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

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 Abeta and alpha-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.

journal_name

Acta Neuropathol

journal_title

Acta neuropathologica

authors

Powers JM,Byrne NP,Ito M,Takao M,Yankopoulou D,Spillantini MG,Ghetti B

doi

10.1007/s00401-003-0719-9

subject

Has Abstract

pub_date

2003-08-01 00:00:00

pages

181-7

issue

2

eissn

0001-6322

issn

1432-0533

journal_volume

106

pub_type

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