Demographic, neurological and behavioural characteristics and brain perfusion SPECT in frontal variant of frontotemporal dementia.
Isabelle Le Ber
(1, 2, 3)
,
Eric Guedj
(4)
,
Audrey Gabelle
(1)
,
Patrice Verpillat
(2)
,
Magali Volteau
(5)
,
Catherine Thomas-Anterion
(6)
,
Marielle Decousus
(7)
,
Didier Hannequin
(8, 9)
,
Pierre Vera
(10)
,
Lucette Lacomblez
(11, 3)
,
Agnès Camuzat
(2)
,
Mira Didic
(12, 13)
,
Michèle Puel
(7)
,
Jean-Albert Lotterie
(14)
,
Véronique Golfier
(15)
,
Anne-Marie Bernard
(16)
,
Martine Vercelletto
(17)
,
Christine Magne
(18)
,
François Sellal
(19, 20)
,
Izzie Namer
(21)
,
Bernard-François Michel
(17)
,
Jacques Pasquier
(18)
,
François Salachas
(3, 12, 13)
,
Jean Bochet
(16)
,
Alexis Brice
(2, 3, 21, 22)
,
Marie-Odile Habert
(23)
,
Bruno Dubois
(1, 5, 22)
1
Centre de neuropsychologie et du langage
2 Neurologie et thérapeutique expérimentale
3 Fédération des Maladies du Système Nerveux
4 Service Central de Biophysique et Médecine Nucléaire
5 Neuro-anatomie fonctionnelle du comportement et de ses troubles
6 Service de Neurologie [CHU de Saint-Étienne]
7 Neurologie générale et maladies inflammatoires du système nerveux [Toulouse]
8 Service de neurologie [Rouen]
9 Génétique médicale et fonctionnelle du cancer et des maladies neuropsychiatriques
10 Service de médecine nucléaire [Rouen]
11 Service de Pharmacologie médicale [CHU Pitié-Salpêtrière]
12 Service de neurologie et de neuropsychologie
13 Epilepsies, Lesions Cerebrales et Systemes Neuraux de la Cognition
14 Service de Médecine Nucléaire - Pierre-Paul Riquet [CHU Toulouse]
15 Service de Neurologie
16 Service de médecine nucléaire [Rennes]
17 Service de neurologie [Nantes]
18 Service de Médecine Nucléaire [Nantes]
19 Service de Neurologie [Strasbourg]
20 Laboratoire de signalisation moléculaire et neurodégénerescence
21 Service de médecine nucléaire [Strasbourg]
22 Service de Neurogériatrie
23 Service de Médecine Nucléaire [Saint-Quentin]
2 Neurologie et thérapeutique expérimentale
3 Fédération des Maladies du Système Nerveux
4 Service Central de Biophysique et Médecine Nucléaire
5 Neuro-anatomie fonctionnelle du comportement et de ses troubles
6 Service de Neurologie [CHU de Saint-Étienne]
7 Neurologie générale et maladies inflammatoires du système nerveux [Toulouse]
8 Service de neurologie [Rouen]
9 Génétique médicale et fonctionnelle du cancer et des maladies neuropsychiatriques
10 Service de médecine nucléaire [Rouen]
11 Service de Pharmacologie médicale [CHU Pitié-Salpêtrière]
12 Service de neurologie et de neuropsychologie
13 Epilepsies, Lesions Cerebrales et Systemes Neuraux de la Cognition
14 Service de Médecine Nucléaire - Pierre-Paul Riquet [CHU Toulouse]
15 Service de Neurologie
16 Service de médecine nucléaire [Rennes]
17 Service de neurologie [Nantes]
18 Service de Médecine Nucléaire [Nantes]
19 Service de Neurologie [Strasbourg]
20 Laboratoire de signalisation moléculaire et neurodégénerescence
21 Service de médecine nucléaire [Strasbourg]
22 Service de Neurogériatrie
23 Service de Médecine Nucléaire [Saint-Quentin]
Audrey Gabelle
- Function : Author
- PersonId : 757644
- ORCID : 0000-0002-7648-9194
Didier Hannequin
- Function : Author
- PersonId : 842408
Mira Didic
- Function : Author
- PersonId : 757436
- ORCID : 0000-0001-7067-536X
Marie-Odile Habert
- Function : Author
- PersonId : 758316
- ORCID : 0000-0002-7719-9746
Bruno Dubois
- Function : Author
- PersonId : 893169
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.