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April 01, 2014; 82 (13) Article

Quantitative application of the primary progressive aphasia consensus criteria

Meredith R. Wicklund, Joseph R. Duffy, Edythe A. Strand, Mary M. Machulda, Jennifer L. Whitwell, Keith A. Josephs
First published March 5, 2014, DOI: https://doi.org/10.1212/WNL.0000000000000261
Meredith R. Wicklund
From the Division of Behavioral Neurology (M.R.W., K.A.J.), Speech Pathology, Department of Neurology (J.R.D., E.A.S.), Division of Neurocognitive Disorders, Department of Psychiatry and Psychology (M.M.M.), and Division of Radiology Research, Department of Radiology (J.L.W.), Mayo Clinic, Rochester, MN.
MD
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Joseph R. Duffy
From the Division of Behavioral Neurology (M.R.W., K.A.J.), Speech Pathology, Department of Neurology (J.R.D., E.A.S.), Division of Neurocognitive Disorders, Department of Psychiatry and Psychology (M.M.M.), and Division of Radiology Research, Department of Radiology (J.L.W.), Mayo Clinic, Rochester, MN.
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Edythe A. Strand
From the Division of Behavioral Neurology (M.R.W., K.A.J.), Speech Pathology, Department of Neurology (J.R.D., E.A.S.), Division of Neurocognitive Disorders, Department of Psychiatry and Psychology (M.M.M.), and Division of Radiology Research, Department of Radiology (J.L.W.), Mayo Clinic, Rochester, MN.
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Mary M. Machulda
From the Division of Behavioral Neurology (M.R.W., K.A.J.), Speech Pathology, Department of Neurology (J.R.D., E.A.S.), Division of Neurocognitive Disorders, Department of Psychiatry and Psychology (M.M.M.), and Division of Radiology Research, Department of Radiology (J.L.W.), Mayo Clinic, Rochester, MN.
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Jennifer L. Whitwell
From the Division of Behavioral Neurology (M.R.W., K.A.J.), Speech Pathology, Department of Neurology (J.R.D., E.A.S.), Division of Neurocognitive Disorders, Department of Psychiatry and Psychology (M.M.M.), and Division of Radiology Research, Department of Radiology (J.L.W.), Mayo Clinic, Rochester, MN.
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Keith A. Josephs
From the Division of Behavioral Neurology (M.R.W., K.A.J.), Speech Pathology, Department of Neurology (J.R.D., E.A.S.), Division of Neurocognitive Disorders, Department of Psychiatry and Psychology (M.M.M.), and Division of Radiology Research, Department of Radiology (J.L.W.), Mayo Clinic, Rochester, MN.
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Citation
Quantitative application of the primary progressive aphasia consensus criteria
Meredith R. Wicklund, Joseph R. Duffy, Edythe A. Strand, Mary M. Machulda, Jennifer L. Whitwell, Keith A. Josephs
Neurology Apr 2014, 82 (13) 1119-1126; DOI: 10.1212/WNL.0000000000000261

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Abstract

Objective: To determine how well the consensus criteria could classify subjects with primary progressive aphasia (PPA) using a quantitative speech and language battery that matches the test descriptions provided by the consensus criteria.

Methods: A total of 105 participants with a neurodegenerative speech and language disorder were prospectively recruited and underwent neurologic, neuropsychological, and speech and language testing and MRI in this case-control study. Twenty-one participants with apraxia of speech without aphasia served as controls. Select tests from the speech and language battery were chosen for application of consensus criteria and cutoffs were employed to determine syndromic classification. Hierarchical cluster analysis was used to examine participants who could not be classified.

Results: Of the 84 participants, 58 (69%) could be classified as agrammatic (27%), semantic (7%), or logopenic (35%) variants of PPA. The remaining 31% of participants could not be classified. Of the unclassifiable participants, 2 clusters were identified. The speech and language profile of the first cluster resembled mild logopenic PPA and the second cluster semantic PPA. Gray matter patterns of loss of these 2 clusters of unclassified participants also resembled mild logopenic and semantic variants.

Conclusions: Quantitative application of consensus PPA criteria yields the 3 syndromic variants but leaves a large proportion unclassified. Therefore, the current consensus criteria need to be modified in order to improve sensitivity.

GLOSSARY

agPPA=
agrammatic variant of PPA;
AOS=
apraxia of speech;
BNT=
Boston Naming Test;
DKEFS=
Delis-Kaplan Executive Function System Card Sort;
lvPPA=
logopenic variant of PPA;
MoCA=
Montreal Cognitive Assessment battery;
PPA=
primary progressive aphasia;
PPAOS=
primary progressive apraxia of speech;
PPTT=
Pyramids and Palm Trees Test;
Rey-O=
Rey-Osterrieth Complex Figure Test;
svPPA=
semantic variant of PPA;
WAB=
Western Aphasia Battery–revised;
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.

  • Editorial, page 1108

  • Supplemental data at Neurology.org

  • Received July 5, 2013.
  • Accepted in final form November 19, 2013.
  • © 2014 American Academy of Neurology
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