Date: Monday, June 5, 2017
Session Title: Parkinsonism, MSA, PSP (Secondary and Parkinsonism-Plus)
Session Time: 1:45pm-3:15pm
Location: Exhibit Hall C
Objective: To elucidate the natural history of the PSP clinical phenotype
Background: Recently, various clinical types of PSP have been reported, but details on their natural course have not yet been evaluated.The JALPAC project is a Japanese longitudinal observational study of a cohort of patients with PSP and CBD that has, to date (12/11/2016), enrolled 152 participants from 41 institutions (UMIN18468). The most frequent clinical phenotype is Richardson’s syndrome (RS) (n = 54), followed by CBD/CBS (n = 37), PSP-parkinsonism (PSP-P) (n = 13), PSP-pure akinesia with gait freezing (PSP-PAGF) (n = 12), and PSP-cerebellar ataxia (PSP-C) (n = 9).
Methods: The following milestones were selected from the PSP rating scale items (the score level 2 or 3): falls, urinary incontinence, disorientation, dysarthria, dysphagia, voluntary saccades (upward and downward), gait, and postural stability. For each clinical phenotype, the interval from initial symptom onset to each milestone and total score of over 50 were investigated. Kaplan–Meier and log-rank tests were implemented for the comparisons.
Results: In PSP-P, intervals from the onset to all milestones were significantly longer than those in RS. In PSP-PAGF, intervals from the onset to milestones except dysarthria and postural stability were significantly longer than those in RS. Intervals from the onset to gait with assistance were significantly shorter in PSP-C than in RS. Intervals from the onset to total score of over 50 were significantly longer in PSP-P and PSP-PAGF than in RS.
Conclusions: PSP-P and PSP-PAGF showed a slower progression to many milestones, and PSP-C showed a more rapid worsening of mobility than RS. The number of patients, except for those with RS, was relatively small; hence, we continue enrolling participants in the JALPAC project.
References: JALPAC study group
To cite this abstract in AMA style:I. Aiba, T. Ikeuchi, H. Takigawa, T. Shimohata, T. Tokuda, M. Morita, O. Onodera, S. Murayama, K. Hasegawa, K. Nakashima. Progression of milestones by clinical types in progressive supranuclear palsy: a longitudinal observational study of a cohort of patients with PSP/CBD (the JALPAC project) [abstract]. Mov Disord. 2017; 32 (suppl 2). https://www.mdsabstracts.org/abstract/progression-of-milestones-by-clinical-types-in-progressive-supranuclear-palsy-a-longitudinal-observational-study-of-a-cohort-of-patients-with-pspcbd-the-jalpac-project/. Accessed December 5, 2023.
« Back to 2017 International Congress
MDS Abstracts - https://www.mdsabstracts.org/abstract/progression-of-milestones-by-clinical-types-in-progressive-supranuclear-palsy-a-longitudinal-observational-study-of-a-cohort-of-patients-with-pspcbd-the-jalpac-project/