The right to choose: stories from the rare dementias
Reviewed by Anna Volkmer

People with primary progressive aphasia (PPA) experience an insidious onset and gradual decline in language on a background of lesser or no cognitive impairment, hence a language-led dementia. There are three different PPA variants that correspond with three different clinical presentations; namely logopenic PPA (lvPPA), presenting as difficulties in word retrieval, non-fluent agrammatic PPA (nfvPPA), presenting as an apraxia and/or agrammatism, and semantic PPA (svPPA), presenting as a dissolution in word meaning. In this article, the authors present a series of cases to illustrate the complexities around autonomy in decision-making that can arise when a person has this diagnosis. They described a woman with nfvPPA whose husband wanted to make decisions on her behalf as he did not understand she was able to participate in decision-making, despite her apraxia, as well as a man with lvPPA who decided with his wife that he should no longer drive but ran into difficulties on his ride-share en route to therapy. They also described a woman who had svPPA who started spontaneously visiting family in other states, often forgetting essential travel necessities or misunderstanding flight times. Her family struggled to support her independence without making her isolated. The authors reflect on the role of the health professionals in supporting people and their families to achieve a balance between autonomy in decision-making capacity and making decisions in a person’s best interest when they are no longer able to do this themselves.

Ethical and practical challenges of the communication and behavioural manifestations of primary progressive aphasia.
Tippett DC, Hillis AE.
SEMINARS IN SPEECH AND LANGUAGE
2020;41(3):249-56.