Creutzfeldt-Jakob Disease Signs and Symptoms
- Jun 25, 2011
CJD is diagnosed by a clinical neurological exam and electroencephalography (EEG), which shows characteristic spikes called triphasic sharp waves. Magnetic resonance imaging (MRI) or computed tomography scans (CT) should be done to exclude other forms of dementia, and in CJD typically shows atrophy or loss of brain tissue. Lumbar puncture, or spinal tap, may be done to rule out other causes of dementia (as cell count, chemical analysis, and other routine tests are normal in CJD) and to identify elevated levels of marker proteins known as 14-3-3. Another marker, neuron-specific enolase, may also be increased in CJD. CJD is conclusively diagnosed after death by brain autopsy. Scientists are investigating whether testing lymphatic tissue such as the tonsil may be an early tool in vCJD diagnosis. Additionally, recent studies have suggested that other blood tests may be useful as well.
There is no cure for CJD, and no treatment that slows the progression of the disease. Drug therapy and nursing care are aimed at minimizing psychiatric symptoms and increasing patient comfort. However, the rapid progression of CJD frustrates most attempts at treatment, since decreasing cognitive function and more prominent behavioral symptoms develop so quickly. Despite the generally grim prognosis, a few CJD patients progress more slowly and live longer than the average; for these patients, treatment will be more satisfactory. Scientists are investigating whether some medicines that can “break” the abnormal protein form may be useful and whether a vaccine could help.
Creutzfeldt-Jakob disease is invariably fatal, with death following symptom onset by an average of eight months. About 5% of patients live longer than two years. Death from vCJD has averaged approximately 12 months after onset.
There is no known way to prevent sporadic CJD, by far the most common type. Not everyone who inherits the gene mutation for familial CJD will develop the disease, but at present, there is no known way to predict who will and who won’t succumb. The incidence of iatrogenic CJD has fallen with recognition of its sources, the development of better screening techniques for infected tissue, and the use of sterilization techniques for surgical instruments that inactivate prion proteins.
Strategies for prevention of vCJD are a controversial matter, as they involve a significant sector of the agricultural industry and a central feature of the diet in many countries. The infectious potential of contaminated meat is unknown, because the ability to detect prions within meat is limited. Surveillance of North American herds strongly suggests there is no BSE here, and strict regulations on imports of European livestock make future outbreaks highly unlikely. Therefore, avoidance of all meat originating in North America, simply on grounds of BSE risk, is a personal choice unsupported by current data. The ban on the export of British beef continues in countries of the European Union, although some herds in these countries have developed low levels of infection as well.
By Larry I. Lutwick MDThe Gale Group Inc.
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