10 research outputs found
Symptom-led staging for semantic and non-fluent/agrammatic variants of primary progressive aphasia. Alzheimer's & Dementia
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 Alzheimerâs 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 (âPPA-Squaredâ).
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
Symptom-based staging for logopenic variant primary progressive aphasia
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 âexploratoryâ survey (34 UK caregivers). Feedback from this survey informed the development of a âconsolidationâ 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. âMilestoneâ 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