The French Reference Centre for Rare Dementias
(Frontotemporal Dementia, Primary Progressive Aphasia,
Progressive Supranuclear Palsy, Corticobasal Degeneration)
The “National Plan for Rare Diseases, 2005-2008” led to the creation of reference centres (RC), labeled by the French Ministry of Health and Social Care, devoted to the patients affected by rare diseases. The reference centres are organized around highly specialized medical teams that are comprised of a group of professionals with multidisciplinary skills. The creation of the reference centres aims to facilitate the diagnosis of rare diseases, to improve the care and support for the patients, to make their access to specialized consultations easier, to provide medical information, and to coordinate research on these diseases.
The “Reference Centre for Rare Dementias” was created in July 2007. It is located at the Memory and Alzheimer’s disease Institute at the Pitié-Salpêtrière Hospital (Paris). It is led by the Pr. Bruno Dubois and is coordinated by the Dr. Isabelle Le Ber. This reference centre is dedicated to patients affected by four rare pathologies : Frontotemporal Dementia (FTD), Primary Progressive Aphasia (PPA), Progressive Supranuclear Palsy (PSP), and Corticobasal Degeneration (CBD).
The principal aims of the “Reference Centre for Rare Dementias” are to improve the diagnosis of these diseases and to elaborate a strategy for therapeutic, psychological, and social support, adapted to each patient according to his pathology. The centre is also aimed at leading and coordinating a network of health and medico-social correspondents ; to be a privileged voice on behalf of associations for the sick ; to participate in training and teaching healthcare professionals, the patients, and their families ; and to coordinate research on these diseases.
The recent structuration of the Reference Centre makes it possible to propose a treatment and care plan specific to each patient and their families. The RC introduced multidisciplinary consultations (MDC) dedicated to each of the pathologies concerned by the centre. The MDCs allow for patients and families to meet with a team of medical specialists (a neurologist, social worker, clinical psychologist, neuropsychologist, and speech therapist) that allow to evaluate different aspects of the disease on the same day and in the same place, and to bring psychological support to the caregiver. The MDCs complement the traditional specialized consultations already offered by the RC for Rare Dementias : consultations with neurologists, psychologists, social workers, speech therapists, neuropsychologists, genetic counseling and informational sessions explaining the science and symptoms of the disease.
The support for the patients and families is also focused on psycho-education groups led by Mr. Hergueta. The groups bring together a dozen participants (groups of patients, groups of spouses, groups of children) all impacted by the same pathology, over the course of a dozen meetings each year that are held at the Pitié-Salpêtrière Hospital. A specific theme is addressed at each session (social care, behavioral problems, scientific aspects, etc.), where experts are invited to speak and answer questions of the patients or families.
In order to provide information about the pathologies a website (www.cref-demrares.fr, in French) dedicated to the patients and families affected by FTD, PPA, PSP, and CBD was created by healthcare professionals. The website includes practical informations about the Reference Centre, the system of medical and psychosocial support provided by the Reference Centre and informations on the pathologies treated at the Reference Centre, as well as links to social assistance and patient associations on the website. Furthermore, the Reference Centre has prepared informational leaflets on the pathologies for the patients and caregivers, which will soon be downloadable from the website.
The RC also strengthens ties with patient associations (France-Alzheimer and PSP France), and participates to the creation of an association dedicated to patients affected by FTD (France-DFT).
The reference centre works in collaboration with 12 regional centres that were determined in 2008 : Angers, Bordeaux, Dijon, Lille, Limoges, Lyon-Saint Etienne, Montpellier, Marseille, Poitiers, Rouen, Strasbourg, Toulouse. These qualified centres allow for patients to benefit from specialized consultations close to their homes. The 12 regional centres and the Reference Centre are committed to sharing identical diagnostic, social support, and therapeutic procedures and to develop joint research projects on FTD, PPA, PSP, and CBD. This ensures that the quality of care for the patients affected by rare diseases is the same throughout France. The information and addresses for the regional centres are available on the website www.cref-demrares.fr. The structuration of a national network, formed by the Reference Centre and the regional centres allowed the development of research projects on a national scale.
Reference Centre Team
Supervisor : Pr. Bruno Dubois
Director : Dr. Isabelle Le Ber, Dr. Marc Teichmann
Neurologists : Pr. Bruno Dubois (FTD, PPA, PSP, CBD), Dr. Isabelle Le Ber (FTD), Dr. Anne-Marie Bonnet (PSP, CBD), Dr. Marc Teichmann (PPA)
Clinical Psychologist : Thierry Hergueta, Velina Negovanska, Sarah Boucault
Clinical Research Assistant : Vanessa Mourlon
Social Worker : Pierre Arnoulin, Yasmina Belmekki
Neuropsychologists : Aurélie Guignebert, Céline Chamayou, Dalila Samri
Speech Therapists : Marie Noguès, Sophie Férieux, Christine Pointon
Genetic Diagnosis : Isabelle Le Ber (genetic counseling), Fabienne Clot (molecular analysis)
Health Education : Martine Galipaud
Nurse : Monique Foulonneau, Anne Besnard, Catherine Poisson
Reference Centre Address :
Centre de référence des Démences Rares (Reference Centre for Rare Diseases)
IM2A- Fédération des maladies du système nerveux
47 boulevard de l’hôpital
75651 PARIS cedex 13
Mail : email@example.com
Regional Centre Addresses :
They are accessible on the website for the Reference Centre for Rare Dementias :