Creutzfeldt-Jakob Disease Surveillance Data for Reporting Years 2000-2008
A total of 119 people died from CJD during 2000-2008. Texas has had one variant CJD case. Investigators have concluded that the patient was a former resident of the UK where exposure was likely to have occurred.
CJD cases* in Texas by gender and age 2000–2008†
Characteristics 2000 2001 2002 2003 2004 2005 2006 2007 2008 Total
Gender Male 10 8 3 6 10 7 7 5 8 64
Female 4 6 4 10 3 8 2 9 8 54
< 55 3 0 3 4 3 4 3 3 3 26
≥ 55 11 14 4 12 10 11 6 11 13 92
* based on data as of March 30, 2009
† Includes cases of possible, probable and confirmed sporadic CJD and confirmed familial CJD
Reporting Creutzfeldt-Jakob Disease
Several Texas laws (Tex. Health & Safety Code, Chapters 81, 84 and 87) require specific information regarding notifiable conditions be provided to the Texas Department of State Health Services (DSHS). Health care providers, hospitals, laboratories, schools, and others are required to report patients who are suspected of having a notifiable condition (25 Tex. Admin. Code §97.2 ).
Report Creutzfeldt-Jakob Disease (CJD) within one week. Reporting Forms >>
CJD became a notifiable condition in 1998 in Texas. Suspected cases of CJD should be reported to local health departments by dialing 1-800-705-8868.
In September 1997, the National Prion Disease Pathology Surveillance Center (NPDPSC) was established at the Division of Neuropathology of Case Western Reserve University to, among other functions, assist clinicians in the diagnosis of prion disease. The NPDPSC assists clinicians by analyzing cerebrospinal fluid, blood, and brain tissue. Information about diagnostic services, protocols for various CJD testing, and specimen submission can be obtained at
or by contacting the director, Dr. Pierluigi Gambetti or staff at the Institute of Pathology, Case Western Reserve University, 2085 Adelbert Road, Room 419, Cleveland, Ohio 44106; Phone, 216/368-0587; Fax, 216/368-4090; E-mail, firstname.lastname@example.org .
Physicians are strongly encouraged to confirm the diagnosis of CJD by arranging for an autopsy following the death of the person suspected of having CJD. This is especially important if the person had an onset at age less than 55. Please contact the center above for assistance or specimen submission.
Have There Been Any Cases of “Mad Cow Disease” in the United States? Bovine spongiform encephalopathy (BSE) or “mad cow disease” is a disease specific to cattle. There have been three cases of BSE in cattle identified in the United States. The first case of BSE was identified in Washington State (2002) and the cow was found to have been imported from Canada. In 2004 BSE was identified in a Texas-born cow representing the first native case in the United States. A second native case was reported in an Alabama bovine in 2006.
Have There Been Any Cases of Variant CJD in the United States? Yes, there have been three cases of variant CJD identified in humans in the United States. The first case-patient was born in the United Kingdom in the late 1970s and lived there until relocating to Florida in 1995. Onset of symptoms began in 2001 and the patient died in 2004. The second case–patient was born and raised in England before moving to Texas in 2001. Symptoms began in 2005 and were confirmed neuropathologically (by autopsy) in 2006 by experts in the United Kingdom. Both the first and second case-patients are believed to have been exposed to the BSE agent while residing in the United Kingdom during the defined period of risk (1980–1996). The third case–patient was born and raised in Saudi Arabia and resided in the United States since 2005. Variant CJD was neuropathologically confirmed by biopsy in 2006. Investigators believe that exposure most likely occurred from consumption of contaminated cattle while residing in Saudi Arabia as a child.
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Friday, November 30, 2007
CJD QUESTIONNAIRE USA CWRU AND CJD FOUNDATION
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.
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