Journal Article

Demographic, neurological and behavioural characteristics and brain perfusion SPECT in frontal variant of frontotemporal dementia

Isabelle Le Ber, Eric Guedj, Audrey Gabelle, Patrice Verpillat, Magali Volteau, Catherine Thomas-Anterion, Marielle Decousus, Didier Hannequin, Pierre Véra, Lucette Lacomblez, Agnès Camuzat, Mira Didic, Michèle Puel, Jean-Albert Lotterie, Véronique Golfier, Anne-Marie Bernard, Martine Vercelletto, Christine Magne, François Sellal, Izzie Namer, Bernard-François Michel, Jacques Pasquier, François Salachas, Jean Bochet, Alexis Brice, Marie-Odile Habert and Bruno Dubois

in Brain

Published on behalf of The Guarantors of Brain

Volume 129, issue 11, pages 3051-3065
ISSN: 0006-8950
Published online November 2006 | e-ISSN: 1460-2156 | DOI: http://dx.doi.org/10.1093/brain/awl288
Demographic, neurological and behavioural characteristics and brain perfusion SPECT in frontal variant of frontotemporal dementia

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We conducted a French multicentric cross-sectional study to describe in detail the demographic, neurological and behavioural characteristics of the frontal variant of frontotemporal dementia (fvFTD) and to characterize the pattern of brain perfusion SPECT in comparison to a healthy control group. A total of 68 fvFTD patients had technetium-99m-ECD brain perfusion SPECT at inclusion, 61 of which also underwent an in-depth evaluation including 70 items assessing behaviour, language and affect/emotion at onset and at inclusion. The mean age-at-onset was 60.4 ± 7.8 years (35–75). Twenty-six per cent of the patients were older than 65 at onset. A positive familial history consistent with an autosomal dominant inheritance was found in 18% of the patients. At onset, the behavioural profile was predominantly inert in 25% of the patients, disinhibited in 18% and mixed in others. The behavioural features progressed to predominantly mixed or inert forms. Although, inertia was associated with predominant medial frontal and cingulate hypoperfusion, and patients with disinhibition exhibited predominant ventromedial prefrontal and temporal hypoperfusion, there were no major clinical differences between disinhibited and inert patients. Forty-five per cent of the deceased patients survived <6 years (short survival), and 34% of the patients survived >8 years (long survival). This shows that the final outcome of fvFTD is highly variable. No clinical factors predictive of short or long survival were identified. Unexpected, however, was the finding that brainstem hypoperfusion distinguished patients with a short survival from patients with long survival. In conclusion, this study shows that fvFTD is clinically a rather homogeneous entity. It also provides evidence that different behavioural presentations at onset are related to different anatomical localizations of degenerative damage. Finally, it demonstrates the prognostic value of brainstem hypoperfusion in a subgroup of patients with a short survival.

Keywords: frontotemporal lobar degeneration; fvFTD; frontotemporal dementia; MAPT gene; SPECT; SPM

Journal Article.  8522 words.  Illustrated.

Subjects: Neurology ; Neuroscience

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