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

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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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Acknowledgements

This study was supported by the Ministère de l’Enseignement National, de la Recherche et de la Technologie (MENRT/EA 2691) and INSERM. The authors wish to thank Monique Henneron, Véronique Dumetz, Annick Wattez and Véronique Vervaeck for technical assistance.

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Authors and Affiliations

  1. INSERM U422, Faculté de Médecine, Université de Lille 2, 1 place de Verdun, 59045 Cedex, Lille, France

    Claude-Alain Maurage, Nicolas Sergeant, Susanna Schraen-Maschke, Bernard Sablonnière & André Delacourte

  2. EA 2691, Faculté de Médecine et Université de Lille 2, 59045 Cedex, Lille, France

    Florence Lebert, Marie-Magdeleine Ruchoux & Florence Pasquier

  3. Department of Neuropathology, Centre Hospitalier Régional Universitaire, 59037 cedex, Lille, France

    Claude-Alain Maurage & Marie-Magdeleine Ruchoux

  4. Department of Biochemistry and Molecular Biology, Centre Hospitalier Régional Universitaire, 59037 cedex, Lille, France

    Susanna Schraen-Maschke & Bernard Sablonnière

  5. Department of Neurology, Centre Hospitalier Régional Universitaire, 59037 cedex, Lille, France

    Florence Lebert & Florence Pasquier

Authors
  1. Claude-Alain Maurage
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  2. Nicolas Sergeant
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  3. Susanna Schraen-Maschke
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  4. Florence Lebert
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  8. André Delacourte
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Correspondence to André Delacourte.

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Maurage, CA., Sergeant, N., Schraen-Maschke, S. et al. Diffuse form of argyrophilic grain disease: a new variant of four-repeat tauopathy different from limbic argyrophilic grain disease. Acta Neuropathol 106, 575–583 (2003). https://doi.org/10.1007/s00401-003-0762-6

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  • DOI: https://doi.org/10.1007/s00401-003-0762-6

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