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Abstract
Objective: To disentangle the clinical heterogeneity of nonsemantic variants of primary progressive aphasia (PPA) and to identify a coherent linguistic-anatomical marker for the logopenic variant of PPA (lv-PPA).
Methods: Key speech and language features of 14 cases of lv-PPA and 18 cases of nonfluent/agrammatic variant of PPA were systematically evaluated and scored by an independent rater blinded to diagnosis. Every case underwent a structural MRI and a Pittsburgh compound B (PiB)-PET scan, a putative biomarker of Alzheimer disease. Key speech and language features that showed association with the PiB-PET status were entered into a hierarchical cluster analysis. The linguistic features and patterns of cortical thinning in each resultant cluster were analyzed.
Results: The cluster analysis revealed 3 coherent clinical groups, each of which was linked to a specific PiB-PET status. The first cluster was linked to high PiB retention and characterized by phonologic errors and cortical thinning focused on the left superior temporal gyrus. The second and third clusters were characterized by grammatical production errors and motor speech disorders, respectively, and were associated with low PiB brain retention. A fourth cluster, however, demonstrated nonspecific language deficits and unpredictable PiB-PET status.
Conclusions: These findings suggest that despite the clinical and pathologic heterogeneity of nonsemantic variants, discrete clinical syndromes can be distinguished and linked to specific likelihood of PiB-PET status. Phonologic errors seem to be highly predictive of high amyloid burden in PPA and can provide a specific clinical marker for lv-PPA.
GLOSSARY
- ACE-R=
- Addenbrooke's Cognitive Examination revised;
- AD=
- Alzheimer disease;
- ICC=
- International Consensus Criteria;
- lv-PPA=
- logopenic variant of primary progressive aphasia;
- nfv-PPA=
- nonfluent/agrammatic variant of primary progressive aphasia;
- PiB=
- Pittsburgh compound B;
- PPA=
- primary progressive aphasia;
- sv-PPA=
- semantic variant of primary progressive aphasia
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 September 18, 2013.
- Accepted in final form February 3, 2014.
- © 2014 American Academy of Neurology
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