Mechanisms and prevention of sudden death in multiple system atrophy

Shimohata, Takayoshi; Aizawa, Naotaka; Nakayama, Hideaki; Taniguchi, Hisanori; Ohshima, Yasuyoshi; Okumura, Hitoshi; Yokoseki, Akio; Inoue, Makoto; Nishizawa, Masatoyo

doi:10.1016/j.parkreldis.2016.04.011

Cited by 37 publications

(30 citation statements)

References 45 publications

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“…progressive neurodegenerative disease and can affect esophageal function variably with disease progression. Sudden death is the most serious consequence of MSA 18 and has been reported to be the second most common cause of death in MSA. 2 Respiratory disorders frequently occur during sleep, sometimes resulting in sleep apnea and possibly sudden death in patients with MSA.…”

Section: Discussionmentioning

confidence: 99%

Esophageal Dysmotility is Common in Patients With Multiple System Atrophy

et al. 2020

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Objectives: Esophageal dysmotility (ED) in patients with multiple system atrophy (MSA) are poorly understood. This study aimed to investigate the prevalence of ED in patients with MSA and to assess the relationship of esophageal abnormalities with other clinical findings and characteristics in these patients. Methods: A retrospective chart review was conducted to identify patients with MSA and to compare them to the elderly controls without MSA (65+ years) who underwent a videofluorographic esophagram from 2014 to 2019. Disease type, disease severity, vocal fold mobility impairment, abnormal deglutitive proximal esophageal contraction (ADPEC), and intra-esophageal stasis (IES) were reviewed and compared between groups. Results: Thirty-seven patients with MSA were identified. The median age was 63 and 26 (70%) were male. These patients were matched to 22 elderly adults with presbylarynx but not MSA (median age 77, 68% male). In MSA patients, cerebellar variant type was predominant (59%), and ADPEC was recognized in 18 patients (49%). Disease severity level (P = 0.028) and existence of IES (P = 0.046) were associated with higher risks of developing ADPEC. The prevalence of IES was significantly higher in patients with MSA (95%) compared to controls without MSA (46%) (P < 0.001). Disease severity level and the existence of IES were significantly associated with the presence of ADPEC (p < 0.05). Conclusion: ADPEC and IES were significantly more common in MSA than in elderly subjects without MSA. MSA severity is associated with the development of ADPEC. The data suggest that esophageal motility is predominantly affected in MSA.

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Section: Discussionmentioning

confidence: 99%

Esophageal Dysmotility is Common in Patients With Multiple System Atrophy

et al. 2020

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“…Obstructive SA is more frequent than is central SA, occurring in up to 40% of patients with MSA [ 19 ]. Sudden death is not uncommon in patients with MSA and usually occurs during sleep [ 2 , 20 ], but it is unclear whether the sudden death results from VFMI and/or central hypoventilation or from SA or other causes. Considering that obstructive SA can be partly caused by laryngeal constriction, including VFMI, based on our findings, vocal fold movement should be inspected, especially in patients with MSA-P.…”

Section: Discussionmentioning

confidence: 99%

Heightened risk of early vocal fold motion impairment onset and dysphagia in the parkinsonian variant of multiple system atrophy: a comparative study

Tsuchiya

Ueha

Suzuki

et al. 2020

Clinical Parkinsonism & Related Disorders

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Objective We compared differences in frequency and timing of onset of the following clinical events between the cerebellar and parkinsonian variants of multiple system atrophy (MSA-C and MSA-P, respectively): type of operation including tracheostomy and/or aspiration prevention surgery, vocal fold motion impairment (VFMI), sleep apnea (SA), introduction of mechanical ventilation (MV), and dysphagia. The risks of these events cooccurring with either MSA-C or MSA-P were compared. Methods We retrospectively assessed clinical outcomes only of patients with MSA who presented at the Department of Otolaryngology of the University of Tokyo Hospital between 2008 and 2018. The proportion and timing of onset events between MSA-C and MSA-P and risks of onset were compared using chi-square tests and Cox proportional hazard models adjusted for age, sex, and disease severity, respectively. Results We identified 113 patients (median age: 60 years, 72 men [64%]). The frequency and timing of VFMI, SA, MV, dysphagia, and surgeries were 55 patients (49%) and 76 (95% CI 61–91) months after MSA onset, 85 (75%) and 41 (32–50), 36 (32%) and 100 (73–127), 77 (68%) and 43 (36–50), and 25 (22%) and 102 (84–120), respectively. Twenty-seven patients (24%) had MSA-P and higher risk of VFMI ( p < .001), SA ( p = .030), and dysphagia ( p = .017) than did patients with MSA-C. Conclusion While MSA-P is less common, it may involve heightened risk of VFMI and dysphagia early onset. Thus, careful follow-up for VFMI, SA, and dysphagia may be needed for these patients. Criteria for Rating Diagnostic Accuracy Studies Class II.

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“…Drooling is linked to bradykinesia/hypokinesia involving the oral preparatory and propulsive phases of swallowing but pharyngeal/orofacial dystonia may play an additional role [3,11,30]. Changes in posture during oral food intake, changes in eating habits (e.g., avoidance of a particular food consistency), unusual prolongation of mealtimes and weight loss are also signs that deserve clinical attention [11,36]. Frequent fever of unexplained origin, coughing, bronchitis and pneumonia may be investigated as possible consequences of aspiration.…”

Section: Tablementioning

confidence: 99%

Dysphagia in multiple system atrophy consensus statement on diagnosis, prognosis and treatment

Calandra‐Buonaura

Alfonsi

Vignatelli

et al. 2021

Parkinsonism & Related Disorders

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Multiple system atrophy (MSA) is a neurodegenerative disorder characterized by a combination of autonomic failure plus cerebellar syndrome and/or parkinsonism. Dysphagia is a frequent and disabling symptom in MSA and its occurrence within 5 years of motor onset is an additional diagnostic feature. Dysphagia can lead to aspiration pneumonia, a recognized cause of death in MSA. Guidelines for diagnosis and management of dysphagia in MSA are lacking. An International Consensus Conference among experts with methodological support was convened in Bologna to reach consensus statements for the diagnosis, prognosis, and treatment of dysphagia in MSA. Abnormalities of the oral and pharyngeal phases of swallowing, esophageal dysfunction and aspiration occur in MSA and worsen as the disease progresses. According to the consensus, dysphagia should be investigated through available screening questionnaires and clinical and instrumental assessment (videofluoroscopic study or fiberoptic endoscopic evaluation of swallowing and manometry) at the time of MSA diagnosis and periodically thereafter. There is evidence that dysphagia is associated with poor survival in MSA, however effective treatments for dysphagia are lacking. Compensatory strategies like diet modification, swallowing maneuvers and head postures should be applied and botulinum toxin injection may be effective in specific conditions. Percutaneous endoscopic gastrostomy may be performed when there is a severe risk of malnutrition and pulmonary complications, but its impact on survival is undetermined. Several research gaps and unmet needs for research involving diagnosis, prognosis, and treatment were identified.

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Mechanisms and prevention of sudden death in multiple system atrophy

Cited by 37 publications

References 45 publications

Esophageal Dysmotility is Common in Patients With Multiple System Atrophy

Esophageal Dysmotility is Common in Patients With Multiple System Atrophy

Heightened risk of early vocal fold motion impairment onset and dysphagia in the parkinsonian variant of multiple system atrophy: a comparative study

Dysphagia in multiple system atrophy consensus statement on diagnosis, prognosis and treatment

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