Espo's  Hope

Cure CJD

About CJD

What is CJD?


Creutzfeldt-Jakob Disease (CJD) is a prion disease, a group of rare, invariably fatal brain disorders which occur both in humans and certain animals. They first came to public attention in the mid 1980s in the form of the BSE epidemic in the United Kingdom. BSE (bovine spongiform encephalopathy) is a prion disease in cattle. Tissue from infected animals may have contaminated cattle feed, leading to the silent spread of the BSE epidemic. There is also a theory that BSE came from feed contaminated with scrapie, the long established sheep prion disease. Inevitably, concern over whether BSE could pass to humans mounted.

 
In humans the best known of the prion diseases is Creutzfeldt-Jakob Disease (CJD), which reportedly affects around one person per million per year. In the United States this translates to 250-300 new cases per year. It is well known that CJD is very difficult to diagnose leading to speculation that the one case per million report may be incorrect. Most of the cases are "classical" or "sporadic" CJD (sCJD), occurring for no, as yet, known reason. The sporadic form accounts for approximately 85% of the cases, the familial form approximately 15%. There have also been a few cases which have occurred from contamination via medical procedures; this type is known as iatrogenic or Acquired CJD. Finally over the last few years, another type of Acquired CJD called variant (vCJD) has been identified in young people. CJD has been linked to ingestion of beef tainted with BSE (bovine spongiform encephalopathy), most cases have occurred in the United Kingdom




Creutzfeldt-Jakob Disease (CJD) is a rare, fatal brain disorder. The incidence of CJD cases worldwide is one case, per million, per population. In the United States this statistic translates to approximately 300 new cases per year. Eighty-five percent of the cases are sporadic, meaning there is no known cause at present.


There are three types of CJD:

 Sporadic (sCJD)

 Familial (fCJD)

 Acquired (vCJD or iCJD)


In the early stages of the disease, CJD patients may exhibit failing memory, behavior changes, impaired coordination and/or visual disturbances. As the illness progresses, mental deterioration becomes more pronounced, and involuntary movements, blindness, weakness of extremities, and, ultimately, coma may occur. sCJD usually occurs later in life and typically leads to death within a few weeks or months to one year following the onset of symptoms


Causes of CJD


Sporadic CJD

  • Unknown


Familial CJD

  • Genetic mutation inherited from a parent


Iatrogenic CJD

  • Contaminated surgical instruments
  • Contaminated dura mater transplant
  • Contaminated corneal transplant
  • Contaminated human growth hormone


Variant CJD

  • Contaminated beef
  • Contaminated blood or blood plasma transfusion


The sporadic form of CJD is the most prevalent form of CJD affecting approximately 300 new people in the United States each year. The familial form of CJD accounts for 10-15% of the cases. Acquired CJD, which includes iatrogenic and variant CJD, accounts for less than 1% of all cases. More information can be found on www.cjdfoundation.org. As of May 2009, there are no known cases of endemic vCJD in the United States.

Diagnosis of CJD is very difficult and is often made from clinical observation and/or process of elimination of other diseases. The diagnosis of CJD can only be confirmed through a brain biopsy or autopsy. Cerebral spinal fluid testing positive for a 14-3-3 or tau protein is often used to confirm a possible diagnosis, this test, however, can be ambiguous.


Public Health Concerns

CJD is NOT “Mad Cow Disease.” Bovine Spongiform Encephalopathy (BSE), the technical term for Mad Cow Disease, occurs only in cows.The first documented case of BSE found in the United States occurred in Washington State in December 2003 in a cow imported from Canada. The first endemic case was found in Texas and was announced in 2005. Eating infected beef is widely believed to be the cause of the variant form of CJD (vCJD) in humans. vCJD usually affects young people. An endemic case has not yet been documented as originating in the United States.Is the public at risk of exposure to CJD? Although most Americans have never heard of Creutzfeldt-Jakob Disease, they have heard of “Mad Cow Disease” and fear it. They do not know what it is, but know it is catastrophic. The confirmation of a case of vCJD originating in the U.S. would likely lead to a chain reaction of panic-driven decisions and policymaking.


For more information on CJD, please visit:


CJD Foundation Website


The CJD Foundation holds an annual conference in Washington DC during July where researchers from around the world attend and provide updates in their efforts to find a treatment and potential cure for CJD.

The attached 2 minute video is from the conference taking place July 14, 2017. It is one of the best and easiest explanations of what CJD does to the brain and of a potential treatment.


It is critical the government continues to provide funding for research. Write to cure CJD.org voicing your support that the antibody gif CJD be released for use in humans. It's been held up by regulatory agencies in the UK (where antibody treatment was discovered) for 5+ years.

Also write to your congressmen asking them to support expanded funding for CJD and neuro-degenerative research and to keep the NPDPSC funded.  



   Click above for more information

                                                                  "Faces of CJD"