title: Demographic, neurological and behavioural characteristics and brain perfusion SPECT in frontal variant of frontotemporal dementia.
abstract: 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.
authors.affiliation: AP-HP, Hôpital de la Salpêtrière, Centre de Neuropsychologie UPMC, Paris, France.
authors.forename: Isabelle
authors.initials: I
authors.lastname: Le Ber
authors.forename: Eric
authors.initials: E
authors.lastname: Guedj
authors.forename: Audrey
authors.initials: A
authors.lastname: Gabelle
authors.forename: Patrice
authors.initials: P
authors.lastname: Verpillat
authors.forename: Magali
authors.initials: M
authors.lastname: Volteau
authors.forename: Catherine
authors.initials: C
authors.lastname: Thomas-Anterion
authors.forename: Marielle
authors.initials: M
authors.lastname: Decousus
authors.forename: Didier
authors.initials: D
authors.lastname: Hannequin
authors.forename: Pierre
authors.initials: P
authors.lastname: Véra
authors.forename: Lucette
authors.initials: L
authors.lastname: Lacomblez
authors.forename: Agnès
authors.initials: A
authors.lastname: Camuzat
authors.forename: Mira
authors.initials: M
authors.lastname: Didic
authors.forename: Michèle
authors.initials: M
authors.lastname: Puel
authors.forename: Jean-Albert
authors.initials: JA
authors.lastname: Lotterie
authors.forename: Véronique
authors.initials: V
authors.lastname: Golfier
authors.forename: Anne-Marie
authors.initials: AM
authors.lastname: Bernard
authors.forename: Martine
authors.initials: M
authors.lastname: Vercelletto
authors.forename: Christine
authors.initials: C
authors.lastname: Magne
authors.forename: François
authors.initials: F
authors.lastname: Sellal
authors.forename: Izzie
authors.initials: I
authors.lastname: Namer
authors.forename: Bernard-François
authors.initials: BF
authors.lastname: Michel
authors.forename: Jacques
authors.initials: J
authors.lastname: Pasquier
authors.forename: François
authors.initials: F
authors.lastname: Salachas
authors.forename: Jean
authors.initials: J
authors.lastname: Bochet
authors.forename: Alexis
authors.initials: A
authors.lastname: Brice
authors.forename: Marie-Odile
authors.initials: MO
authors.lastname: Habert
authors.forename: Bruno
authors.initials: B
authors.lastname: Dubois
mesh_terms: D000328:Adult; D017668:Age of Onset; D000368:Aged; D001921:Brain / Q000000981:diagnostic imaging* / Q000503:physiopathology; D001931:Brain Mapping / Q000379:methods; D001933:Brain Stem / Q000000981:diagnostic imaging; D002560:Cerebrovascular Circulation; D003430:Cross-Sectional Studies; D003704:Dementia / Q000000981:diagnostic imaging / Q000235:genetics / Q000523:psychology*; D018450:Disease Progression; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009483:Neuropsychological Tests; D011379:Prognosis; D012920:Social Behavior Disorders / Q000209:etiology*; D016019:Survival Analysis; D015899:Tomography, Emission-Computed, Single-Photon