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Jennifer L. Whitwell, BA; Keith A. Josephs, MST, MD; Martin N. Rossor, MD, FRCP; John M. Stevens, DRACR, FRCR; Tamas Revesz, MD; Janice L. Holton, MB, ChB, PhD; Safa Al-Sarraj, MSc, FRCPath; Alison K. Godbolt, MA, MRCP; Nick C. Fox, MD, MRCP; Jason D. Warren, FRACP

Arch Neurol. 2005;62:1402-1408.

Background  The pathologic substrates of frontotemporal dementia (FTD) are difficult to predict in vivo.

Objective  To determine whether different pathologic substrates of FTD have distinct patterns of regional atrophy on magnetic resonance imaging (MRI).

Design  Retrospective case study.

Setting  The Institute of Neurology, University College London, and the Institute of Psychiatry, King’s College London.

Patients  Twenty-one cases of FTD selected on pathologic grounds (9 with ubiquitin-positive [tau- and -synuclein–negative] inclusions [FTD-U], 7 with Pick disease [PiD], and 5 with familial FTD with tau exon 10+16 mutations [tau exon 10+16]) and 20 healthy controls were studied.

Main Outcome Measures  Patterns of gray matter atrophy in each group as assessed by voxel-based morphometry (VBM) and a blinded visual assessment of each MRI study.

Results  All pathologic substrates were associated with atrophy that involved the inferior and medial temporal and inferior frontal lobes. Additionally, specific VBM signatures were identified for each subgroup: FTD-U was associated with asymmetric (left > right) temporal lobe atrophy, PiD was associated with severe dorsolateral bifrontal atrophy, and tau exon 10+16 was associated with asymmetric (right > left) medial temporal lobe atrophy. The VBM findings were supported by blinded visual assessment.

Conclusion  These findings suggest that MRI patterns of regional gray matter atrophy constitute signatures of tissue pathology in FTD.

Author Affiliations: Dementia Research Centre, Institute of Neurology, University College London, London, England (Ms Whitwell and Drs Josephs, Rossor, Godbolt, Fox, and Warren); Department of Neurology, Mayo Clinic, Rochester, Minn (Dr Josephs); Division of Neuroscience and Psychological Medicine, Imperial College, London (Dr Rossor); Departments of Neuroradiology (Dr Stevens) and Molecular Neuroscience (Drs Revesz and Holton), Institute of Neurology, London; and Department of Neuropathology, Institute of Psychiatry, King’s College London, London (Dr Al-Sarraj).

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