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

Autor:	Martine Vercelletto, Jacques Pasquier, Agnès Camuzat, Michèle Puel, François Salachas, Patrice Verpillat, Audrey Gabelle, Jean-Albert Lotterie, Lucette Lacomblez, Jean Bochet, Isabelle Le Ber, Marie-Odile Habert, Didier Hannequin, Bruno Dubois, Mira Didic, François Sellal, Marielle Decousus, Pierre Vera, Catherine Thomas-Antérion, Véronique Golfier, M. Volteau, Eric Guedj, Alexis Brice, Anne-Marie Bernard, Christine Magne, Bernard-François Michel, Izzie Jacques Namer
Přispěvatelé:	CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Neurologie et thérapeutique expérimentale, Université Pierre et Marie Curie - Paris 6 (UPMC)-IFR70-Institut National de la Santé et de la Recherche Médicale (INSERM), Service Central de Biophysique et Médecine Nucléaire, Assistance Publique - Hôpitaux de Marseille (APHM)- Hôpital de la Timone [CHU - APHM] (TIMONE), Neuro-anatomie fonctionnelle du comportement et de ses troubles, Service de Neurologie [CHU de Saint-Étienne], Centre Hospitalier Universitaire de Saint-Etienne [CHU Saint-Etienne] (CHU ST-E)-Université Jean Monnet - Saint-Étienne (UJM), Neurologie générale et maladies inflammatoires du système nerveux [Toulouse], Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Service de neurologie [Rouen], CHU Rouen, Normandie Université (NU)-Normandie Université (NU), Génétique médicale et fonctionnelle du cancer et des maladies neuropsychiatriques, Université de Rouen Normandie (UNIROUEN), Normandie Université (NU)-Normandie Université (NU)-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de médecine nucléaire [Rouen], CRLCC Haute Normandie-Centre de Lutte Contre le Cancer Henri Becquerel Normandie Rouen (CLCC Henri Becquerel), Service de neurologie et de neuropsychologie, Université de la Méditerranée - Aix-Marseille 2-Assistance Publique - Hôpitaux de Marseille (APHM)- Hôpital de la Timone [CHU - APHM] (TIMONE), Epilepsies, Lesions Cerebrales et Systemes Neuraux de la Cognition, Université de la Méditerranée - Aix-Marseille 2-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de Médecine Nucléaire - Pierre-Paul Riquet [CHU Toulouse], Pôle imagerie médicale [CHU Toulouse], Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Service de Neurologie, Centre hospitalier Saint-Brieuc, Service de médecine nucléaire [Rennes], CRLCC Eugène Marquis (CRLCC), Service de neurologie [Nantes], Université de Nantes (UN)-Centre hospitalier universitaire de Nantes (CHU Nantes)-Hôpital Guillaume-et-René-Laennec [Saint-Herblain], Service de Médecine Nucléaire [Nantes], Hôpital Laennec, Service de Neurologie [Strasbourg], CHU Strasbourg-Hopital Civil, Laboratoire de signalisation moléculaire et neurodégénerescence, Université Louis Pasteur - Strasbourg I-IFR37-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de médecine nucléaire [Strasbourg], CHU Strasbourg, Service de Neurogériatrie, Assistance Publique - Hôpitaux de Marseille (APHM)-Hôpital Sainte-Marguerite [CHU - APHM] (Hôpitaux Sud ), Service de Médecine Nucléaire [Saint-Quentin], Polyclinique Saint-Claude, French research network on FTD/FTD-MND, Centre de neuropsychologie et du langage, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Sorbonne Université (SU), Fédération des Maladies du Système Nerveux, Service de neurologie, Hôpital Bellevue, Service de Pharmacologie médicale [CHU Pitié-Salpêtrière], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Assistance Publique - Hôpitaux de Marseille (APHM)-Hôpital Sainte Marguerite, Breton, Céline, Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Institut National de la Santé et de la Recherche Médicale (INSERM)-IFR70-Université Pierre et Marie Curie - Paris 6 (UPMC), Université Toulouse III - Paul Sabatier (UT3), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-CHU Toulouse [Toulouse]-Hôpital Purpan [Toulouse], CHU Toulouse [Toulouse], Service de pharmacologie médicale [CHU Pitié-Salpêtrière], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Service de Médecine Nucléaire [Toulouse]
Jazyk:	angličtina
Rok vydání:	2006
Předmět:	Male Pathology MESH: Social Behavior Disorders Cross-sectional study Perfusion scanning Neuropsychological Tests 0302 clinical medicine Age of Onset MESH: Brain Mapping MESH: Aged Brain Mapping 0303 health sciences MESH: Middle Aged Brain MESH: Neuropsychological Tests Frontotemporal lobar degeneration MESH: Cerebrovascular Circulation Middle Aged Prognosis MESH: Dementia 3. Good health Cerebrovascular Circulation MESH: Survival Analysis Disease Progression Cardiology Female MESH: Disease Progression [SDV.NEU]Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC] medicine.symptom Psychology Perfusion Frontotemporal dementia Adult medicine.medical_specialty MESH: Age of Onset MESH: Prognosis MESH: Tomography Emission-Computed Single-Photon 03 medical and health sciences MESH: Brain MESH: Cross-Sectional Studies Internal medicine medicine Humans [SDV.NEU] Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC] Survival analysis Aged 030304 developmental biology Tomography Emission-Computed Single-Photon MESH: Humans Social Behavior Disorders MESH: Adult medicine.disease Survival Analysis MESH: Male Cross-Sectional Studies Disinhibition MESH: Brain Stem Dementia Neurology (clinical) Age of onset MESH: Female 030217 neurology & neurosurgery Brain Stem
Zdroj:	Brain-A Journal of Neurology Brain-A Journal of Neurology, 2006, 129 (Pt 11), pp.3051-65. ⟨10.1093/brain/awl288⟩ Brain-A Journal of Neurology, Oxford University Press (OUP), 2006, 129 (Pt 11), pp.3051-65. ⟨10.1093/brain/awl288⟩
ISSN:	0006-8950 1460-2156
DOI:	10.1093/brain/awl288⟩
Popis:	International audience; 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 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.
Databáze:	OpenAIRE
Externí odkaz:	https://explore.openaire.eu/search/publication?articleId=doi_dedup___::e6964d516be1d8e843dc4ec4305c5a12 https://www.hal.inserm.fr/inserm-00475850 Zobrazit plný text záznamu