Daytime Hypoxemia, Sleep-Disordered Breathing, and Laryngopharyngeal Findings in Multiple System Atrophy

Brain Research Institute, Niigata University, Niahi-niigata, Niigata, Japan
JAMA Neurology (Impact Factor: 7.42). 07/2007; 64(6):856-61. DOI: 10.1001/archneur.64.6.856
Source: PubMed


The mechanism underlying nocturnal sudden death in patients with MSA remains unclear. It may be explained by upper airway obstruction, such as vocal cord abductor paralysis; an impairment of the respiratory center, such as Cheyne-Stokes respiration; or an impaired hypoxemic ventilatory response.
To investigate the mechanism of sleep-disordered breathing in multiple system atrophy (MSA).
We recruited 21 patients with probable MSA who were admitted sequentially to our hospital, and performed daytime blood gas analysis, pulmonary function tests, polysomnography, and fiberoptic laryngoscopy during wakefulness and with the patient under anesthesia.
A decrease in arterial oxygen pressure and an increase in alveolar-arterial oxygen gradient significantly correlated with disease duration (P = .045 and .046, respectively). Polysomnography demonstrated Cheyne-Stokes respiration in 3 (15%) of 20 patients. Fiberoptic laryngoscopy during wakefulness showed that 3 (14%) of the 21 patients exhibited vocal cord abductor paralysis, and laryngoscopy under anesthesia showed that 9 (45%) of 20 patients exhibited vocal cord abductor paralysis. Laryngoscopy under anesthesia also revealed that 11 (55%) of 20 patients showed upper airway obstruction in places other than the vocal cords, including obstruction at the base of the tongue or soft palate. In addition, it demonstrated novel laryngopharyngeal findings, such as floppy epiglottis and airway obstruction at the arytenoid.
We observed daytime hypoxemia with an increased alveolar-arterial oxygen gradient, Cheyne-Stokes respiration, and novel abnormal laryngopharyngeal movements in patients with MSA. We also found that laryngoscopy under anesthesia might be useful for evaluating upper airway obstruction. The significance of these findings to the mechanism of sudden death in those with MSA needs to be examined.

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Available from: Takayoshi Shimohata
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    • "Debates continue whether the stridor occurs as a result of dystonic vocal cord or bilateral vocal fold paresis [63] [64] [65]. However , false negative findings may be obtained from this examination, requiring the examination under anesthesia and/or esophageal pressure monitoring in highly suspected cases [61] [66] [67]. Prompt intervention of SDBs is essential since they may underlie the mechanism of sudden death in these patients. "
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    ABSTRACT: Although nocturnal disturbances are increasingly recognized as an integral part of the continuum of daytime manifestations of Parkinson's disease (PD), there is still little evidence in the medical literature to support the occurrence of these complex phenomena in patients with atypical parkinsonian disorders (APDs). Based on the anatomical substrates in APDs, which are considered to be more extensive outside the basal ganglia than in PD, we might expect that patients with APDs encounter the whole range of nocturnal disturbances, including motor, sleep disorders, autonomic dysfunctions, and neuropsychiatric manifestations at a similar, or even greater, frequency than in PD. This article is a review of the current literature on the problems at nighttime of patients with progressive supranuclear palsy, multiple system atrophy, corticobasal degeneration, and dementia with Lewy bodies. MEDLINE, life science journals and online books were searched by querying appropriate key words. Reports were included if the studies were related to nocturnal manifestations in APDs. Forty articles fulfilled the selection criteria. Differences between these symptoms in APDs and PD are highlighted, given the evidence available about each manifestation. This analysis of nocturnal manifestations of APDs suggests the need for future studies to address these issues to improve the quality of life not only of patients with APDs but the caregivers who encounter the challenges of supporting these patients on a daily basis.
    Full-text · Article · Apr 2014
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    • "Moreover, around 20% of MSA patients succumb to nocturnal sudden death which may be related to stridor or other respiratory disturbances including sleep apnea [9]. SA is mostly obstructive, and is mixed or central in some MSA patients [2] [3] [4] [5] [6] [7] [8]. Continuous positive airway pressure has been suggested as an effective therapy for SA without distinguishing between central, obstructive or mixed origins [7] [10] [11]. "
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    ABSTRACT: Objective To assess the diagnostic accuracy of portable polygraphy (PG) for the detection of sleep apnea (SA) in multiple system atrophy (MSA). Methods Thirty consecutive patients with probable MSA underwent PG (overnight recording of nasal flow, thoracic/abdominal movements and pulse oximetry), followed four weeks later by full polysomnography (PSG) (reference standard). The accuracy of PG was first assessed using the same threshold as for PSG (apnea–hypopnea index [AHI] ⩾5), then for all possible AHI thresholds using the area under the receiver operating characteristics (AUROC) curve. Inter-rater reliability of PG was assessed using the kappa coefficient. Results Among 30 patients enrolled, seven were excluded for technical problems on PG or PSG and 23 were included in the main analysis. Eight out of 23 had an AHI ⩾5 on PSG. With the same threshold, sensitivity, specificity, positive and negative predictive values of PG for the diagnosis of SA were 87.5% (95% confidence interval: 47–99), 80% (52–96), 70% (35–93) and 92.3% (64–99) respectively. The kappa between PG raters was 0.75 (0.49–1.00) indicating good agreement. The AUROC was 0.93 (0.82–1.00). No association was found between sleep and excessive daytime sleepiness questionnaires and SA. Conclusions Portable PG seems to be valuable for ruling out SA in MSA.
    Full-text · Article · Apr 2014 · Sleep Medicine
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    • "However, the mechanism underlying nocturnal sudden death in patients with MSA remains unclear. It may be explained by upper-airway obstruction, such as vocal cord abductor paralysis; an impairment of the respiratory center, such as Cheyne-Stokes respiration; or an impaired hypoxemic ventilatory response [13]. It was reported by laryngeal electromyography that patients with multiple-system atrophy and autonomic failure can present unequivocal evidence of denervation of the posterior cricoarytenoid, partial denervation in the cricopharyngeal sphincter, in the interarytenoid, cricopharyngeal sphincter and may have respiratory obstruction requiring a tracheostomy, as it happened with our patient. "
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    ABSTRACT: Background. MSA (Multiple System Atrophy) may be associated either with Parkinsonism or with cerebellar ataxia (MSA-c subtype). It is considered a rare disease, but many patients are misdiagnosed as suffering from idiopathic Parkinson's disease. In this paper, we report a case of a patient admitted with respiratory failure and vocal cords paralysis due to MSA-c. Case Report. A 79-year-old Caucasian woman was admitted in March 2010 with dyspnea, asthenia, stridor, and respiratory failure needing noninvasive ventilation. She had orthostatic blood pressure decline, constipation, insomnia, daytime sleepiness, and snoring. The neurologic examination revealed cerebellar ataxia. A laryngoscopy revealed vocal cord paralysis in midline position and tracheostomy was performed. The Brain Magnetic Resonance Imaging revealed atrophy of middle cerebellar peduncles and pons with the "hot cross bun sign." Conclusion. Although Multiple-system atrophy is a rare disease, unexplained respiratory failure, bilateral vocal cord paralysis, or stridor should lead to consider MSA as diagnosis.
    Full-text · Article · Sep 2010 · Case Reports in Medicine
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