Sleep and breathing in neuromuscular disease

Bourke, SC; Gibson, Gretchen

doi:10.1183/09031936.02.01302001a

Cited by 151 publications

(113 citation statements)

References 77 publications

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“…Noktürnal hipoventilasyon semptom ve bulguları uykusuzluk, kabus görme, sabah baş ağrısı, gün boyu uyuklama, yorgunluk, bilişsel fonksiyonlarda yavaşlamadır. 25 …”

Section: Tablo 2 Solunum Kas Zayıflığı Ile Ilişkili Problemlerunclassified

“…Uyku-uyanıklık anomalileri veya noktürnal solunum yetmezliği bulgu ve semptomları görülen hastalarda gece boyu polisomnografi veya solunum poligrafisi önerilir. 25 Nöromusküler hastalıklarda solunum fonksiyonlarının değerlendirilmesi, çok yönlü düşünmeyi ve klinik karar vermeyi gerektiren dinamik bir süreçtir. Hastanın rutin kontrollere düzenli olarak katılması, günlük yaşamındaki değişimleri izlemesi ve gözlemlerini sağlık profesyonelleriyle paylaşması cesaretlendirilmelidir.…”

Section: Uyku Değerlendirmesiunclassified

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Nöromusküler Hastalıklarda Solunum Fonksiyonlarının Değerlendirilmesi: Derleme

Yeldan

Aslan²

2014

HSP

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Nöromusküler hastalıklarda kas iskelet sistemi problemlerinin yanı sıra solunum kas zayıflığı ve buna bağlı olarak solunum disfonksiyonu gelişebilmektedir. Solunumsal komplikasyonlar, özellikle kronik ve hızlı ilerleyen nöromusküler hastalıklarda, morbidite ve mortalitenin en önemli nedenlerindendir. Bu hastalarda vital kapasite azalır, restriktif solunum paterni görülür. Solunum kas zayıflığı nedeniyle, nöromusküler hastalığı olan kişilerde yorgunluk, nefes darlığı, sekresyon birikimi, tekrarlayan alt solunum yolu infeksiyonları, solunum yetmezliği, pulmoner hipertansiyon ve akut veya kronik korpulmonale gelişebilir. Uyku ile ilişkili solunumsal problemler de başlangıçta uyku sırasında görülürken hastalığın ilerlemesiyle birlikte uyanıklık dönemine de yansır. Nöromusküler hastalıklarda sistematik klinik değerlendirme, erken dönemde hastanın fiziksel kapasitesindeki yetersizlik nedeniyle maskelenen solunumsal semptom ve bulguların saptanmasında esastır. Bu derleme nöromusküler hastalıklarda karşılaşılan solunum problemleri ile anamnez, fizik muayene, solunum kas gücü ölçümleri, arter kan gazları analizi, tepe öksürük akımı ölçümü, radyolojik değerlendirme, spirometrik ölçümler ve uyku başlıkları altında değerlendirmeyi kapsamlı olarak gözden geçirecektir. Anahtar Kelimeler: Nöromusküler hastalıklar, solunum, solunumsal değerlendirme AbstractRespiratory muscle weakness and consequently respiratory dysfunction may occur as well as musculoskeletal problems in patients with neuromuscular diseases. Respiratory complications are the most important cause of morbidity and mortality particularly in chronic and rapidly progressive neuromuscular diseases. Vital capacity is reduced and a restrictive ventilatory pattern is seen in these patients. Due to respiratory muscle weakness; fatigue, breathlessness, secretion retention, recurrent lower respiratory tract infections, respiratory failure, pulmonary hypertension, and acute or chronic corpulmonale may occur in patients with neuromuscular diseases. While respiratory problems may manifest initially during sleep, it also reflect to awake period with progression of diseases. Systematic clinical evaluation of neuromuscular diseases is essential for the detection of respiratory signs and symptoms which are masked because of physical capacity inability of patients in early stage. This review will evaluate comprehensively the assessment of respiratory problems which are encountered in neuromuscular diseases under the headings of history, physical examination, respiratory muscle strength measurements, arterial blood gas analysis, peak cough flow measurements, radiologic evaluation, spirometry and sleep.

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“…Noktürnal hipoventilasyon semptom ve bulguları uykusuzluk, kabus görme, sabah baş ağrısı, gün boyu uyuklama, yorgunluk, bilişsel fonksiyonlarda yavaşlamadır. 25 …”

Section: Tablo 2 Solunum Kas Zayıflığı Ile Ilişkili Problemlerunclassified

Section: Uyku Değerlendirmesiunclassified

Nöromusküler Hastalıklarda Solunum Fonksiyonlarının Değerlendirilmesi: Derleme

Yeldan

Aslan²

2014

HSP

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“…For example, a forced vital capacity of Ͻ40% to 50% of predicted or Ͻ1 L are associated with nocturnal hypoventilation and the need for noninvasive ventilation, independent of clinical symptoms. 1,[19][20][21] The use of such support heralds increased risk for diminished airway clearance and the need to augment the patient's weakened cough.…”

Section: Measurement Of Cough Effectivenessmentioning

confidence: 99%

Airway Clearance in Duchenne Muscular Dystrophy

Kravitz

2009

Pediatrics

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This is a summary of the presentation on airway clearance in neuromuscular disorders presented as part of the program on airway clearance in Duchenne muscular dystrophy at the 30th annual Carrell-Krusen Neuromuscular Symposium on February 20, 2008. Pediatrics 2009123:S231-S235 D UCHENE MUSCULAR DYSTROPHY (DMD) is an X-linked recessive hereditary disease that affects both skeletal and cardiac muscle. [1][2][3][4][5] The protein dystrophin is made in inadequate amounts, which leads to the break down of muscle cell walls and the cell's ultimate death. This loss of muscle leads to numerous problems including axial skeletal muscle weakness and the loss of ambulation, diaphragmatic weakness with recurrent pneumonias and progressive respiratory failure, and cardiomyopathy with arrhythmias. [2][3][4][5] The combination of cardiopulmonary failure leads to early death, with the mean life span of affected males being ϳ25 years. 1,2,[4][5][6][7] Although there is currently no cure for DMD, supportive therapies can markedly decrease the morbidity of this condition, thus prolonging the life span of these young men and improving their quality of life. This review will focus on airway clearance and cough augmentation as ways of decreasing the incidence of atelectasis, pneumonia, and respiratory failure associated with this muscular dystrophy. NORMAL COUGHCoughing works by helping to clear airway secretions from the lungs. [8][9][10][11] These secretions act as a protective layer covering the bronchi and bronchioles. They trap environmental particulate matter as well as bacteria and viruses that are inhaled during respiration. 8,11 Cilia protruding from bronchial epithelial cells move this protective layer in a proximal direction. 11 Coughing augments the ciliary clearance of these secretions, with the ultimate expectoration or swallowing of this material. 9 Coughing consists of 3 components: an inspiratory phase; a contraction phase; and an expiratory phase. 8,10 During the inspiratory phase, the patient inhales, usually to 60% to 90% of total lung capacity. 2,3,10,12,13 In the contraction phase, the patient's glottis closes and the expiratory muscles begin to contract. This phase lasts only 0.2 seconds; however, during this time, elevated intrathoracic pressures are reached. 8 During the final (expiratory) phase of a cough, the glottis opens, and there is a sudden expiratory rush of air. It is during this phase of the cough that large shearing forces are generated, cleaving off parts of the secretary lining and carrying with them the trapped foreign material. 8,10 During this expiratory phase, velocities as high as 12 m/second can be reached, with flow rates of 360 to 1000 L/minute being obtained for a normal cough. 2,3,8,10,12 Many conditions can lead to an increase in airway secretions. In pediatrics, 2 of the more common etiologies of increased airway secretions include pneumonia and asthma. These illnesses are associated with an increase in airway inflammation and a resultant increase in secretion volume. One of...

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“…Шейно-грудная форма дебюта БАС имелась у 23 пациентов, бульбарная -у 26, пояснично-кре-стцовая -у 12. Наибольший интерес для исследования нарушений дыхания во сне (НДС) и качества ночного сна представляют заболевания, сопровождающиеся изменением мышечной силы и/или тонуса, при которых НДС нередко своевремен-но не выявляются [1][2][3][4]. …”

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The severity of respiratory disorders in different forms of amyotrophic lateral sclerosis

Рушкевич

Чечик

et al. 2017

RJTAO

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Рушкевич Ю.Н., Чечик Н.М., Лихачев С.А., Меркуль О.В., Корбут Т.В. ГУ «Республиканский научно-практический центр неврологии и нейрохирургии» Министерства здравоохраненияРеспублики Беларусь, Минск, Беларусь 220114, Республика Беларусь, Минск, ул. Ф. Скорины, 24 Нарушения дыхания во сне (НДС) значительно ухудшают качество жизни пациента с боковым амиотрофическим склерозом (БАС) и способствуют присоединению серьезных осложнений со стороны дыхательной и сердечно-сосудистой систем. Цель исследования -выявление ранних признаков НДС у пациентов с разными формами дебюта БАС с помощью комплексной оцен-ки сна. Пациенты и методы. Анкетирование с использованием комплексного теста на выявление расстройств сна было проведено у 65 па-циентов: 39 из них страдали БАС (соотношение мужчин и женщин -25/14, возраст -59 [51; 66] лет) и 26 вошли в контрольную группу (13/13; 54 [43; 59] 62 [55; 67] года. Длительность заболевания с момента появления первых симптомов -12 [8,9; 27,1] мес. Индекс массы тела достигал 25,7 [23,3; 28,7] кг/м 2 . Оценка по ALSFRSR [9] на момент включения в исследование в основной груп-пе составляла 34,32 [32; 38] балла. Шейно-грудная форма дебюта БАС имелась у 23 пациентов, бульбарная -у 26, пояснично-кре-стцовая -у 12. Наибольший интерес для исследования нарушений дыхания во сне (НДС) и качества ночного сна представляют заболевания, сопровождающиеся изменением мышечной силы и/или тонуса, при которых НДС нередко своевремен-но не выявляются [1][2][3][4]. года соответственно). Анкета состоит из 50 вопросов, результаты оцениваются в баллах. Скрининговое портативное полисомнографическое исследование было проведено пациентам с впервые установленным диагнозом БАС на относительно ранних стадиях болезни. Обследован 61 пациент: 32 женщины и 29 мужчин, медиана (Ме и 25-я и 75-я процен-тили) возраста составила Скрининговую диагностику сна проводили с помощью портативного полисомнографа Polymate YH-1000C (BMC, Китай), регист-рировали назофарингеальный поток (движение воздуха через носовую и ротовую полости); торакоабдоминальные движения (дви-жения грудной и брюшной стенки); насыщение гемоглобина артериальной крови кислородом (сатурация, SpO2); звук храпа посред-ством микрофона, положение тела во сне (актография). Результаты. В ходе комплексного скринингового исследования дыхания во сне показана недооценка жалоб и симптомов субклини-Боковой амиотрофический склероз (БАС) -быстро прогрессирующее дегенеративное заболевание ЦНС, при котором поражаются преимущественно центральные и пе-риферические мотонейроны, кортико-спинальные пути. Дебют заболевания приходится на зрелый трудоспособный возраст, как правило, на 5-е десятилетие, несколько чаще страдают мужчины [5].Клинические проявления БАС в большинстве слу-чаев представлены неловкостью или слабостью в одном из дистальных отделов конечностей (кисть или стопа), реже -в проксимальных отделах (плечо, бедро) с вовле-чением поясных мышц, развиваются асимметрично. В дальнейшем мышечная слабость распространяется на все отделы и становится генерализованной. Примерно у 25...

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Sleep and breathing in neuromuscular disease

Cited by 151 publications

References 77 publications

Nöromusküler Hastalıklarda Solunum Fonksiyonlarının Değerlendirilmesi: Derleme

Nöromusküler Hastalıklarda Solunum Fonksiyonlarının Değerlendirilmesi: Derleme

Airway Clearance in Duchenne Muscular Dystrophy

The severity of respiratory disorders in different forms of amyotrophic lateral sclerosis

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