Decline in activities of daily living in the rarer dementias

Rarer dementias are associated with atypical symptoms and younger onset, which result in a higher burden of care. We provide a review of the global literature on longitudinal decline in activities of daily living (ADLs) in dementias that account for less than 10% of dementia diagnoses. Published studies were identified through searches conducted in Medical Literature Analysis and Retrieval System Online (MEDLINE), Excerpta Medica Database (Embase), Excerpta Medica Care (Emcare), PsycINFO, and Cumulative Index in Nursing and Allied Health Literature (CINAHL). The search criteria included terms related to ‘rarer dementias’, ‘activities of daily living’ and ‘longitudinal or cross-sectional studies’ following a predefined protocol registered. Studies were screened, and those that met the criteria were citation searched. Quality assessments were performed, and relevant data were extracted. 20 articles were selected, of which 19 focused on dementias within the frontotemporal dementia/primary progressive aphasia spectrum, while one addressed posterior cortical atrophy. Four studies were cross-sectional and 16 studies were longitudinal, with a median duration of 2.2 years. The Disability Assessment for Dementia was used to measure decline in 8 of the 20 studies. The varied sequences of ADL decline reported in the literature reflect variation in diagnostic specificity between studies and within-syndrome heterogeneity. Most studies used Alzheimer’s disease staging scales to measure decline, which cannot capture variant-specific symptoms. To enhance care provision in dementia, ADL scales could be deployed postdiagnosis to aid treatment and planning. This necessitates staging scales that are variant-specific and span the disease course from diagnosis to end of life. PROSPERO registration number: CRD42021283302.