Clinical and Genetic Study of Neurodegenerative Disorders With Cognitive Impairment

NCT ID: NCT00149175

Last Updated: 2025-01-23

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

COMPLETED

Total Enrollment

2256 participants

Study Classification

OBSERVATIONAL

Study Start Date

2002-12-31

Study Completion Date

2020-12-31

Brief Summary

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Patients with different types of dementia will be recruited and evaluated in national hospital departments for their usual neurological follow-ups. A blood sample will be proposed in the field of this research project, and the biological material will be stored at the DNA and Cell Bank of Institut de Fédératif Recherche (IFR) of Neurosciences (Pitié-Salpêtrière Hospital, Paris). The clinical research network is already set up for Alzheimer's disease and frontotemporal dementias, which permits an evaluation according to a clinical standardized protocol.

Among these disorders, a monogenic sub-group has been identified. In Alzheimer's disease, it is associated with the APP, PSEN1 and PSEN2 genes, which account only for 75% of the familial forms with early onset. In frontotemporal dementias, the tau gene mutations account only for 10% of the cases with an autosomal dominant inheritance. The identification of familial forms with a genetic inquiry in the relatives is essential for a greater knowledge of the molecular bases of forms not caused by the known genes, using linkage approaches and candidate gene analysis. The familial forms are also useful for identifying the modifier genes.

In the multifactorial forms, the aim is to assemble a wide cohort of patients and controls matched for localizing and identifying susceptibility genetic factors. The strategies will use a candidate gene approach, and in the near future, studies of single nucleotide polymorphisms (SNPs) spread out in the whole genome. Meanwhile, similar approaches, particularly with candidate genes, could be used for identifying predictive factors of tolerance and response to the treatment.

Finally, correlations will be performed with seric markers according to each kind of dementia.

Specialized clinical teams in diagnosis and follow-up in dementias are assembled for this project, and in the study of neurological disorders of genetic origin.

Detailed Description

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Conditions

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Alzheimer's Disease Dementia Neurodegenerative Disorders

Study Design

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Observational Model Type

CASE_CONTROL

Study Time Perspective

PROSPECTIVE

Study Groups

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Neurodegenerative disorders with cognitive impairment

Blood sampling, skin biopsy

Intervention Type OTHER

Blood sampling, skin biopsy in the field of the medical follow-up

Control

Blood sampling, skin biopsy

Intervention Type OTHER

Blood sampling, skin biopsy in the field of the medical follow-up

At risk reactive

Blood sampling, skin biopsy

Intervention Type OTHER

Blood sampling, skin biopsy in the field of the medical follow-up

Interventions

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Blood sampling, skin biopsy

Blood sampling, skin biopsy in the field of the medical follow-up

Intervention Type OTHER

Eligibility Criteria

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Inclusion Criteria

* Patients presenting with a neurodegenerative disorder with cognitive impairment controls (without signs of the disease), matched with sex and age with the patients
* Relatives for the familial cases

Exclusion Criteria

* Pregnant women
* Minors
* Persons refusing to sign the informed consent
Minimum Eligible Age

18 Years

Maximum Eligible Age

90 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

Yes

Sponsors

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Aventis Pharmaceuticals

INDUSTRY

Sponsor Role collaborator

Institut National de la Santé Et de la Recherche Médicale, France

OTHER_GOV

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Principal Investigators

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Alexis Brice, MD

Role: PRINCIPAL_INVESTIGATOR

Assistance Publique - Hôpitaux de Paris, University Paris 6

Locations

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CHU de la Côte de Nacre

Caen, , France

Site Status

Hôpital Sainte-Marguerite

Marseille, , France

Site Status

Hôpital Guillaume et René Laennec

Nantes, , France

Site Status

Hôpital de l'Archet

Nice, , France

Site Status

Pitié-Salpêtrière Hospital - Centre du Langage et de Neuropsychologie

Paris, , France

Site Status

Pitié-Salpêtrière Hospital - Fédération de Neurologie

Paris, , France

Site Status

Pitié-Salpêtrière Hospital

Paris, , France

Site Status

Hôpital Pontchaillou

Rennes, , France

Site Status

Hôpital Charles Nicolle

Rouen, , France

Site Status

Centre Hospitalier

Saint-Brieuc, , France

Site Status

Hôpital Bellevue

Saint-Etienne, , France

Site Status

Hôpital Civil

Strasbourg, , France

Site Status

Countries

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France

References

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Le Ber I, Guedj E, Gabelle A, Verpillat P, Volteau M, Thomas-Anterion C, Decousus M, Hannequin D, Vera P, Lacomblez L, Camuzat A, Didic M, Puel M, Lotterie JA, Golfier V, Bernard AM, Vercelletto M, Magne C, Sellal F, Namer I, Michel BF, Pasquier J, Salachas F, Bochet J; French research network on FTD/FTD-MND; Brice A, Habert MO, Dubois B. Demographic, neurological and behavioural characteristics and brain perfusion SPECT in frontal variant of frontotemporal dementia. Brain. 2006 Nov;129(Pt 11):3051-65. doi: 10.1093/brain/awl288.

Reference Type BACKGROUND
PMID: 17071924 (View on PubMed)

van der Zee J, Le Ber I, Maurer-Stroh S, Engelborghs S, Gijselinck I, Camuzat A, Brouwers N, Vandenberghe R, Sleegers K, Hannequin D, Dermaut B, Schymkowitz J, Campion D, Santens P, Martin JJ, Lacomblez L, De Pooter T, Peeters K, Mattheijssens M, Vercelletto M, Van den Broeck M, Cruts M, De Deyn PP, Rousseau F, Brice A, Van Broeckhoven C. Mutations other than null mutations producing a pathogenic loss of progranulin in frontotemporal dementia. Hum Mutat. 2007 Apr;28(4):416. doi: 10.1002/humu.9484.

Reference Type BACKGROUND
PMID: 17345602 (View on PubMed)

Baba Y, Baker MC, Le Ber I, Brice A, Maeck L, Kohlhase J, Yasuda M, Stoppe G, Bugiani O, Sperfeld AD, Tsuboi Y, Uitti RJ, Farrer MJ, Ghetti B, Hutton ML, Wszolek ZK. Clinical and genetic features of families with frontotemporal dementia and parkinsonism linked to chromosome 17 with a P301S tau mutation. J Neural Transm (Vienna). 2007 Jul;114(7):947-50. doi: 10.1007/s00702-007-0632-9. Epub 2007 Feb 23.

Reference Type BACKGROUND
PMID: 17318302 (View on PubMed)

Le Ber I, van der Zee J, Hannequin D, Gijselinck I, Campion D, Puel M, Laquerriere A, De Pooter T, Camuzat A, Van den Broeck M, Dubois B, Sellal F, Lacomblez L, Vercelletto M, Thomas-Anterion C, Michel BF, Golfier V, Didic M, Salachas F, Duyckaerts C, Cruts M, Verpillat P, Van Broeckhoven C, Brice A; French Research Network on FTD/FTD-MND. Progranulin null mutations in both sporadic and familial frontotemporal dementia. Hum Mutat. 2007 Sep;28(9):846-55. doi: 10.1002/humu.20520.

Reference Type BACKGROUND
PMID: 17436289 (View on PubMed)

Guedj E, Le Ber I, Lacomblez L, Dubois B, Verpillat P, Didic M, Salachas F, Vera P, Hannequin D, Lotterie JA, Puel M, Decousus M, Thomas-Anterion C, Magne C, Vercelletto M, Bernard AM, Golfier V, Pasquier J, Michel BF, Namer I, Sellal F, Bochet J, Volteau M, Brice A, Meininger V; French Research Network on FTD/FTD-MND; Habert MO. Brain spect perfusion of frontotemporal dementia associated with motor neuron disease. Neurology. 2007 Jul 31;69(5):488-90. doi: 10.1212/01.wnl.0000266638.53185.e7. No abstract available.

Reference Type BACKGROUND
PMID: 17664410 (View on PubMed)

Related Links

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Other Identifiers

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DGS2004/0525

Identifier Type: REGISTRY

Identifier Source: secondary_id

RBM02-59

Identifier Type: -

Identifier Source: org_study_id

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