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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.
GLOSSARY
- ANOVA=
- analysis of variance;
- eTIV=
- estimated total intracranial volume;
- IFG=
- inferior frontal gyrus;
- LH=
- left hemisphere;
- PPA=
- primary progressive aphasia;
- PPA-G=
- primary progressive aphasia–agrammatic;
- PPA-L=
- primary progressive aphasia–logopenic;
- PPA-S=
- primary progressive aphasia–semantic;
- PSTC=
- perisylvian temporal cortex;
- RH=
- right hemisphere;
- ROI=
- region of interest;
- V1=
- visit 1;
- V2=
- visit 2;
- WAB-AQ=
- Western Aphasia Battery–Aphasia Quotient
Footnotes
Go to Neurology.org for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
Supplemental data at Neurology.org
- Received March 18, 2014.
- Accepted in final form June 30, 2014.
- © 2014 American Academy of Neurology
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