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Introduction Pediatric sleep-related hypoventilation is a clinical pattern in which ventilatory insufficiency occurs in the setting of either severe OSA (obstructive hypoventilation) or obesity hypoventilation (sleep related hypoventilation). We are reporting a case describing the management of overlapping obstructive hypoventilation due to uncontrolled severe OSA and pediatric obesity hypoventilation syndrome. Report of case(s) The patient is a full term African American 5 y/o male with past medical history of obesity and prediabetes who was referred to discuss sleep related problems after rapid onset of weight gain.He gained 76 lbs over 8-month period; during this time his aunt noticed apneic events with the development of loud snoring.Initial workup was negative for abnormal findings,MRI w/w/o was unremarkable and TSH, cortisol, GH, IGF-1 and prolactin were all within normal limits. X-ray bone age study showed skeletal age appropriate for patient's chronologic age.Upper airway exam showed severe tonsillar hypertrophy 3+ with Mallampati class 4.The diagnostic PSG demonstrated the presence of 12 obstructive, 151 central, and 61 mixed apneas.There were 214 hypopneas resulting in an Apnea\Hypopnea Index of 60.5 events per hour.Mean oxygen saturation was 91.3%.The lowest oxygen saturation during sleep was 64.0%.Time spent ≤88% oxygen saturation was 119.7 minutes (26.2%). End tidal CO2 range was between 50-100 mmHg for 30% of study.The titration study revealed that the optimal treatment modality was BiPAP ST. Conclusion This case focuses on a five-year-old male who experienced rapid onset weight gain and presented with snoring, excessive daytime sleepiness and apneic events and was found to have profound hypoventilation with hypopnea predominant severe obstructive sleep apnea.The initial differential diagnosis of pediatric sleep-related hypoventilation includes ROHHAD, congenital central hypoventilation syndrome, primary central sleep apnea and obesity hypoventilation syndrome.The most common cause of hypoventilation during sleep in children remains OSA.The stepwise approach to management in such a case includes tonsillectomy with adenoidectomy,along with use of BiPAP. After the T&A the patient should have repeat PSG with capnography, if hypercarbia persists on post procedure PSG, he will need to continue BiPAP therapy with appropriate weight loss program. Early recognition evaluation, and treatment of obstructive sleep apnea in the pediatric population is imperative to prevent long-term consequences. Support (if any)

Introduction Narcolepsy is a sparsely found disease with a prevalence of 0.02-0.07% worldwide. Most patients are diagnosed much later than symptom onset with a gap of several years to even decades. The inability to diagnose and lack of awareness of narcolepsy leads to poor quality of life for the patient and family, poor school performance, and increased medical and socioeconomic burden. Hence, we report a case of a child inflicted with these woes looking for an improved quality of life. Report of case(s) A 12-year-old boy with past medical history of mild intermittent asthma, tonsillectomy, and adenoidectomy, initially presented to Allergy-Immunology for asthma management where it was revealed he was dealing with snoring and excessive daytime sleepiness since age 9. Patient was sent for Polysomnography (PSG) which revealed an Apnea Hypopnea Index (AHI) of 0.4 and Oxygen Nadir of 91%. Patient was referred to Pediatric ENT for evaluation, but further surgical intervention was not indicated. Patient continued to deal with excessive daytime sleepiness (Epworth Sleepiness Scale of 20/24) and multiple detention visits due to sleeping in class. Mother was distressed from daily conversations with school officials. Patient was referred to Sleep Medicine and found to have no cataplexy episodes, episodic symptoms, hypersexuality, or hyperphagia. Multiple Sleep Latency Test (MSLT) was ordered. Patient’s PSG night prior to MSLT revealed an AHI of 0.8 and Oxygen Nadir of 91%. Urine Drug Screen prior to MSLT was negative. MSLT revealed 2 naps demonstrating Sleep-Onset REM Periods and average mean sleep latency of 5 minutes. Patient was started on Methylphenidate ER 10mg with increase in alertness. Further treatment modification and workup is ongoing. Conclusion Our patient experienced a delay in diagnosis of nearly three years. The patient dealt with multiple detention visits and the mother experienced harassment from the school. Increasing awareness in the school system and patient population is necessary to mitigate prejudice against the pediatric narcolepsy population. Furthermore, increased research into new biomarkers and technologies for Narcolepsy type symptoms will be critical to decreasing the gap in diagnosis. Support (if any)

Introduction Sleep disordered breathing is very common in Arnold Chiari malformation but Biots’s breathing has not been reported in these cases. Report of Cases: 3-year-old with history of Arnold Chiari II malformation with hydrocephalus (s/p VP shunt and surgical decompression), spina bifida myelomeningocele (s/p in utero repair), subglottic stenosis with prior tracheostomy and eventual decannulation presented for evaluation of sleep disordered breathing. Presenting symptoms included witnessed apneas, cyanosis, daytime sleepiness and frequent awakenings. Physical examination was largely unremarkable. Initial PSG demonstrated complex sleep apnea, with an AHI 20.6, REM AHI 57.8 per hour of sleep, ETCO2 peak of 69 cm H20 and an O2 saturation nadir of 34%, with sleep related hypoventilation/hypoxemia and Biot's breathing in the absence of opioid use and CNS infection. During titration study, Biot’s breathing, complex apnea and sleep related hypoventilation/hypoxemia responded well to BiPAP ST of IPAP 18 cm H20 and EPAP 14 cm H20 BUR of 12 and oxygen of 1 L/min. She later developed intolerance to BiPAP due to high pressures and was decreased to BIPAP 13/11 cm H2O. Later the patient discontinued the use of BIPAP due to intolerance and was switched to night time O2 at 3-4 L/min. Per the parents, the patient has been maintaining her oxygen saturation in the absence of BIPAP therapy with oxygen use. Due to COVID, patient was unable to follow up but will be scheduled for a repeat PSG in the near future. She followed with Neurosurgery for Arnold Chiari II and they recommended no surgical intervention at this time due to functional VP shunt. Conclusion This is an atypical presentation of Biot’s breathing in the absence of CNS infections and opioid use in a patient with Arnold Chiari malformation II. Patient has complex sleep apnea, initially well controlled with BiPAP ST, but developed BiPAP intolerance. She is on oxygen with good control of hypoxemia in the absence of BiPAP therapy. Support (If Any)

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

Introduction Since iron is a cofactor used in the synthesis of CNS dopamine, some of which is then converted into norepinephrine, CNS iron deficiency will have an impact on the availability of dopamine and norepinephrine. Medications for narcolepsy and hypersomnia, such as stimulants and wakefulness medications, are dependent on the availability of these neurotransmitters to achieve their clinical response. Unrecognized and untreated nutritional deficiencies may contribute to inadequate responses to treatment of these sleep disorders. Report of Cases: A 26-year-old female with a history of iron deficiency since her pre-kindergarten health evaluation had a history of inadequate responses to stimulants and wakefulness medications. Additional labs were drawn to evaluate for nutritional deficiencies that could be contributing to the combination of fatigue and hypersomnolence. These revealed continued severe iron deficiency along with vitamin B6, B12, and D deficiencies. The treatment plan then expanded to include treatment of the nutritional deficiencies and a trial of pitolisant, which works to increase histamine levels in the brain. Sadly, pitolisant resulted in intolerable headaches, so it was discontinued. Conclusion This author feels that patients with hypersomnia warrant a broader approach that includes evaluating for nutritional deficiencies which may contribute to fatigue and hypersomnolence. In this case, the plan was to address the patient’s nutritional deficiencies and switch to pitolisant which mediates increased CNS histamine levels. This was unsuccessful due to the side effect of increased frequency and intensity of headaches related to pitolisant. Treating her iron deficiency with iron infusions should increase CNS synthesis of dopamine and norepinephrine. She may now have a better response to stimulants or wakefulness medications which depend on the availability of these neurotransmitters. She may also benefit from combination therapy by adding sodium oxybate to one of these medications. Support (If Any)

Introduction Central sleep apnea is commonly seen in patients with heart failure. Here we present a case demonstrating shifting of predominant apneic events from central to obstructive type after placement of left ventricular assist device (LVAD) in end stage heart failure patient. Report of case(s) Case Presentation: 66 year-old African American male has past medical history of chronic congestive heart failure diabetes, hypertension, paroxysmal atrial fibrillation, anemia, hypothyroidism, chronic kidney disease and sleep apnea. Prior to his LVAD placement, his left ventricular ejection fraction (EF) was Conclusion This case report highlights not only the improvement of the sleep apnea in CHF treated with LVAD but also shows the shift of apneic events from predominantly central to obstructive type post LVAD. Support (if any) 1. Henein MY, Westaby S, Poole-Wilson PA, Cowie MR, Simonds AK. Resolution of central sleep apnoea following implantation of a left ventricular assist device. Int J Cardiol. 2010 Feb 4;138(3):317–9. PMID: 18752859. 2. Köhnlein T, Welte T, Tan LB, Elliott MW. Central sleep apnoea syndrome in patients with chronic heart disease: a critical review of the current literature. Thorax. 2002 Jun;57(6):547–54. PMID: 12037232 3. Monda C, Scala O, Paolillo S, Savarese G, Cecere M, D’Amore C, Parente A, Musella F, Mosca S, Filardi PP. Apnee notturne e scompenso cardiaco: fisiopatologia, diagnosi e terapia [Sleep apnea and heart failure: pathophysiology, diagnosis and therapy]. G Ital Cardiol (Rome). 2010 Nov;11(11):815–22. Italian. PMID: 21348318.

Introduction Introduction/Background: PAP Intolerance is a common initial complaint among new PAP users, with mask issues and difficulty with pressure level leading the list. In clinical practice, feelings of claustrophobia and anxiety are usually cited as the reason for PAP intolerance. Sometimes craniofacial impairments such as congenital malformations or change in facial architecture after a major accident or surgery may play a role. Our case describes our experience with a patient afflicted with severe sinonasal cutaneous Sarcoidosis. Report of case(s) Case Description: A 43 year old AAF with a PMH of morbid obesity, depression and cutaneous sinonasal Sarcoidosis was referred to Sleep Medicine by ENT in late May 2020 with complaints of snoring, witnessed apneas, excessive daytime sleepiness, fatigue, and non-restorative sleep for >20 years. The patient was first diagnosed with cutaneous Sarcoidosis limited to the internal sinonasal region around 2015 and managed by ENT until the lesions spread to her face. Due to this, the patient developed depression and became increasingly non-complaint with her medical regimens. As a result, her sarcoidosis and other co-morbidities worsened. Diagnostic Polysomnogram (PSG) in May 2020 showed presence of Hypopnea predominant Obstructive Sleep Apnea (OSA) with AHI 35.9 and O2 desaturation to 58%. Subsequently, the patient was placed on auto CPAP 6–20 cm H2O with a DreamWear FFM. At follow up in September 2020, her sarcoidosis had spread to her upper lip area, so she was prescribed a Fit Life mask to avoid this region. Follow up in December 2020 revealed improvement in Sarcoidosis and accompanying vast improvement in objective compliance though she reverted to using her DreamWear FFM due to “mask seal issues. An AirFit f20 mask was then given to help avoid the active lesions on her upper lip. Conclusion: Discussion/Conclusion In patients with compromised facial architecture, special attention must be paid to the type of mask interface used so it does not aggravate sensitive areas which may lead to poor compliance with PAP therapy. Instructions were given for her to alternate between her three masks—the Fit Life, the DreamWear FFM and the AirFit f20 FFM—in an effort to reduce irritation on her face over time. Support (if any)

We report two cases of adult males with sleep-related eating disorder (SRED), with durations of 3 and 7 years, and without associated psychiatric history. In both cases, the use of low-dose (25 mg) sertraline taken at bedtime resulted in immediate, full and sustained resolution of symptoms at the latest follow-ups. The sertraline efficacy was of particular benefit for the patient reported on in case 2 who was a commercial airline pilot subjected to a highly restricted list of Federal Aviation Administration-approved medications. Risk factors for SRED included smoking cessation and work-related stress in case 1, and a history of sleepwalking and work-related circadian disruptions and partial sleep deprivations in case 2. Sertraline therapy of SRED is considered within a review of all current pharmacologic therapies of SRED. CITATION: Varghese R, Rey de Castro J, Liendo C, Schenck CH. Two cases of sleep-related eating disorder responding promptly to low-dose sertraline therapy. J Clin Sleep Med. 2018;14(10):1805–1808.

OBJECTIVEObstructive sleep apnea (OSA) is associated with the progression of abdominal and thoracic aortic aneurysms. However, the role of OSA in the overall outcome of intracranial aneurysms (IAs) has not yet been established. Authors of this report investigated the role of OSA in the overall outcome of IAs.METHODSRadiological and clinical data on patients (from 2010 through 2015) with confirmed IA were retrospectively reviewed. Significant differences between the OSA and non-OSA groups were determined using a chi-square test. Logistic regression analysis was performed to identify the predictors of an unfavorable IA outcome.RESULTSAmong the 283 patients with confirmed IAs, 45 patients (16%) were positively screened for OSA, a proportion that was significantly higher than the prevalence of OSA in nonaneurysmal neurosurgical patients (4%, p = 0.008). The percentage of patients with hypertension (p = 0.018), a body mass index ≥ 30 kg/m2 (p < 0.0001), hyperlipidemia (p = 0.034), diabetes mellitus (p = 0.005), chronic heart disease (CHD; p = 0.024), or prior stroke (p = 0.03) was significantly higher in the OSA group than in the non-OSA group. Similarly, the percentage of wide-necked aneurysms (p = 0.00001) and patients with a poor Hunt and Hess Grade IV–V (p = 0.01) was significantly higher in the OSA group than in the non-OSA group. In addition, the percentage of ruptured aneurysms (p = 0.03) and vasospasms (p = 0.03) was significantly higher in the OSA group. The percentage of patients with poor modified Rankin Scale (mRS) scores (3–6) was significantly higher in the OSA group (p = 0.03). A separate cohort of patients with ruptured IAs showed similar results. In both univariate (p = 0.01) and multivariate (p = 0.04) regression analyses, OSA was identified as an individual predictor of an unfavorable outcome. In addition, hypertension and prior stroke were revealed as predictors of a poor IA outcome.CONCLUSIONSComplications of IA such as rupture and vasospasm are often the consequence of uncontrolled OSA. Overall outcome (mRS) of IAs is also affected by the co-occurrence of OSA. Therefore, the coexistence of OSA with IA affects the outcome of IAs. Obstructive sleep apnea is a risk factor for a poor outcome in IA patients.

Introduction Congestive heart failure and sleep-disordered breathing frequently coexist. Many of these patients are referred for polysomnography(PSG). Left ventricular assist device (LVAD) is increasingly used as a destination therapy or as a bridge to transplant. This can lead to artifacts in EEG and EKG. The artifact can mask pathological waves or over-reading of pathology. We present a case report of a patient who underwent a PSG on LVAD. Report of case(s) Sixty-six-year old male with severe heart failure with reduced ejection fraction (EF) of 15 % presented with snoring and witnessed apnea. The patient was diagnosed with predominant central sleep apnea (92%) but never initiated BPAP/ST due to an unstable heart. He was placed on LVAD (HeartMate III LVAD, oscillatory speed of 5300 rpm/83.3Hz) as the heart status deteriorated. Repeat polysomnography was done for persistent snoring but improved witnessed apneas revealed a newly appearing artifact. EEG showed a diffuse low amplitude,6–7 Hz frequency waves, and a regularly appearing high voltage sharp-peaked wave. The morphology of alpha wave, K wave, spindles, and delta waves could not be appreciated and hence was unable to perform sleep staging. EKG had electrical artifacts. All troubleshooting maneuvers were unsuccessful in eliminating artifacts. The artifact is generated by the impeller rotational speed of LVAD that ranges from 2400 rpm to 10,00 rpm(oscillation frequencies of 40 Hz to 166.7 Hertz,1Hz == 60 rpm). The device’s artificial pulse causes additional minor peaks. EEG artifact can be improved by moving the amplifier away from the heart and repositioning the patient. Adjusting the low-frequency filter can affect the nasal pressure tracing, delta waves, and stage 3 recognition. Reducing the high-frequency filter frequency by 10 from the LVAD oscillation frequency improves the EKG signal. But this may cut off the frequency component of pathologies like left ventricular hypertrophy. Conclusion The LVAD has been increasingly used in the United States. It is important to improve awareness regarding the artifacts among sleep techs and physicians. Unresolved artifacts may lead to missing serious pathologies in EEG and EKG and also can lead to misreading the waves as pathological leading to unnecessary treatment. Support (if any) An image of EEG and EKG

Introduction Dementia with Lewy bodies (DLB) is one of the most common types of degenerative dementia after Alzheimer’s dementia. The core clinical features for diagnosis includes cognitive fluctuations, visual hallucinations, rapid eye movement (REM) sleep behavior disorder (RBD), and parkinsonism. Other symptoms include daytime drowsiness, longer daytime naps, prolonged staring spells, and episodes of disorganized speech. REM behavioral disorder (RBD) is commonly associated with DLB, occurring in 85 percent of individuals, often early in the course of the disease. It can precede the clinical diagnosis of DLB by up to 20 years. Report of case(s) A seventy-six-year-old female with a history of well controlled obstructive sleep apnea was diagnosed with REM behavioral disorder in 2012. She had presented with episodes of screaming, attempt to ambulate during sleep, resulting in injury. Her polysomnogram revealed evidence of REM without atonia and a screaming episode during REM. Her RBD symptoms were controlled with clonazepam and melatonin with less frequency of the RBD episodes. The patient gradually started noticing memory issues and by January 2020 she was diagnosed with dementia and was initiated on Aricept. Within 7 months of diagnosis of dementia, she started reporting vivid hallucinations that were not threatening or violent compared to her violent content of RBD. Physical exam revealed impaired cognitive function and mild intermittent resting tremor of the right hand. The neurological exam was normal including normal tone, strength, and gait. She also reported repeated falls and fractures. The diagnosis of Lewy body dementia was made based on the presence of 2 core clinical features. Conclusion The current management of these conditions is mainly symptomatic. In the evolution of neurodegenerative disorder, RBD precedes other conditions like LBD, parkinsonism, etc. Research suggests that alpha-synuclein neurodegeneration is the common pathology behind these conditions. The understanding that RBD presents at the beginning of the evolution, provides us with a unique opportunity for preemptive treatment to prevent further degeneration in turn preventing the debilitation consequence like dementia, parkinsonism, neuroleptic sensitivity, and dysautonomia. Further research is needed for developing these early interventional strategies. Support (if any) NOne

Introduction Floppy eye lid syndrome (FES) is known to be associated with Obstructive sleep apnea (OSA) and chronic progressive external ophthalmoplegia (CPEO) is a rare genetic disorder with mitochondrial myopathy that may present with isolated eye lid ptosis in the initial stages. In a patient with loud snoring and obesity, treating obstructive sleep apnea may improve Floppy eyelid syndrome. Report of case(s) 52-year-old African – American male with past medical history of Hypertension, obesity, glaucoma, CPEO status bilateral blepharoplasty with failed surgical treatment. Patient was referred to Sleep medicine team to rule out Obstructive Sleep Apnea aa a cause of possible underlying FES and residual ptosis. On exam, patient was noted to have bilateral brow and eyelid ptosis and mild ataxic gait. MRI brain with and without contrast was unremarkable. Deltoid muscle biopsy was suggestive of possible congenital myopathy and mild denervation atrophy. Polysomnogram showed severe OSA with AHI of 74.1 per hour and patient was initiated on Auto CPAP at a pressure setting of 7–20 cm H2O. CPAP treatment improved snoring, OSA and subjective symptoms of excessive day time sleepiness but did not improve the residual ptosis. Conclusion Treatment of severe OSA in a patient previously diagnosed with CPEO and failed surgical treatment with bilateral blepharoplasty, did not alter the course of residual ptosis/ floppy eyelids even though his other sleep apnea symptoms have improved. Support (if any) 1. McNab AA. Floppy eyelid syndrome and obstructive sleep apnea. Ophthalmic Plast Reconstr Surg. 1997 Jun;13(2):98–114. doi: 10.1097/00002341-199706000-00005. PMID: 9185193.

Introduction Abnormal movements and behaviors during sleep are part of a larger group of nocturnal events that may occur during the sleep-wake cycle and/or the transitions into and out of sleep. We are presenting the case of OSA-related confusional arousals associated with sleep-writing and sleep-talking during REM-sleep. Report of case(s) 46 year old female with history of CAD, HTN, RLS, Anxiety, Depression, and REM predominant OSA (AHI of 2.9 per hour of sleep, REM AHI of 40 per hour of sleep, and oxygen saturation nadir of 91%), noncompliant with PAP therapy, returned to reestablish sleep medicine care and restart PAP therapy. The patient reported worsening of her OSA symptoms while being without PAP therapy. In addition to traditional OSA symptoms (snoring, frequent nighttime awakenings, restless legs, daytime sleepiness/fatigue), she reported episodes of sleep-talking, sleep-writing with demonstrated evidence of these events in her personal diary. She maintains a collective diary that incorporates her blood pressure readings, her weight loss accounts with records of meals in a day, as well as a separate log of letters that she will write and mail. In this diary, she has noticed sleep writing instances in each field, with no recollections subsequently on the act of writing them. The patient reported that these episodes of sleep writing would occur 3–4 times in a week, during this period of PAP noncompliance. Last reported instance of her sleep writing was October 2020. After re-initiation of PAP therapy, the patient has not reported further episodes of sleep-writing or sleep-talking. Conclusion The sleep-writing is a very rare clinical symptom in the presentation of REM-predominant OSA as well as in REM/NREM parasomnias. We were not able to come across a case of it in the sleep literature review. Sleep-talking is a well-documented phenomenon. Confusional arousals may be responsible for symptoms of sleep-writing and sleep-talking in this case. We may repeat a sleep study with split protocol and parasomnia montage using AutoBipap if needed to investigate further. Further research should be done to explore the nature and correlation of sleep-writing in clinical practice. Support (if any) N/A

Optimal treatment of intracranial aneurysms (IAs) in elderly patients has not yet been well established. We have investigated the clinical and radiological outcomes and predictors of unfavorable outcome of IAs in elderly patients. Radiological and clinical data of 85 elderly patients from 2010 through 2015 were retrospectively reviewed. Significant differences between the groups were determined by a chi-square test. Regression analysis was performed to identify the predictors of unfavorable outcome. Among the 85 patients with IAs, the number of patients with >7 mm size aneurysm (p = 0.01), diabetes mellitus (DM) (p = 0.02), smoking (0.009) and Hunt and Hess grade 4–5 (p = 0.003) was significantly higher in the ruptured group compared to the unruptured group. Similarly, the number of patients who underwent clipping was higher in the ruptured aneurysm group (p = 0.01). The overall clinical outcome was comparatively better in the unruptured group (p = 0.03); however, microsurgical clipping of aneurysms provides a significantly higher rate of complete aneurysmal occlusion (p = 0.008). Overall, there was no significant difference in outcome in respect to treatment approach. In regression analysis, hypertension (HTN), obstructive sleep apnea (OSA), prior stroke, ruptured aneurysms and partial occlusion of aneurysms were identified as predictors of unfavorable outcome of IAs. Intracranial aneurysms in elderly patients reveals that endovascular treatment provides better clinical outcome; however, microsurgical clipping yields higher complete occlusion. Retreatment of residual aneurysms was comparatively more in the coiling group. Practice pattern has shifted from clipping to coiling for aneurysms in posterior circulation but not for aneurysms in anterior circulation.

Cervicogenic headache affects a significant portion of the entire population. This type of headache especially with atypical presentation is often hard to diagnose and manage since its etiopathophysiology is not been yet well understood. We have investigated the prevalence of cervicogenic headache with atypical presentation and discussed the etiology of it, and the outcome of surgical intervention on this type of headache in patients with cervical degenerative disease.Radiological and clinical data of 160 patients (from 2001 through 2016) were retrospectively reviewed. Significant differences between the groups were determined by chi-square test. Logistic regression analysis was performed to identify the predictors of unfavorable outcome.In this study, 10% of the patients had atypical presentation of cervicogenic headache. In overall cohort, after surgical intervention, there was significant improvement in symptoms and pain control, whether the presentation is typical or atypical. Sixty-one percent of the patients had no complaints, and 90% of the patients were headache-free (p0.0001). Sixty-nine percent of the patients were free of neck, shoulder and extremity pain, and visual analogue scale pain score was reduced by 7 points (pre-op, 8.4 vs. last follow-up, 1.5, p0.0001). However, number of patients with reduced headache was significantly higher in the group with typical presentation of headache (90.1%) compared to group with atypical (80%) presentation, p=0.04. In this study, female gender, smoking, obesity and depression were identified as predictors of overall unfavourable outcome. In addition, in a separate analysis, smoking and depression were revealed as risk factors for persistent headache.A notable portion of patients with cervicogenic headache can have an atypical presentation mimicking a primary type headache. However, cervicogenic headaches with atypical presentation can be difficult to diagnose and manage at the initial visit of the patients. Etiopathophysiology of this type of headache could be explained by the theories including discogenic, convergence and sensitization-desensitization theories. When cervicogenic headache is accompanied with CDD, performing ACDF or laminectomy would be the treatment of choice. Surgical intervention can also relieve the accompanying neck, shoulder and extremity pain with minimal complications. Lastly, outcomes of surgical intervention depend on the patients' morbidities including obesity, smoking and depression.

Introduction No standard of care exists for management of sleep disorders/sleep disordered breathing (SDB) and Mitochondrial myopathies. Our case report describes our experience with this condition in a pediatric patient. Report of Case A ten-year-old middle eastern male with PMH of Mitochondrial myopathy was referred to Sleep Medicine by ENT with reported snoring, witnessed apneic spells and daytime fatigue. Flexible Video Laryngoscopy did not reveal tonsillar or adenoid hypertrophy so it was postulated that his OSA was a result of his craniofacial abnormalities and global hypotonia. Genetic workup showed Mitochondrial complex II & III deficiency from a variant in SNAPC4 gene and PURA gene leading to failure to thrive, severe developmental delay, generalized muscle weakness, bradycardia requiring pacemaker, global hypotonia requiring nocturnal oxygen for chronic respiratory failure and G-tube placement due to difficulty with feedings. Diagnostic PSG showed severe OSA with AHI of 11.3, RDI 11.8, REM AHI 56.0, REM RDI 56, and minimum oxygen saturation 85%. Subsequent PAP titration led to initiation of BIPAP therapy with settings of Auto-BiPAP EPAP min 5, IPAP max 20; PSmin 4; PSmax 6 cm H2O and continuation of nocturnal home oxygen. Sleep fragmentation improved to an arousal index of 3.1 with BIPAP. The patient and caregiver presented to the Sleep Medicine clinic 1 month after the PAP titration study with objective report showing >4 hours use >70% of the time and subjective satisfaction with BIPAP therapy with improvement in snoring and apnea. Conclusion Mitochondrial disorders lead to a deficiency of ATP affecting all organ systems and is most recognizable in the form of neuromuscular impairments. Neuromuscular impairments can translate into SDB issues such as OSA. Patients with genetic conditions such as mitochondrial myopathy should be routinely screened and evaluated for SDB and treated if warranted to significantly improve morbidity, mortality and quality of life.

Introduction: The aim of this paper was to measure the loop gain (LG) in central apneas (CA), in workers workers exposed to chronic intermittently high altitude during the first, third and fifth night shift. To asses if the loop gain is attenuated as an indirect surrogate of acclimatization. Material and Methods: This is a secondary crosssectional and descriptive study applied to high tonnage trucks's drivers that work in an open-pit mine located at 4300 m.a.s.l. The drivers alternate ten working days at mine site with ten resting days at home. We used a type III device. The acquisition was taken during the first, third and fifth night while workers were sleeping at mine site. We measured the respiratory cycle and the LG. Inclusion criteria: a) At least ≥3 consecutive CA separated by an interapnea recovery breaths, b) CA Index≥5 and c) More than 50% of events were CA. In order to be included in the analysis, workers must fulfil requirements a, b and c in the first night. We used non-parametric tests for the comparison of continuous values between groups. Results: Seventeen workers, age 41.6±9.7 and all were male. BMI 27.2±1.9 kg/m2, Epworth Sleepiness Scale 4.8±3.5, and living home altitude 1426±1498 m.a.s.l. The values for the first, third and fifth nights were: AHI 33.4±16.8, 21.6±16.3 and 27.9±16.9 (p=0.012), CAI 20.4±13.5, 9.5±9.8, 14.2±14.9 (p=0.022), MediaO2Sat 83.6±2.1, 84.9±2.1, 83.8±3.5 (p=0.019) and LG 1.95±1.88, 2.10±1.20, 3.35±4.23 (p=0.028). Conclusions: Workers had high respiratory index with clear predominance of central events, and profound desaturation. The LG value was over 1 and documented ventilatory instability. This variable showed absence of attenueation of LG and CA Index which could be suggestive that acclimatization was not reached. Funding Statement: Study sponsored by Compania Minera Antamina. Declaration of Interests: All the authors declare no conflict of interest. Ethics Approval Statement: Drivers signed an informed consent. The Comite de Etica de Investigacion from the Universidad Catolica de Santa Maria approved and authorized the study. The study was carried out according to the Declaration of Helsinski.

Introduction Weill-Marchesani Syndrome (WMS) is a rare systemic genetic connective tissue disorder which usually presents with symptoms of short stature, limited joint movement, and eye problems such as glaucoma and microspherophakia. This genetic condition is associated with fibrous tissue hyperplasia. WMS is inherited as autosomal dominant or autosomal recessive patterns in families leading to a variability in presenting phenotype. Few papers have been written on airway management during anesthesia but as far as we know, this is the first case report on obstructive sleep apnea management in a patient with WMS. Report of Case A 9 year old boy with a past medical history of Methylene THF Reductase deficiency, von Willebrand’s Disease, seizure disorder, premature birth, developmental delays and Weill-Marchesani syndrome was referred to Sleep Medicine due to tonsillar hypertrophy (3+), snoring and witnessed apneas. Upon physical examination, patient had mid-facial hypoplasia, retropositioning of the mandible, high arched palate, Mallampti class IV, maxillary hypoplasia and mandibular hypoplasia. He had been evaluated by ENT which determined the patient to be too high risk due to his medical conditions for T&A. The patient had a polysomnogram in 2018 indicating OSA with an apnea-hypopnea index of 4.2 and a minimum oxygen saturation of 91%. After a CPAP titration study, the patient was started on Auto CPAP of 5-15 cmH2O and has shown improvement in symptoms based on subjective and objective compliance report. Patient has been able to tolerate PAP therapy well with 100% compliance greater than 4 hours per night. Conclusion This case is the first illustrating OSA in a patient with Weill-Marchesani Syndrome. In WMS, the causes of OSA are not only due to tonsillar hypertrophy, but multifactorial, including craniofacial abnormalities. Given the high risk of surgical complications in WMS patients, PAP therapy appears to be a reasonable option for OSA management.

The need for speeding up the diagnosis and treatment of sleep-disordered breathing has increased in recent years. In order to achieve that, a split-night protocol has been implemented, in which the patient with suspected sleep-disordered breathing undergoes a diagnostic and therapeutic study in one night. Elshaug et al. (1), by using the split-night protocol, were able to reduce the time to initiate continuous positive airway pressure (CPAP) therapy by 15% in patients with severe obstructive sleep apnea. However, reviewing the limitations of said protocol is necessary to make it more streamline.

Purpose The severity of obstructive sleep apnoea (OSA) ranges from mild or moderate to severe sleep apnoea. However, there is no information available on the clinical characteristics associated with cases involving more than 100 events per hour. This is a preliminary report and our goal was to characterise the demographics and sleep characteristics of patients with Extreme OSA and compare with patients with sleep apnoea of lesser severity. We hypothesised that patients with Extreme OSA (AHI>100) is associated with an increased comorbidities and/or risk factors. Methods We carried out a case-control study on male patients with OSA who were seen in a private hospital in Lima, Peru between 2006 and 2012. Cases were identified if their apnoea/hypopnea index (AHI) was higher than 100 (Extreme OSA), and four controls were selected per case: two with 15–29 AHI and two with 30–50 AHI, matched according to case diagnosis dates. We evaluated demographic, past medical history, and oxygen saturation variables Results We identified 19 cases that were matched with 54 controls. In the multivariate model, only arterial hypertension, neck circumference, age, and over 10% in SatO2Hb≤90% in total sleep time (T90) were associated with Extreme OSA. Arterial hypertension had an OR=6.31 (CI95%: 1.71–23.23) of Extreme OSA. Each 5-cm increment in neck circumference was associated with an increase of OR=4.34 (CI95%: 1.32–14.33), while T90>10% had an OR=19.68 (CI95%: 4.33–89.49). Age had a marginal relevance (OR=0.95; CI95%: 0.92–0.99) Conclusion Our results suggest that arterial hypertension, neck circumference, and over 10% SatO2Hb≤90% in total sleep time were associated with a higher probability of Extreme OSA. We recommend investigators to study this population of Extreme OSA looking for an early diagnosis and the identification of prognostic factors in comparison with moderate to severe levels.

Introduction: Obstructive sleep apnea (OSA) is associated with the progression of abdominal and thoracic aortic aneurysms. However, the role of OSA in the overall outcome of intracranial aneurysms (IAs) has not yet been established. We have investigated the role of OSA in the progression and outcome of intracranial aneurysms. Methods: Radiological and clinical data of 283 patients (from 2010 through 2015) were retrospectively reviewed. Significant differences between the OSA and non-OSA groups were determined by a chi-square test. Logistic regression analysis was performed to identify the predictors of unfavorable outcome. Results: Among the 283 patients with IAs, 45 (16%) patients were positively screened for OSA, a proportion that was significantly higher than the prevalence of OSA in the general population (4%), p=0.008. Number of patients with hypertension (p=0.018), BMI ≥ 30 (p Conclusions: The complications of IAs such as rupture and vasospasms are influenced by presence of OSA. Overall outcome (mRS) of IAs is also affected by the concurrence of OSA. Therefore, coexistence of OSA in patients with IAs affects progression as well as outcome of IAs. Lastly, OSA serves as an individual risk factor for poor outcome in these patients.

STUDY OBJECTIVES: By measuring the apnea length, ventilatory phase, respiratory cycle length, and loop gain, we can further characterize the central apneas of high altitude (CAHA). METHODS: Sixty-three drivers of all-terrain vehicles, working in a Peruvian mine located at 2,020 meters above sea level (MASL), were evaluated. A respiratory polygraph was performed in the first night they slept at high altitude. None of the subjects were exposed to oxygen during the test or acetazolamide in the preceding days of the test. RESULTS: Sixty-three respiratory polygraphs were performed, and 59 were considered for analysis. Forty-six (78%) were normal, 6 (10%) had OSA, and 7 (12%) had CAHA. Key data from subjects include: residing altitude: 341 +/- 828 MASL, Lake Louise scoring: 0.4 +/- 0.8, Epworth score: 3.4 +/- 2.7, apneahypopnea index: 35.7 +/- 19.3, CA index: 13.4 +/- 14.2, CA length: 14.4 +/- 3.6 sec, ventilatory length: 13.5 +/- 2.9 sec, cycle length: 26.5 +/- 4.0 sec, ventilatory length/CA length ratio 0.9 +/- 0.3 and circulatory delay 13.3 +/- 2.9 sec. Duty ratio media [ventilatory duration/cycle duration] was 0.522 +/- 0 0.128 [0.308-0.700] and loop gain was calculated from the duty ratio utilizing this formula: LG = 2pi / [(2piDR-sin(2piDR)]. All subjects have a high loop gain media 2.415 +/- 1.761 [1.175-6.260]. Multiple correlations were established with loop gain values, but the only significant correlation detected was between central apnea index and loop gain. CONCLUSIONS: Twelve percent of the studied population had CAHA. Measurements of respiratory cycle in workers with CAHA are more similar to idiopathic central apneas rather than Hunter-Cheyne-Stokes respiration. Also, there was a high degree of correlation between severity of central apnea and the degree of loop gain. The abnormal breathing patterns in those subjects could affect the sleep quality and potentially increase the risk for work accidents.

Pulsus paradoxus (PP) is a decrease in systolic blood pressure greater than 10 mm Hg during inspiration that occurs in various medical conditions. Using polysomnography pulse oximetry signal, photoplethysmography variations of the amplitude of the pulse pressure within the respiratory cycle were observed. There is a proportional relationship between the changes of inspiratory waveform values and the generated PP. A 59-year-old male underwent polysomnography that showed sleep hypoxemia, obstructive sleep-disordered breathing (apnea hypopnea index [AHI] = 5.1and respiratory disturbance index [RDI] = 87.9), with variations of pulse pressure induced primarily by inspiration. The highest variations in the pulse wave were observed in NREM sleep during obstructive respiratory events and in biocalibration during nasal breathing. The lowest variations occurred after the correction of inspiratory obstructive events and during biocalibration when asked to hold his breath.

We present a case of supine respiratory failure due to a bilateral diaphragmatic paralysis associated with brachial neuritis secondary to thoracic herpes zoster. Fluoroscopy in both the standing and supine positions revealed bilateral diaphragmatic paralysis accentuated in the supine position. To our knowledge, this is the first case of thoracic herpes zoster associated with brachial neuritis and bilateral diaphragmatic paralysis.

Oleg Y Chernyshev,1 David E McCarty,1 Douglas E Moul,2 Cesar Liendo,1 Gloria C Caldito,1 Sai K Munjampalli,1 Roger E Kelley,3 Andrew L Chesson Jr1 1Division of Sleep Medicine, Department of Neurology, Louisiana State University Health Sciences Center at Shreveport, LA 2Sleep Disorders Center, Cleveland Clinic, Cleveland, OH, 3Department of Neurology, Tulane University, New Orleans, LA, USA Introduction: Prompt diagnosis of obstructive sleep apnea (OSA) after acute ischemic stroke (AIS) is critical for optimal clinical outcomes, but in-laboratory conventional polysomnograms (PSG) are not routinely practical. Though portable out-of-center type III cardiopulmonary sleep studies (out-of-center cardiopulmonary sleep testing [OCST]) are widely available, these studies have not been validated in patients who have recently suffered from AIS. We hypothesized that OCST in patients with AIS would yield similar results when compared to conventional PSG. Methods: Patients with AIS had simultaneous type III OCST and PSG studies performed within 72 hours from symptom onset. The accuracy of OCST was compared to PSG using: chi-square tests, receiver operatory characteristic curves, Bland–Altman plot, paired Student's t-test/Wilcoxon signed-rank test, and calculation of sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV). Results: Twenty-one out of 23 subjects with AIS (age 61±9.4 years; 52% male; 58% African-American) successfully completed both studies (9% technical failure). Nearly all (95%) had Mallampati IV posterior oropharynx; the mean neck circumference was 16.8±1.6 in. and the mean body mass index (BMI) was 30±7 kg/m2. The apnea hypopnea index (AHI) provided by OCST was similar to that provided by PSG (19.8±18.0 vs 22.0±22.7, respectively; P=0.49). On identifying subjects by OCST with an AHI ≥5 on PSG, OCST had the following parameters: sensitivity 100%, specificity 85.7%, PPV 93%, and NPV 100%. On identifying subjects with an AHI ≥15 on PSG, OCST parameters were as follows: sensitivity 100%, specificity 83.3%, PPV 81.8%, and NPV 100%. Bland–Altman plotting showed an overall diagnostic agreement between OCST and PSG modalities for an AHI cutoff >5, despite fine-grained differences in estimated AHIs. Conclusion: Compared with PSG, OCST provides similar diagnostic information when run simultaneously in AIS patients. OCST is a reliable screening tool for early diagnosis of OSA in AIS patients. Keywords: portable clinical screening, obstructive sleep apnea diagnosis, acute ischemic stroke, sleep testing in stroke, acute sleep medicine

This study aims to assess the association between excessive daytime sleepiness (EDS) and variables extracted from the pulse-oximetry signal obtained during overnight polysomnography.A cross-sectional design was used to study the relation between four hypoxemia variables and EDS as determined by Epworth Sleepiness Scale scores (ESSS) in 200 consecutive patients, newly diagnosed with obstructive sleep apnea (OSA), as defined by an apnea-hypopnea index (AHI)≥ 15. Hypoxemia measurements were compared between sleepy (ESSS ≥ 10) and nonsleepy (ESSS10) patients before and after dichotomizing the cohort for each hypoxemia variable (and for AHI) such that there were 35 (165) patients in each of the corresponding higher (lower) subcohorts. The hypoxemia variables were combined into a biomarker, and its accuracy for predicting sleepiness in individual patients was evaluated. We planned to interpret prediction accuracy above 80 % as evidence that hypoxemia predicted EDS.Hypoxemia was unassociated with sleepiness in OSA patients with AHI in the range of 15 to 50. In patients with AHI50, the hypoxemia biomarker (but not individual hypoxemia variables) predicted sleepiness with 82 % accuracy.Nocturnal hypoxemia as determined by a polyvariable biomarker reliably predicted EDS in patients with severe OSA (AHI50), indicating that oxygen fluctuation had a direct role in the development of EDS in patients with severe OSA.

Background We describe a patient with cirrhotic liver disease and atrial fibrillation who was treated with spironolactone and digoxin. He was hospitalized because of an incidental finding of a high serum digoxin level (4.2 μg/L), but he remained asymptomatic without emerging arrhythmias. Despite discontinuation of both drugs, his serum digoxin level persisted at or above 3.0 μg/L for ~5 weeks, drawing into question the accuracy of the digoxin assay. Methods Additional digoxin methods gave lower, discrepant results, providing evidence of an assay interference, and several possible sources of digoxin false positivity were evaluated. This included assessment of the contribution of digoxin-like immunoreactive factor (DLIF), digoxin metabolites, and spironolactone. Because the routine digoxin assay used a monoclonal antibody, we also tested for another hypothetical interference: human heterophilic (“anti-mouse”) antibodies. Results We found no contribution from DLIF, dogoxin antibodies, or spironolactone to the apparent digoxin results. However, the use of protein A to complex and selectively remove immunoglobulin G molecules markedly lowered the apparent digoxin value, as did the less specific process of ultrafiltration. Conclusions These results suggest a previously unreported cause of digoxin false positivity: heterophilic antibodies, which have been reported to bind murine monoclonal antibodies in other assays. Because newer digoxin assays now use murine monoclonal antibodies, the possible presence of heterophilic, anti-mouse antibodies should now be considered in the interpretation of a high digoxin level. Clinical Pharmacology & Therapeutics (1996) 60, 593–598; doi

Pneumocystis carinii pneumonia in a 52-year-old homosexual AIDS patient is described because of its unusual manifestations of multiple cavitary and noncavitary peripheral pulmonary nodules and spontaneous pneumothorax. Such manifestations might become more common in the future because of the improvement of diagnostic techniques and therapeutic measures.