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      Leukoaraiosis Severity Predicts Rate of Decline in Primary Progressive Aphasia

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          Abstract

          BACKGROUND:

          The rate of decline in language in Primary Progressive Aphasia (PPA) is highly variable and difficult to predict at baseline. The severity of diffuse white matter disease (leukoaraiosis), a marker of overall brain health, may substantially influence the rate of decline.

          AIMS:

          To test the hypothesis that leukoaraiosis is associated with a steeper decline in naming in PPA.

          METHODS AND PROCEDURES:

          In this longitudinal, observational study, 29 individuals with PPA (all variants) were administered the Boston Naming Test (BNT) at baseline and 1 year later. Two raters evaluated leukoaraiosis on baseline MRI, using the Cardiovascular Health Study scale. We evaluated the effects of leukoaraiosis severity, age, education, and baseline BNT on decline measured by change in BNT accuracy with multivariable linear regression. We also evaluated the effects of these variables on the dichotomized outcome of faster decline in BNT (worst 50%) versus slower decline (best 50%) using logistic regression.

          RESULTS:

          Together, leukoaraiosis, age, education, and baseline BNT score predicted change in BNT score (F(3, 25) = 8.12; p=0.0006). Change in BNT score was predicted by severity of leukoaraiosis (t =−3.81; p=0.001) and education (t= −2.45; p=0.022), independently of the other variables. When we dichotomized outcome into upper 50 th percentile versus lower 50 th percentile (faster decline), faster decline was predicted by all variables together (chi squared = 13.91; p = 0.008). However, only leukoaraiosis independently predicted outcome (OR=2.80; 95%CI: 1.11 to 7.03). For every 1 point increase on the CHS rating scale, there was 2.8 times higher chance of showing faster decline in naming.

          CONCLUSION:

          Severity of leukoaraiosis is associated with steeper decline in naming in PPA. This imaging marker can aide in prognosis and planning by caregivers and stratification of participants in clinical trials.

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          Author and article information

          Journal
          8708531
          25619
          Aphasiology
          Aphasiology
          Aphasiology
          0268-7038
          1464-5041
          21 March 2019
          23 March 2019
          2020
          01 January 2021
          : 34
          : 3
          : 365-375
          Affiliations
          [1 ]Department of Neurology, Johns Hopkins University School of Medicine
          [2 ]Department of Physical Medicine & Rehabilitation, Johns Hopkins University School of Medicine
          [3 ]Department of Otolaryngology & Head & Neck Surgery, Johns Hopkins University School of Medicine
          [4 ]Department of Cognitive Science, Johns Hopkins University
          Author notes
          Corresponding Author: Argye E. Hillis, MD, Department of Neurology, Physical Medicine & Rehabilitation, and Cognitive Science, 600 N Wolfe Street, Phipps 446, Johns Hopkins University School of Medicine, Baltimore, MD 21287, USA, argye@ 123456jhmi.edu , Phone: +1 410-614-2381, Fax: +1 410-614-9807
          [*]

          Mr. Odolil has no academic degree; he is an undergraduate student

          Article
          PMC7202552 PMC7202552 7202552 nihpa1523962
          10.1080/02687038.2019.1594152
          7202552
          32377026
          b83ba24b-586f-4a07-b625-6f2c70e9b1a5
          History
          Categories
          Article

          primary progressive aphasia,white matter disease,prognosis,leukoaraiosis

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