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Open Access 01-03-2022 | Frontotemporal Dementia | Original Communication

The natural history of primary progressive aphasia: beyond aphasia

Authors: Hulya Ulugut, Simone Stek, Lianne E. E. Wagemans, Roos J. Jutten, Maria Antoinette Keulen, Femke H. Bouwman, Niels D. Prins, Afina W. Lemstra, Welmoed Krudop, Charlotte E. Teunissen, Bart N. M. van Berckel, Rik Ossenkoppele, Frederik Barkhof, Wiesje M. van der Flier, Philip Scheltens, Yolande A. L. Pijnenburg

Published in: Journal of Neurology | Issue 3/2022

Abstract

Introduction

Primary progressive aphasia (PPA) is divided into three prototypical subtypes that are all characterized by their single core symptom of aphasia. Although later in their course, other cognitive, behavioral, and motor domains may become involved, little is known about the progression profile of each subtype relative to the other subtypes.

Methods

In this longitudinal retrospective cohort study, based on the recent biomarker-supported diagnostic criteria, 24 subjects diagnosed with semantic variant (svPPA), 22 with non-fluent variant (nfvPPA), and 18 with logopenic variant (lvPPA) were collected and followed up for 1–6 years. Symptom distribution, cognitive test and neuropsychiatric inventory scores, and progression into another syndrome were assessed.

Results

Over time, lvPPA progressed with broader language problems (PPA-extended) and nfvPPA progressed to mutism, whereas semantic impairment remained the major problem in svPPA. Apart from linguistic problems, svPPA developed pronounced behavioral disturbances, whereas lvPPA exhibited a greater cognitive decline. By contrast, in nfvPPA motor deficits were more common. Furthermore, within 5 years (IQR = 2.5) after clinical onset, 65.6% of the patients additionally fulfilled the clinical criteria for another neurodegenerative syndrome (PPA-plus). Fourteen out of 24 (58%) svPPA patients additionally met the diagnostic criteria of behavioral variant frontotemporal dementia (5.1 years, IQR = 1.1), whereas the clinical features of 15/18 (83%) lvPPA patients were consistent with Alzheimer disease dementia (4.5 years IQR = 3.4). Furthermore, 12/22 (54%) of the subjects with the nfvPPA progressed to meet the diagnostic criteria of corticobasal syndrome, progressive supranuclear palsy, or motor neuron disease (5.1 years IQR = 3.4).

Discussion

Despite aphasia being the initial and unique hallmark of the syndrome, our longitudinal results showed that PPA is not a language limited disorder and progression differs widely for each subtype, both with respect to the nature of symptoms and disease duration.

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Title: The natural history of primary progressive aphasia: beyond aphasia
Authors: Hulya Ulugut
Simone Stek
Lianne E. E. Wagemans
Roos J. Jutten
Maria Antoinette Keulen
Femke H. Bouwman
Niels D. Prins
Afina W. Lemstra
Welmoed Krudop
Charlotte E. Teunissen
Bart N. M. van Berckel
Rik Ossenkoppele
Frederik Barkhof
Wiesje M. van der Flier
Philip Scheltens
Yolande A. L. Pijnenburg
Publication date: 01-03-2022
Publisher: Springer Berlin Heidelberg
Keywords: Frontotemporal Dementia
Frontotemporal Dementia
Dementia
Dementia
Aphasia
Progressive Supranuclear Palsy
Published in: Journal of Neurology / Issue 3/2022
Print ISSN: 0340-5354
Electronic ISSN: 1432-1459
DOI: https://doi.org/10.1007/s00415-021-10689-1

Keynote webinar | Spotlight on medication adherence

Springer Medicine

The natural history of primary progressive aphasia: beyond aphasia

Abstract

Introduction

Methods

Results

Discussion

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Introduction

Methods

Results

Discussion

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