Local Health Departments:
Please report all clinical cases diagnosed by a neurologist
or confirmed by tissue pathology of human TSEs including:
• Sporadic CJD (sCJD), also known as Classic
Creutzfeldt-Jakob Disease (CJD)
• Familial CJD (fCJD) or inherited CJD
• Iatrogenic CJD (iCJD)
• Variant Creutzfeldt-Jakob Disease (vCJD),
originally called new variant CJD (nvCJD)
• Gerstmann-Straussler-Scheinker Syndrome
(GSS)
• Fatal Familial Insomnia (FFI)
• Kuru
Reporting a Case:
Typically a local health department (LHD) will receive notification
of a possible case of CJD from one or more of four different
sources. You may be sent 1)
a report of an abnormal 14-3-3 protein or positive test for
abnormal tau protein from cerebrospinal fluid (CSF); 2)
notification of a case from a healthcare provider; 3)
a death certificate listing CJD as a cause of death; or 4)
abnormal brain biopsy or autopsy results indicating prion disease.
1) Report of abnormal 14-3-3 protein or tau
protein (see How is CJD
Diagnosed?)
The
National Prion Disease Pathology Surveillance Center (NPDPSC)
based at Case Western Reserve University is the laboratory in
the United States that does the majority of diagnostic testing
for CJD and other prion diseases. The NPDPSC has begun testing
all CSF sent for 14-3-3 testing for tau protein as well. NPDPSC
or one of the reference laboratories that send CSF samples to
the NPDPSC may forward you a copy of an abnormal 14-3-3 or tau
report.
Detection of the 14-3-3 protein or tau protein in
CSF is NOT definitive for the diagnosis of prion disease. A
positive result can occur in non-prion diseases or in a sample
that is contaminated by blood. Pathological studies of brain
material to detect protease resistant prion remain the only
way to make a definitive diagnosis of prion diseases.
When you receive an abnormal CSF test result for the 14-3-3
or tau protein, you should contact
the ordering physician and ascertain whether a neurologist thinks
that the patient may have or have had CJD. It is frequently the
case that by the time the report reaches you, the clinicians will
have made another more common and alternate diagnosis. In addition,
even if an exact diagnosis has not been made, but the patient
has fully recovered, a prion disease has been ruled out. If
the neurologist NO LONGER suspects CJD, no report is necessary.
If, however, the diagnosis of CJD is still being entertained,
a report must be filed. (Case
report form)
If the patient is still alive, the LHD can
play an important role in getting a definitive diagnosis by
alerting clinicians that free shipping and testing of biopsy
and autopsy material can be arranged through the NPDPSC. Arrangements
for these services and other details regarding the collection
and shipment of clinical specimens, as well as additional resources
available at the NPDPSC can be obtained from its website (http://www.cjdsurveillance.com)
or by calling (216) 368-0587.
If the patient has died, please obtain hospital
discharge summaries, biopsy and autopsy reports, EEG and MRI
reports, neurologist notes, and the death certificate. These
documents should be submitted along with the case report.
2) Notification of a Case from a Healthcare Provider
If you are notified of a case of CJD or prion disease by a
healthcare provider, then you should fill out a CJD
report form and obtain the appropriate records.
If at all possible, it is especially important to retrieve the
notes and opinions of the neurologist, EEG and MRI reports and
any biopsy or autopsy information that exists. Again, if
the patient is still alive, the LHD can alert clinicians
that free shipping and testing of biopsy and autopsy material
can be arranged through the NPDPSC.
3) Notification of a Case from A Death Certificate
If you are notified of a possible case of CJD from a Death
Certificate then please file a CJD
report form and requisition all appropriate patient records.
Autopsy and biopsy results and neurologist and MRI reports are
particularly important.
4) Notification of a Case from Biopsy or Autopsy Report
If you are notified of a case of CJD through receipt of an
abnormal pathology report indicating the presence of CJD, please
file a CJD
case report and requisition all appropriate medical records
and, in the case of autopsy, the death certificate.
CASE REPORT FORM
The case report form is available on-line at http://www.cdph.ca.gov/pubsforms/forms/Pages/CD-Report-Forms.aspx
under CJD. Please attach hospital discharge summaries, MRI,
EEG, pathology, biopsy and lab report(s), and a copy of the
death certificate and mail to:
CA Department of Public Health
Surveillance and Statistics Section
MS 7306
P.O. Box 997377, Sacramento, CA 95899-7377
If assistance is needed in case investigation, please contact
the CA Emerging Infections Program-CJD Surveillance Project
at (510) 451-1344
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