Acta Neuropathologica

, Volume 106, Issue 6, pp 575–583 | Cite as

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

  • Claude-Alain Maurage
  • Nicolas Sergeant
  • Susanna Schraen-Maschke
  • Florence Lebert
  • Marie-Magdeleine Ruchoux
  • Bernard Sablonnière
  • Florence Pasquier
  • André DelacourteEmail author
Regular Paper


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.


Tau protein Argyrophilic grain dementia Argyrophilic grain Coiled body Tauopathy 



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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Copyright information

© Springer-Verlag 2003

Authors and Affiliations

  • Claude-Alain Maurage
    • 1
    • 3
  • Nicolas Sergeant
    • 1
  • Susanna Schraen-Maschke
    • 1
    • 4
  • Florence Lebert
    • 2
    • 5
  • Marie-Magdeleine Ruchoux
    • 2
    • 3
  • Bernard Sablonnière
    • 1
    • 4
  • Florence Pasquier
    • 2
    • 5
  • André Delacourte
    • 1
    Email author
  1. 1.INSERM U422Faculté de Médecine, Université de Lille 2LilleFrance
  2. 2.EA 2691Faculté de Médecine et Université de Lille 2LilleFrance
  3. 3.Department of NeuropathologyCentre Hospitalier Régional UniversitaireLilleFrance
  4. 4.Department of Biochemistry and Molecular BiologyCentre Hospitalier Régional UniversitaireLilleFrance
  5. 5.Department of NeurologyCentre Hospitalier Régional UniversitaireLilleFrance

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