Nosology of Primary Progressive Aphasia and the Neuropathology of Language

M. Marsel Mesulam*, Christina Coventry, Eileen H. Bigio, Changiz Geula, Cynthia Thompson, Borna Bonakdarpour, Tamar Gefen, Emily J. Rogalski, Sandra Weintraub

*Corresponding author for this work

Research output: Chapter in Book/Report/Conference proceedingChapter

16 Scopus citations


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.

Original languageEnglish (US)
Title of host publicationAdvances in Experimental Medicine and Biology
Number of pages17
StatePublished - 2021

Publication series

NameAdvances in Experimental Medicine and Biology
ISSN (Print)0065-2598
ISSN (Electronic)2214-8019


  • Alzheimer
  • Asymmetry
  • Broca
  • Grammar
  • Language
  • Naming
  • Selective vulnerability
  • Wernicke
  • Word comprehension

ASJC Scopus subject areas

  • Biochemistry, Genetics and Molecular Biology(all)


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