Demographic, neurological and behavioural characteristics and brain perfusion SPECT in frontal variant of frontotemporal dementia.
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.
Le Ber
Isabelle
I
AP-HP, Hôpital de la Salpêtrière, Centre de Neuropsychologie UPMC, Paris, France.
Guedj
Eric
E
Gabelle
Audrey
A
Verpillat
Patrice
P
Volteau
Magali
M
Thomas-Anterion
Catherine
C
Decousus
Marielle
M
Hannequin
Didier
D
Véra
Pierre
P
Lacomblez
Lucette
L
Camuzat
Agnès
A
Didic
Mira
M
Puel
Michèle
M
Lotterie
Jean-Albert
JA
Golfier
Véronique
V
Bernard
Anne-Marie
AM
Vercelletto
Martine
M
Magne
Christine
C
Sellal
François
F
Namer
Izzie
I
Michel
Bernard-François
BF
Pasquier
Jacques
J
Salachas
François
F
Bochet
Jean
J
Brice
Alexis
A
Habert
Marie-Odile
MO
Dubois
Bruno
B
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