Frequency of nocturnal sudden death in patients with multiple system atrophy

Shimohata, Takayoshi; Ozawa, Tetsutaro; Nakayama, Hideaki; Tomita, Masahiko; Shinoda, Hideo; Nishizawa, Masatoyo

doi:10.1007/s00415-008-0941-4

Cited by 69 publications

(50 citation statements)

References 14 publications

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“…Because patients with food stagnation within the esophagus did not necessarily report subjective symptoms other than dysphagia, evaluation of the esophageal phase on videofluorography is necessary. We previously demonstrated that patients with MSA frequently died suddenly during sleep and that upper airway obstruction might not fully explain the mechanism of sudden death [13].…”

Section: Discussionmentioning

confidence: 99%

Esophageal Involvement in Multiple System Atrophy

et al. 2015

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The prevalence of esophageal involvement and its impact on clinical manifestations in patients with multiple system atrophy (MSA) remains unknown. We recruited 16 consecutive patients with dysphagia associated with MSA (MSA group) and 16 consecutive patients with dysphagia associated with amyotrophic lateral sclerosis (ALS group). We assessed the presence or absence of food stagnation within the esophagus using videofluorography. Food stagnation within the esophagus was observed in 16 patients (100 %; 7 severe, 9 mild) in the MSA group and in 4 patients (25 %; 4 mild) in the ALS group (P < 0.001). Follow-up videofluorography revealed that food stagnation in patients with MSA could exacerbate during the disease course. Patients with MSA and severe food stagnation showed a wide range of intraesophageal stasis by videofluorography. Among the 16 patients in the MSA group, 4 developed aspiration pneumonia and 1 died of suffocation associated with food regurgitation during continuous positive airway pressure therapy. In conclusion, food stagnation within the esophagus occurs more frequently in MSA patients with dysphagia than in ALS patients with dysphagia. Because food stagnation can cause serious complications such as aspiration pneumonia and suffocation, patients with MSA should be evaluated by videofluorography, especially those with stagnation in the esophageal phase.

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Section: Discussionmentioning

confidence: 99%

Esophageal Involvement in Multiple System Atrophy

et al. 2015

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“…The high frequency (71 %) of floppy epiglottis in MSA reported in this study could suggest that MSA patients are prone to present this type of epiglottic abnormality, probably related to an abnormal laryngeal tone. Since it has been reported that MSA patients could experience sudden death despite CPAP use [55], this study raises the possibility that downward displacement of the epiglottis by CPAP could cause upper airway obstruction and thus result in choking to death. However, floppy epiglottis should be not only observed under sedation with propofol, but also during physiologic sleep.…”

Section: Obstructive Apneamentioning

confidence: 87%

“…In MSA, tridor during wakefulness is a particularly harmful condition that can lead to subacute episodes of dramatic respiratory failure and death [52,Class III]. Also the presence of stridor during sleep is considered a life-threatening condition since it has been associated with decreased survival and higher rates of sudden death during sleep [54,Class III,55].…”

Section: Sleep-related Breathing Disorders Stridormentioning

confidence: 99%

Sleep Dysfunction in Multiple System Atrophy

Ferini‐Strambi

Marelli

2012

Curr Treat Options Neurol

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Sleep disorders in multiple system atrophy (MSA) are common manifestation and include reduced and fragmented sleep, excessive daytime sleepiness, REM sleep behaviour disorder (RBD), and sleep-disordered breathing. Of these, RBD is the most common (affecting 90 %-100 % of patients with MSA) and is regarded as a red flag for MSA. RBD, as well as stridor during sleep, may be the initial manifestation of the disease, occurring several years before the waking motor and dysautonomic onset. Sleep disorders occur in both MSA with predominant parkinsonism (MSA-P) and MSA with predominant cerebellar ataxia (MSA-C). Treatment strategies in patients with MSA presenting difficulties in initiating and maintaining sleep need to be highly individualized. Clonazepam has been found to be successful in treating RBD symptoms at the dose of 0.25 to 2.0 mg given approximately 30 min before bedtime. In case of comorbid obstructive sleep apnea, zopiclone (at the dose from 3.75 to 7.5 mg each night) or melatonin (with a recommended dose of 3 to 12 mg at bedtime) may be alternative treatments. An increased survival in MSA patients with stridor may be obtained both with continuous positive airway pressure (CPAP) and tracheostomy. Since tracheostomy is an invasive surgical procedure, not easily accepted by the patient, CPAP therapy should be considered first. However, tracheostomy is first indicated when stridor is present during wakefulness because of the high risk of respiratory failure and death. In MSA, obstructive sleep apnea (OSA) occurs more frequently than central sleep apnea, ranging from 15 % to 37 % of the cases. CPAP is an effective treatment for eliminating obstructive sleep apnea in MSA patients, even if the adaptation to the device may be difficult in advanced cases.

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“…Treatment, such as continuous positive airway pressure therapy or a tracheotomy, is effective. However, these treatments do not always prevent sudden death in patients with MSA, suggesting a mechanism such as central hypoventilation, other than upper airway obstruction, may play a role (Shimohata et al, 2008 …”

Section: Sleep Apnea Syndrome (Sas)mentioning

confidence: 99%