Baseline demographics and clinical data. The Progressive Supranuclear Palsy Rating Scale (PSPRS), Montreal Cognitive Assessment (MoCA), Mini-Mental State Examination (MMSE), and Clinical Dementia Rating (CDR) scores are presented as Mean ± SD, with the percent of the group tested in parenthesis.
Total PSPRS Clinical Score
The Progressive Supranuclear Palsy Rating Scale (PSPRS) data is plotted for the individual CBD (green dots), PSP (orange dots), and healthy control subjects (the black line indicates the group mean). Statistical significance was assessed by one-way ANOVA and Tukey's test for multiple comparisons. *** p<0.001; **** p<0.0001
Data Sources
4-Repeat Tauopathy Neuroimaging Initiative (4RTNI) and Frontotemporal Lobar Degeneration Neuroimaging Initiative (FTLDNI)
Data used in the preparation of this presentation were obtained from the 4-Repeat Tauopathy Neuroimaging Initiative (4RTNI) database and the Frontotemporal Lobar Degeneration Neuroimaging Initiative (FTLDNI). 4RTNI was launched in early 2011 and is funded through the National Institute of Aging and The Tau Research Consortium. The primary goal of 4RTNI is to identify neuroimaging and biomarker indicators for disease progression in the 4-repeat tauopathy neurodegenerative diseases, progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD). FTLDNI is also founded through the National Institute of Aging, and started in 2010. The primary goals of FTLDNI are to identify neuroimaging modalities and methods of analysis for tracking frontotemporal lobar degeneration (FTLD) and to assess the value of imaging versus other biomarkers in diagnostic roles. The Principal Investigator of 4RTNI is Dr. Adam Boxer, MD, PhD, at the University of California, San Francisco. The data is the result of collaborative efforts at four sites in North America. For more information on 4RTNI, please visit: 4RTNI Study. The Principal Investigator of NIFD is Dr. Howard Rosen, MD at the University of California, San Francisco. The data is the result of collaborative efforts at three sites in North America. For up-to-date information on participation and protocol, please visit: NIFD Study.
Data collection and sharing for this project was funded by the 4-Repeat Tauopathy Neuroimaging Initiative (4RTNI) (National Institutes of Health Grant R01 AG038791) and through generous contributions from the Tau Research Consortium, and by the Frontotemporal Lobar Degeneration Neuroimaging Initiative (National Institutes of Health Grant R01 AG032306). The study is coordinated through the University of California, San Francisco, Memory and Aging Center. 4RTNI and FTLDNI data are disseminated by the Laboratory for Neuro Imaging at the University of Southern California.
The CBD data used for analysis was obtained from the 4-Repeat Tauopathy Neuroimaging Initiative (4RTNI) database, while the cognitively normal healthy control (HC) subject data was obtained from the Frontotemporal Lobar Degeneration Neuroimaging Initiative (FTLDNI), which is a companion study to 4RTNI. A primary goal of these studies is to identify neuroimaging and biomarker indicators of disease progression in 4-repeat tauopathy neurodegenerative diseases.
As outlined in the Table, 48 CBD participants were included in the analysis, compared to a set of 117 healthy controls. All subjects were evaluated by MRI at baseline, 6 month follow-up, and 12 month follow-up visits, with clinical rating scales assessed at similar intervals. Participants in the CBD group have higher PSPRS & CDR scores and lower MMSE & MoCA scores compared to the healthy control participants. While the total PSPRS clinical scores for the PSP and CBD subjects within the 4RTNI study are substantially higher than controls, there is also a significant difference in the total PSPRS between the disease groups, with CBD subjects having significantly lower PSPRS scores as illustrated in the bar graph. This reduction in total PSPRS is primarily contributed by the PSPRS-Bulbar subscore in CBD (Brittain, 2019). The healthy controls had an MMSE score of >29, a CDR score of almost 0, and no subjective cognitive complaints. To avoid bias, only images from control subjects obtained with a similar scanning acquisition protocol were included in our analysis.