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Neurology. 2014 Sep 23;83(13):1184-91. doi: 10.1212/WNL.0000000000000824. Epub 2014 Aug 27.

Asymmetry of cortical decline in subtypes of primary progressive aphasia.

Author information

1
From the Cognitive Neurology and Alzheimer's Disease Center (E.R., A.M., C.W., S.W., M.-M.M.), and Departments of Psychiatry and Behavioral Sciences (D.C., S.W.), Preventative Medicine (A.R.), and Neurology (M.-M.M.), Northwestern University Feinberg School of Medicine, Chicago, IL. erogalski@gmail.com.
2
From the Cognitive Neurology and Alzheimer's Disease Center (E.R., A.M., C.W., S.W., M.-M.M.), and Departments of Psychiatry and Behavioral Sciences (D.C., S.W.), Preventative Medicine (A.R.), and Neurology (M.-M.M.), Northwestern University Feinberg School of Medicine, Chicago, IL.

Abstract

OBJECTIVE:

The aim of this study was to provide quantitative measures of changes in cortical atrophy over a 2-year period associated with 3 subtypes of primary progressive aphasia (PPA) using whole-brain vertex-wise and region-of-interest (ROI) neuroimaging methods. The purpose was to quantitate disease progression, establish an empirical basis for clinical expectations, and provide outcome measures for therapeutic trials.

METHODS:

Changes in cortical thickness and volume loss as well as neuropsychological performance were assessed at baseline and 2-year follow-up in 26 patients who fulfilled criteria for logopenic (8 patients), agrammatic (10 patients), and semantic (8 patients) PPA subtypes. Whole-brain vertex-wise and ROI imaging analysis were conducted using the FreeSurfer longitudinal pipeline.

RESULTS:

Clinical deficits and cortical atrophy patterns showed distinct patterns of change among the subtypes over 2 years. Results confirmed that progression for each of the 3 subtypes showed left greater than right hemisphere asymmetry. An ROI analysis also revealed that progression was greater within, rather than outside, the language network.

CONCLUSIONS:

Preferential neurodegeneration of the left hemisphere language network is a common denominator for all 3 PPA subtypes, even as the disease progresses. Using a focal cortical language network ROI as an outcome measure of disease progression appears to be more sensitive than whole-brain or ventricular volume measures of change and may be helpful for designing future clinical trials in PPA.

PMID:
25165386
PMCID:
PMC4176026
DOI:
10.1212/WNL.0000000000000824
[Indexed for MEDLINE]
Free PMC Article

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