Frontal lobe dementia with novel tauopathy: Sporadic multiple system tauopathy with dementia

Eileen H. Bigio, Anne M. Lipton, Shu Hui Yen, Mike L. Hutton, Matt Baker, Parimala Nacharaju, Charles L. White, Peter Davies, Wenlang Lin, Dennis W. Dickson

Research output: Contribution to journalArticle

66 Scopus citations

Abstract

We present a novel tauopathy in a patient with a 10-yr history of progressive frontal lobe dementia and a negative family history. Autopsy revealed mild atrophy of frontal and parietal lobes and severe atrophy of the temporal lobes. There were occasional filamentous tau-positive inclusions, but more interesting were numerous distinctive globular neuronal and glial tau-positive inclusions in both gray and white matter of the neocortex. Affected subcortical regions included substantia nigra, globus pallidus, subthalamic nucleus, and cerebellar dentate nucleus, in a distribution similar to progressive supranuclear palsy (PSP), but without significant accompanying neuronal loss or gliosis. Predominantly straight filaments were detected by electron microscopy (EM), while other inclusions were similar to fingerprint bodies. No twisted ribbons were detected. Immuno-EM studies revealed that only the filamentous inclusions were composed of tau. Immunoblotting of sarkosyl-insoluble tau revealed 2 major bands of 64 and 68 kDa. Blotting analysis after dephosphorylation revealed predominantly 4-repeat tau. Sequence analysis of tau revealed that there were no mutations in either exons 9-13 or the adjacent intronic sequences. The unique cortical tau pathology in this case of sporadic multiple system tauopathy with dementia adds a new pathologic profile to the spectrum of tauopathies.

Original languageEnglish (US)
Pages (from-to)328-341
Number of pages14
JournalJournal of Neuropathology and Experimental Neurology
Volume60
Issue number4
DOIs
StatePublished - 2001
Externally publishedYes

Keywords

  • Corticobasal ganglionic degeneration
  • Dementia
  • FTDP-17
  • Frontal lobe dementia
  • Frontotemporal degeneration
  • Progressive supranuclear palsy
  • Tauopathy

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Neurology
  • Clinical Neurology
  • Cellular and Molecular Neuroscience

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    Bigio, E. H., Lipton, A. M., Yen, S. H., Hutton, M. L., Baker, M., Nacharaju, P., White, C. L., Davies, P., Lin, W., & Dickson, D. W. (2001). Frontal lobe dementia with novel tauopathy: Sporadic multiple system tauopathy with dementia. Journal of Neuropathology and Experimental Neurology, 60(4), 328-341. https://doi.org/10.1093/jnen/60.4.328