The primary progressive aphasias (PPA) present complex and diverse challenges of diagnosis, management and prognosis. A clinically-informed, syndromic staging system for PPA would take a substantial step toward meeting these challenges. This study addressed this need using detailed, multi-domain mixed-methods symptom surveys of people with lived experience in a large international PPA cohort. We administered structured online surveys to caregivers of patients with a canonical PPA syndromic variant (nonfluent/agrammatic (nvPPA), semantic (svPPA) or logopenic (lvPPA)). In an exploratory survey, a putative list and ordering of verbal communication and nonverbal functioning (nonverbal thinking, conduct and wellbeing, physical) symptoms was administered to 118 caregiver members of the UK national PPA Support Group. Based on feedback, we expanded the symptom list and created six provisional clinical stages for each PPA subtype. In a consolidation survey, these stages were presented to 110 caregiver members of UK and Australian PPA Support Groups, and refined based on quantitative and qualitative feedback. Symptoms were retained if rated as present by a majority (at least 50%) of respondents representing that PPA syndrome, and assigned to a consolidated stage based on majority consensus; the confidence of assignment was estimated for each symptom as the proportion of respondents in agreement with the final staging for that symptom. Qualitative responses were analysed using framework analysis. For each PPA syndrome, six stages ranging from 1 (Very mild) to 6 (Profound) were identified; earliest stages were distinguished by syndromic hallmark symptoms of communication dysfunction, with increasing trans-syndromic convergence and dependency for basic activities of daily living at later stages. Spelling errors, hearing changes and nonverbal behavioural features were reported at early stages in all syndromes. As the illness evolved, swallowing and mobility problems were reported earlier in nfvPPA than other syndromes, while difficulty recognising familiar people and household items characterised svPPA and visuospatial symptoms were more prominent in lvPPA. Overall confidence of symptom staging was higher for svPPA than other syndromes. Across syndromes, functional milestones were identified as key deficits that predict the sequence of major daily life impacts and associated management needs. Qualitatively, we identified five major themes encompassing 15 subthemes capturing respondents' experiences of PPA and suggestions for staging implementation. This work introduces a prototypical, symptom-led staging scheme for canonical PPA syndromes: the PPA Progression Planning Aid (PPA-squared). Our findings have implications for diagnostic and care pathway guidelines, trial design and personalised prognosis and treatment for people living with these diseases.
Objective: To study how activities of daily living (ADLs) decline over the progressive course of rarer dementias (prevalence below 10%), in a systematic review of the literature. Methods: Relevant studies were identified by searching Medline, Embase, Emcare, PsycINFO and Cinahl. The databases were searched for terms relating to (rarer dementias) AND (activities of daily living) AND (longitudinal OR cross-sectional studies), using a pre-established protocol registered with the international prospective register of systematic reviews (registration: CRD42021283302). Results: A total of 579 articles were screened for relevant content, of which 20 full-text publications were included in the analysis. Nineteen studies were about rarer dementias on the frontotemporal dementia/primary progressive aphasia spectrum, and one was about posterior cortical atrophy. Long term description of decline was limited to just seven studies following patients for longer than five years. The rate of decline, sequence of symptom onset, and symptom duration were also highlighted. Conclusion: Descriptions of ADL progression were inadequately long term, covering an average of 3.5 years from symptom onset, and lacked phenotypic specificity. The literature disproportionately studied dementias on the frontotemporal dementia spectrum. To facilitate better care, more longitudinal data, quantitative analyses, and development of rarer dementia-specific ADL scales is needed. Given the low prevalence of rarer dementias, big data analyses may never be applicable and so personalised medicine approaches should be pursued, including innovative possibilities in digital biomarkers such as from wearable technology.
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