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

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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.

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Acknowledgements

The authors thank Tina Blazey for her usual outstanding secretarial assistance; Dr. Mark Forman, Neuropathology, University of Pennsylvania, for confirmatory α-synuclein and ubiquitin immunostaining in our patient and Dr. Peter Davies, Albert Einstein College of Medicine, for his generous donation of Alz-50, PHF-1, TG3, PG5, CP3 and CP13 antibodies. We also thank L. Miravalle and J.R. Murrell for their efforts. Supported in part by P30AG10133.

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Authors and Affiliations

  1. Department of Pathology and Laboratory Medicine, University of Rochester School of Medicine and Dentistry, Box 626, 601 Elmwood Avenue, Rochester, NY, 14642, USA

    J. M. Powers, N. P. Byrne & M. Ito

  2. Department of Pathology and Laboratory Medicine, Indiana University Medical Center, Indianapolis, Indiana, USA

    M. Takao & B. Ghetti

  3. Centre for Brain Repair, University of Cambridge, Cambridge, UK

    D. Yankopoulou & M. G. Spillantini

Authors
  1. J. M. Powers
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  2. N. P. Byrne
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  3. M. Ito
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  4. M. Takao
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  5. D. Yankopoulou
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  6. M. G. Spillantini
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  7. B. Ghetti
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Correspondence to J. M. Powers.

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Powers, J.M., Byrne, N.P., Ito, M. et al. A novel leukoencephalopathy associated with tau deposits primarily in white matter glia. Acta Neuropathol 106, 181–187 (2003). https://doi.org/10.1007/s00401-003-0719-9

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  • DOI: https://doi.org/10.1007/s00401-003-0719-9

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