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From: TSS ()
Subject: Creutzfeldt-Jakob Disease Surveillance in Texas 2000-2006
Date: December 16, 2007 at 2:33 pm PST

Sunday, December 16, 2007

Creutzfeldt-Jakob Disease Surveillance in Texas 2000-2006

Abstract


Creutzfeldt-Jakob disease (CJD) is a rare neurodegenerative disease caused by
prions. The disease is usually fatal within a year and there is currently no known
treatment or cure. CJD has been a reportable condition in Texas since 1998. There
are currently four known types: sporadic, familial, variant, and iatrogenic. Diagnosis
is complex and direct examination of brain tissue is required for case confirmation
and identification of the type of CJD. During 2000–2006, there were 181 cases of
confirmed, probable, or possible CJD diagnosed among Texas residents, including
88 cases of sporadic CJD, 5 cases of familial CJD, and 1 case of variant CJD. Most
case-patients were male (55%) and 55 years of age or older (78%). The Texas
Department of State Health Services is available to assist health care professionals
with arrangement of free diagnostic testing and to provide resources for family
support.

snip...


Texas Surveillance


CJD has been a reportable condition in
Texas since 1998. The majority of CJD
cases are reported to the Texas
Department of State Health Services
(DSHS) by test or autopsy reports
received from the Prion Center. A few
cases are discovered by routine death
certificate review and some cases are
reported by a local or regional health
department, health care provider, or
family member. Copies of results from
all CJD-related testing performed by the
Prion Center are sent to DSHS as a part
of routine surveillance procedures.
DSHS reviews test results and
investigates all cases with positive test
results including those with elevated
CSF 14-3-3 protein levels. Table 1
summarizes the number of cases for all
types of CJD in Texas (2000–2006).
Each year in Texas, the total number of
confirmed, probable, or possible
sporadic CJD cases has remained
somewhat constant (mean=13; range=
6–15). In addition, on average, one case
of familial CJD was diagnosed each year
in Texas during 2000–2006, all from
separate families. Only 1 case of variant
CJD has ever been diagnosed in Texas.
The patient was a former resident of the
United Kingdom, where the exposure
was likely to have occurred. Texas has a
population of 23 million, and since the
national rate of sporadic CJD is about 1
per million, it is expected that
approximately 23 cases of CJD would
occur each year in the state. Therefore,
it is believed that CJD is currently underreported
in Texas. Table 2 describes
sporadic and familial CJD cases in
Texas according to gender and age. Of
the patients diagnosed with CJD during
2000–2006, 58% were male and 79%
were 55 years of age or older. Figure 1
depicts the number of CJD cases per
county for the years 2000–2006. Note
that higher numbers of cases are located
in counties with higher populations.

CJD should be suspected and reported
to DSHS in individuals who meet the
following criteria:


1) Dementia of early onset (younger
than 55 years of age) or
2) Rapidly progressive dementia
and one of more of the following:
• Movement disorder
• Painful sensory symptoms
• Visual disturbances or
3) Diagnosed by a physician as
having CJD


Case Classification


The World Health Organization (WHO)
established recommended standards for
all types of CJD surveillance in 1997
(Table 3). The United States, including
Texas, has adopted these surveillance
standards to determine case
classification for sCJD, fCJD and iCJD.
For vCJD, CDC has developed
diagnostic case criteria for use in the
United States (Table 3). Direct
examination of brain tissue through either
biopsy or autopsy is highly
recommended for all types of CJD and is
required for confirmation of sporadic
CJD and variant CJD. A sCJD case is
determined to be ‘probable’ if there is
enough diagnostic testing to suggest
CJD but no autopsy has been
performed. Patients considered for


probable sCJD must exhibit progressive
dementia and have a typical EEG and/or
elevated CSF 14-3-3 protein. In addition
they must display at least 2 of 4 clinical
features: myoclonus, visual or cerebellar
disturbance, akinetic mutism, and/or
pyramidal/extrapyramidal dysfunction.
For a case to be listed as ‘possible’, the
patient must have progressive dementia
and at least 2 of the listed clinical
features, disease duration of less than 2
years and either no EEG testing or EEG
atypical for CJD. Iatrogenic CJD must
have a known risk factor, e.g., high risk
surgery or cadaver-derived pituitary
hormone. Familial CJD is considered
confirmed in a patient with a
neuropsychiatric disorder if a first degree
relative had confirmed or probable CJD
or a disease-specific prion protein gene
mutation has been identified. Table 3
describes the case classification for
each type of CJD.


Conclusions


CJD is an emerging disease that may be
misdiagnosed and appears to be underreported
in Texas. As with other
emerging diseases, the partnership of
the medical community and public health
epidemiologists provides a framework to
share information and expertise among
local, state, federal and international
colleagues and to detect and respond to


diseases that are rapidly fatal, difficult to
diagnose, and rare. Enhanced
surveillance will provide a better
understanding of the epidemiology of this
disease and its impact and opportunities
for mitigation.
Recognition of possible CJD and
reporting by health professionals is an
integral part of CJD surveillance in
Texas. CJD is a reportable disease in
Texas. All suspected cases should be
reported within one week to the Texas
Department of State Health Services,
Infectious Disease Control Unit.
Confirmation and typing of the disease
requires neuropathological confirmation
by direct examination of brain tissue,
usually postmortem, by the Prion Center.
It is important to establish the precise
type of CJD to help monitor disease
occurrence especially for variant CJD.
Physicians should strongly consider
arranging for autopsies of suspected or
clinically-diagnosed CJD patients. The
Texas Department of State Health
Services can assist health professionals
by arranging free diagnostic testing and
providing resources for family support.


References...snip...end...TSS

Table 1. Cases of Creutzfeldt-Jakob disease in Texas*§
Type 2000 2001 2002 2003 2004 2005 2006 Total
Sporadic
Confirmed 9 8 4 6 7 8 5 47
Probable 2 4 1 5 5 6 3 26
Possible 3 1 1 4 1 0 0 10
Subtotal 14 13 6 15 13 14 8 83
Familial 0 1 1 1 0 1 1 5
Iatrogenic 0 0 0 0 0 0 0 0
Variant 0 0 0 0 0 0 1† 1
Total 14 14 7 16 13 15 10 89
*Based on data as of June 15, 2007.
† Confirmed in United Kingdom and reported to Texas Department of State
Health Services
through Centers for Disease Control and Prevention.
§ Based on year of death.

Table 2. Creutzfeldt-Jakob disease cases* in Texas by gender and age
2000–2006†§
Characteristics 2000 2001 2002 2003 2004 2005 2006 Total
Gender
Male 10 8 3 6 10 7 7 51
Female 4 6 4 10 3 8 2 37
Age (years)
<55>55 11 14 4 12 10 11 7 68
* Based on data as of June 15, 2007.
† Includes cases of possible, probable and confirmed sporadic CJD and
confirmed familial CJD.
§ Based on year of death.


Volume 64/Number 8/ November 5, 2007

http://www.dshs.state.tx.us/idcu/epilink/volume_64/issue_8/docs/640804.pdf


please notice Texas 2006 ;

† Confirmed in United Kingdom and reported to Texas Department of State
Health Services
through Centers for Disease Control and Prevention.


Only 1 case of variant
CJD has ever been diagnosed in Texas.
The patient was a former resident of the
United Kingdom, where the exposure
was likely to have occurred. Texas has a
population of 23 million, and since the
national rate of sporadic CJD is about 1
per million, it is expected that
approximately 23 cases of CJD would
occur each year in the state. Therefore,
it is believed that CJD is currently underreported
in Texas. ...END...TSS


also see ;


http://www.dshs.state.tx.us/idcu/disease/creutzfeldt-jakob/data/

Creutzfeldt-Jakob Disease in Northeast Texas,

J.A. Rawlings,*1 K.A.
Hendricks1, O.M. Nuno1, D.A. Brown1, D.A. Evans2, Texas Department of
Health, 1Austin and 2Tyler, Texas


Creutzfeldt-Jacob Disease (CJD), a transmissible spongiform
encephalopathy, is caused by prions composed of proteinaceous material
devoid of nucleic acid. CJD occurs sporadically (generally 1
case/1,000,000 population per year) in older patients (average age of
65) and is characterized by rapidly progressive dementia, accompanied by
severe muscle spasms and incoordination. Death usually occurs within 3
to 12 months (average 7 months). CJD activity in Texas, which has a
population of nearly 19 million, appeared to be typical. The statewide
death rate for 1995 and 1996 was just under 1/1,000,000. In April of
1997, the Texas Department of Health became aware of an increased number
of possible CJD cases in a 23-county area of NE Texas with a population
of just over one million. After review of medical and pathology records,
four patients were identified with definite classic CJD and three were
identified with probable CJD. Dates of death for the eight patients were
from April, 1996 through mid-July 1997. The patients were from 46
through 65 years of age; four were male and three were female. A
case-control study to identify risks for CJD in NE Texas has been initiated.


http://www.jifsan.umd.edu/tse/Rawlings.htm

JOURNAL OF NEUROLOGY

MARCH 26, 2003

snip...


CJD and all human TSEs must be made reportable in every

state and internationally. I hope that the CDC does not continue to

expect us to still believe that the 85%+ of all CJD cases which are

sporadic are all spontaneous, without route/source.

http://www.neurology.org/cgi/eletters/60/2/176#535


North American Equity Research

New York

13 January 2004


BSE (Mad Cow) Update:


Do Reports of sCJD Clusters Matter?


snip... see full text ;

http://cjdtexas.blogspot.com/


Terry S. Singeltary Sr.
P.O. Box 42
Bacliff, Texas USA 77518




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