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Progression of brain atrophy in PSP and CBS over 6 months and 1 year

Title: Progression of brain atrophy in PSP and CBS over 6 months and 1 year
Authors: Dutt, Shubir; Binney, Richard J; Heuer, Hilary W; Luong, Phi; Attygalle, Suneth; Bhatt, Priyanka; Marx, Gabe A; Elofson, Jonathan; Tartaglia, Maria C; Litvan, Irene; McGinnis, Scott M; Dickerson, Bradford C; Kornak, John; Waltzman, Dana; Voltarelli, Lisa; Schuff, Norbert; Rabinovici, Gil D; Kramer, Joel H; Jack, Clifford R; Miller, Bruce L; Rosen, Howard J; Boxer, Adam L; Williams, David; Lafontaine, Anne Louise; Marras, Connie; Jog, Mandar; Panisset, Michael; Lang, Anthony; Parker, Lesley; Stewart, Alistair J; Corvol, Jean-Christophe; Azulay, Jean-Philippe; Couratier, Philippe; Mollenhauer, Brit; Lorenzl, Stefan; Ludolph, Albert; Benecke, Reiner; Hoglinger, Gunter; Lipp, Axel; Reichmann, Heinz; Woitalla, Dirk; Chan, Dennis; Zermansky, Adam; Burn, David; Lees, Andrew; Gozes, Illana; Boxer, Adam; Lobach, Iryna V; Roberson, Erik; Honig, Lawrence; Zamrini, Edward; Pahwa, Rajesh; Bordelon, Yvette; Driver-Dunkley, Erika; Lessig, Stephanie; Lew, Mark; Womack, Kyle; Boeve, Brad; Ferrara, Joseph; 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; Knopman, David S; Schneider, Lon S; Doody, Rachelle S; Koestler, Mary; Van Deerlin, Viviana; Randolph, Christopher; Whitaker, Steve; Hirman, Joe; Gold, Michael; Morimoto; Bruce H
Source: Neurology, vol 87, iss 19
Publisher Information: eScholarship, University of California
Publication Year: 2016
Collection: University of California: eScholarship
Subject Terms: 32 Biomedical and Clinical Sciences (for-2020); 3202 Clinical Sciences (for-2020); Alzheimer's Disease Related Dementias (ADRD) (rcdc); Biomedical Imaging (rcdc); Brain Disorders (rcdc); Dementia (rcdc); Frontotemporal Dementia (FTD) (rcdc); Aging (rcdc); Neurosciences (rcdc); Alzheimer's Disease including Alzheimer's Disease Related Dementias (AD/ADRD) (rcdc); Clinical Trials and Supportive Activities (rcdc); Neurodegenerative (rcdc); Clinical Research (rcdc); Rare Diseases (rcdc); Acquired Cognitive Impairment (rcdc); 2.1 Biological and endogenous factors (hrcs-rac); Neurological (hrcs-hc); Aged (mesh); Atrophy (mesh); Basal Ganglia (mesh); Cerebral Cortex (mesh); Disease Progression (mesh); Female (mesh); Follow-Up Studies (mesh); Humans (mesh); Image Processing; Computer-Assisted (mesh); Magnetic Resonance Imaging (mesh); Male (mesh); Middle Aged (mesh)
Time: 2016 - 2025
Description: OBJECTIVE: To examine the utility and reliability of volumetric MRI in measuring disease progression in the 4 repeat tauopathies, progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS), to support clinical development of new tau-directed therapeutic agents. METHODS: Six- and 12-month changes in regional MRI volumes and PSP Rating Scale scores were examined in 55 patients with PSP and 33 patients with CBS (78% amyloid PET negative) compared to 30 normal controls from a multicenter natural history study. Longitudinal voxel-based morphometric analyses identified patterns of volume loss, and region-of-interest analyses examined rates of volume loss in brainstem (midbrain, pons, superior cerebellar peduncle), cortical, and subcortical regions based on previously validated atlases. Results were compared to those in a replication cohort of 226 patients with PSP with MRI data from the AL-108-231 clinical trial. RESULTS: Patients with CBS exhibited greater baseline atrophy and greater longitudinal atrophy rates in cortical and basal ganglia regions than patients with PSP; however, midbrain and pontine atrophy rates were similar. Voxel-wise analyses showed distinct patterns of regional longitudinal atrophy in each group as compared to normal controls. The midbrain/pons volumetric ratio differed between diagnoses but remained stable over time. In both patient groups, brainstem atrophy rates were correlated with disease progression measured using the PSP Rating Scale. CONCLUSIONS: Volume loss is quantifiable over a period of 6 months in CBS and PSP. Future clinical trials may be able to combine CBS and PSP to measure therapeutic effects.
Document Type: article in journal/newspaper
File Description: application/pdf
Language: unknown
Relation: qt01c909rx; https://escholarship.org/uc/item/01c909rx; https://escholarship.org/content/qt01c909rx/qt01c909rx.pdf
DOI: 10.1212/wnl.0000000000003305
Availability: https://escholarship.org/uc/item/01c909rx; https://escholarship.org/content/qt01c909rx/qt01c909rx.pdf; https://doi.org/10.1212/wnl.0000000000003305
Rights: public
Accession Number: edsbas.1338F8BF
Database: BASE