Sleep-disordered breathing at an early stage of amyotrophic lateral sclerosis

doi:10.1016/S0022-510X(99)00044-1

Journal of the Neurological Sciences

Volume 164, Issue 1, 15 March 1999, Pages 37-43

https://doi.org/10.1016/S0022-510X(99)00044-1 Get rights and content

Abstract

Eighteen amyotrophic lateral sclerosis (ALS) patients having neither respiratory complaints nor subjective symptoms of sleep disturbance were studied by using an ambulatory multi-parameter monitoring system during sleep. They were divided into two groups: 11 patients with predominantly bulbar form and seven with non-bulbar form. After performing daytime pulmonary function tests, the presence of sleep-disordered breathing (SDB) was evaluated by using a portable device. ALS patients did not show significant SDB as a whole, and the respiratory disturbance index (RDI) was not significantly different between the bulbar group and the non-bulbar group. However, three patients of the bulbar group showed significant SDB, and the patterns of apnea/hypopnea suggested that both bulbar weakness and minimal diaphragmatic weakness might cause SDB in ALS patients at an early clinical stage. Multi-parameter respiratory monitoring during sleep should be included in the routine evaluation of ALS patients at an early clinical stage, especially those with predominantly bulbar involvement, in order to predict early respiratory failure.

Introduction

Neuromuscular diseases of various etiologies are often accompanied by respiratory disturbance due to weakness of the respiratory muscles [21]. Patients mildly affected by these diseases may show respiratory disturbance only during sleep or sleep-disordered breathing (SDB) while they still manifest no clinical signs of respiratory dysfunction in the daytime [16], [18]. SDB was observed also in patients with amyotrophic lateral sclerosis (ALS) who presented with advanced bulbar symptoms or respiratory muscle weakness [7], [8]. These previous studies, however, dealt with ALS patients at different clinical stages or at various lengths of time after the clinical onset, and furthermore those patients lacked detailed neurological information. In patients with ALS at an early clinical stage in particular, monitoring of respiratory function during sleep has not been reported to our knowledge.

In ALS, not only the muscles subserving inspiration and/or expiration itself but also those involved in bulbar function may cause SDB when affected. For example, bulbar involvement with brainstem infarction influences the incidence and severity of SDB [1]. In this study, therefore, in order to evaluate the respiratory function during sleep in patients at an early stage of ALS presenting no daytime respiratory symptoms, we investigated the occurrence and degree of SDB by using a multi-parameter EdenTrace II recorder. The data were analyzed with special emphasis being placed on the presence or absence of bulbar symptoms.

Section snippets

Subjects and methods

We studied 18 patients with ALS (seven women and 11 men) who were at an early clinical stage and had no respiratory complaints or subjective symptoms of sleep disturbance. Their age ranged from 45 to 75 years (mean±SD, 57.6±8.1 years). All of them were admitted to our hospital for neurological evaluation between January 1996 and July 1997. Inclusion criteria were: firstly, they suffered from definite or probable ALS according to the diagnostic criteria (see below) and, secondly, they were

Results

The patients consisted of 11 bulbar and seven non-bulbar patients according to the above criteria. None of the patients had respiratory complaints or subjective symptoms of sleep disturbance. All were normotensive with a normal electrocardiogram, and had no history of cardiovascular diseases. The age of the bulbar and non-bulbar group ranged from 46 to 68 years (mean±SD, 58.2±7.3) and from 45 to 75 years (56.6±9.8), respectively. The duration of illness from the onset of clinical symptoms to

Discussion

The advantage of using ambulatory equipment like that used in the present study is that it enables us to study a substantial number of neurological patients, especially in circumstances where a full polysomnographic (PSG) study is unable to be performed routinely due to lack of laboratory technicians. Although the ambulatory equipment precludes us from monitoring EEG, electrooculogram and chin electoromyogram, all of which are essential for sleep staging, a high level of agreement was

Acknowledgements

The authors are grateful to Dr Motoharu Ohi and Dr Kazuo Chin of the Chest Disease Research Institute of Kyoto University for their advice on pulmonary function tests. This study was partly supported by Grants-in-Aid for Scientific Research (A) 09308031 and (A) 08558083, and for Scientific Research on Priority Areas 08279106 from the Japan Ministry of Education, Science, Sports and Culture, Research for the Future Program from the Japan Society for the Promotion of Science JSPS-RFTF97L00201,

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Sleep-disordered breathing occurs frequently in neuromuscular diseases and chest wall disorders as a result of the effects of normal sleep on ventilation and additional challenges imposed by specific disorders. Patterns of sleep-disordered breathing include sleep-related hypoventilation and hypoxemia as well as sleep apnea. Sleep-related hypoventilation precedes diurnal respiratory failure and clinical presentation is often nonspecific. Polysomnography may be useful to identify patterns and severity of sleep-disordered breathing and to titrate therapy. Noninvasive positive pressure ventilation is considered first line therapy in patients with progressive restrictive thoracic disorders and respiratory insufficiency.
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According to Dean et al., the difference in AHI between polio survivors with or without bulbar sequelae was significant only in terms of central events (4.29 vs. 0.67, P = 0.016) [39]. This finding suggests that bulbar involvement may play a role both in CSA and OSA genesis in the same way as it does in amyotrophic lateral sclerosis (by impaired diaphragmatic and upper airway dilator muscles, respectively) [43]. Moreover, PLMs or obstructive event-related μ-arousals are known to promote central events, but no study has properly addressed this issue or the consequences and management of CSA in polio survivors.
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This review focused on current knowledge of the prevalence of sleep disorders in polio survivors, their features, predictive factors and management.
Articles were searched in PubMed and the Cochrane Library up to March 2018.
Articles needed to 1) be written in English; 2) include only participants with previous poliomyelitis or post-polio syndrome diagnosis; and 3) involve any form of sleep disorders. Articles about isolated fatigue or non-specific sleep complaints as well as non-polio specific articles (neuromuscular disorders) were not included in the qualitative analysis.
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Follow-up of polio survivors should include systematic screening for sleep disorders because they are associated with adverse consequences. Sleep disorder evaluation and management should improve the long-term survival and quality of life of polio survivors. Methodologically robust clinical trials are needed, but the decreasing prevalence and large clinical spectrum of the disease may complicate the creation of comparable groups.
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Patients with NMD have fragmented sleep, low sleep efficiency, increased phase 1, reduced REM sleep, central and mixed apnea, hypoventilation and, less frequently, obstructive sleep apneas.35,36 HMV can improve respiratory parameters, survival and quality of life.37–40 Improved oxygenation has also been described.41
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Airway clearance is very important in patients with HMV and many patients, particularly those with reduced peak cough flow, require airway clearance (manual or assisted) or assisted cough techniques (manual or mechanical) and suctioning procedures, in addition to ventilation.
In the case of invasive HMV, good tracheostomy cannula management is essential for success.
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Other techniques, such as bronchoscopy or nutritional support, may be required in patients on HMV, particularly if percutaneous gastrostomy is required.
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Otras técnicas que pueden ser requeridas en pacientes con VMD son la fibrobroncoscopia o el soporte nutricional (especialmente si se requiere la colocación de una sonda de gastrostomía).
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Central sleep apnea is characterized by insufficient drive to breathe during sleep and is associated with major comorbidity. There are many forms of central sleep apnea including the classic Cheyne–Stokes breathing pattern, which is relatively common in patients with advanced heart failure. This article focuses on the other forms of central sleep apnea that may occur in conjunction with various medical conditions including brain stem tumors/abnormalities, chronic pain/cancer/drug abuse, congenital central hypoventilation syndrome, insomnia, neuromuscular conditions, obesity hypoventilation syndrome, periodic leg movement disorder, renal failure, and stroke.
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Sleep-disordered breathing at an early stage of amyotrophic lateral sclerosis

Abstract

Introduction

Section snippets

Subjects and methods

Results

Discussion

Acknowledgements

Chest

Mayo Clin Proc

J Neurol Sci

Chest

Sleep apnea as a feature of bulbar stroke

Stroke

Sleep apnea, hypopnea and oxygen desaturation in normal subjects

N Engl J Med

Effect of sleep position on sleep apnea severity

Sleep

Polysomnographic studies in amyotrophic lateral sclerosis

J Neurol Sci

Sleep positions in the young adult and their relationship with the subjective quality of sleep

Sleep

Respiratory complications and their management in motor neuron disease

Brain