Clinical Trial: The Role of the Coagulation Pathway at the Synapse in Prion Diseases
Study Status: Not yet recruiting
Recruit Status: Not yet recruiting
Study Type: Observational
Official Title: The Role of the Coagulation Pathway at the Synapse in Prion Diseases
Brief Summary:
The study hypothesis is that that the deleterious effect of prions on the brain may be mediated (at least partially) by activation of serine proteases involved in the coagulation system. If this is true, then measurement of the activity of the coagulation system may be a marker of disease onset (in at higher risk individuals such as E200K* carriers) and for disease progression or activity in affected individuals. In addition, modulation of the coagulation system activity may be a potential tool for therapeutic intervention.
*E200K- E200K mutation (Glu to Lys substitution) in the prion protein gene
Detailed Summary:
We plan to collect Cerebrospinal fluid (CSF) samples for thrombin activity assay in order to test whether there is a difference in thrombin activity in the CSF between CJD (Creutzfeldt-Jakob disease) and non-CJD patients. CSF samples will be obtained from two sources 1. Patients with familial or sporadic CJD and control patients with other neurodegenerative disorders (e.g. SDAT**, NPH) that will be evaluated in Sheba Medical Center 2. From our collaborating group of Prof. Zerr in the German Prion Referral Center at the University of Gottingen which has a collection of thousands of CSF samples from patients with familial and sporadic CJD as well as ideal controls with other degenerative brain disease.
The study has 2 sections:
-
Prospective part in which we plan to recruit 25 patients with CJD and 25 patients with other types of dementia from Sheba Medical Center (SMC). Prior to inclusion in the study a senior neurologist will interview the patient and will verify that he fully understands the objectives of the study and he is mentally qualified to sign the informed consent form (severely demented patients who will not be able to adequately consider the participation in the study will be excluded).
Cognitive performance will be evaluated using the Mini-mental Status Examination and Frontal Assessment Battery scales.
No clinical data other than the cognitive assessment and those needed for the clinical work up will be especially collected for this study.
- Retrospective part in which CSF samples from CJD patients and patients with other type of dementia will be shipped to us from our collaborators in Germany ans w
Sponsor: Sheba Medical Center
Current Primary Outcome: Thrombin activity assay [ Time Frame: 10 years ]
Original Primary Outcome: Same as current
Current Secondary Outcome:
Original Secondary Outcome:
Information By: Sheba Medical Center
Dates:
Date Received: June 22, 2015
Date Started: June 2015
Date Completion: July 2025
Last Updated: June 23, 2015
Last Verified: June 2015
