variant Creutzfeldt-Jakob disease CJD is a relatively new strain of "Mad Cow" disease, which is infecting humans, outside of the United States (1). CJD transmission is rare but most commonly is through infected tissue such as cornea transplants or the use of non-sterilized surgical equipment (2). There have been reported cases of CJD in adolescents in the United Kingdom, thought to have been transmitted through growth hormones which were extracted from the pituitary glands of cadavers (2). Consumption of meat tainted by Bovine spongiform encepalopathy is another vehicle of CJD transmission (4). In 10%-15% of reported cases heredity is the means of transmission, caused by a mutation in the PrP gene (7). The most common reservoir is infected cattle or their bone marrow (4).
The pathogen responsible for CJD transmission is the prion (5). A prion is an "abnormal isoform of a cellular glycoprotein" (1). The primary characteristic of the prion is that it has the ability to alter healthy protein molecules into dangerous life threatening spongifor encephalopathies (5).
At this time the most effective test unfortunately is by obtaining a brain biopsy from the infected individual and testing for characteristic changes in the brain tissue (5). Infected brains display a spongy degeneration, additionally; differences in brain tissue can be detected microscopically (8). The only other test that has been successful in diagnosis is electroencephalogram EEG. EEG measures brain wave activity and typically can detect abnormal wave patterns (5).
CJD was first detected in 1986 by, "Gerald A. H. Wells and John W. Wilesmith of the Central Veterinary Laboratory in Weybridge England" (10). I was unable to find any information pertaining to the significance of the name of this disease.
Early symptoms of CJD may include muscle tremors, coordination problems and an altered personality, similar to Alzheimer (2). As this disease progresses, symptoms worsen to: hallucinations, speech impairment, sleepiness, dramatic disorientation and muscle stiffness (2). Symptoms accelerate rapidly and death can occur in as little as 3-7 months (2). CJD has a normal incubation time of 1 to 2 years (2).
The reason that the prion is so different to any virus or bacteria is because it contains not nucleic acids (DNA or RNA) (10). The prion that is responsible for CJD is unlike any known pathogen; it is resistant to both chemical and physical treatments, such as being resistant to heat up to 100ƒC (3). "The agent has remained elusive, and its identity is currently unknown"(3).
CJD control is primarily handled by the examination and control of cattle that exhibit signs of this disease, if the animals exhibit symptoms they are immediately destroyed and incinerated, in order to cut down on disease transmission (1). If a health provider is caring for an infected individual, the best ways to control the spreading of CJD is: washing hands, face mask, disposal of bedclothes/sheets, wearing surgical gloves and disinfecting any instrumentation with a diluted chloride bleach solution (9). Unfortunately there is no known treatment for CJD, it is inevitable a fatal disease (5).
References:
1. Center for Disease Control. 'BSE and CJD Information and Resources." 04/18/02. URL: http://www.cdc.gov/ncidod/diseases/cjd/cjd.htm assessed on 04/23/02.
2. Yahoo Health. "Creutzfeldt-Jakob disease." 2002. URL: http://health.yahoo.com/health/dc/000788/0.html assessed on 04/23/02.
3. Creutzfeldt-Jakob Disease Foundation Inc."CJD Agent Theories." 1999. URL: http://cjdfoundation.org/Agent.html assessed on 04/29/02.
4. Food and Drug Administration. "Prions and Transmissible Spongiform Encephalopathies." 01/01. URL: http://www.cfsan.fda.gov/~mow/prion.html assessed on 04/29/02.
5. Creutzfeldt-Jakob Disease Foundation Inc."CJD info." 1999. URL: http://www.cjdfoundation.org/CJDInfo.html assessed on 04/29/02
6. Prionics. "The Pathogen Causing Prion Diseases."URL: http://prionics.ch/prionics-e.htm assessed on 04/23/02.
7. Prionics." ìClassic Creutzfeldt-Jakob Disease." URL: http://prionics.ch/prionics-e.htm assessed on 04/23/02
8. Virtual Hospital."British Beef and Dementia." 1996. URL: http://www.vh.org/Patients/IHB/IntMed/ABA30/1996/Madcow.html assessed 04/29/02
9. National Institute of Neurological Disorders and Stroke."Creutzfeldt-Jakob Disease."03/15/02 http://www.ninds.nih.gov/health_and_medical/pubs/creutzfeldt-jakob_disease_fact_sheet.htm assessed 05/01/02
10. ScientificAmerican.com. "The Prion Disease." 05/29/00. URL: http://www.sciam.com/0896issue/prion.html assessed on 05/01/02