Mahale Rohan R, Krishnan Syam, Divya K P, Jisha V T, Kishore Asha
Department of Neurology, Sree Chitra Tirunal Institute of Medical Sciences and Technology (SCTIMST), Trivandrum, Kerala, India.
Achutha Menon Centre for Health Science Studies (AMCHSS), Sree Chitra Tirunal Institute of Medical Sciences and Technology (SCTIMST), Trivandrum, Kerala, India.
Ann Indian Acad Neurol. 2021 Jan-Feb;24(1):56-62. doi: 10.4103/aian.AIAN_611_20. Epub 2021 Jan 19.
Progressive supranuclear palsy (PSP) is a clinically heterogeneous disease characterized by supranuclear gaze palsy and varying combinations of Parkinsonism, gait disturbances, postural instability, and fronto-limbic cognitive dysfunction. A major challenge in clinical diagnosis is the existence of subtypes whose clinical features overlap with those of other Parkinsonian disorders.
To categorize patients of PSP into its using the recently proposed movement disorder society criteria (2017) and to determine the prognosis of the PSP subtypes.
Demographic and clinical data of patients diagnosed with PSP over a 21 year period were collected by review of medical records and categorized into its subtypes. Subtype prognosis was assessed from the interval between disease onset and attainment of the first of 5 clinical disability milestones namely wheelchair dependency, unintelligible speech, severe dysphagia, severe cognitive impairment, and urinary catheterization.
When categorized into subtypes, out of the 334 patients with PSP, PSP-RS predominated (72%), followed by PSP-parkinsonism (PSP-P) (13.5%), PSP-corticobasal syndrome (PSP-CBS) (5.1%), PSP-frontal (PSP-F) (4.2%), PSP-progressive gait freezing (PSP-PGF) (4.2%), PSP-postural instability (PSP-PI) (0.6%), and PSP-speech/language (PSP-SL) (0.3%). PSP-P reaches the milestones of wheelchair dependency, unintelligible speech, and dysphagia later than other subtypes.
PSP-RS was the commonest and PSP-OM the rarest PSP subtype in our retrospective PSP cohort analysis. PSP-P had a better prognosis than all other subtypes of PSP. A large proportion of these cases would remain unclassified using NINDS-SPSP (1996) criteria.
进行性核上性麻痹（PSP）是一种临床异质性疾病，其特征为核上性凝视麻痹以及帕金森综合征、步态障碍、姿势不稳和额颞叶认知功能障碍的不同组合。临床诊断中的一个主要挑战是存在一些亚型，其临床特征与其他帕金森病性疾病重叠。
根据最近提出的运动障碍协会标准（2017年）对PSP患者进行分类，并确定PSP各亚型的预后。
通过查阅病历收集21年间诊断为PSP的患者的人口统计学和临床数据，并将其分类为各亚型。从疾病发作到达到5个临床残疾里程碑中的第一个里程碑（即轮椅依赖、言语不清、严重吞咽困难、严重认知障碍和导尿）的时间间隔来评估亚型预后。
在分为各亚型的334例PSP患者中，PSP-RS占主导（72%），其次是PSP-帕金森综合征（PSP-P）（13.5%）、PSP-皮质基底节综合征（PSP-CBS）（5.1%）、PSP-额叶型（PSP-F）（4.2%）、PSP-进行性步态冻结（PSP-PGF）（4.2%）、PSP-姿势不稳（PSP-PI）（0.6%）和PSP-言语/语言型（PSP-SL）（0.3%）。PSP-P比其他亚型更晚达到轮椅依赖、言语不清和吞咽困难的里程碑。
在我们的回顾性PSP队列分析中，PSP-RS是最常见的PSP亚型，而PSP-OM是最罕见的亚型。PSP-P的预后优于PSP的所有其他亚型。使用美国国立神经疾病与中风研究所-PSP（1996年）标准，这些病例中有很大一部分将无法分类。
