Severity dependent distribution of impairments in PSP and CBS: Interactive Visualizations

Autor: Brittain, Claire, McCarthy, Andrew, investigators, PROPSPERA, Tolosa, E., Buongiorno, M. T., Bargalló, N., Burguera, J. A., Martinez, I., Ruiz-Mart'ınez, J., Narrativel, I., Vivancos, F., Ybot, I., Aguilar, M., 4RNTI-1authors, Quilez, P., Boada, M., Lafuente, A., Hernandez, I., López-Lozano, J. J., Mata, M., Kupsch, A., Lipp, A., Ebersbach, G., Schmidt, T., PSP, Tau Restoration on, Hahn, K., Höllerhage, M., Oertel, W. H., Respondek, G., Stamelou, M., Reichmann, H., Wolz, M., Schneider, C., Klingelhöfer, L., Berg, D., Williams, David, Maetzler, W., Srulijes, K. K., Ludolph, A., Kassubek, J., Steiger, M., Tyler, K., Burn, D. J., Morris, L., Lees, A., Ling, H., Lafontaine, Anne Louise, Hauser, R., McClain, T., Truong, D., Jenkins, S., Litvan, I., Houghton, D., Ferrara, J., Bordelon, Y., Gratiano, A., Golbe, L., Marras, Connie, Mark, M., Uitti, R., Ven Gerpen, J., Jog, Mandar, Panisset, Michael, Lang, Anthony, Parker, Lesley, Irizarry, Michael C, Stewart, Alistair J, Corvol, Jean-Christophe, Azulay, Jean-Philippe, Couratier, Philippe, Mollenhauer, Brit, Lorenzl, Stefan, Ludolph, Albert, Benecke, Reiner, Lipp, Axel, Reichmann, Heinz, McDermott, Dana, Woitalla, Dirk, Chan, Dennis, Zermansky, Adam, Burn, David, Lees, Andrew, Gozes, Illana, Boxer, Adam, Miller, Bruce L, Lobach, Iryna V, Roberson, Erik, Biglan, Kevin, Honig, Lawrence, Zamrini, Edward, Pahwa, Rajesh, Bordelon, Yvette, Driver-Dunkley, Erika, Lessig, Stephanie, Lew, Mark, Womack, Kyle, Boeve, Brad, Ferrara, Joseph, Höglinger, Günter U, Hillis, Argyle, Kaufer, Daniel, Kumar, Rajeev, Xie, Tao, Gunzler, Steven, Zesiewicz, Theresa, Dayalu, Praveen, Golbe, Lawrence, Grossman, Murray, Jankovic, Joseph, McGinnis, Scott, Santiago, Anthony, Tuite, Paul, Isaacson, Stuart, Leegwater-Kim, Julie, Litvan, Irene, Knopman, David S, Schneider, Lon S, Doody, Rachelle S, Del Ser, Teodoro, Golbe, Lawrence I, Roberson, Erik D, Koestler, Mary, Jack, Clifford R, Van Deerlin, Viviana, Randolph, Christopher, Whitaker, Steve, Hirman, Joe, Gold, Michael, Morimoto, Bruce H, Boxer, Adam L, Nuebling G, Georg, Hensler, Mira, Paul, Sabine, Zwergal, Andreas, Heuer, Hilary W, Tartaglia, Maria C, McGinnis, Scott M, Dickerson, Bradford C, Kornak, John, Group, AL-108-231 Study, Schuff, Norbert, Rabinovici, Gil D, Rosen, Howard J, Gómez, J. C., Tijero, B., Berganzo, K., Garc'ıa de Yebenes, J., Lopez Sendón, J. L., Garcia, G.
Jazyk: angličtina
Rok vydání: 2018
Předmět:
0301 basic medicine
Male
Activities of daily living
physiopathology [Supranuclear Palsy
Progressive]
Disease
Severity of Illness Index
diagnosis [Supranuclear Palsy
Progressive]
Predictive models
0302 clinical medicine
diagnosis [Basal Ganglia Diseases]
Aged
80 and over
Neurodegenerative Diseases
Syndrome
Middle Aged
Corticobasal syndrome
Prognosis
Neurology
Disease Progression
Female
Tauopathy
Supranuclear Palsy
Progressive
Natural history study
PSP rating scale
medicine.medical_specialty
physiopathology [Basal Ganglia Diseases]
diagnosis [Neurodegenerative Diseases]
Models
Neurological
Article
Progressive supranuclear palsy
03 medical and health sciences
Physical medicine and rehabilitation
Basal Ganglia Diseases
Rating scale
medicine
Humans
ddc:610
Aged
Interactive visualizations
business.industry
physiopathology [Neurodegenerative Diseases]
Data Visualization
medicine.disease
Gait
eye diseases
Clinical trial
030104 developmental biology
Neurology (clinical)
Geriatrics and Gerontology
business
030217 neurology & neurosurgery
Zdroj: Parkinsonism & related disorders 60, 138-145 (2019). doi:10.1016/j.parkreldis.2018.08.025
PARKINSONISM & RELATED DISORDERS
r-IIS La Fe. Repositorio Institucional de Producción Científica del Instituto de Investigación Sanitaria La Fe
instname
ISSN: 1353-8020
DOI: 10.1016/j.parkreldis.2018.08.025
Popis: Background: Progressive supranuclear palsy (PSP)-Richardson's Syndrome and Corticobasal Syndrome (CBS) are the two classic clinical syndromes associated with underlying four repeat (4R) tau pathology. The PSP Rating Scale is a commonly used assessment in PSP clinical trials; there is an increasing interest in designing combined 4R tauopathy clinical trials involving both CBS and PSP. Objectives: To determine contributions of each domain of the PSP Rating Scale to overall severity and characterize the probable sequence of clinical progression of PSP as compared to CBS. Methods: Multicenter clinical trial and natural history study data were analyzed from 545 patients with PSP and 49 with CBS. Proportional odds models were applied to model normalized cross-sectional PSP Rating Scale, estimating the probability that a patient would experience impairment in each domain using the PSP Rating Scale total score as the index of overall disease severity. Results: The earliest symptom domain to demonstrate impairment in PSP patients was most likely to be Ocular Motor, followed jointly by Gait/Midline and Daily Activities, then Limb Motor and Mentation, and finally Bulbar. For CBS, Limb Motor manifested first and ocular showed less probability of impairment throughout the disease spectrum. An online tool to visualize predicted disease progression was developed to predict relative disability on each subscale per overall disease severity. Conclusion: The PSP Rating Scale captures disease severity in both PSP and CBS. Modelling how domains change in relation to one other at varying disease severities may facilitate detection of therapeutic effects in future clinical trials.
Databáze: OpenAIRE
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