Non-invasive positive pressure ventilation for laryngeal contraction disorder during sleep in multiple system atrophy

doi:10.1016/j.jns.2006.03.008

Journal of the Neurological Sciences

Volume 247, Issue 1, 15 August 2006, Pages 53-58

https://doi.org/10.1016/j.jns.2006.03.008 Get rights and content

Abstract

We examined the usefulness of non-invasive positive pressure ventilation (NPPV) in the management of nocturnal laryngeal stridor associated with vocal cord dysfunction in five cases of multiple system atrophy (MSA). First, the patients were investigated during sleep induced by a minimal dose of propofol. Laryngoscopy showed paradoxical vocal cord movement resulting in inspiratory stridor. Electromyographic (EMG) study revealed synchronized bursts in the thyroarytenoid muscles and diaphragm in every inspiratory phase whenever the stridor emerged. NPPV was initiated after paradoxical movement was recognized with laryngoscopy. The NPPV mask was equipped with an additional channel for laryngoscopic monitoring. The optimal pressure for treatment was determined according to laryngoscopic and EMG findings. Next, NPPV was applied to natural sleep using the conditions determined in propofol-induced sleep. In all cases, NPPV eliminated nocturnal stridor and oxygen desaturation during natural sleep. Laryngoscopic observation during induced sleep is recommended as a useful procedure to titrate the optimal pressure for NPPV therapy. Since central hypoventilation progresses in the course of MSA, the choice of NPPV rather than continuous positive airway pressure should be encouraged to treat laryngeal contraction disorder associated with MSA.

Introduction

Multiple system atrophy (MSA) may manifest a full spectrum of disordered breathing during sleep, including obstructive sleep apnea syndrome, central sleep apnea syndrome, vocal cord dysfunction, and respiratory dysrhythmias [1], [2]. Nocturnal stridor is the most characteristic sleep breathing problem. The stridor is a harsh or strained, high-pitched, inspiratory sound usually distinguishable from snoring [3]. If untreated, sudden death may occur during sleep [1], [4], [5]. Although pathological and electrophysiological studies have been used to investigate the possible mechanisms [6], [7], the pathogenesis of stridor remains controversial. If laryngeal inspiratory stridor is demonstrated by laryngoscopy, tracheostomy or continuous positive airway pressure (CPAP) has been recommended as the standard treatment [1], [8]. The latter may be a more acceptable approach to eliminate stridor for most patients. However, the use of CPAP is not always successful for treating breathing disorders in MSA [5]. Alternatively, non-invasive positive pressure ventilation (NPPV) such as biphasic positive airway pressure (BiPAP) seems to be suitable for subduing laryngeal inspiratory stridor in our experience.

The aims of the present study were: (1) to investigate laryngeal inspiratory stridor correlated with vocal cord dysfunction by laryngoscopic and electromyographic (EMG) study under propofol-induced sleep; (2) to describe the method of titrating the optimal pressures to eliminate stridor by NPPV during induced sleep; and (3) to confirm the effectiveness the optimal settings of NPPV during natural sleep in MSA patients.

Section snippets

Subjects

We enrolled five MSA patients (Table 1) to examine the application of NPPV to treat sleep breathing disorder. Polysomnography (PSG) (Embla Polysomnograph, Medcare, Iceland) demonstrated oxygen desaturation with laryngeal inspiratory stridor during sleep in all the patients. Signed informed consent was obtained from all patients after explaining further experimental procedures in detail.

The PSG parameters were monitored as follows: brain activity by four electroencephalographic montages (C3-A2,

All patients

Insufficient opening of the glottis was present during wakefulness in all patients (Fig. 1A).

During propofol-induced sleep, laryngoscopy showed paradoxical vocal cord movement resulting in inspiratory stridor (Fig. 1B). The EMG study revealed synchronized bursts in TA muscles and diaphragm in every inspiratory phase when inspiratory stridor emerged (Fig. 2). Loud high-pitched inspiratory stridor associated with retraction of the sternum was evoked from the onset of optimal sleep and persisted

Discussion

Two different mechanisms may underlie vocal cord dysfunction in MSA. First, neuronal degeneration in nucleus ambiguus may give rise to motor neuron weakness of the abducting posterior cricoarytenoid muscles. Indeed, pathological study revealed a selective neurogenic atrophy in the abductor muscles [1]. Clinical observation reported that insufficient opening of the laryngeal aperture may be present even during wakefulness in the advanced stage [5]. Second, abnormal over-activity of the adductor

Conclusion

In MSA patients with nocturnal stridor, paradoxical vocal cord movement is accompanied by inspiratory phasic activation of adductor muscle. Application of NPPV is effective to subdue paradoxical vocal cord movement and improve oxygen desaturation. Consideration the progression of central hypoventilation in the course of MSA, NPPV rather than CPAP should be encouraged to treat laryngeal contraction disorder associated with MSA.

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Nocturnal stridor is a frequent symptom in MSA associated with respiratory failure and sudden death during sleep (Iranzo, 2007; Silber & Levine, 2000). Small studies support the use of home non-invasive positive pressure ventilation (NPPV) (Nonaka et al., 2006) and continuous positive airway pressure (CPAP) ventilation (Iranzo et al., 2004). Tolerability of this kinds of treatment is well, especially early in the disease (Ghorayeb, Yekhlef, Bioulac, & Tison, 2005).
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Nocturnal stridor is associated with respiratory failure and sudden death during sleep (Iranzo, 2007). Treatment recommendations emerging from several small studies include home non-invasive positive pressure ventilation (NPPV), which was reported to eliminate nocturnal stridor and oxygen desaturation during natural sleep in all patients studied (Nonaka et al., 2006). Continuous positive airway pressure (CPAP) ventilation has been used with comparable success (Iranzo et al., 2004).
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Approximately 30% of patients with MSA have nocturnal inspiratory stridor, which may seriously disrupt sleep of caregivers, even if not sleeping in the same room with the patient. Some reports have suggested positive effects of non-invasive ventilation approaches inclucding continuous positive airway pressure or non-invasive positive pressure ventilation such as biphasic positive airway pressure in MSA patients with inspiratory stridor.145-147 These non-invasive ventilation approaches are not always possible, however, and may be associated with worsening of respiration in sleep in some patients.28
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Non-invasive positive pressure ventilation for laryngeal contraction disorder during sleep in multiple system atrophy

Abstract

Introduction

Section snippets

Subjects

All patients

Discussion

Conclusion

Chest

Lancet

Laryngeal abductor paralysis in multiple system atrophy

Brain

Peculiar snoring in patients with multiple system atrophy: its sound source, acoustic characteristics, and diagnostic significance

Ann Otol Rhinol Laryngol

Abnormal respiration and sudden death during sleep in multiple system atrophy with autonomic failure

Neurology

Stridor and death in multiple system atrophy

Mov Disord

Laryngeal electromyography with separated surface electrodes in patients with multiple system atrophy presenting vocal cord paralysis

J Neurol