National Prion Disease Pathology Surveillance Center
June 1, 1998
The American Association of Neuropathologists [AANP] has
established a National Prion Disease Pathology Surveillance Center at Case
Western Reserve University (CWRU) in Cleveland, Ohio. This effort is
being collaborated with the AANP, CWRU and the Centers for Disease Control
and Prevention (CDC) in Atlanta, Georgia. The Director for this program
will be Dr. P Gambetti, Professor and Director, Division of Neuropathology,
CWRU, Cleveland, OH; and co-directed by Dr. B. Ghetti, Indiana University
Medical Center, Indianapolis, IN.
A major objective of this project is to monitor the
presence or absence of new variant Creutzfeldt-Jakob disease (nvCJD) and
other emerging human prion diseases in the United States through systematic
review of pathologic data and brain tissues of suspected or proven cases of
human prion diseases. Initially, the center will collect pathology reports
of autopsy or biopsy-confirmed cases of sporadic, familial, and iatrogenic CJD, as well as of other prion diseases, such as
Gerstmann-Straussler-Scheinker syndrome (GSS) and fatal familial insomnia (FFI).
These cases will then be reported to the CDC to augment its national CJD
surveillance, which is mainly based on analysis of multiple cause of death
data derived from routinely submitted death certificates.
The surveillance center has issued letters to
neuropathologists requesting participation in the program. Requests for
information pertaining to all cases diagnosed as CJD, GSS, or FFI from
January 1, 1994 to June 1, 1998, including any cases examined directly or in
consultation. Information submitted should include the date of death or
biopsy, the month of onset of the disease, and the patient's date of birth
or age at death. There is also a questionnaire provided for submissions.
For ongoing surveillance, the Center is requesting samples, if possible, at
least five grams of fresh, unfixed tissue from the cerebral cortex and
cerebellum. Please contact the individuals listed below for further
information and copies of the questionnaires.
Dr. Pierluigi Gambetti, M.D. at CWRU
Email: cjdsurv@cwru.edu
Email cjdsurveillance@cwru.edu
www.cjdsurveillance.com
Telephone: 216/368-0587
Fax: 216/268-2546
All correspondence and shipments should be addressed to:
Pierluigi Gambetti, M.D.
Division of Neuropathology, Room 419
Case Western Reserve University
2085 Adelbert Road
Cleveland, OH 44106
Federal Express Account numbers can be obtained from CWRU
for shipments.
Results from testing of samples from individual patients
will be mailed to the sender. A copy of the immunostained slides will also
be made available. CDC will issue summary reports on the prevalence of CJD
in the United States and other findings of this surveillance effort. If
reports of individual case materials are considered for publication, senders
will be offered authorship.
Comments:
Members of CJD Voice are sure the incidence rate of CJD is far higher than
that which is published. It is my personal and most sincere hope if what we
believe is true, that this program excels beyond the purpose of solely
detecting nvCJD and does indeed show cause and proof that the true incidence
of CJD (ALL FORMS) is extremely higher than one per million. AND, if this
should prove true, that the CDC will mandate reporting of CJD in all
states!!! Sincerely and waiting and watching with anticipation........ Liz.
