Understanding and living with primary progressive aphasia: Current progress and challenges for the future

Background: Primary progressive aphasia (PPA) is a progressive language disorder in which aphasia is the first and most prominent symptom of degenerative brain disease. PPA has received increasing attention in the scientific literature over the past 30 years, but there remains a relative lack of awareness and understanding of it in the wider clinical community. As editors of the volume, Clinical Perspectives on Primary Progressive Aphasia, we invited the contributing authors to provide an up-to-date survey of research on a range of topics that are relevant to clinical practice in PPA.

Aims: The aim of this article is to address some key questions that may arise when an individual receives a diagnosis of PPA and to direct readers to additional sources of information in this volume and elsewhere that will allow them to gain further knowledge about topics of interest.

Main Contribution: We address the following questions: (1) What is PPA? (2) How is PPA diagnosed? (3) What happens to a person’s language when they have PPA? (4) How will the disease progress over time? (5) How does PPA impact a person’s life and the life of their family and friends? (6) What treatments and support are available? (7) What other services should we be providing?

Conclusions: Considerable progress has been made in our understanding of PPA and the relationship between the symptomatology, progression, pathology, and genetics of PPA. However, there are many challenges remaining, particularly in terms of ensuring that people with PPA and their families and friends receive optimal information and support at diagnosis and appropriate interventions and/or management strategies throughout their journey with PPA.