Sleep-Disordered Breathing in Patients with Motor Neurone Disease: One Size Does Not Fit all

2021 ◽  
pp. 1-8
Author(s):  
Vinod Aiyappan ◽  
Peter Catcheside ◽  
Nick Antic ◽  
Graham Keighley-James ◽  
Jeremy Mercer ◽  
...  

Introduction: Sleep-disordered breathing (SDB) in patients with motor neurone disease (MND) is normally attributed to hypoventilation due to muscle weakness. However, we have observed different patterns of SDB among MND patients referred for non-invasive ventilation, which do not appear to be explained by respiratory muscle weakness alone. Aim: The aim of this study was to examine the characteristics of SDB in MND. Methods: This is a retrospective analysis of sleep studies (using polysomnography [PSG]), pulmonary function tests, and arterial blood gases in MND patients referred to a tertiary sleep medicine service for clinical review. Sleep apnoeas were characterised as obstructive or central, and to further characterise the nature of SDB, hypopnoeas were classified as obstructive versus central. Results: Among 13 MND patients who had a diagnostic PSG, the mean ± SD age was 68.9 ± 9.8 years, BMI 23.0 ± 4.3 kg/m2, forced vital capacity 55.7 ± 20.9% predicted, and partial pressure of CO2 (arterial blood) 52.7 ± 12.1 mm Hg. A total of 38% of patients (5/13) showed evidence of sleep hypoventilation. The total apnoea/hypopnoea index (AHI) was (median [interquartile range]) 44.4(36.2–56.4)/h, with 92% (12/13) showing an AHI >10/h, predominantly due to obstructive events, although 8% (1/13) also showed frequent central apnoea/hypopnoeas. Conclusions: Patients with MND exhibit a wide variety of SDB. The prevalence of obstructive sleep apnoea (OSA) is surprising considering the normal BMI in most patients. A dystonic tongue and increased upper-airway collapsibility might predispose these patients to OSA. The wide variety of SDB demonstrated might have implications for ventilator settings and patients’ outcomes.

SLEEP ◽  
2021 ◽  
Vol 44 (Supplement_2) ◽  
pp. A224-A225
Author(s):  
Fayruz Araji ◽  
Cephas Mujuruki ◽  
Brian Ku ◽  
Elisa Basora-Rovira ◽  
Anna Wani

Abstract Introduction Achondroplasia (ACH) occurs approximately 1 in 20,000–30,000 live births. They are prone to sleep disordered breathing specifically due to the upper airway stenosis, enlarged head circumference, combined with hypotonia and limited chest wall size associated with scoliosis at times. The co-occurrence of sleep apnea is well established and can aide in the decision for surgical intervention, however it is unclear at what age children should be evaluated for sleep apnea. Screening is often delayed as during the daytime there is no obvious gas exchange abnormalities. Due to the rareness of this disease, large studies are not available, limiting the data for discussion and analysis to develop guidelines on ideal screening age for sleep disordered breathing in children with ACH. Methods The primary aim of this study is to ascertain the presence of sleep disorder breathing and demographics of children with ACH at time of first polysomnogram (PSG) completed at one of the largest pediatric sleep lab in the country. The secondary aim of the study is to identify whether subsequent polysomnograms were completed if surgical interventions occurred and how the studies differed over time with and without intervention. Retrospective review of the PSGs from patients with ACH, completed from 2017–2019 at the Children’s Sleep Disorders Center in Dallas, TX. Clinical data, demographics, PSG findings and occurrence of interventions were collected. Results Twenty-seven patients with the diagnosis of ACH met criteria. The average age at the time of their first diagnostic PSG was at 31.6 months of age (2.7 years), of those patients 85% had obstructive sleep apnea (OSA),51% had hypoxemia and 18% had hypercapnia by their first diagnostic sleep study. Of those with OSA, 50% were severe. Majority were females, 55%. Most of our patients were Hispanic (14%), Caucasian (9%), Asian (2%), Other (2%), Black (0%). Each patient had an average of 1.9 PSGs completed. Conclusion Our findings can help create a foundation for discussion of screening guidelines. These guidelines will serve to guide primary care physicians to direct these patients to an early diagnosis and treatment of sleep disordered breathing. Support (if any):


PEDIATRICS ◽  
1996 ◽  
Vol 98 (5) ◽  
pp. 871-882 ◽  
Author(s):  
Christian Guilleminault ◽  
Rafael Pelayo ◽  
Damien Leger ◽  
Alex Clerk ◽  
Robert C. Z. Bocian

Objective. To determine whether upper airway resistance syndrome (UARS) can be recognized and distinguished from obstructive sleep apnea syndrome (OSAS) in prepubertal children based on clinical evaluations, and, in a subgroup of the population, to compare the efficacy of esophageal pressure (Pes) monitoring to that of transcutaneous carbon dioxide pressure (tcPco2) and expired carbon dioxide (CO2) measurements in identifying UARS in children. Study Design. A retrospective study was performed on children, 12 years and younger, seen at our clinic since 1985. Children with diagnoses of sleep-disordered breathing were drawn from our database and sorted by age and initial symptoms. Clinical findings, based on interviews and questionnaires, an orocraniofacial scale, and nocturnal polygraphic recordings were tabulated and compared. If the results of the first polygraphic recording were inconclusive, a second night's recording was performed with the addition of Pes monitoring. In addition, simultaneous measurements of tcPco2 and endtidal CO2 with sampling through a catheter were performed on this second night in 76 children. These 76 recordings were used as our gold standard, because they were the most comprehensive. For this group, 1848 apneic events and 7040 abnormal respiratory events were identified based on airflow, thoracoabdominal effort, and Pes recordings. We then analyzed the simultaneously measured tcPCo2 and expired CO2 levels to ascertain their ability to identify these same events. Results. The first night of polygraphic recording was inconclusive enough to warrant a second recording in 316 of 411 children. Children were identified as having either UARS (n = 259), OSAS (n = 83), or other sleep disorders (n = 69). Children with small triangular chins, retroposition of the mandible, steep mandibular plane, high hard palate, long oval-shaped face, or long soft palate were highly likely to have sleep-disordered breathing of some type. If large tonsils were associated with these features, OSAS was much more frequently noted than UARS. In the 76 gold standard children, Pes, tcPco2, and expired CO2 measurements were in agreement for 1512 of the 1848 apneas and hypopneas that were analyzed. Of the 7040 upper airway resistance events, only 2314 events were consonant in all three measures. tcPco2 identified only 33% of the increased respiratory events identified by Pes; expired CO2 identified only 53% of the same events. Conclusions. UARS is a subtle form of sleep-disordered breathing that leads to significant clinical symptoms and day and nighttime disturbances. When clinical symptoms suggest abnormal breathing during sleep but obstructive sleep apneas are not found, physicians may, mistakenly, assume an absence of breathing-related sleep problems. Symptoms and orocraniofacial information were not useful in distinguishing UARS from OSAS but were useful in distinguishing sleep-disordered breathing (UARS and OSAS) from other sleep disorders. The analysis of esophageal pressure patterns during sleep was the most revealing of the three techniques used for recognizing abnormal breathing patterns during sleep.


Author(s):  
Guy M. Hatch ◽  
Liza Ashbrook ◽  
Aric A. Prather ◽  
Andrew D. Krystal

Pulse oximetry is the current standard for detecting drops in arterial blood oxygen saturation (SpO2) associated with obstructive sleep apnea and hypopnea events in polysomnographic (PSG) testing. In cases of hypoxic challenge, such as occurs during apneic events, regulatory mechanisms restrict blood flow to the skin to preferentially maintain SpO2 for more vital organs. As a result, a measure related to skin tissue oxygenation is likely to be more sensitive to inadequate breathing during sleep than pulse oximetry. Energy Conversion Monitoring (ECM) provides a method for measuring skin tissue oxygen-dependent energy conversion and, as such, is promising for more sensitively detecting sleep disordered breathing (SDB) events compared to pulse oximetry. We hypothesized that ECM would detect hypoxia occurring with SDB events associated with drops in SpO2 but also would detect hypoxic challenge occurring with SDB events not associated with drops in SpO2 (hypopneas defined by a drop in nasal pressure occurring in conjunction with an arousal, respiratory-related arousals, and primary snoring). Primary snoring is of particular interest with respect to the potential of ECM because it is statistically associated with co-morbidities of SDB, such as hypertension, but is not considered pathological because of the lack of a proximal measure of pathology occurring with PSG. In this article we review ECM technology and methodology, present preliminary data indicating that it detects hypoxia occurring in the skin during SDB events that is not detected as blood desaturation by pulse oximetry, and make the case that it is a promising tool for identifying pathology occurring at the mild end of the SDB spectrum.


SLEEP ◽  
2021 ◽  
Vol 44 (Supplement_2) ◽  
pp. A1-A2
Author(s):  
Carla Freire ◽  
Huy Pho ◽  
Jacob Ramsey ◽  
Stone Streeter ◽  
Ryo Kojima ◽  
...  

Abstract Introduction Obstructive sleep apnea (OSA) is characterized by recurrent periods of upper airway obstruction. The prevalence of OSA exceeds 50% in obese individuals and in 10–20% of obese patients OSA coexists with obesity hypoventilation syndrome (OHS) defined as daytime hypercapnia and hypoventilation during sleep attributed to the depressed control of breathing. There is no effective pharmacotherapy for OSA and OHS. Leptin is a potent respiratory stimulant and a potential therapeutic candidate. However, diet-induced obesity (DIO) results in reduced permeability of the blood-brain barrier (BBB) for leptin. Previous studies have shown that the BBB can be penetrated by exosomes, natural nanoparticles that can be used as drug delivery vehicles. In this study, we aimed to determine if exosomes overcome the BBB and treat OSA and OHS in DIO mice. Methods o examine the ability of exosomes to cross the BBB, male, lean (n=5) and DIO (n=5) C57BL/6J mice were injected with fluorescent exosomes or saline into the lateral tail vein. After 4h fluorescent exosomes biodistribution was evaluated by an in vitro imaging system (IVIS). Saline injected mice images were used for background adjustment. A separate subgroup of male, DIO (n=10) and lean (n=10) mice were headmounted with EEG and nuchal EMG leads. Sleep studies were performed in a plethysmography chamber and mice received saline, empty exosomes, free leptin, or leptin-loaded exosomes in a crossover manner. Results Exosomes were successfully delivered to the brain and the transport across the BBB was more efficient in DIO mice with 2-times greater relative fluorescence units measured in DIO when compared to lean mice (p<0.005). In DIO mice, exosomal leptin induced dramatic 1.7-2.2-fold increases in minute ventilation and 1.5-2.0-fold increases in maximal inspiratory flow during both flow-limited (upper airway/sleep apnea) and non-flow limited breathing (control of breathing) (p<0.05). In contrast, free leptin had no effect. Lean mice did not present significant sleep disordered breathing and no differences were observed between groups. Conclusion We demonstrated that exosomes overcome the BBB and that leptin-loaded exosomes treat OSA and OHS in DIO mice. Support (if any) R01HL 128970, R01HL 138932, R61 HL156240, U18 DA052301, FAPESP 2018/08758-3


Author(s):  
Jennifer Janusz ◽  
Ann Halbower

Pediatric sleep disorders have been gaining awareness among practitioners due to their potential for cognitive, behavioral, and somatic effects (Gozal 2008; Moore et al. 2006). Sleep-disordered breathing (SDB) is commonly seen in children and encompasses a range of disorders, in primary snoring to obstructive sleep apnea (Marcus 2000). Sleep-disordered breathing is characterized by partial or complete upper airway obstruction during sleep due to collapse or narrowing of the pharynx. This can result in sleep fragmentation due to brief arousals during the night, as well as disruption or cessation of airflow (Blunden and Beebe 2006; Halbower and Mahone 2006). This chapter describes the neuropsychological and behavioral consequences of SDB, comorbid disorders, and effects of treatment. Sleep-disordered breathing is considered a spectrum of airflow limitation, from mild to severe. For instance, primary snoring (PS), defined as snoring without oxygen desaturation or sleep arousals, is at the mild end of the spectrum. Upper airway resistance syndrome (UARS), in the middle of the spectrum, is characterized by increased negative intrathoracic pressure with sleep arousals and sleep fragmentation but no oxygen desaturations (Bao and Guilleminault 2004; Garetz 2008; Lumeng and Chervin 2008). In obstructive sleep apnea (OSA), at the severe end of the spectrum, there are repeated episodes of blockage of the airway with changes in oxygenation. Obstructive sleep apnea results from a combination of factors, including anatomical obstruction from adenoids, tonsils, or a narrow pharynx, and decreased neuromuscular tone required to maintain airway patency (Arens and Marcus 2004). An overnight polysomnogram (PSG) completed in a sleep laboratory and measuring sleep–wake states, respiration, movement, blood levels of oxygen and carbon dioxide, and cardiac activity, is considered the “gold standard” for the diagnosis of OSA (American Academy of Pediatrics 2002). The PSG is used to diagnose respiratory events, cardiac changes, and arousals from different sleep states. Respiratory events include obstructive apneas and hypopneas. Obstructive apnea events are episodes of complete airway obstruction, while hypopneas are partial obstructions or airflow limitations (Garetz 2008; Redline et al. 2007).


2001 ◽  
Vol 91 (6) ◽  
pp. 2758-2766 ◽  
Author(s):  
Y. Tagaito ◽  
V. Y. Polotsky ◽  
M. J. Campen ◽  
J. A. Wilson ◽  
A. Balbir ◽  
...  

To investigate the pathophysiological sequelae of sleep-disordered breathing (SDB), we have developed a mouse model in which hypoxia was induced during periods of sleep and was removed in response to arousal or wakefulness. An on-line sleep-wake detection system, based on the frequency and amplitude of electroencephalograph and electromyograph recordings, served to trigger intermittent hypoxia during periods of sleep. In adult male C57BL/6J mice ( n= 5), the sleep-wake detection system accurately assessed wakefulness (97.2 ± 1.1%), non-rapid eye movement (NREM) sleep (96.0 ± 0.9%) and rapid eye movement (REM) sleep (85.6 ± 5.0%). After 5 consecutive days of SDB, 554 ± 29 (SE) hypoxic events were recorded over a 24-h period at a rate of 63.6 ± 2.6 events/h of sleep and with a duration of 28.2 ± 0.7 s. The mean nadir of fraction of inspired O2 (Fi O2 ) on day 5 was 13.2 ± 0.1%, and 137.1 ± 13.2 of the events had a nadir Fi O2 <10% O2. Arterial blood gases confirmed that hypoxia of this magnitude lead to a significant degree of hypoxemia. Furthermore, 5 days of SDB were associated with decreases in both NREM and REM sleep during the light phase compared with the 24-h postintervention period. We conclude that our murine model of SDB mimics the rate and magnitude of sleep-induced hypoxia, sleep fragmentation, and reduction in total sleep time found in patients with moderate to severe SDB in the clinical setting.


2017 ◽  
Vol 50 (6) ◽  
pp. 1700985 ◽  
Author(s):  
Athanasios G. Kaditis ◽  
Maria Luz Alonso Alvarez ◽  
An Boudewyns ◽  
Francois Abel ◽  
Emmanouel I. Alexopoulos ◽  
...  

The present statement was produced by a European Respiratory Society Task Force to summarise the evidence and current practice on the diagnosis and management of obstructive sleep disordered breathing (SDB) in children aged 1–23 months. A systematic literature search was completed and 159 articles were summarised to answer clinically relevant questions. SDB is suspected when symptoms or abnormalities related to upper airway obstruction are identified. Morbidity (pulmonary hypertension, growth delay, behavioural problems) and coexisting conditions (feeding difficulties, recurrent otitis media) may be present. SDB severity is measured objectively, preferably by polysomnography, or alternatively polygraphy or nocturnal oximetry. Children with apparent upper airway obstruction during wakefulness, those with abnormal sleep study in combination with SDB symptoms (e.g.snoring) and/or conditions predisposing to SDB (e.g.mandibular hypoplasia) as well as children with SDB and complex conditions (e.g.Down syndrome, Prader–Willi syndrome) will benefit from treatment. Adenotonsillectomy and continuous positive airway pressure are the most frequently used treatment measures along with interventions targeting specific conditions (e.g.supraglottoplasty for laryngomalacia or nasopharyngeal airway for mandibular hypoplasia). Hence, obstructive SDB in children aged 1–23 months is a multifactorial disorder that requires objective assessment and treatment of all underlying abnormalities that contribute to upper airway obstruction during sleep.


2003 ◽  
Vol 95 (5) ◽  
pp. 1761-1766 ◽  
Author(s):  
Jason P. Kirkness ◽  
Melanie Madronio ◽  
Rosie Stavrinou ◽  
John R. Wheatley ◽  
Terence C. Amis

Lowering surface tension (γ) of upper airway lining liquid (UAL) reduces upper airway opening (anesthetized humans) and closing (anesthetized rabbits) pressures. We now hypothesize that in sleeping obstructive sleep apnea hypopnea syndrome (OSAHS) patients lowering γ of UAL will enhance upper airway stability and decrease the severity of sleep-disordered breathing. Nine OSAHS patients [respiratory disturbance index (RDI): 49 ± 8 (SE) events/h, diagnostic night] participated in a two-part, one-night, polysomnography study. In the first part, upper airway closing pressures (during non-rapid eye movement sleep, Pcrit) were measured and samples of UAL (awake) were obtained before and after 2.5 ml of surfactant (Exosurf, Glaxo Smith Kline) was instilled into the posterior pharynx. The γ of UAL was determined with the use of the “pull-off” force technique. In the second part, subjects received a second application of 2.5 ml of surfactant and then slept the remainder of the night (205 ± 30 min). Instillation of surfactant decreased the γ of UAL from 60.9 ± 3.1 mN/m (control) to 45.2 ± 2.5 mN/m (surfactant group) ( n = 9, P < 0.001). Pcrit decreased from 1.19 ± 1.14 cmH2O (control) to -0.56 ± 1.15 cmH2O (surfactant group) ( n = 7, P < 0.02). Compared with the second half of diagnostic night, surfactant decreased RDI from 51 ± 8 to 35 ± 8 events/h ( n = 9, P < 0.03). The fall in RDI (ΔRDI) correlated with the fall in γ of UAL (Δγ) (ΔRDI = 1.8 × Δγ, r = 0.68, P = 0.04). Hypopneas decreased ∼50% from 42 ± 8 to 20 ± 5 events/h ( n = 9, P < 0.03, paired t-test). The γ of UAL measured the next morning remained low at 49.5 ± 2.7 mN/m ( n = 9, P < 0.001, ANOVA, compared with control). In conclusion, instillation of surfactant reduced the γ of UAL in OSAHS patients and decreased Pcrit and the occurrence of hypopneas. Therapeutic manipulation of γ of UAL may be beneficial in reducing the severity of sleep-disordered breathing in OSAHS patients.


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