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Vol. 17, No. 4, 2004
Issue release date: June 2004
Dement Geriatr Cogn Disord 2004;17:274–276
(DOI:10.1159/000077153)

Frontotemporal Dementia Linked to Chromosome 3

Brown J. · Gydesen S. · Johannsen P. · Gade A. · Skibinski G. · Chakrabarti L. · Brun A. · Spillantini M. · Yancopoulou D. · Thusgaard T. · Sorensen A. · Fisher E. · Collinge J.
aDepartment of Neurology, Addenbrooke’s Hospital, Cambridge, bMRC Prion Unit, Institute of Neurology, London, UK; cDepartment of Psychiatry, Central Hospital, Holbaek, dPET Centre, Department of Neurology, Aarhus University Hospitals, Aarhus, eDepartment of Psychology, University of Copenhagen, Rigshospitalet, fInstitute of Medical Genetics, Panum Institute, Copenhagen, Denmark; gDepartment of Pathology, University Hospital of Lund, Lund, Sweden

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Abstract

A large pedigree with autosomal dominant frontotemporal dementia has been identified. Positional cloning has linked the disease gene to the pericentromeric region of chromosome 3. Clinical, neuropsychological, imaging, pathological and molecular genetic data are presented. The genetic mutation responsible for the disease has not been identified.



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References

  1. Gydesen S, Hagen S, Klinken L, Abelskov J, Sorensen SA: Neuropsychiatric studies in a family with presenile dementia different from Alzheimer and Pick disease. Acta Psychiatr Scand 1987;76:276–284.
  2. Brown J, Ashworth A, Gydesen S, Sorensen A, Rossor M, Hardy J, Collinge J: Familial non-specific dementia maps to chromosome 3. Hum Mol Genet 1995;4:1625–1628.
  3. Gydesen S, Brown J, Brun A, Chakrabarti L, Gade A, Johannsen P, Rossor M, Thusgaard T, Grove A, Yancopoulou D, Spillantini MG, Fisher E, Collinge J, Sorensen SA: Chromosome 3 linked frontotemporal dementia (FTD-3). Neurology 2002;59:1585–1594.
  4. Brun A, Englund B, Gustafson L, Passant U, Mann DMA, Neary D, Snowden JS: Consensus statement – Clinical and neuropathological criteria for frontotemporal dementia. J Neurol Neurosurg Psychiatry 1994;57:416–418.


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