Donker Kaat L, Boon A J W, Kamphorst W, Ravid R, Duivenvoorden H J, van Swieten J C
Department of Neurology, Erasmus Medical Center, Rotterdam, The Netherlands.
Neurology. 2007 Aug 21;69(8):723-9. doi: 10.1212/01.wnl.0000267643.24870.26.
Progressive supranuclear palsy (PSP) is a progressive hypokinetic rigid disorder with supranuclear gaze palsy and frequent falls. Although clinical consensus criteria are available, an atypical presentation may lead to clinical misdiagnosis in the initial phase. In the present study we investigated the clinical presentation of PSP and its relationship to initial clinical diagnosis and survival.
We ascertained patients with PSP in a prospective cohort by nationwide referral from neurologists and nursing home physicians. All patients underwent a structural interview and clinical examination before entering the study. Medical records were reviewed for the presence of symptoms during the first 2 years.
A total of 152 patients ascertained between 2002 and 2005 fulfilled the international consensus criteria for PSP. Categorical principal component analysis of clinical symptoms within the first 2 years showed apart from a cluster of typical PSP symptoms, the clustering of cognitive dysfunction and behavioral changes. Further analysis showed that 20% of patients had a predominant frontal presentation with less than two other PSP symptoms. Survival analysis showed that this subgroup had a similar prognosis to that of the total group of patients with PSP.
There exists a subgroup of patients with progressive supranuclear palsy (PSP) with a predominant frontal presentation, who progressed into typical PSP over the course of the disease.
进行性核上性麻痹（PSP）是一种伴有核上性凝视麻痹和频繁跌倒的进行性运动减少性僵硬疾病。尽管有临床共识标准，但非典型表现可能导致疾病初期的临床误诊。在本研究中，我们调查了PSP的临床表现及其与初始临床诊断和生存的关系。
我们通过神经病学家和疗养院医生的全国转诊，在前瞻性队列中确定PSP患者。所有患者在进入研究前均接受了结构化访谈和临床检查。回顾医疗记录以了解前两年内症状的存在情况。
2002年至2005年间确定的152例患者符合PSP的国际共识标准。对前两年内临床症状进行的分类主成分分析显示，除了一组典型的PSP症状外，还存在认知功能障碍和行为改变的聚类。进一步分析表明，20%的患者以额叶表现为主，伴有少于两种其他PSP症状。生存分析表明，该亚组的预后与PSP患者总群体相似。
存在一组以额叶表现为主的进行性核上性麻痹（PSP）患者，他们在疾病过程中进展为典型的PSP。
