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01-12-2003 | Regular Paper

Diffuse form of argyrophilic grain disease: a new variant of four-repeat tauopathy different from limbic argyrophilic grain disease

Authors: Claude-Alain Maurage, Nicolas Sergeant, Susanna Schraen-Maschke, Florence Lebert, Marie-Magdeleine Ruchoux, Bernard Sablonnière, Florence Pasquier, André Delacourte

Published in: Acta Neuropathologica | Issue 6/2003

Abstract

Argyrophilic grain disease (AGD) is characterized by the occurrence of argyrophilic grains and coiled bodies in brain tissue, mainly in limbic areas located in the temporal lobe. Recent biochemical data have shown that inclusions in AGD consist of aggregates of pathological microtubule-associated tau protein isoforms of 64/69 kDa. We report here a study on two AGD patients, belonging to a series of demented patients affected by several tauopathies, prospectively followed until death. In both patients, clinical, neuropathological and biochemical investigations clearly demonstrated AGD. Diffuse tau pathology was shown by Gallyas’ silver stain, tau immunohistochemistry and tau protein variant biochemical analysis, not only in temporal lobes but also in all cortical and subcortical areas that were assessed. Primary motor, primary sensory, and associative brain cortices were involved, as well as brain stem, but not cerebellum. We suggest that “diffuse” AGD might be a subgroup of AGD, the specific profile of which is different from that of “limbic” AGD.

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Title: Diffuse form of argyrophilic grain disease: a new variant of four-repeat tauopathy different from limbic argyrophilic grain disease
Authors: Claude-Alain Maurage
Nicolas Sergeant
Susanna Schraen-Maschke
Florence Lebert
Marie-Magdeleine Ruchoux
Bernard Sablonnière
Florence Pasquier
André Delacourte
Publication date: 01-12-2003
Publisher: Springer-Verlag
Published in: Acta Neuropathologica / Issue 6/2003
Print ISSN: 0001-6322
Electronic ISSN: 1432-0533
DOI: https://doi.org/10.1007/s00401-003-0762-6

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Diffuse form of argyrophilic grain disease: a new variant of four-repeat tauopathy different from limbic argyrophilic grain disease

Abstract

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Abstract

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