Demographic, neurological and behavioural characteristics and brain perfusion SPECT in frontal variant of frontotemporal dementia
1 AP-HP, Hôpital de la Salpêtrière, Centre de Neuropsychologie UPMC, Paris 2 INSERM U679, Hôpital de la Salpêtrière UPMC, Paris 3 AP-HP, Hôpital de la Salpêtrière, Fédération des maladies du système nerveux UPMC, Paris 4 INSERM U610, Hôpital de la Salpêtrière UPMC, Paris 5 AP-HP, Hôpital de la Salpêtrière, Service de Pharmacologie UPMC, Paris 6 Université Pierre et Marie Curie 6 UPMC, Paris 7 AP-HP, Hôpital de la Salpêtrière, Département de Génétique, Cytogénétique et Embryologie UPMC, Paris 8 Service de Médecine Nucléaire, Hôpital de la Pitié-Salpêtrière UPMC, Paris 9 Service Central de Biophysique et Médecine Nucléaire, Hôpital de la Timone, APHM and Centre d'Exploration Métabolique par Résonance Magnétique, Université de la Méditerranée 10 Service de Neurologie et Neuropsychologie, Hôpital de la Timone, et Laboratoire de Neurophysiologie et de Neuropsychologie INSERM U-751, Marseille 11 Service de Neurogériatrie, Hôpital Sainte Marguerite, Institut Paoli-Calmettes Marseille 12 Département de Médecine Nucléaire, Institut Paoli-Calmettes Saint-Étienne, Marseille 13 Service de Neurologie, Hôpital Bellevue 14 Service de Médecine Nucléaire, Hôpital Bellevue Saint-Étienne 15 Département de Neurologie et INSERM U614, Rouen University Hospital Rouen, France 16 Service de Médecine Nucléaire, Centre Henri Becquerel Rouen, France 17 Service de Neurologie CHU Purpan, Hôpital Rangueil Toulouse 18 Service de Médecine Nucléaire, Hôpital Rangueil Toulouse 19 Service de Neurologie, Centre Hospitalier Saint Brieuc Saint Brieuc 20 Service de Médecine Nucléaire, Centre Eugène Marquis Rennes 21 Service de Neurologie, CHU Guillaume et René Laënnec 22 Service de Médecine Nucléaire, Hôpital Guillaume et René Laënnec Nantes 23 Service de Neurologie, Hôpitaux Universitaires et INSERM U692 Strasbourg 24 Service de Médecine Nucléaire, Hôpital de Hautepierre Strasbourg 25 Service de Médecine Nucléaire, Polyclinique Saint-Claude Saint-Quentin, France
Correspondence to: Prof. Bruno Dubois, Fédération de Neurologie, Hôpital de la Salpêtrière, 47, Boulevard de l'hôpital, 75 651 Paris Cedex 13, France E-mail: bruno.dubois{at}psl.ap-hop-paris.fr
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.
Key Words: frontotemporal lobar degeneration; fvFTD; frontotemporal dementia; MAPT gene; SPECT; SPM
Abbreviations: FTLD, frontotemporal lobar degeneration; fvFTD, frontal variant of frontotemporal dementia; MND, motor neuron disease; PNFA, progressive non-fluent aphasia
.
Received July 10, 2006. Revised September 12, 2006. Accepted September 13, 2006.
*These authors contributed equally to this work.
**Details regarding French research network on FTD/FTD-MND are provided in the Acknowledgements section.
CiteULike 
Connotea 
Del.icio.us What's this?
This article has been cited by other articles:
|
|
 |
|
  G. Zamboni, E. D. Huey, F. Krueger, P. F. Nichelli, and J. Grafman Apathy and disinhibition in frontotemporal dementia: Insights into their neural correlates Neurology, September 2, 2008; 71(10): 736 - 742. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M. F. Mendez, E. C. Lauterbach, S. M. Sampson, and ANPA Committee on Research An Evidence-Based Review of the Psychopathology of Frontotemporal Dementia: A Report of the ANPA Committee on Research J Neuropsychiatry Clin Neurosci, May 1, 2008; 20(2): 130 - 149. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 I. Le Ber, A. Camuzat, D. Hannequin, F. Pasquier, E. Guedj, A. Rovelet-Lecrux, V. Hahn-Barma, J. van der Zee, F. Clot, S. Bakchine, et al. Phenotype variability in progranulin mutation carriers: a clinical, neuropsychological, imaging and genetic study Brain, March 1, 2008; 131(3): 732 - 746. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 L. Young and M. Koenigs Investigating emotion in moral cognition: a review of evidence from functional neuroimaging and neuropsychology Br. Med. Bull., December 1, 2007; 84(1): 69 - 79. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
E. Guedj, I. L. Ber, L. Lacomblez, B. Dubois, P. Verpillat, M. Didic, F. Salachas, P. Vera, D. Hannequin, J-A Lotterie, et al. BRAIN SPECT PERFUSION OF FRONTOTEMPORAL DEMENTIA ASSOCIATED WITH MOTOR NEURON DISEASE Neurology, July 31, 2007; 69(5): 488 - 490. [Full Text] [PDF]
|
|