Creutzfeldt-Jakob Disease and Other Prion Diseases

There are several types of CJD: sporadic, genetic or familial, iatrogenic or acquired, and variant.

• Sporadic CJD (sCJD): Around 85–95% of CJD cases are sCJD. This form of CJD occurs for unknown reasons. Most people affected by sCJD will die within one year of symptoms beginning. Roughly half of those affected die in the first four to five months.
• Genetic or familial (fCJD): Familial CJD makes up 5–15% of cases of CJD. It occurs when a person inherits a gene from a parent that increases their risk of developing the disease. Symptoms often appear at an earlier age compared to sCJD.
• Iatrogenic or acquired CJD (iCJD): Less than 1% of CJD cases are iCJD. This type of CJD results from exposure to the abnormal protein during a medical procedure. It occurs when medical equipment that has been in contact with nervous tissues (for example, the brain and spinal cord) is used without proper cleaning on those same types of tissues in an unaffected person. Other sources of transmission include tissue grafts, or contaminated hormone donation. The age that symptoms begin varies based on when the procedures were performed. CJD is not spread through normal everyday contact with someone who has the disease. Due to improvements in sterilization protocols and surgical methods, cases of iCJD are now very rare.
• Variant CJD: vCJD makes up less than 1% of CJD cases. It is caused by eating from cattle sick with bovine spongiform encephalopathy (BSE or "mad cow disease"). Most reported cases originated from an outbreak of BSE in cattle beginning in the 1980s that extended through the 1990s in the United Kingdom. Cases of vCJD began to be diagnosed in 1996. Due to improved surveillance efforts, BSE is now very rare and cases of vCJD are even rarer. There have been four cases of vCJD in the United States, all in people who were likely exposed outside of the country. No cases of vCJD have been identified among Wisconsin residents. Early symptoms of vCJD include behavioral and emotional changes and painful sensations to touch. These symptoms occur before the typical neurological symptoms of CJD. Compared to sCJD, vCJD affects a younger population and has a longer duration of symptoms onset and survival time.

Symptoms of CJD include:

• Rapidly progressive dementia.
• Paralysis (not able to move certain body parts).
• Loss of muscle control.
• Vision complications.
• Speech difficulties.
• Death (usually within a year).

CJD may be suspected when a patient experiences the common neurologic symptoms typical of the disease and other illnesses have been ruled out. There are tests available to diagnose a probable case of CJD. The only way to confirm a diagnosis is through an autopsy. Autopsies can also determine if there is a risk for a parent to pass the disease to their children. Autopsies are available free of charge through the National Prion Disease Pathology Surveillance Center.

•  The National Prion Disease Pathology Surveillance Center (NPDPSC) is the national testing location for definitive diagnosis of CJD. The center offers autopsy services and can cover the costs of all autopsy-related services. Contact the NPDPSC Autopsy Coordination Program for autopsy related questions and planning. Visit the NPDPSC website for more information on the services they provide and more about prion diseases.
• The Creutzfeldt-Jakob Disease Foundation helps guide and inform families experiencing a potential or confirmed CJD diagnosis. They offer a helpline (800-659-1991) for support. Additional resources for families navigating a CJD diagnosis can be found on their website.
• Local Aging and Disability Resource Centers (ADRCs) can be a resource for families looking for assistance after a diagnosis. They can help find palliative care, provide advices on benefits, or answer general questions on what life after a disabling diagnosis can look like. See a list of all ADRCs in Wisconsin and the various services they provide.
• ClinicalTrials.gov provides information on clinical studies. Find studies on prion diseases or Creutzfeldt Jakob Disease using the condition/disease search bar.

DHS Creutzfeldt-Jakob Disease Fact Sheet, P-42183 (PDF)

Centers for Disease Control and Prevention—About Prion Diseases

National Institute of Neurological Disorders and Stroke—Creutzfeldt-Jakob Disease 

• Gerstmann-Sträussler-Scheinker syndrome (GSS) is a very rare prion disease that is passed from a parent to a child. It is characterized by neurologic symptoms such as a loss of coordination and eventual dementia. Symptoms usually start at an earlier age, between 35 and 50 years old. Life expectancy ranges from 2–10 years.
• Fatal familial insomnia (FFI) is a very rare prion disease that is passed from a parent to a child. FFI leads to a disruption of sleep patterns, eventually leading to clinical insomnia. Other symptoms include memory loss and motor function complications. The average duration of the disease is approximately 18 months.
• Sporadic fatal insomnia (sFI) is a very rare form of prion disease that occurs for an unknown reason. Symptoms are similar to FFI.
• Kuru is an acquired prion disease that was localized to a tribe in New Guinea. People mainly got sick with kuru from eating the brain and spinal cord of deceased family members who were sick with the disease. Symptoms include motor function complications, tremors, cognitive decline, and death. Death usually occurs within a year of symptoms beginning. Through the guidance of the New Guinea government, these practices no longer occur. The last reported case of Kuru was in 2005.

• Bovine spongiform encephalopathy (BSE), commonly referred to as “mad cow disease,” is found in cattle. Symptoms include changes in aggression or temperament, weight loss, loss of coordination, and eventual death. Eating beef harvested from cattle sick with BSE caused variant CJD in humans. This prompted further research into animal prion diseases and the potential for affected animals to cause illness in people. Due to precautions taken in the United States, there have been fewer than ten cases of BSE in the United States in the last 20 years. Infected cattle are prevented from entering the food supply.
• Chronic wasting disease (CWD) affects animals such as deer, elk, and moose. Symptoms include severe weight loss, lethargy, a lack of fear of humans, motor function complications and death. To date, there have been no cases of CWD in people. However, there is concern that CWD could affect people. CWD is closely related to BSE, which has caused disease in people. As a result, both the Centers for Disease Control and Prevention (CDC) and DHS recommend not eating venison from deer that test positive for CWD. The Wisconsin Department of Natural Resources (DNR) provides a robust CWD testing service for hunters. Find more information on the disease and a breakdown of CWD cases in Wisconsin on the DNR's website.
• Scrapie affects sheep and goats. Symptoms include muscle control difficulties, changes in behavior and temperament, weight loss, excessive rubbing and/or itching, and death. It has not been identified in humans.

CJD is a Wisconsin disease surveillance category II disease:

• Cases should be reported to the patients local health department electronically, through the Wisconsin Electronic Disease Surveillance System (WEDSS) by mail or fax using an Acute or Communicable Disease Case Report, F-44151 (PDF) within 72 hours of recognition of a case.
• General information on communicable disease reporting.
• Report All Prion Diseases, P-01610 (PDF): Flyer for infection preventionists on reporting prion diseases.
• CJD EpiNet, P-01913 (PDF): A case reporting and investigation protocol for state health professionals. Note: There is no local public health follow up of a prion disease report. All investigations are done by the state public health department.

• Prion disease resources for health professionals and autopsy coordination provided by the NPDPSC.
• Prion Disease Information for Wisconsin Medical Providers, P-01262 (PDF): Information from DHS for medical providers on reporting and diagnosing prion diseases.
• Report All Prion Diseases, P-01610 (PDF): A flyer for infection preventionists on reporting prion diseases.
• ClinicalTrials.gov: A resource that provides information on clinical studies

• CDC information on proper infection prevention measures.
• WHO infection control guidelines.
• NPDPSC Infection Control Practices.

• A short documentary outlining the process of working with the body of and arranging a funeral for someone that died due to CJD.
• CDC information for funeral directors and crematory practitioners.
• WHO infection control guidelines.