Sleep polygraphic parameters in neuromuscular diseases

Pradella, Márcia

doi:10.1590/s0004-282x1994000400004

Cited by 14 publications

(4 citation statements)

References 16 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…There are very few studies of sleep patterns in patients with SMA, and especially in subjects with SMA1 [9]. In a polysomnographic (PSG) study of 32 neuromuscular patients, four with a form of SMA sleep architecture revealed an increase in stage 1 sleep coupled with a decrease or absence of rapid eye movement (REM) sleep [10].…”

Section: Introductionmentioning

confidence: 99%

Sleep architecture in infants with spinal muscular atrophy type 1

Verrillo¹,

Bruni²,

Pavone³

et al. 2014

Sleep Medicine

View full text Add to dashboard Cite

Section: Introductionmentioning

confidence: 99%

Sleep architecture in infants with spinal muscular atrophy type 1

Verrillo¹,

Bruni²,

Pavone³

et al. 2014

Sleep Medicine

View full text Add to dashboard Cite

“…Furthermore, sleep latency was found to be significantly prolonged in SMA type 1 patients, but no data are available regarding sleep latency in SMA type 2 and 3 ( 42 ). Regarding NREM sleep, N1 and N2 were increased in SMA type 1, and stages N1was increased in SMA type 2 ( 24 , 41 , 42 ), while slow-wave sleep (sleep stage N3) were decreased ( 42 ). However, stage N2 sleep proportion is found to be lower in SMA type 1 compared to types 2–3, and stages N1-3 increased parallelly with age ( 34 ).…”

Section: Respiratory Function and Sleep In Smamentioning

confidence: 95%

“…Indeed, very few studies investigated sleep patterns in patients with SMA. Scattered reports of a few patients revealed a decrease or absence of REM sleep together with an increase of stage 1 sleep in SMA patients ( 41 ). Furthermore, sleep latency was found to be significantly prolonged in SMA type 1 patients, but no data are available regarding sleep latency in SMA type 2 and 3 ( 42 ).…”

Section: Respiratory Function and Sleep In Smamentioning

confidence: 99%

Sleep and sleep-related breathing disorders in patients with spinal muscular atrophy: a changing perspective from novel treatments?

Abati,

Mauri,

Rimoldi

et al. 2024

Front. Neurol.

View full text Add to dashboard Cite

Spinal Muscular Atrophy (SMA) is an inherited neuromuscular disorder characterized by progressive muscle weakness and atrophy, resulting from the degeneration of motor neurons in the spinal cord. A critical aspect of SMA is its impact on respiratory function. As the disease progresses, respiratory muscles, in particular intercostal muscles, become increasingly affected, leading to breathing difficulties and respiratory failure. Without intervention, many children with SMA type 1 die from respiratory failure before their second year of life. While assisted ventilation has improved survival, it often results in ventilator dependence. The development of new SMN-augmenting therapies has renewed optimism, but their long-term impact on respiratory function is uncertain, and non-invasive respiratory support remains an important part of SMA management. Despite the importance of respiratory support in SMA, knowledge regarding sleep disorders in this population is limited. This review aims to synthesize existing literature on sleep and sleep-related breathing disorders in patients with SMA, with a focus on SMA type 1. We summarize evidence of sleep-disordered breathing and respiratory failure in SMA, as well as outcomes and survival benefits associated with non-invasive or invasive ventilation with or without pharmacological therapies. We also discuss current knowledge regarding the effects of novel disease-modifying therapies for SMA on respiratory function and sleep. In conclusion, optimal care for children with SMA requires a multidisciplinary approach that includes neurology and respiratory specialists. This review highlights the importance of monitoring sleep and respiratory function in SMA, as well as the potential benefits and challenges associated with assisted ventilation combined with new therapies.

show abstract

“…Buna ek olarak, REM uyku evre süresinde artış da bildirilmiştir (10). SMA tip 1 ve tip 2 hastalarında da UE'de azalma ile birlikte, yüzeyel uyku evre sürelerinde (N1 ve N2) artış, ve REM uyku evre sürelerinde ise azalma bildirilmiştir (11)(12)(13)(14)(15). Bizim çalışmamızda ise, DMD tanısı alan hastalarda uykunun makro yapısı incelendiğinde, aynı yaş grubundan oluşan sağlıklı kontrol grubuna kıyasla, REML uzun ve toplam REM uyku evre süresinin kısa olduğu izlendi; ancak bu fark istatistiksel anlamlı değere ulaşmadı.…”

Section: Sonuçunclassified

Evaluation of Sleep Structure and Sleep-related Disorders in Pediatric Patients Diagnosed with Duchenne Muscular Dystrophy and Spinal Muscular Atrophy

Arkalı¹,

Şenel²,

Kılıç³

et al. 2020

jtsm

View full text Add to dashboard Cite

Objective: Duchenne muscular dystrophy (DMD) is a hereditary neuromuscular disorder in which respiratory problems arise secondary to the involvement of skeletal muscles. Spinal muscular atrophy (SMA) is a disorder group characterized by the involvement of anterior horn cells. The most common respiratory problems in DMD and SMA patients are pharyngeal muscle weakness in sleep and sleep-related respiratory disorders. In this study, we evaluated sleep structure and sleep-related disorders in pediatric age group diagnosed with DMD and SMA. Materials and Methods: In pediatric patients who were diagnosed to have DMD or SMA upon genetic analysis between 2014 and 2018 were performed polysomnographic investigations in our Sleep and Disorder Units. Results: Age (p=0.074), gender (p=0.065) or Body Mass index (p=0.473) were compatible between the three groups, including 13 DMD patients, seven SMA patients, and six healthy control children. In DMD patients, although rapid eye movement (REM) sleep phase latency was longer and REM sleep phase duration was shorter compared to both healthy controls and SMA patients; it was not statistically significant. In SMA patients, total sleep time and deep non-REM (NREM) sleep duration were shorter than both healthy controls and DMD patients; though this difference was not statistically significant. Pediatric Obstructive Sleep Apnea syndrome (OSAS) was detected in three DMD patients (23.1%) and six SMA patients (85.7%), no pediatric OSAS was detected in the control group (p=0.010). Mean Apnea-hypopnea index was 2.15+4.60/ hr in DMD patients, 7.43+6.19/hr in SMA patients and 0.33+0.52/hr in control group (p=0.018). Conclusion: In our study, it was found that sleep structure is disturbed and pediatric OSAS is more commonly encountered in DMD and SMA patient groups, which two disorders causing muscle and respiratory problems due to muscular and neurogenic involvement.

show abstract

Sleep polygraphic parameters in neuromuscular diseases

Cited by 14 publications

References 16 publications

Sleep architecture in infants with spinal muscular atrophy type 1

Sleep architecture in infants with spinal muscular atrophy type 1

Sleep and sleep-related breathing disorders in patients with spinal muscular atrophy: a changing perspective from novel treatments?

Evaluation of Sleep Structure and Sleep-related Disorders in Pediatric Patients Diagnosed with Duchenne Muscular Dystrophy and Spinal Muscular Atrophy

Contact Info

Product

Resources

About