Hardy, CJD;
Taylor-Rubin, C;
Taylor, B;
Harding, E;
Gonzalez, AS;
Jiang, J;
Thompson, L;
... Warren, JD; + view all
(2024)
Symptom-based staging for logopenic variant primary progressive aphasia.
European Journal of Neurology
, Article e16304. 10.1111/ene.16304.
(In press).
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Abstract
Background and purpose: Logopenic variant primary progressive aphasia (lvPPA) is a major variant presentation of Alzheimer's disease (AD) that signals the importance of communication dysfunction across AD phenotypes. A clinical staging system is lacking for the evolution of AD-associated communication difficulties that could guide diagnosis and care planning. Our aim was to create a symptom-based staging scheme for lvPPA, identifying functional milestones relevant to the broader AD spectrum. Methods: An international lvPPA caregiver cohort was surveyed on symptom development under an ‘exploratory’ survey (34 UK caregivers). Feedback from this survey informed the development of a ‘consolidation’ survey (27 UK, 10 Australian caregivers) in which caregivers were presented with six provisional clinical stages and feedback was analysed using a mixed-methods approach. Results: Six clinical stages were endorsed. Early symptoms included word-finding difficulty, with loss of message comprehension and speech intelligibility signalling later-stage progression. Additionally, problems with hearing in noise, memory and route-finding were prominent early non-verbal symptoms. ‘Milestone’ symptoms were identified that anticipate daily-life functional transitions and care needs. Conclusions: This work introduces a new symptom-based staging scheme for lvPPA, and highlights milestone symptoms that could inform future clinical scales for anticipating and managing communication dysfunction across the AD spectrum.
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