Classification of primary progressive aphasia: challenges and complexities

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Abstract

Primary progressive aphasia (PPA) is classified into three variants, logopenic variant PPA (lvPPA), nonfluent agrammatic PPA (nfaPPA), and semantic variant PPA (svPPA), based on clinical (syndromic) characteristics with support from neuroimaging and/or underlying neuropathology. Classification of PPA variants provides information valuable to disease management. International consensus criteria are widely employed to identify PPA subtypes; however, classification is complex, and some individuals do not fit neatly into the subtyping scheme. In this review, diagnostic challenges and their implications are discussed, possible explanations for these challenges are explored, and approaches to address PPA classification are considered.

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Version 3 of the Alzheimer Disease Centers’ Neuropsychological Test Battery in the Uniform Data Set (UDS)

Sandra Weintraub, Lilah Besser, Hiroko H. Dodge … (2018)

Introduction: The neuropsychological battery of the Uniform Data Set (UDSNB) was implemented in 2005 by the National Institute on Aging (NIA) Alzheimer Disease Centers program to measure cognitive performance in dementia and mild cognitive impairment due to Alzheimer Disease. This paper describes a revision, the UDSNB 3.0. Methods: The Neuropsychology Work Group of the NIA Clinical Task Force recommended revisions through a process of due diligence to address shortcomings of the original battery. The UDSNB 3.0 covers episodic memory, processing speed, executive function, language, and constructional ability. Data from 3602 cognitively normal participants in the National Alzheimer Coordinating Center database were analyzed. Results: Descriptive statistics are presented. Multivariable linear regression analyses demonstrated score differences by age, sex, and education and were also used to create a normative calculator available online. Discussion: The UDSNB 3.0 neuropsychological battery provides a valuable non proprietary resource for conducting research on cognitive aging and dementia.

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Semantic dementia. Progressive fluent aphasia with temporal lobe atrophy.

S Oxbury, Barbara E. Funnell, Heather Patterson … (1992)

We report five patients with a stereotyped clinical syndrome characterized by fluent dysphasia with severe anomia, reduced vocabulary and prominent impairment of single-word comprehension, progressing to a stage of virtually complete dissolution of the semantic components of language. A marked reduction in the ability to generate exemplars from restricted semantic categories (e.g. animals, vehicles, etc.) was a consistent and early feature. Tests of semantic memory demonstrated a radically impoverished knowledge about a range of living and man-made items. In contrast, phonology and grammar of spoken language were largely preserved, as was comprehension of complex syntactic commands. Reading showed a pattern of surface dyslexia. Autobiographical and day-to-day (episodic) memory were relatively retained. Non-verbal memory, perceptual and visuospatial abilities were also strikingly preserved. In some cases, behavioural and personality changes may supervene; one patient developed features of the Kluver-Bucy Syndrome. Radiological investigations have shown marked focal temporal atrophy in all five patients, and functional imaging by single positron emission tomography and positron emission tomography (one case) have implicated the dominant temporal lobe in all five. In the older literature, such cases would have been subsumed under the rubric of Pick's disease. Others have been included in series with progressive aphasia. We propose the term semantic dementia, first coined by Snowden et al. (1989), to designate this clinical syndrome.

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Primary progressive aphasia: clinicopathological correlations.

Murray Grossman (2010)

Primary progressive aphasia (PPA) is a disorder of declining language that is a frequent presentation of neurodegenerative diseases such as frontotemporal lobar degeneration. Three variants of PPA are recognized: progressive nonfluent aphasia, semantic dementia, and logopenic progressive aphasia. In an era of etiology-specific treatments for neurodegenerative conditions, determining the histopathological basis of PPA is crucial. Clinicopathological correlations in PPA emphasize the contributory role of dementia with Pick bodies and other tauopathies, TDP-43 proteinopathies, and Alzheimer disease. These data suggest an association between a specific PPA variant and an underlying pathology, although many cases of PPA are associated with an unexpected pathology. Neuroimaging and biofluid biomarkers are now emerging as important adjuncts to clinical diagnosis. There is great hope that the addition of biomarker assessments to careful clinical examination will enable accurate diagnosis of the pathology associated with PPA during a patient's life, and that such findings will serve as the basis for clinical trials in this spectrum of disease.

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Author and article information

Contributors

Donna C. Tippett: Role: ConceptualizationRole: Writing – Original Draft PreparationRole: Writing – Review & Editing

ORCID: https://orcid.org/0000-0001-9485-9745

Journal

Journal ID (nlm-ta): F1000Res

Journal ID (iso-abbrev): F1000Res

Journal ID (pmc): F1000Research

Title: F1000Research

Publisher: F1000 Research Limited (London, UK )

ISSN (Electronic): 2046-1402

Publication date (Electronic): 30 January 2020

Publication date Collection: 2020

Volume: 9

Electronic Location Identifier: F1000 Faculty Rev-64

Affiliations

[1 ]Departments of Neurology, Otolaryngology - Head and Neck Surgery, and Department of Physical Medicine and Rehabilitation, Johns Hopkins University School of Medicine, Baltimore, Maryland, 21287, USA

Author notes

[a ] dtippet1@ 123456jhmi.edu

No competing interests were disclosed.

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Donna C. Tippett https://orcid.org/0000-0001-9485-9745

Article

DOI: 10.12688/f1000research.21184.1

PMC ID: 6993834

SO-VID: c607360d-bfcc-41b7-a656-45d3685fd781

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This is an open access article distributed under the terms of the Creative Commons Attribution Licence, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

History

Date accepted : 21 January 2020

Funding

Funded by: NIH NIDCD

Award ID: R01DC011317

This work was supported by NIH grant R01DC011317 from NIA and NIDCD awarded to Rhonda B. Friedman and Aaron Meyer.

The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.

Classification of primary progressive aphasia: challenges and complexities

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Most cited references 62

Version 3 of the Alzheimer Disease Centers’ Neuropsychological Test Battery in the Uniform Data Set (UDS)

Semantic dementia. Progressive fluent aphasia with temporal lobe atrophy.

Primary progressive aphasia: clinicopathological correlations.

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