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[多系统萎缩的预后——有无气管切开术的生存时间]

[Prognosis of multiple system atrophy--survival time with or without tracheostomy].

作者信息

Kurisaki H

机构信息

Department of Neurology, National Tokyo Hospital.

出版信息

Rinsho Shinkeigaku. 1999 May;39(5):503-7.

Abstract

Prognosis of 21 patients with multiple system atrophy (MSA) who deceased or received tracheostomy is described. The percentage of patients with MSA among the cases of spinocerebellar degeneration was 40% in National Tokyo Hospital. There were 12 women and 9 men, and the mean age at onset was 56 years. Seventy-four percent of MSA patients was olivopontocerebellar atrophy (OPCA), 22% was striatonigral degeneration (SND). The mean age of 17 deceased patients (10 women, and 7 men) was 65.5 years. Ten patients did not undergo tracheostomy and deceased, and 11 patients underwent tracheostomy, among whom 4 patients are still alive. Mean duration of illness from onset to death (without tracheostomy) or tracheostomy was 6.8 years. Cause of death of patients who did not undergo tracheostomy was related to paresis of the larynx or pharynx, for example, aspiration pneumonia due to dysphagia, vocal cord paralysis and sudden death. Some of those who underwent tracheostomy deceased for causes which were not directly related to MSA such as cerebral hemorrhage or uremia, but others seem to be related to some problems of respiratory center such as central chronic respiratory failure, or sudden death (sometimes it happened after infection, but the obstruction of the respiratory tract was not always present at autopsy).

摘要

本文描述了21例多系统萎缩(MSA)患者的预后情况,这些患者已死亡或接受了气管切开术。在东京国立医院的脊髓小脑变性病例中,MSA患者的比例为40%。患者中有12名女性和9名男性,平均发病年龄为56岁。74%的MSA患者为橄榄体脑桥小脑萎缩(OPCA),22%为纹状体黑质变性(SND)。17例死亡患者(10名女性和7名男性)的平均年龄为65.5岁。10例患者未接受气管切开术并死亡,11例患者接受了气管切开术,其中4例仍存活。从发病到死亡(未行气管切开术)或气管切开术的平均病程为6.8年。未接受气管切开术的患者的死因与喉或咽麻痹有关,例如吞咽困难导致的吸入性肺炎、声带麻痹和猝死。一些接受气管切开术的患者死于与MSA无直接关系的原因,如脑出血或尿毒症,但其他患者似乎与呼吸中枢的一些问题有关,如中枢性慢性呼吸衰竭或猝死(有时发生在感染后,但尸检时呼吸道梗阻并不总是存在)。

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