突触核蛋白病
Synucleinopathies.
作者信息
Coon Elizabeth A, Singer Wolfgang
出版信息
Continuum (Minneap Minn). 2020 Feb;26(1):72-92. doi: 10.1212/CON.0000000000000819.
PURPOSE OF REVIEW
This article reviews the α-synucleinopathies pure autonomic failure, multiple system atrophy, dementia with Lewy bodies, and Parkinson disease with respect to autonomic failure.
RECENT FINDINGS
The pattern and severity of autonomic involvement in the synucleinopathies is related to differences in cellular deposition and neuronal populations affected by α-synuclein aggregation, which influences the degree and manifestation of autonomic failure. Clinical and laboratory autonomic features distinguish the different synucleinopathies based on pattern and severity. These features also determine which patients are at risk for evolution from pure autonomic failure to the synucleinopathies with prominent motor involvement, such as multiple system atrophy, dementia with Lewy bodies, or Parkinson disease.
SUMMARY
Autonomic failure is a key feature of the synucleinopathies, with varying type and degree of dysfunction from predominantly peripheral involvement in the Lewy body disorders to central involvement in multiple system atrophy.
综述目的
本文就自主神经功能衰竭方面，对α-突触核蛋白病（纯自主神经功能衰竭、多系统萎缩、路易体痴呆和帕金森病）进行综述。
最新发现
突触核蛋白病中自主神经受累的模式和严重程度与α-突触核蛋白聚集所影响的细胞沉积和神经元群体差异有关，这会影响自主神经功能衰竭的程度和表现。临床和实验室自主神经特征基于模式和严重程度区分不同的突触核蛋白病。这些特征还能确定哪些患者有从纯自主神经功能衰竭发展为伴有明显运动受累的突触核蛋白病的风险，如多系统萎缩、路易体痴呆或帕金森病。
总结
自主神经功能衰竭是突触核蛋白病的关键特征，功能障碍的类型和程度各不相同，从路易体疾病主要累及外周到多系统萎缩累及中枢。
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