Multiple System Atrophy
Multiple System Atrophy
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DOI：
10.1007/978-3-030-62263-3_23
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发表时间：
2021
期刊：
影响因子：
--
通讯作者：
R. Vetrugno
中科院分区：
文献类型：
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作者：
R. Vetrugno
多系统萎缩(MSA)是一种进行性和衰弱的神经退行性疾病，以帕金森病、小脑性共济失调和自主神经衰竭为临床特征，影响纹状体、橄榄桥小脑和自主神经系统。MSA的主要病理特征是少突胶质细胞中存在胶质细胞质内含物(GCI)。GCI主要由α-突触核蛋白聚集体组成，因此被认为是一种α-突触核病症，与帕金森病和路易体痴呆并存。睡眠障碍发生在以帕金森综合征为主的MSA(MSA-P)和以小脑性共济失调(MSA-C)为主的MSA。其中，RBD是最常见的，影响了90%-100%的MSA患者，并被视为在近一半的患者中提前几年出现觉醒运动症状和自主神经衰竭的危险信号。SDB表现为中枢性低通气量，反映继发于桥延髓呼吸中枢退行性变的呼吸自控功能受损，更常见的表现为喘鸣和阻塞性睡眠呼吸暂停，继发于喉部狭窄，继发于联合声带外展肌麻痹和吸气时过度内收肌激活。夜间响声是MSA患者的一种危及生命的情况，与呼吸衰竭和睡眠中猝死有关。治疗主要针对帕金森症和自主神经衰竭。此外，表现为睡眠障碍的MSA患者的治疗策略需要高度个体化。目前还没有有效的神经保护疗法。
Multiple system atrophy (MSA) is a progressive and debilitating neurodegenerative disease characterized by the clinical triad of parkinsonism, cerebellar ataxia, and autonomic failure, impacting on striatonigral, olivopontocerebellar, and autonomic systems. The key pathological hallmark of MSA is the presence of glial cytoplasmic inclusions (GCI) in oligodendrocytes. GCI comprise insoluble proteinaceous filaments composed chiefly of α-synuclein aggregates and, therefore, MSA is regarded as an α-synucleinopathy along with Parkinson’s disease (PD) and dementia with Lewy bodies.Sleep disorders are common in MSA and include reduced and fragmented sleep, excessive daytime sleepiness, REM sleep behavior disorder (RBD), and sleep-disordered breathing (SDB). Sleep disorders occur in both MSA with predominant parkinsonism (MSA-P) and MSA with predominant cerebellar ataxia (MSA-C). Of these, RBD is the most common affecting 90–100% of patients with MSA and is regarded as a red flag preceding in near half of the patients the onset of waking motor symptoms and autonomic failure by several years. SDB manifests as central hypoventilation that reflects impaired automatic control of ventilation secondary to degeneration of the pontomedullary respiratory center and more commonly as stridor and obstructive sleep apnea due to larynx narrowing secondary to combined vocal cord abductor paralysis and excessive adductor activation during inspiration. Nocturnal stridor is a life-threatening condition in MSA associated with respiratory failure and sudden death during sleep.Therapy mainly targets parkinsonism and autonomic failure. Moreover, treatment strategies in patients with MSA presenting with sleep disorders need to be highly individualized. No effective neuroprotective therapy is available.