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Abstract
Objective: A brief speech expression protocol that can be administered and scored without special training would aid in the differential diagnosis of the 3 principal forms of primary progressive aphasia (PPA): nonfluent/agrammatic PPA, logopenic variant PPA, and semantic variant PPA.
Methods: We used a picture-description task to elicit a short speech sample, and we evaluated impairments in speech-sound production, speech rate, lexical retrieval, and grammaticality. We compared the results with those obtained by a longer, previously validated protocol and further validated performance with multimodal imaging to assess the neuroanatomical basis of the deficits.
Results: We found different patterns of impaired grammar in each PPA variant, and additional language production features were impaired in each: nonfluent/agrammatic PPA was characterized by speech-sound errors; logopenic variant PPA by dysfluencies (false starts and hesitations); and semantic variant PPA by poor retrieval of nouns. Strong correlations were found between this brief speech sample and a lengthier narrative speech sample. A composite measure of grammaticality and other measures of speech production were correlated with distinct regions of gray matter atrophy and reduced white matter fractional anisotropy in each PPA variant.
Conclusions: These findings provide evidence that large-scale networks are required for fluent, grammatical expression; that these networks can be selectively disrupted in PPA syndromes; and that quantitative analysis of a brief speech sample can reveal the corresponding distinct speech characteristics.
GLOSSARY
- AOS=
- apraxia of speech;
- bvFTD=
- behavioral-variant frontotemporal dementia;
- FA=
- fractional anisotropy;
- FDR=
- false discovery rate;
- GM=
- gray matter;
- lvPPA=
- logopenic variant primary progressive aphasia;
- MLU=
- mean length of utterance;
- naPPA=
- nonfluent/agrammatic primary progressive aphasia;
- PPA=
- primary progressive aphasia;
- svPPA=
- semantic variant primary progressive aphasia;
- WM=
- white matter
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 www.neurology.org
- Received December 23, 2012.
- Accepted in final form April 8, 2013.
- © 2013 American Academy of Neurology
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