A novel leukoencephalopathy associated with tau deposits primarily in white matter glia

James M. Powers, N. P. Byrne, M. Ito, M. Takao, D. Yankopoulou, M. G. Spillantini, Bernardino Ghetti

Research output: Contribution to journalArticle

28 Citations (Scopus)

Abstract

A 79-year-old woman had a 10-year history of dementia, initially presenting as non-fluent aphasia. Magnetic resonance imaging showed frontal atrophy (left greater than right) and hyperintense foci within white matter. Neuropathologically, there was severe frontal atrophy due to cortical neuronal loss with spongy change and to an even greater loss of white matter that contained prominent eosinophilic deposits. The deposits were immunoreactive for phosphorylated tau, non-reactive for Aβ and α-synuclein and equivocally or weakly reactive for ubiquitin. They stained with the Gallyas, Bielschowsky, and Bodian techniques. Ultrastructural examination revealed the deposits to be composed of straight filaments with a diameter of approximately 10 nm, primarily in white matter glia. Moderate loss of neurons in substantia nigra and numerous argyrophilic threads in gray and particularly white matter were noted. The precise relationship between this disorder and other frontotemporal degenerations/tauopathies, as well as the pathogenetic basis of the leukoencephalopathy, remains to be determined.

Original languageEnglish
Pages (from-to)181-187
Number of pages7
JournalActa Neuropathologica
Volume106
Issue number2
DOIs
StatePublished - Aug 2 2003

Fingerprint

Leukoencephalopathies
Neuroglia
Atrophy
Synucleins
Tauopathies
Aphasia
Substantia Nigra
Ubiquitin
Dementia
Magnetic Resonance Imaging
Neurons
White Matter

Keywords

  • Frontotemporal degeneration
  • Leukoencephalopathy
  • Progressive aphasia
  • Tauopathy

ASJC Scopus subject areas

  • Clinical Neurology
  • Pathology and Forensic Medicine
  • Neuroscience(all)

Cite this

Powers, J. M., Byrne, N. P., Ito, M., Takao, M., Yankopoulou, D., Spillantini, M. G., & Ghetti, B. (2003). A novel leukoencephalopathy associated with tau deposits primarily in white matter glia. Acta Neuropathologica, 106(2), 181-187. https://doi.org/10.1007/s00401-003-0719-9

A novel leukoencephalopathy associated with tau deposits primarily in white matter glia. / Powers, James M.; Byrne, N. P.; Ito, M.; Takao, M.; Yankopoulou, D.; Spillantini, M. G.; Ghetti, Bernardino.

In: Acta Neuropathologica, Vol. 106, No. 2, 02.08.2003, p. 181-187.

Research output: Contribution to journalArticle

Powers, JM, Byrne, NP, Ito, M, Takao, M, Yankopoulou, D, Spillantini, MG & Ghetti, B 2003, 'A novel leukoencephalopathy associated with tau deposits primarily in white matter glia', Acta Neuropathologica, vol. 106, no. 2, pp. 181-187. https://doi.org/10.1007/s00401-003-0719-9
Powers JM, Byrne NP, Ito M, Takao M, Yankopoulou D, Spillantini MG et al. A novel leukoencephalopathy associated with tau deposits primarily in white matter glia. Acta Neuropathologica. 2003 Aug 2;106(2):181-187. https://doi.org/10.1007/s00401-003-0719-9
Powers, James M. ; Byrne, N. P. ; Ito, M. ; Takao, M. ; Yankopoulou, D. ; Spillantini, M. G. ; Ghetti, Bernardino. / A novel leukoencephalopathy associated with tau deposits primarily in white matter glia. In: Acta Neuropathologica. 2003 ; Vol. 106, No. 2. pp. 181-187.
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