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Nosology of Primary Progressive Aphasia and the Neuropathology of Language

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Frontotemporal Dementias

Part of the book series: Advances in Experimental Medicine and Biology ((AEMB,volume 1281))

Abstract

Primary progressive aphasia (PPA) is a dementia syndrome associated with several neuropathologic entities, including Alzheimer’s disease (AD) and all major forms of frontotemporal lobar degeneration (FTLD). It is classified into subtypes defined by the nature of the language domain that is most impaired. The asymmetric neurodegeneration of the hemisphere dominant for language (usually left) is one consistent feature of all PPA variants. This feature offers unique opportunities for exploring mechanisms of selective vulnerability in neurodegenerative diseases and the neuroanatomy of language. This chapter reviews some of the current trends in PPA research as well as the challenges that remain to be addressed on the nosology, clinicopathologic correlations, and therapy of this syndrome.

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Acknowledgments

SUPPORT: R01 DCOO8552 and K23 DC014303 from the National Institute of Deafness and Communication Disorders; P30 AG013854 and R01 AG056258 from the National Institute on Aging, R01 NS085770 from the National Institute of Neurological Disorders and Stroke, the Davee Foundation and the Jeanine Jones Fund.

Author information

Authors and Affiliations

  1. Mesulam Center for Cognitive Neurology and Alzheimer’s Disease; Department of Neurology, Northwestern University, Chicago, IL, USA

    M. -Marsel Mesulam & Borna Bonakdarpour

  2. Mesulam Center for Cognitive Neurology and Alzheimer’s Disease, Northwestern University, Chicago, IL, USA

    Christina Coventry, Changiz Geula & Sandra Weintraub

  3. Mesulam Center for Cognitive Neurology and Alzheimer’s Disease; Department of Pathology, Northwestern University, Chicago, IL, USA

    Eileen H. Bigio

  4. Mesulam Center for Cognitive Neurology and Alzheimer’s Disease; Department of Communication Sciences and Disorders; Department of Neurology, Northwestern University, Evanston, IL, USA

    Cynthia Thompson

  5. Mesulam Center for Cognitive Neurology and Alzheimer’s Disease; Department of Psychiatry, Northwestern University, Chicago, IL, USA

    Tamar Gefen & Emily J. Rogalski

Authors
  1. M. -Marsel Mesulam
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  2. Christina Coventry
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  3. Eileen H. Bigio
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  4. Changiz Geula
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  5. Cynthia Thompson
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  7. Tamar Gefen
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  8. Emily J. Rogalski
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  9. Sandra Weintraub
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Correspondence to M. -Marsel Mesulam .

Editor information

Editors and Affiliations

  1. Department of Pathology and Laboratory Medicine, Indiana University, Indianapolis, IN, USA

    Bernardino Ghetti

  2. International Centre for Genetic Engineering and Biotechnology (ICGEB), Trieste, Italy

    Emanuele Buratti

  3. Department of Neurology, Mayo Clinic, Rochester, MN, USA

    Bradley Boeve

  4. VIB Center for Molecular Neurology University of Antwerp-CDE Antwerpen, Belgium, Department of Neuroscience Mayo Clinic Jacksonville, FL, USA, Antwerpen, Belgium

    Rosa Rademakers

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Mesulam, M.M. et al. (2021). Nosology of Primary Progressive Aphasia and the Neuropathology of Language. In: Ghetti, B., Buratti, E., Boeve, B., Rademakers, R. (eds) Frontotemporal Dementias . Advances in Experimental Medicine and Biology, vol 1281. Springer, Cham. https://doi.org/10.1007/978-3-030-51140-1_3

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