The patterns of progression in primary progressive aphasia—Implications for assessment and management

Abstract

Background: Primary progressive aphasia (PPA) is a progressive language disorder with preserved cognitive function for at least 2 years from onset. The main variants currently distinguished are: non-fluent/agrammatic (nfvPPA), semantic (svPPA), and logopenic (lvPPA). Patients with initial language presentation may subsequently develop other symptoms, such as behavioural dysfunction or apraxia. The clinical pattern of PPA depends on the location of atrophy, the underlying pathology, and the stage of the disease.

Aims: This review aims at characterising longitudinal changes in clinical presentations of different PPA variants and at presenting implications of these changes for the assessment, diagnosis, and management.

Main contribution: The three PPA variants differ not only in terms of language impairment, but also with regard to cognitive and behavioural profile. Apraxia and rigidity frequently occur in the course of nfvPPA. Patients with lvPPA seem to follow the pattern of aphasic Alzheimer’s disease, where language impairment is accompanied by episodic memory deficit. Individuals diagnosed with svPPA often develop behavioural dysfunction similar to that observed in behavioural variant of frontotemporal dementia.

Conclusions: Implications for patient care are dependent on PPA variant and on the stage of the disease. In svPPA, emphasis should be on the management of semantic and behavioural problems in daily life. Caregivers of nfvPPA patients should be informed about the possible emergence of apraxia and other movement disorders. In contrast, families of individuals with lvPPA should be made aware of and trained to cope with an episodic memory decline and possible progression to other varieties of PPA.

Keywords:

• Primary progressive aphasia
• Pick complex
• Frontotemporal dementia
• Semantic dementia
• Progressive non-fluent aphasia
• Alzheimer’s disease

MH and EJS were supported by Polish Ministry of Science and Higher Education scholarship during the preparation of the manuscript.

Notes

¹ The pathological aggregation of tau protein in the human brain characteristic for a class of neurodegenerative diseases.

² Transactive response DNA binding protein 43, a cellular protein which in humans is encoded in TARDBP gene.

³ Pittsburgh Compound-B—novel amyloid-imaging positron emission tomography tracer, which can be used in positron emission tomography scans to image beta-amyloid plaques in neuronal tissue.