Osaki Yasushi, Morita Yukari, Miyamoto Yuka, Furuta Kounosuke, Furuya Hirokazu
Department of Neurology Kochi Medical School Hospital Kochi Japan.
Department of Emergency Medicine Kochi Medical School Hospital Kochi Japan.
Neurol Clin Neurosci. 2017 May;5(3):86-90. doi: 10.1111/ncn3.12122. Epub 2017 Apr 17.
Early clinical diagnosis of progressive supranuclear palsy (PSP) remains challenging.
We attempted to identify any sign or symptom to diagnose PSP earlier.
A total of 401 patients, 40 with PSP and 361 with other neurodegenerative disorders, were included. We followed these patients for at least 1 year since 2009. We reviewed the signs and symptoms of patients with PSP in a standardized manner, and observed four manifestations: "vertical supranuclear gaze abnormality," "movement disorders," "pseudobulbar palsy" and "dementia of frontal type." Features, such as symmetric parkinsonism, freezing of gait, postural instability, dysarthria and/or dysphagia, or dementia of frontal type, were considered core clinical features.
In patients with PSP, "movement disorders" was the most common manifestation, whereas "vertical supranuclear gaze abnormality" was uncommon during the early disease course. A total of 16 patients fulfilled the National Institute for Neurological Disorders and Stroke and Society for PSP criteria for possible PSP at their first clinic visit. Of the remaining 24 patients, 15 presented with one or more core clinical features before fulfilling the criteria for possible PSP; nine patients had a clinical diagnosis of PSP but never fulfilled the criteria. A total of 49 of the 361 patients with other neurodegenerative disorders had core clinical features. A comparison showed that freezing of gait differentiated the groups the best over the disease course.
Freezing of gait is an early feature that might improve the clinical diagnosis of PSP, whereas vertical supranuclear gaze abnormality is not.
进行性核上性麻痹（PSP）的早期临床诊断仍然具有挑战性。
我们试图确定任何能够更早诊断PSP的体征或症状。
共纳入401例患者，其中40例患有PSP，361例患有其他神经退行性疾病。自2009年以来，我们对这些患者进行了至少1年的随访。我们以标准化方式回顾了PSP患者的体征和症状，并观察到四种表现：“垂直核上性凝视异常”、“运动障碍”、“假性球麻痹”和“额叶型痴呆”。诸如对称性帕金森症、步态冻结、姿势不稳、构音障碍和/或吞咽困难，或额叶型痴呆等特征被视为核心临床特征。
在PSP患者中，“运动障碍”是最常见的表现，而“垂直核上性凝视异常”在疾病早期并不常见。共有16例患者在首次就诊时符合美国国立神经疾病与中风研究所及PSP协会制定的可能PSP标准。在其余24例患者中，15例在符合可能PSP标准之前出现了一种或多种核心临床特征；9例患者临床诊断为PSP，但从未符合标准。361例患有其他神经退行性疾病的患者中共有49例具有核心临床特征。比较显示，在整个疾病过程中，步态冻结对区分这些组的效果最佳。
步态冻结是一个早期特征，可能有助于改善PSP的临床诊断，而垂直核上性凝视异常则不然。
