Clinical outcomes of progressive supranuclear palsy and multiple system atrophy
Clinical outcomes of progressive supranuclear palsy and multiple system atrophy
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DOI：
10.1093/brain/awn065
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发表时间：
2008-05-01
期刊：
影响因子：
14.5
通讯作者：
Lees, A. J.
中科院分区：
文献类型：
--
作者：
O'Sullivan, S. S.;Massey, L. A.;Lees, A. J.
进行性核上性麻痹（PSP）和多系统萎缩（MSA）的预后预测因素尚未确定。这两种疾病的亚型已经提出了早期临床特征的基础上。我们进行了一项回顾性病历审查，以调查病理证实的PSP和MSA病例的自然史。确定了生存数据和几个临床相关的里程碑，即：频繁跌倒、认知障碍、言语不清、严重吞咽困难、行动依赖轮椅、导尿管的使用和住院治疗。根据早期症状，我们将PSP分为Richardson综合征（RS）和PSP-帕金森综合征（PSP-P）。MSA病例根据是否存在早期自主神经功能衰竭进行细分。110例PSP中69例（62.7）被归类为RS，29例（26.4）被归类为PSP-P。83例MSA中，42例（53.2）在发病2年内出现自主神经功能衰竭。PSP的发病年龄较MSA大（P < 0.001），但病程与MSA相似。PSP患者达到第一个临床里程碑的时间早于MSA患者（P < 0.001）。PSP组较MSA组更早出现规则性福尔斯跌倒（P < 0.001）、言语不清（P = 0.04）和认知功能障碍（P = 0.03）。在PSP中，RS表型、男性、发病年龄较大和从疾病发作到达到第一个临床里程碑的时间间隔较短都是疾病持续时间较短至死亡的独立预测因素。与PSP-P患者相比，RS患者在疾病发作后较短的时间间隔内也达到了临床里程碑。在MSA早期自主神经功能衰竭中，女性性别，发病年龄较大，从疾病发作到达到第一个临床里程碑的时间间隔较短，并且未接受住院治疗是预测疾病持续时间较短直至死亡的独立因素。至第一个临床里程碑的时间是一个有用的生存预后预测因子。我们证实RS的病程不如PSP-P，MSA患者早期自主神经功能衰竭与生存期缩短相关。
Prognostic predictors have not been defined for progressive supranuclear palsy (PSP) and multiple system atrophy (MSA). Subtypes of both disorders have been proposed on the basis of early clinical features. We performed a retrospective chart review to investigate the natural history of pathologically confirmed cases of PSP and MSA. Survival data and several clinically relevant milestones, namely: frequent falling, cognitive disability, unintelligible speech, severe dysphagia, dependence on wheelchair for mobility, the use of urinary catheters and placement in residential care were determined. On the basis of early symptoms, we subdivided cases with PSP into Richardsons syndrome (RS) and PSP-parkinsonism (PSP-P). Cases of MSA were subdivided according to the presence or absence of early autonomic failure. Sixty-nine (62.7) of the 110 PSP cases were classified as RS and 29 (26.4) as PSP-P. Of the 83 cases of MSA, 42 (53.2) had autonomic failure within 2 years of disease onset. Patients with PSP had an older age of onset (P < 0.001), but similar disease duration to those with MSA. Patients with PSP reached their first clinical milestone earlier than patients with MSA (P < 0.001). Regular falls (P < 0.001), unintelligible speech (P = 0.04) and cognitive impairment (P = 0.03) also occurred earlier in PSP than in MSA. In PSP an RS phenotype, male gender, older age of onset and a short interval from disease onset to reaching the first clinical milestone were all independent predictors of shorter disease duration to death. Patients with RS also reached clinical milestones after a shorter interval from disease onset, compared to patients with PSP-P. In MSA early autonomic failure, female gender, older age of onset, a short interval from disease onset to reaching the first clinical milestone and not being admitted to residential care were independent factors predicting shorter disease duration until death. The time to the first clinical milestone is a useful prognostic predictor for survival. We confirm that RS had a less favourable course than PSP-P, and that early autonomic failure in MSA is associated with shorter survival.