Creutzfeldt-Jakob Disease/Bovine Spongiform Encephalopathy
Incidence
The incidence of classical CJD is approximately 1 in 1,000,000. CJD in persons under 30 years old has an incidence of 5 in 1,000,000,000.
Sequelae
The disease is uniformly fatal in cases that are recognized with clinical syndrome.
Cost
Unknown. The recent withdrawal of blood products by the American Red Cross due to possible contamination by infected blood donors was estimated to be a $50 million loss. The Canadian Red Cross also did a massive recall of blood plasma products.
Transmission
Most cases are sporadic with unknown mechanism of transmission. Five percent of cases are familial. CJD induced inadvertently by medical treatment include a) following surgical procedures, b) following cadaveric corneal or dura transplants and c) following growth hormone therapy derived from cadaveric pituitary glands.
Risk Groups
- Classical CJD is predominantly a disease of the elderly with peak number of deaths at age 65-69
- familial CJD
- iatrogenic (see Transmission)
Surveillance
The diagnostic error in clinical diagnosis of CJD is 25 percent with reluctance on the part of pathologists to confirm the diagnosis by necropsy.
Trends
Since incubation of BSE in animals is 2.5 to 8 years, the full impact of potential human infections that occurred before 1989 may not be realized. If the assumption of the mode of transmission to humans is correct, current intervention strategies, i.e. ceasing to feed animal offal to cattle, should remove any future risk.
Challenges
Detection method for the genetic or metabolic products that are unique to the transmissible agent. Identify the transmissible agent.
Opportunities
National repository for tissues from CJD cases. National data collection of CJD cases for more solid definition of disease. Education of beef producers.
Research Priorities
Priorities include a) to develop a test to detect antibodies to and the antigen of the transmissible agent for use in examination of sera samples, b) identification of the transmissible agent and c) to develop a national pathological and epidemiological surveillance project for BSE/CJD.
Year 2000 Objectives
No existing objectives. The goals should be to identify the agent and develop a detection method for better epidemiological data.
May 1996