Alzheimer's & dementia : the journal of the Alzheimer's Association · 2025
Language impairment is associated with faster progression in progressive supranuclear palsy-Richardson syndrome.
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Garcia-Cordero Indira, Vargas-Gonzalez Juan Camilo, Hadian Mohsen, Bayram Ece, Rodriguez-Porcel Federico, Couto Blas, Iyer Jay, Golbe Lawrence I, Stephen Christopher D, Pantelyat Alexander, Dale Marian L, McFarland Nikolaus, Xie Tao, Swan Matthew, Kang Kyurim, Gunzler Douglas, Wills Anne-Marie, Boxer Adam, Litvan Irene, Lang Anthony, Tartaglia Maria Carmela
Аннотация
INTRODUCTION: Cognitive impairment is common but often overlooked due to motor symptoms in progressive supranuclear palsy-Richardson syndrome (PSP-RS). This study investigates whether cognitive deficits predict disease progression in PSP-RS.
METHODS: A total of 146 PSP-RS from the Tilavonemab trial were evaluated at baseline and over 52 weeks using the PSP-Rating Scale (PSPRS), the Repeatable Battery for the Assessment of Neuropsychological Status (RBANS), and the Unified Parkinson's Disease Rating Scale Part-II (UPDRS-II). Multiple linear regression analyses were performed between the RBANS, UPDRS-II, and the PSPRS change. Clinical scores, gray matter volumes, and neurofilament-light chain (NfL) were compared using analyses of covariance (ANCOVAs) and linear mixed models between language score-groups.
RESULTS: Lower RBANS-language at baseline predicted greater PSPRS worsening over time. The low language-score group showed poorer cognitive performance, elevated NfL, and reduced gray matter volume in language-related areas.
DISCUSSION: Speech/language deficits predict worse prognosis in PSP-RS, emphasizing the value of including language scores in clinical trials.
HIGHLIGHTS: Speech and language deficits predict a worse prognosis in progressive supranuclear palsy-Richardson syndrome (PSP-RS). Lower language scores are associated with worse cognitive performance over time. Lower language scores related to higher neurofilament-light chain (NfL) at baseline. The low language-score group presented greater atrophy in language-related brain areas. Stratifying PSP-RS cases using language scores may improve clinical trials.
METHODS: A total of 146 PSP-RS from the Tilavonemab trial were evaluated at baseline and over 52 weeks using the PSP-Rating Scale (PSPRS), the Repeatable Battery for the Assessment of Neuropsychological Status (RBANS), and the Unified Parkinson's Disease Rating Scale Part-II (UPDRS-II). Multiple linear regression analyses were performed between the RBANS, UPDRS-II, and the PSPRS change. Clinical scores, gray matter volumes, and neurofilament-light chain (NfL) were compared using analyses of covariance (ANCOVAs) and linear mixed models between language score-groups.
RESULTS: Lower RBANS-language at baseline predicted greater PSPRS worsening over time. The low language-score group showed poorer cognitive performance, elevated NfL, and reduced gray matter volume in language-related areas.
DISCUSSION: Speech/language deficits predict worse prognosis in PSP-RS, emphasizing the value of including language scores in clinical trials.
HIGHLIGHTS: Speech and language deficits predict a worse prognosis in progressive supranuclear palsy-Richardson syndrome (PSP-RS). Lower language scores are associated with worse cognitive performance over time. Lower language scores related to higher neurofilament-light chain (NfL) at baseline. The low language-score group presented greater atrophy in language-related brain areas. Stratifying PSP-RS cases using language scores may improve clinical trials.