Functional disability in primary progressive aphasia

Abstract

Background: In primary progressive aphasia (PPA), assessment of language predominates over assessment of functional impairment in activities of daily living (ADLs) in clinical and research environments. Most of the knowledge on functional disability in PPA relies largely on anecdotal experience and limited numbers of studies published to date.

Aims: (1) To describe the different patterns of ADL functional disability in the main PPA variants: semantic variant, nonfluent aphasia, and the more recently defined logopenic variant; (2) to draw relations between functional disability, cognitive, and behavioural symptoms in the PPAs; (3) to examine the impact of functional disability on carer burden, and (4) to provide specific strategies to address the described problems.

Main Contribution: Profiles of disease progression are described from a functional perspective, as well as the relationship (or lack thereof) between functional disability and cognitive and behavioural symptoms. Dementia-management strategies for carers and professionals in overcoming day-to-day difficulties are provided, and the impact of functional deficits on those around the patient, including their spouses and children, are discussed.

Conclusions: Patterns of ADL functional disability and their progression vary between PPA subtypes. Understanding these different profiles of impairment is critical to the development of tailored interventions. There is a range of therapeutic strategies which can be trialled to promote improved ADL functioning, which in turn may also help in reducing levels of carer burden in PPA.

Keywords:

• Primary progressive aphasia
• Semantic variant
• Semantic dementia
• Nonfluent variant
• Progressive nonfluent aphasia
• Activities of daily living
• Behaviour

EM was a recipient of a National Health Medical Research Council of Australia Early Career Fellowship [APP1016399] at the time the study was conducted; she is currently a recipient of an Alzheimer Association USA New Investigator Research [grant number NIRP-12-258380]. SA is funded by the NIHR Oxford Biomedical Research Centre.