O'Sullivan S S, Massey L A, Williams D R, Silveira-Moriyama L, Kempster P A, Holton J L, Revesz T, Lees A J
Reta Lila Weston Institute of Neurological Studies, University College London, London, UK.
Brain. 2008 May;131(Pt 5):1362-72. doi: 10.1093/brain/awn065. Epub 2008 Apr 2.
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 'Richardson's 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.
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明确。基于早期临床特征，已提出这两种疾病的亚型。我们进行了一项回顾性病历审查，以研究经病理证实的PSP和MSA病例的自然病史。确定了生存数据以及几个临床相关的关键节点，即：频繁跌倒、认知障碍、言语不清、严重吞咽困难、依赖轮椅行动、使用导尿管以及入住疗养院。根据早期症状，我们将PSP病例细分为“理查森综合征”（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病例中，69例（62.7%）被归类为RS，29例（26.4%）为PSP-P。83例MSA病例中，42例（53.2%）在疾病发作后2年内出现自主神经功能衰竭。PSP患者发病年龄较大（P < 0.001），但病程与MSA患者相似。PSP患者比MSA患者更早达到首个临床关键节点（P < 0.001）。PSP患者中，经常跌倒（P < 0.001）、言语不清（P = 0.04）和认知障碍（P = 0.03）也比MSA患者出现得更早。在PSP中，RS表型、男性、发病年龄较大以及从疾病发作到达到首个临床关键节点的时间间隔较短，都是疾病持续时间至死亡较短的独立预测因素。与PSP-P患者相比，RS患者从疾病发作到达到临床关键节点的时间间隔也较短。在MSA中，早期自主神经功能衰竭、女性、发病年龄较大、从疾病发作到达到首个临床关键节点的时间间隔较短以及未入住疗养院是预测疾病持续时间至死亡较短的独立因素。达到首个临床关键节点的时间是生存的一个有用的预后预测指标。我们证实，RS的病程比PSP-P更差，并且MSA中的早期自主神经功能衰竭与较短的生存期相关。
