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多系统萎缩非运动型变体的病理学描述。

Pathological description of a non-motor variant of multiple system atrophy.

作者信息

Gaig C, Iranzo A, Tolosa E, Vilaseca I, Rey M J, Santamaria J

机构信息

Neurology Service, Hospital Clinic de Barcelona, C/Villarroel 170, Barcelona 08036, Spain.

出版信息

J Neurol Neurosurg Psychiatry. 2008 Dec;79(12):1399-400. doi: 10.1136/jnnp.2008.145276.

Abstract

Multiple system atrophy (MSA) is a neurodegenerative disorder that usually presents clinically as a combination of parkinsonism, cerebellar syndrome and autonomic failure. Patients with MSA can present other clinical features, such as inspiratory stridor and rapid eye movement (REM) sleep behaviour disorder (RBD). We report a patient with pathologically confirmed MSA who presented with a longstanding history of stridor, RBD and autonomic disturbances but did not develop overt parkinsonism or cerebellar signs. This case illustrates that MSA may present clinically without its cardinal motor symptoms, and that stridor and RBD may be clues to recognise the disease in a patient with autonomic failure.

摘要

多系统萎缩(MSA)是一种神经退行性疾病,临床上通常表现为帕金森综合征、小脑综合征和自主神经功能衰竭的组合。MSA患者可能出现其他临床特征,如吸气性喘鸣和快速眼动(REM)睡眠行为障碍(RBD)。我们报告一例经病理证实的MSA患者,该患者有长期的喘鸣、RBD和自主神经功能障碍病史,但未出现明显的帕金森综合征或小脑体征。该病例表明,MSA在临床上可能不出现其主要运动症状,喘鸣和RBD可能是识别自主神经功能衰竭患者中该疾病的线索。

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