Neuroimaging in Frontotemporal Dementia: Heterogeneity and Relationships with Underlying Neuropathology

Neurotherapeutics. 2021 Apr;18(2):728-752. doi: 10.1007/s13311-021-01101-x. Epub 2021 Aug 13.

Abstract

Frontotemporal dementia encompasses a group of clinical syndromes defined pathologically by degeneration of the frontal and temporal lobes. Historically, these syndromes have been challenging to diagnose, with an average of about three years between the time of symptom onset and the initial evaluation and diagnosis. Research in the field of neuroimaging has revealed numerous biomarkers of the various frontotemporal dementia syndromes, which has provided clinicians with a method of narrowing the differential diagnosis and improving diagnostic accuracy. As such, neuroimaging is considered a core investigative tool in the evaluation of neurodegenerative disorders. Furthermore, patterns of neurodegeneration correlate with the underlying neuropathological substrates of the frontotemporal dementia syndromes, which can aid clinicians in determining the underlying etiology and improve prognostication. This review explores the advancements in neuroimaging and discusses the phenotypic and pathologic features of behavioral variant frontotemporal dementia, semantic variant primary progressive aphasia, and nonfluent variant primary progressive aphasia, as seen on structural magnetic resonance imaging and positron emission tomography.

Keywords: Behavioral variant frontotemporal dementia; FTLD-TDP; FTLD-tau; Frontotemporal dementia; Frontotemporal lobar degeneration; Magnetic resonance imaging; Neuroimaging; Nonfluent agrammatic variant primary progressive aphasia; Positron emission tomography; Progressive nonfluent aphasia; Semantic dementia; Semantic variant primary progressive aphasia; TDP-43; Tau.

Publication types

  • Research Support, N.I.H., Extramural
  • Review

MeSH terms

  • Biomarkers / metabolism
  • Brain / diagnostic imaging*
  • Brain / metabolism*
  • Frontotemporal Dementia / diagnostic imaging*
  • Frontotemporal Dementia / genetics
  • Frontotemporal Dementia / metabolism*
  • Genetic Variation / genetics
  • Humans
  • Magnetic Resonance Imaging / trends
  • Neuroimaging / trends*
  • Positron-Emission Tomography / trends
  • tau Proteins / genetics
  • tau Proteins / metabolism

Substances

  • Biomarkers
  • MAPT protein, human
  • tau Proteins