Creutzfeldt-Jakob Disease[CJD] may have greater public health consequences than the suspected number of confirmed cases might indicate. CJD is not reportable in most states and is often misdiagnosed or omitted from death certificates. CJD is thought to be caused by prions. While the infectivity can be reduced, it is extremely difficult to kill these infectious agents. Normal sterilization procedures do not eliminate contamination.

What Is It?

Creutzfeldt-Jakob Disease (CJD) is a horrendous fatal brain-deteriorating disease for which there is no treatment or cure. Most scientists believe CJD is caused by a prion, which is an abnormal isoform of a host-encoded protein (a protien based molecule with no RNA or DNA). While there are many forms of CJD, recently, an atypical form, labeled nvCJD or new variant CJD, was discovered which appears to be more closely related to the clinical and pathological correlates of Kuru (Kuru was discovered in New Guinea and is said to be caused by canabilism rituals). nvCJD has been related to bovine spongiform encephalopathy [BSE] or as it is more commonly called, Mad Cow Disease (as of yet, not found in the United States). The incubation period for CJD was thought to be decades, however, recent clinical presentations have shown it could be much less.

Who Get's It and How?

Creutzfeldt-Jakob Disease affects both men and women worldwide usually between the ages of 50 to 75 years. The officially stated mortality rate is one to two deaths per one million population per year. However, this figure appears to be an understatement as CJD is often misdiagnosed. In one study by Yale University researchers 13% of Alzheimer patients were found upon autopsy to actually have CJD. A similar study performed at the University of Pittsburgh showed over 5% of Alzheimer's patients were CJD victims. There are three forms of CJD: familial (genetic, about 10-15% of cases), sporadic (cause unknown, about 80-85% of cases) and iatrogenic (through a medical procedure such as contaminated cadaver-derived growth hormones (GH), dura mater recipients, use of contaminated surgical instruments, and corneal transplant recipients, about 1% of cases) While it is theoretically possible the infectious agent may be present in blood, there are no documented cases of transmission by blood in humans. Pooled blood products are withdrawn as a precaution when a donor has been confirmed as a CJD victim or they are at a higher risk of contracting CJD. Unfortunately, in most cases blood products have already been utilized by individuals prior to the withdrawals. The CDC is currently under Congressional mandate to monitor blood product recipients for any evidence of increase of CJD incidence.

What Are The Symptoms of CJD?

The initial symptoms are subtle and ambiguous and can include insomnia, depression, confusion, personality/behavioral changes, strange physical sensations, balance disorders and/or memory, coordination and visual problems. Rapid progressive dementia and usually myoclonus (involuntary, irregular jerking movements) develop as CJD progresses. Also, language, sight, muscular weakness, swallowing and coordination problems worsen. The patient may appear startled and become rigid. In the final stage the patient loses all mental and physical functions. The patient may lapse into a coma and usually dies from an infection like pneumonia precipitated by the bedridden, unconscious state. The duration of CJD from the onset of symptoms to death is usually one year or less.

How Is CJD Diagnosed?

A diagnosis of CJD should be considered when a patient develops a rapid dementia and myoclonus. A 14-3-3 spinal fluid test is over 95% effective in supporting a clinical diagnosis of CJD. (Call Dr. Gibbs at NIH (30l) 496-4821 for more information) Another method of diagnosis is a brain biopsy. However, a brain biopsy may produce a false-negative result if the biopsied area was not affected by CJD. An autopsy with appropriate tests is the only method to achieve a 100% accurate diagnosis of CJD.

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