Pure vascular dementia mimicking the behavioural variant of frontotemporal lobar degeneration : about 3 autopsied cases. V. Deramecourt 1, 2, 3 , F. Lebert 1, 2 , C.A. Maurage 1, 3, 4 , F. Pasquier 1, 2. 1 Univ. Lille Nord de France, EA1046, UDSL, Lille, France
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Pure vascular dementia mimicking the behavioural variant of frontotemporal lobar degeneration : about 3 autopsied cases
V. Deramecourt1, 2, 3, F. Lebert1, 2, C.A. Maurage1, 3, 4, F. Pasquier1, 2
1 Univ. Lille Nord de France, EA1046, UDSL, Lille, France
2 Lille University Hospital, Memory Clinic, French national reference centre for young onset Alzheimer patients, Lille, France
3 Univ. Lille Nord de France, Histology department, UDSL, Lille, France
4 Lille University Hospital, Pathology Department, Functional unit of Neuropathology, Lille, France
Early behavioral disinhibition, apathy, loss of empathy, perseverative behavior, dietary changes and decline in executive tasks are core features of the behavioral variant of frontotemporal lobar degeneration (bvFTD). Recent reports have highlighted atypical cases (phenocopy cases) characterized by a long lasting, very slowly or non progressive presentation of bvFTD but neuropathological data are usually unavailable.
Calcified arteriolosclerosis, perivascular space dilatation and microlacunes in case 2. (pallidum, H&E staining)
Mild atrophy in case 3, no macroscopic visible vascular lesion.
« état criblé » of the putamen in case 3 (H&E staining)
Mild atrophy in case 2.
Scattered white matter discoloration.
* Rascovsky et al, Brain 2011, in press. (http://memory.ucsf.edu/ftd/medical/diagnosis/criteria/multiple/bvftd)