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Nonfluent-Agrammatic Variant Primary Progressive Aphasia

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Hybrid PET/MR Neuroimaging

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Abstract

Nonfluent/agrammatic variant primary progressive aphasia (nfvPPA) is the most diverse of the three PPA syndromes. Classically, patients present with a progressive language-predominant disturbance characterized by agrammatism in language production and apraxia of speech. While pathologically considered a tauopathy in etiology, a variety of other associated underlying pathologies have been identified, including TAR DNA-binding protein 43 (TDP-43) and Alzheimer’s disease (AD) pathologies. Imaging can further support the diagnosis when progressive regional atrophy, metabolic impairment, or abnormal tau PET uptake is present, specifically asymmetric involvement of the inferior frontal, opercular, and insular regions of the dominant hemisphere, commonly the left. This chapter reviews the unique entity of nfvPPA and its currently recognized clinical presentation, diagnostic criteria, multimodal imaging features, histopathologic biomarkers, and treatment options.

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Authors and Affiliations

  1. Department of Radiology, NewYork-Presbyterian Hospital/Weill Cornell Medicine, New York, NY, USA

    Michelle Roytman

  2. Zucker Hillside Hospital, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, The Litwin-Zucker Research Center, The Feinstein Institutes for Medical Research, Manhasset, NY, USA

    Marc L. Gordon

  3. Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, The Feinstein Institutes for Medical Research, Manhasset, NY, USA

    Ana M. Franceschi

  4. Division of Neuroradiology, Department of Radiology, Lenox Hill Hospital, Northwell Health, New York, NY, USA

    Ana M. Franceschi

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  1. Michelle Roytman
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Editors and Affiliations

  1. Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, The Feinstein Institutes for Medical Research, Manhasset, NY, USA

    Ana M. Franceschi

  2. Stony Brook University Hospital, Stony Brook, NY, USA

    Dinko Franceschi

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Roytman, M., Gordon, M.L., Franceschi, A.M. (2022). Nonfluent-Agrammatic Variant Primary Progressive Aphasia. In: Franceschi, A.M., Franceschi, D. (eds) Hybrid PET/MR Neuroimaging. Springer, Cham. https://doi.org/10.1007/978-3-030-82367-2_29

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