학술논문
Symptom-led staging for semantic and non-fluent/agrammatic variants of primary progressive aphasia.
Document Type
Academic Journal
Author
Hardy CJD; Dementia Research Centre, UCL Queen Square Institute of Neurology, UCL, London, UK.; Taylor-Rubin C; Uniting War Memorial Hospital, Sydney, Australia.; Faculty of Medicine, Health and Human Sciences, Macquarie University, Sydney, Australia.; Taylor B; Centre for Medical Image Computing, Department of Computer Science, UCL, London, UK.; Harding E; Dementia Research Centre, UCL Queen Square Institute of Neurology, UCL, London, UK.; Gonzalez AS; Dementia Research Centre, UCL Queen Square Institute of Neurology, UCL, London, UK.; Jiang J; Dementia Research Centre, UCL Queen Square Institute of Neurology, UCL, London, UK.; Thompson L; Uniting War Memorial Hospital, Sydney, Australia.; Kingma R; Uniting War Memorial Hospital, Sydney, Australia.; Chokesuwattanaskul A; Dementia Research Centre, UCL Queen Square Institute of Neurology, UCL, London, UK.; Division of Neurology, Department of Internal Medicine, King Chulalongkorn Memorial Hospital, Bangkok, Thailand.; Cognitive Clinical and Computational Neuroscience Research Unit, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand.; Walker F; HealthAbility, Melbourne, Australia.; Barker S; Dementia Research Centre, UCL Queen Square Institute of Neurology, UCL, London, UK.; Brotherhood E; Dementia Research Centre, UCL Queen Square Institute of Neurology, UCL, London, UK.; Waddington C; Dementia Research Centre, UCL Queen Square Institute of Neurology, UCL, London, UK.; Wood O; Dementia Research Centre, UCL Queen Square Institute of Neurology, UCL, London, UK.; Zimmermann N; Dementia Research Centre, UCL Queen Square Institute of Neurology, UCL, London, UK.; Kupeli N; Marie Curie Palliative Care Research Department, Division of Psychiatry, UCL, London, UK.; Yong KXX; Dementia Research Centre, UCL Queen Square Institute of Neurology, UCL, London, UK.; Camic PM; Dementia Research Centre, UCL Queen Square Institute of Neurology, UCL, London, UK.; Stott J; Dementia Research Centre, UCL Queen Square Institute of Neurology, UCL, London, UK.; ADAPT Lab, Research Department of Clinical, Educational and Health Psychology, UCL, London, UK.; Marshall CR; Preventive Neurology Unit, Queen Mary University of London, London, UK.; Oxtoby NP; Centre for Medical Image Computing, Department of Computer Science, UCL, London, UK.; Rohrer JD; Dementia Research Centre, UCL Queen Square Institute of Neurology, UCL, London, UK.; Volkmer A; Dementia Research Centre, UCL Queen Square Institute of Neurology, UCL, London, UK.; Psychology and Language Sciences (PALS), UCL, London, UK.; Crutch SJ; Dementia Research Centre, UCL Queen Square Institute of Neurology, UCL, London, UK.; Warren JD; Dementia Research Centre, UCL Queen Square Institute of Neurology, UCL, London, UK.
Source
Publisher: John Wiley & Sons, Ltd Country of Publication: United States NLM ID: 101231978 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1552-5279 (Electronic) Linking ISSN: 15525260 NLM ISO Abbreviation: Alzheimers Dement Subsets: MEDLINE
Subject
Language
English
Abstract
Introduction: Here we set out to create a symptom-led staging system for the canonical semantic and non-fluent/agrammatic variants of primary progressive aphasia (PPA), which present unique diagnostic and management challenges not well captured by functional scales developed for Alzheimer's disease and other dementias.
Methods: An international PPA caregiver cohort was surveyed on symptom development under six provisional clinical stages and feedback was analyzed using a mixed-methods sequential explanatory design.
Results: Both PPA syndromes were characterized by initial communication dysfunction and non-verbal behavioral changes, with increasing syndromic convergence and functional dependency at later stages. Milestone symptoms were distilled to create a prototypical progression and severity scale of functional impairment: the PPA Progression Planning Aid ("PPA-Squared").
Discussion: This work introduces a symptom-led staging scheme and functional scale for semantic and non-fluent/agrammatic variants of PPA. Our findings have implications for diagnostic and care pathway guidelines, trial design, and personalized prognosis and treatment for PPA.
Highlights: We introduce new symptom-led perspectives on primary progressive aphasia (PPA). The focus is on non-fluent/agrammatic (nfvPPA) and semantic (svPPA) variants. Foregrounding of early and non-verbal features of PPA and clinical trajectories is featured. We introduce a symptom-led staging scheme for PPA. We propose a prototype for a functional impairment scale, the PPA Progression Planning Aid.
(© 2023 The Authors. Alzheimer's & Dementia published by Wiley Periodicals LLC on behalf of Alzheimer's Association.)
Methods: An international PPA caregiver cohort was surveyed on symptom development under six provisional clinical stages and feedback was analyzed using a mixed-methods sequential explanatory design.
Results: Both PPA syndromes were characterized by initial communication dysfunction and non-verbal behavioral changes, with increasing syndromic convergence and functional dependency at later stages. Milestone symptoms were distilled to create a prototypical progression and severity scale of functional impairment: the PPA Progression Planning Aid ("PPA-Squared").
Discussion: This work introduces a symptom-led staging scheme and functional scale for semantic and non-fluent/agrammatic variants of PPA. Our findings have implications for diagnostic and care pathway guidelines, trial design, and personalized prognosis and treatment for PPA.
Highlights: We introduce new symptom-led perspectives on primary progressive aphasia (PPA). The focus is on non-fluent/agrammatic (nfvPPA) and semantic (svPPA) variants. Foregrounding of early and non-verbal features of PPA and clinical trajectories is featured. We introduce a symptom-led staging scheme for PPA. We propose a prototype for a functional impairment scale, the PPA Progression Planning Aid.
(© 2023 The Authors. Alzheimer's & Dementia published by Wiley Periodicals LLC on behalf of Alzheimer's Association.)