The Laryngoscope · 2025
Гипоглоссальная стимуляция нервной нервы к осложнениям сна и коморбидным неевромускулистым расстройствам.
Клинический случайСтатьяПоддержка исследования: вне правительства СШАНазвание и аннотация: Машинный перевод · не проверен
Обработка статьи · этапы, ошибки и управление
Загрузка состояния…
Ошибки и ограничения
История · было → стало
Состояние обновляется каждые 5 секунд, пока блок открыт. Процент показывается только там, где известен объём работы. Пауза останавливает продолжение этапа; текущий запрос к источнику может завершиться.
Choi Alison Y, Kaffenberger Thomas M, Soose Ryan J, Whelan Rachel L
Аннотация
Гипоглоссальная стимуляция нервной системы (HGNS) является эффективным хирургическим лечением для многих пациентов с апноэ с умеренным или сильным обструктивным сном (OSA), которые не в состоянии выдержать постоянное положительное давление в дыхательных путях. Пациенты с нервно-мышечными расстройствами (НМД) особенно уязвимы к более сложным расстройствам дыхания, связанным со сном, из-за слабости дыхательных путей и связанного с этим риска гиповентиляции. В этом исследовании мы представляем пятерых пациентов с миастенией (n = 2), мышечной дистрофией, харкотом-Мари-Зубом и продвинутым множественным склерозом, которые прошли терапию HGNS для OSA. Во время операции средний возраст пациента составлял 54 ± 18,7 года, а средний показатель БМИ - 24,6 ± 4,6 кг/м2. Среднее сцепление устройств составляло 8 ± 1,1 часа в сутки в течение трех месяцев и 7,2 ± 1,7 часа в ночь в течение 12 месяцев. Средний индекс апнеа-гипопне (AHI) значительно улучшился с дооперационного исходного уровня (34.9 - 8,5, p = 0,03) и 5/6 пациентов имели послеоперационное AHI <5. Шкалы сна Epworth Snighty Schallenge значительно улучшились после работы (13.1-8.5, p = 0,03). Кислородный надир повышался (75,5-85,3, p = 0,84). Наши ограниченные выводы показывают, что HGNS было безопасным и эффективным лечением пяти пациентов с OSA и коморбидом NMD. Перспективные исследования и более крупные размеры выборки оправданы для того, чтобы подтвердить наши выводы и подтвердить, является ли HGNS потенциальным терапевтическим вариантом для этой уникальной группы пациентов. Ларингоскоп, 135:2631-2633, 2025.
Оригинал публикации
Hypoglossal Nerve Stimulation for Obstructive Sleep Apnea and Comorbid Neuromuscular Disorders.
Hypoglossal nerve stimulation (HGNS) is an effective surgical treatment for many patients with moderate-to-severe obstructive sleep apnea (OSA) who are unable to tolerate continuous positive airway pressure. Patients with neuromuscular disorders (NMD) are particularly vulnerable to more complex sleep-related breathing disorders due to respiratory muscle weakness and the associated risk of hypoventilation. In this study, we present five patients with myasthenia gravis (n = 2), muscular dystrophy, Charcot-Marie-Tooth, and advanced multiple sclerosis who underwent HGNS therapy for OSA. At time of surgery, the mean patient age was 54 ± 18.7 years and mean BMI was 24.6 ± 4.6 kg/m2. Mean device adherence was 8 ± 1.1 h/night at three months and 7.2 ± 1.7 h/night at 12 months. Mean apnea-hypopnea index (AHI) improved significantly from preoperative baseline (34.9 to 8.5, p = 0.03) and 5/6 patients had a postoperative AHI <5. Epworth Sleepiness Scale scores significantly improved postoperatively (13.1-8.5, p = 0.03). Oxygen nadir trended upward (75.5-85.3, p = 0.84). Our limited findings demonstrate that HGNS was a safe and effective treatment for five patients with OSA and comorbid NMD. Prospective studies and larger sample sizes are warranted to support our findings and confirm whether HGNS is a potential therapeutic option for this unique patient population. Laryngoscope, 135:2631-2633, 2025.
Перевод полного текста: Перевод в очереди
Читать найденный полный текст
This is an open access article under the terms of the http://creativecommons.org/licenses/by-nc/4.0/ License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited and is not used for commercial purposes.
INTRODUCTION
Hypoglossal nerve stimulation (HGNS) is an established surgical treatment for patients with moderate‐to‐severe obstructive sleep apnea (OSA) who are unable to tolerate continuous positive airway pressure (CPAP). HGNS involves electrical stimulation of the genioglossus and geniohyoid muscles via the hypoglossal nerve, allowing for tongue protrusion and prevention of airway collapse during sleep. Clinical trials have demonstrated significant and durable reductions in Apnea‐Hypopnea Index (AHI) and Epworth Sleepiness Scale (ESS) scores and HGNS has shown improved adherence compared to CPAP.
1
Patients with neuromuscular diseases (NMD), including myasthenia gravis, muscular dystrophy, and Charcot–Marie–Tooth, are particularly vulnerable to sleep‐related breathing disorders due to respiratory muscle weakness and disease‐specific features that contribute to upper airway collapse.
2
Sleep disturbances often lead to a poorer quality of life and OSA has been shown to be an independent risk factor for cardiovascular complications.
3
To our knowledge, there are no studies in the existing literature that have explored HGNS outcomes in patients with OSA and comorbid NMD, excluding Trisomy 21. We present a case report of five patients with OSA and different neuromuscular disorders observing outcomes following HGNS implantation.
CASE REPORT
This study was approved by the local institutional review board (STUDY20050058). Five patients who underwent HGNS implantation at a single institution from 2015 to 2021 were identified to have myasthenia gravis ( n = 2), muscular dystrophy, Charcot–Marie–Tooth, and advanced multiple sclerosis (Table 1 ). At the time of surgery, mean +/− SD patient age was 58.6 ± 17.0 years and BMI was 26.1 ± 3.1 kg/m 2 . For all five patients, there were no elevated end‐tidal CO2 levels or other indicators of hypoventilation on baseline PSGs. One patient with myasthenia gravis had few central apneas on baseline PSG (<25% of AHI), but otherwise, all patients were deemed good surgical candidates. At three‐month follow‐up, mean device adherence was 8.4 ± 1.0 h/night. At 12 months, usage remained high at a mean of 7.0 ± 1.8 h/night. Mean reduction in ESS from baseline was 7.2 ± 5.7 points (range 2–17, p = 0.03) after 12 months.
TABLE 1 Patient Demographic Data. Pt # NMD Diagnosis Age at Time of Surgery Sex Preoperative BMI (kg/m 2 ) Preoperative Sleep Study Type Postoperative Sleep Study Type 1 Myasthenia gravis 66 Male 23.96 PSG HST 2 Muscular dystrophy 60 Female 28.90 PSG HST 3 Myasthenia gravis 77 Female 29.51 PSG PSG 4 Charcot–Marie–Tooth 31 Male 25.77 PSG PSG 5 Multiple Sclerosis 59 Female 22.22 HST PSG Abbreviations: HST = home sleep testing; PSG = Non‐titrated in‐lab polysomnography.
Patient Demographic Data.
Abbreviations: HST = home sleep testing; PSG = Non‐titrated in‐lab polysomnography.
Postoperative PSGs were routinely obtained at around 3 months. Four preoperative sleep studies were performed in‐lab without titration and one was performed as a home sleep test. Three postoperative studies were performed in‐lab without titration and the remainder were home sleep tests. For these studies, hypopneas were defined as reductions in airflow by 30% or greater associated with an oxyhemoglobin desaturation of >4%. The mean AHI at 3 months decreased by 74%, from 36.0 to 9.4 events per hour ( p = 0.03), with the greatest reduction seen in a patient with multiple sclerosis and the smallest reduction seen in a patient with myasthenia gravis (Table 2 ). Four patients had resolution of their OSA, with a postoperative AHI of <5. Mean oxygen nadir trended upward (73.2–85.4, p = 0.84) and mean percentage of time spent in an oxygen saturation under 90% (T90) trended downward (11.9–5.6, p = 0.62). All patients reported decreased snoring at night and no discomfort with stimulation. One patient with myasthenia gravis experienced temporary xerostomia after implantation, but otherwise, there were no procedure or therapy‐related adverse events. All patients were managed as an outpatient and there were no hospital or ED admissions postoperatively.
TABLE 2 12‐month Postoperative Device Adherence Data, Baseline Versus 12‐month Postoperative Epworth Sleepiness Scale Scores, Baseline Versus 3‐month Postoperative Sleep Study Data. Pt # Adherence at 12 Months (Hours/Night) ESS Score at Baseline and 12 Months Total AHI at Baseline and 3 Months (Events/Hour) O2 Nadir at Baseline and 3 Months (%) T90 at Baseline and 3 Months (%) Central Apneas at Baseline and 3 Months 1 8 2, 0 21.3, 4 86, 88 0.5, 2.8 24, 1 2 9.2 14, 9 65.1, 35.6 42, 71 42.3, 15.7 0, 0 3 5.3 10, 3 24.7, 2.8 84, 86 6.4, 9.7 0, 0 4 7.8 9, 4 29.4, 0.4 88, 92 0.15, 0 0, 0 5 4.9 24, 7 39.6, 4.5 66, 90 10, 0 0, 0 Abbreviations: AHI = Apnea Hypopnea Index; ESS = Epworth Sleepiness Scale; O2 nadir = Minimum oxygen saturation recorded during study; T90 = Proportion of sleep time with oxygen saturation below 90%.
12‐month Postoperative Device Adherence Data, Baseline Versus 12‐month Postoperative Epworth Sleepiness Scale Scores, Baseline Versus 3‐month Postoperative Sleep Study Data.
Abbreviations: AHI = Apnea Hypopnea Index; ESS = Epworth Sleepiness Scale; O2 nadir = Minimum oxygen saturation recorded during study; T90 = Proportion of sleep time with oxygen saturation below 90%.
DISCUSSION
To our knowledge, this is the first case report describing patients with NMD who have undergone HGNS implantation. We report significant improvements in OSA severity metrics after HGNS, which suggest that this may be an effective treatment for this unique patient population. Additionally, this cohort retained excellent adherence to device usage at 3 months (8.4 h/night) and 12 months (7.0 h/night), indicating that HGNS was well‐tolerated and patients were able to successfully activate the therapy themselves, or with another person's help.
While Trisomy 21 is not classified as an NMD, several studies have observed the effects of HGNS in these patients who have similar traits of muscle hypotonia and deficits in respiratory function during sleep. A study on long‐term outcomes in patients with Trisomy 21 and OSA found that patients who undergo sleep surgery are at significantly reduced risk for adverse health outcomes, including myocardial infarction, cerebral infarction, ischemic heart disease, essential hypertension, diabetes mellitus, and death, when compared with those who used CPAP.
3
These findings support HGNS as an effective treatment for patients with Trisomy 21 and warrant further investigation into HGNS use in those with other causes of hypotonia. Although our study lacks long‐term polysomnography data, our results may be comparable to studies with larger sample sizes and longer follow‐up. A systematic review and meta‐analysis on HGNS outcomes by Kompelli et al. reported a 57% reduction in mean AHI and 41% reduction in mean ESS across 381 patients at 12 months.
4
Our findings, which showed a greater reduction in mean AHI of 74% after 3 months and reduction in mean ESS of 61% after 12 months, suggest that patients with a component of hypotonia may potentially have a more robust response to HGNS therapy. Interestingly, a recent study on adults with Trisomy 21 reported similar rates of improvement in AHI (76%) and adherence data at 3 months (8.5 h/night) to our study findings.
5
Several limitations to this study should be considered, including its retrospective and single‐site study design. Given that NMD is relatively uncommon among patients otherwise deemed fit for surgical candidacy, our study is also limited by its small sample size. When determining surgical candidacy in patients with NMD, important aspects to consider include presence of central apnea or hypoventilation (elevated end‐tidal CO2) on baseline PSG. Our findings are also limited by lack of long‐term data given that postoperative PSGs were only obtained at the 3‐month time point. In our clinical practice, we generally do not repeat postoperative PSGs unless it is to troubleshoot therapy, thereby also supporting positive patient outcomes after HGNS.
CONCLUSION
Our limited findings demonstrate that HGNS was a safe and effective treatment for five patients with OSA and comorbid NMD. Prospective studies and larger sample sizes are warranted to support our findings and confirm whether HGNS is a potential therapeutic option for this unique patient population.
Editor's Note: This Manuscript was accepted for publication on January 29, 2025.
The authors have no funding, financial relationships, or conflicts of interest to disclose.
RJS: Cryosa Inc (Consultant/Advisory Board), Inspire Medical Systems (Consultant, Grant/Research Support), XII Medical (Consultant/Advisory Board); TMK: Inspire Medical Systems (Consultant), Nyxoah SA (Consultant), Cryosa Inc (Institutional Research Support); All others: None.
BIBLIOGRAPHY
Hypoglossal nerve stimulation (HGNS) is an established surgical treatment for patients with moderate‐to‐severe obstructive sleep apnea (OSA) who are unable to tolerate continuous positive airway pressure (CPAP). HGNS involves electrical stimulation of the genioglossus and geniohyoid muscles via the hypoglossal nerve, allowing for tongue protrusion and prevention of airway collapse during sleep. Clinical trials have demonstrated significant and durable reductions in Apnea‐Hypopnea Index (AHI) and Epworth Sleepiness Scale (ESS) scores and HGNS has shown improved adherence compared to CPAP.
1
Patients with neuromuscular diseases (NMD), including myasthenia gravis, muscular dystrophy, and Charcot–Marie–Tooth, are particularly vulnerable to sleep‐related breathing disorders due to respiratory muscle weakness and disease‐specific features that contribute to upper airway collapse.
2
Sleep disturbances often lead to a poorer quality of life and OSA has been shown to be an independent risk factor for cardiovascular complications.
3
To our knowledge, there are no studies in the existing literature that have explored HGNS outcomes in patients with OSA and comorbid NMD, excluding Trisomy 21. We present a case report of five patients with OSA and different neuromuscular disorders observing outcomes following HGNS implantation.
CASE REPORT
This study was approved by the local institutional review board (STUDY20050058). Five patients who underwent HGNS implantation at a single institution from 2015 to 2021 were identified to have myasthenia gravis ( n = 2), muscular dystrophy, Charcot–Marie–Tooth, and advanced multiple sclerosis (Table 1 ). At the time of surgery, mean +/− SD patient age was 58.6 ± 17.0 years and BMI was 26.1 ± 3.1 kg/m 2 . For all five patients, there were no elevated end‐tidal CO2 levels or other indicators of hypoventilation on baseline PSGs. One patient with myasthenia gravis had few central apneas on baseline PSG (<25% of AHI), but otherwise, all patients were deemed good surgical candidates. At three‐month follow‐up, mean device adherence was 8.4 ± 1.0 h/night. At 12 months, usage remained high at a mean of 7.0 ± 1.8 h/night. Mean reduction in ESS from baseline was 7.2 ± 5.7 points (range 2–17, p = 0.03) after 12 months.
TABLE 1 Patient Demographic Data. Pt # NMD Diagnosis Age at Time of Surgery Sex Preoperative BMI (kg/m 2 ) Preoperative Sleep Study Type Postoperative Sleep Study Type 1 Myasthenia gravis 66 Male 23.96 PSG HST 2 Muscular dystrophy 60 Female 28.90 PSG HST 3 Myasthenia gravis 77 Female 29.51 PSG PSG 4 Charcot–Marie–Tooth 31 Male 25.77 PSG PSG 5 Multiple Sclerosis 59 Female 22.22 HST PSG Abbreviations: HST = home sleep testing; PSG = Non‐titrated in‐lab polysomnography.
Patient Demographic Data.
Abbreviations: HST = home sleep testing; PSG = Non‐titrated in‐lab polysomnography.
Postoperative PSGs were routinely obtained at around 3 months. Four preoperative sleep studies were performed in‐lab without titration and one was performed as a home sleep test. Three postoperative studies were performed in‐lab without titration and the remainder were home sleep tests. For these studies, hypopneas were defined as reductions in airflow by 30% or greater associated with an oxyhemoglobin desaturation of >4%. The mean AHI at 3 months decreased by 74%, from 36.0 to 9.4 events per hour ( p = 0.03), with the greatest reduction seen in a patient with multiple sclerosis and the smallest reduction seen in a patient with myasthenia gravis (Table 2 ). Four patients had resolution of their OSA, with a postoperative AHI of <5. Mean oxygen nadir trended upward (73.2–85.4, p = 0.84) and mean percentage of time spent in an oxygen saturation under 90% (T90) trended downward (11.9–5.6, p = 0.62). All patients reported decreased snoring at night and no discomfort with stimulation. One patient with myasthenia gravis experienced temporary xerostomia after implantation, but otherwise, there were no procedure or therapy‐related adverse events. All patients were managed as an outpatient and there were no hospital or ED admissions postoperatively.
TABLE 2 12‐month Postoperative Device Adherence Data, Baseline Versus 12‐month Postoperative Epworth Sleepiness Scale Scores, Baseline Versus 3‐month Postoperative Sleep Study Data. Pt # Adherence at 12 Months (Hours/Night) ESS Score at Baseline and 12 Months Total AHI at Baseline and 3 Months (Events/Hour) O2 Nadir at Baseline and 3 Months (%) T90 at Baseline and 3 Months (%) Central Apneas at Baseline and 3 Months 1 8 2, 0 21.3, 4 86, 88 0.5, 2.8 24, 1 2 9.2 14, 9 65.1, 35.6 42, 71 42.3, 15.7 0, 0 3 5.3 10, 3 24.7, 2.8 84, 86 6.4, 9.7 0, 0 4 7.8 9, 4 29.4, 0.4 88, 92 0.15, 0 0, 0 5 4.9 24, 7 39.6, 4.5 66, 90 10, 0 0, 0 Abbreviations: AHI = Apnea Hypopnea Index; ESS = Epworth Sleepiness Scale; O2 nadir = Minimum oxygen saturation recorded during study; T90 = Proportion of sleep time with oxygen saturation below 90%.
12‐month Postoperative Device Adherence Data, Baseline Versus 12‐month Postoperative Epworth Sleepiness Scale Scores, Baseline Versus 3‐month Postoperative Sleep Study Data.
Abbreviations: AHI = Apnea Hypopnea Index; ESS = Epworth Sleepiness Scale; O2 nadir = Minimum oxygen saturation recorded during study; T90 = Proportion of sleep time with oxygen saturation below 90%.
DISCUSSION
To our knowledge, this is the first case report describing patients with NMD who have undergone HGNS implantation. We report significant improvements in OSA severity metrics after HGNS, which suggest that this may be an effective treatment for this unique patient population. Additionally, this cohort retained excellent adherence to device usage at 3 months (8.4 h/night) and 12 months (7.0 h/night), indicating that HGNS was well‐tolerated and patients were able to successfully activate the therapy themselves, or with another person's help.
While Trisomy 21 is not classified as an NMD, several studies have observed the effects of HGNS in these patients who have similar traits of muscle hypotonia and deficits in respiratory function during sleep. A study on long‐term outcomes in patients with Trisomy 21 and OSA found that patients who undergo sleep surgery are at significantly reduced risk for adverse health outcomes, including myocardial infarction, cerebral infarction, ischemic heart disease, essential hypertension, diabetes mellitus, and death, when compared with those who used CPAP.
3
These findings support HGNS as an effective treatment for patients with Trisomy 21 and warrant further investigation into HGNS use in those with other causes of hypotonia. Although our study lacks long‐term polysomnography data, our results may be comparable to studies with larger sample sizes and longer follow‐up. A systematic review and meta‐analysis on HGNS outcomes by Kompelli et al. reported a 57% reduction in mean AHI and 41% reduction in mean ESS across 381 patients at 12 months.
4
Our findings, which showed a greater reduction in mean AHI of 74% after 3 months and reduction in mean ESS of 61% after 12 months, suggest that patients with a component of hypotonia may potentially have a more robust response to HGNS therapy. Interestingly, a recent study on adults with Trisomy 21 reported similar rates of improvement in AHI (76%) and adherence data at 3 months (8.5 h/night) to our study findings.
5
Several limitations to this study should be considered, including its retrospective and single‐site study design. Given that NMD is relatively uncommon among patients otherwise deemed fit for surgical candidacy, our study is also limited by its small sample size. When determining surgical candidacy in patients with NMD, important aspects to consider include presence of central apnea or hypoventilation (elevated end‐tidal CO2) on baseline PSG. Our findings are also limited by lack of long‐term data given that postoperative PSGs were only obtained at the 3‐month time point. In our clinical practice, we generally do not repeat postoperative PSGs unless it is to troubleshoot therapy, thereby also supporting positive patient outcomes after HGNS.
CONCLUSION
Our limited findings demonstrate that HGNS was a safe and effective treatment for five patients with OSA and comorbid NMD. Prospective studies and larger sample sizes are warranted to support our findings and confirm whether HGNS is a potential therapeutic option for this unique patient population.
Editor's Note: This Manuscript was accepted for publication on January 29, 2025.
The authors have no funding, financial relationships, or conflicts of interest to disclose.
RJS: Cryosa Inc (Consultant/Advisory Board), Inspire Medical Systems (Consultant, Grant/Research Support), XII Medical (Consultant/Advisory Board); TMK: Inspire Medical Systems (Consultant), Nyxoah SA (Consultant), Cryosa Inc (Institutional Research Support); All others: None.
BIBLIOGRAPHY