11) of the patients in our cohort with 27% (3/11) of patients having some improvement of their excessive daytime sleepiness by 26-50%. Only 18% (2/11) patients reported adverse effects of headaches or increased anxiety with none of the patients reporting SJS-like rashes or insomnia. All patients tolerated the 150 mg daily dosing. No interactions were noted with patients taking OCPs.

Conclusion

Solriamfetol demonstrates similar tolerability, safety and side effect profile within the pediatric population when compared to the adult population and may provide additional benefit in refractory cases of EDS at maximal doses. More studies are needed to validate these observations.

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1217 A Complex Case of Progressive Hypercarbic Respiratory Failure
Authors: Hitchings Joshua , Dorsch Jennifer
Published: April 2024

Introduction

Progressive hypercarbic respiratory failure in the absence of overt intrinsic lung disease, thoracic cage abnormalities, CNS depressants, or neuromuscular disease poses a unique diagnostic challenge. Furthermore, continued respiratory failure in patients compliant with non-invasive ventilation warrants additional evaluation for alternative diagnoses. We present a case which produced a unique diagnostic challenge to the inpatient sleep medicine service.

Report of case(s)

A 46 year-old female with a past medical history significant for T2DM, COVID pneumonia, and seizures presented for acute on chronic hypercarbic respiratory failure in the setting of left hemi-diaphragm paralysis. She experienced multiple (greater than ten) hospitalizations requiring intubation for hypercarbic respiratory failure. Initial work up revealed negative inspiratory force of -40 cm H20. Pulmonary function tests revealed a restrictive pattern with FEV1/FVC of 70%. CT Chest revealed no parenchymal abnormalities. Admission ABG of 7.30/56. She was started on non-invasive ventilation through average volume assured pressure support mode with goal volumes of eight cc/kg. Despite effective ventilatory support with excellent compliance she had increasing requirements leading to near twenty-four hours of total use. Repeat hospitalization occurred for respiratory failure and new left arm weakness which raised concern for underlying neuromuscular disease as etiology. MuSK antibodies were sent and resulted positive at a titer of 1.35nmol/L, consistent with a diagnosis of Myasthenia gravis. The patient was treated with prednisone, plasma exchange, and rituximab with marked improvement in clinical and respiratory status. The patient was successfully weaned down to nocturnal use of non-invasive ventilation with preserved eucarbia.

Conclusion

Myasthenia gravis can lead to marked respiratory muscle weakness and respiratory failure. Common triggers of crisis include infection, medications, electrolyte abnormalities, and thyroid disease. Up to twenty percent of patients present with myasthenic crisis as their initial symptoms. Continued evaluation of forced vital capacity, and negative inspiratory force are important measures to follow for signs of disease progression. This case proved difficult in the setting of recurrent admissions believed to be secondary to hemidiaphragm paralysis and previous COVID infection. It is imperative to maintain a broad differential in working up respiratory failure and consider myasthenia in the proper clinical context.

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