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J Alzheimers Dis. 2016 Jun 18;53(3):1033-42. doi: 10.3233/JAD-160010.

Comparing Longitudinal Behavior Changes in the Primary Progressive Aphasias.

Van Langenhove T1,2,3,4, Leyton CE1,2,5, Piguet O1,2,3, Hodges JR1,2,3.

Author information

1
Neuroscience Research Australia, Sydney, Australia.
2
ARC Centre of Excellence in Cognition and its Disorders, Sydney, Australia.
3
School of Medical Sciences, The University of New South Wales, Sydney, Australia.
4
Institute Born-Bunge, University of Antwerp, Antwerp, Belgium.
5
Faculty of Health Sciences, The University of Sydney, Sydney, Australia.

Abstract

BACKGROUND:

Differentiating between primary progressive aphasia (PPA) variants based on the profile of language deficits can be difficult in a proportion of patients. Further, little is presently know about the pattern of longitudinal changes in behavior in PPA variants.

OBJECTIVE:

To determine the presence of behavioral changes in the main variants of PPA: semantic (sv-PPA), nonfluent/agrammatic (nfv-PPA), and logopenic (lv-PPA), and establish the course of these changes over time.

METHODS:

We measured behavioral changes in 73 prospectively recruited PPA (30 sv-PPA, 22 nfv-PPA, and 21 lv-PPA), as well as 33 behavioral variant frontotemporal dementia (bv-FTD) and 31 Alzheimer's disease (AD) patients, at baseline and after 1 year, using the Cambridge Behavioural Inventory Revised. All included patients had mild dementia severity at baseline.

RESULTS:

Both at baseline and follow-up, sv-PPA exhibited significantly more behavioral disturbances of the type characteristic of bv-FTD compared with other PPA variants. 74% of sv-PPA patients with mild dementia severity exhibited at least one behavior disturbance at baseline, which increased to 84% during follow-up. Behavioral symptoms did not differ between nfv-PPA and lv-PPA groups at baseline. At follow-up, however, empathy loss was significantly more pronounced in nfv-PPA. The prevalence and course of behavioral symptoms in lv-PPA was similar to that found in AD.

CONCLUSIONS:

sv-PPA show more prominent FTD-like behavioral disturbances compared with other PPA variants which typically emerge already early in the disease course. Empathy loss may be an important factor that helps differentiating nfv-PPA from lv-PPA. Our results may allow improved prediction of likely progression in behavioral symptoms across the PPA variants.

KEYWORDS:

Behavioral symptoms; empathy; frontotemporal dementia; longitudinal studies; primary progressive aphasia

PMID:
27340847
DOI:
10.3233/JAD-160010
[Indexed for MEDLINE]

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