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Neurology 2002;59:1585-1594
© 2002 American Academy of Neurology

Chromosome 3 linked frontotemporal dementia (FTD-3)

S. Gydesen, MD, J.M. Brown, MD, A. Brun, MD, L. Chakrabarti, DPhil, A. Gade, PhD, P. Johannsen, MD PhD, M. Rossor, MD, T. Thusgaard, A. Grove, MD, D. Yancopoulou, M.G. Spillantini, PhD, E.M.C. Fisher, PhD, J. Collinge, MD and S.A. Sorensen, MD DMS

From the Frontotemporal Dementia Research in Jutland Association (FreJA),, Denmark; Institute of Medical Genetics (Drs. Gydesen and Sorensen, and T. Thusgaard), The Panum Institute, University of Copenhagen, Denmark; Department of Psychiatry (Dr. Gydesen), Central Hospital, Holbaek, Denmark; Department of Neurology (Dr. Brown), Addenbrooke’s Hospital, Cambridge, United Kingdom; Department of Neuropathology (Dr. Brun), University Hospital, Lund, Sweden; MRC Prion Unit (Drs. Chakrabarti, Fisher, and Collinge), Department of Neurodegenerative Disease, Institute of Neurology, London, United Kingdom; Department of Neurogenetics (Drs. Chakrabarti, Fisher, and Collinge), Imperial College Medical School, London, United Kingdom; Department of Psychology (Dr. Gade), Copenhagen University, Denmark; Memory Disorders Clinic (Dr. Gade), University Hospital, Copenhagen, Denmark; Department of Neurology and PET Centre (Dr. Johannsen), Aarhus University Hospitals, Denmark; Dementia Research Group (Dr. Rossor), National Hospital for Neurology, Queen Square, London, United Kingdom; Department of Pathology (Dr. Grove), Holstebro Hospital, Denmark; and Brain Repair Centre and Department of Neurology (D. Yancopoulou and Dr. Spillantini), University of Cambridge, United Kingdom.

Address correspondence and reprint requests to Dr. J.M. Brown, Department of Neurology, Addenbrooke’s Hospital, Hills Road, Cambridge, CB2 2QQ, UK; e-mail: jmb75{at}medschl.cam.ac.uk

Background: The authors have identified and studied a large kindred in which frontotemporal dementia (FTD) is inherited as an autosomal dominant trait. The trait has been mapped to the pericentromeric region of chromosome 3.

Methods: The authors report on the clinical, neuroimaging, neuropsychological, and pathologic features in this unique pedigree collected during 17 years of study.

Results: Twenty-two individuals in three generations have been affected; the age at onset varies between 46 and 65 years. The disease presents with a predominantly frontal lobe syndrome but there is also evidence for temporal and dominant parietal lobe dysfunction. Late in the illness individuals develop a florid motor syndrome with pyramidal and extrapyramidal features. Structural imaging reveals generalized cerebral atrophy; H215O-PET scanning in two individuals relatively early and late in the disease shows a striking global reduction in cerebral blood flow affecting all lobes. On macroscopic pathologic examination, there is generalized cerebral atrophy affecting the frontal lobes preferentially. Microscopically, there is neuronal loss and gliosis without specific histopathologic features.

Conclusions: FTD-3 shares clinical and pathologic features with other forms of FTD and fulfills international consensus criteria for FTD. There is involvement of the parietal lobes clinically, radiologically, and pathologically in FTD-3 in contrast to some forms of FTD. This more diffuse involvement of the cerebral cortex leads to a distinctive, global pattern of reduced blood flow on PET scanning.




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