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Rare and Deadly Diseases

CJD: Mad Cow's Human Counterpart

Creutzfeld-Jakob disease (CJD) is a rare, fatal brain disease that causes rapid, progressive dementia and associated neuromuscular disturbances. It is a horrible disease that leads to a gruesome death. People experience mood swings, numbness, uncontrollable body movements, and other neurological symptoms. It is similar to Alzheimers, another disease that affects the brain.

The course of the disease takes around 18 months from the first appearance of symptoms to death, although it can be faster in some cases. There is no treatment, and the disease is always fatal. There are three ways people can get CJD: 1) it can occur sporadically; 2) it can be inherited; and 3) it can be transmitted through infection.

Even when it occurs by infection, CJD isn't contagious in the usual sense. Only a few cases have arisen after exposure to an infected individual. Infection of this type occurs as an accidental consequence of a medical procedure that uses tainted human matter or surgical instruments. This can happen with corneal transplants, implantation of electrodes in the brain, or contaminated surgical instruments. Normal human contact is not enough to spread the disease.

Disease Diction

Creutzfeld-Jakob disease is a rare disease—only one person in a million gets it each year.

From Subtle to Severe

There are several common symptoms in CJD patients. The initial stages can be subtle, with symptoms like insomnia, depression, confusion, personality changes, strange physical sensations, and problems with memory, coordination, and sight. As the disease progresses, people experience rapid, progressive dementia and involuntary jerking movements. Problems with language, sight, muscle weakness, and coordination worsen. In the final stages of the disease, the person loses all mental and physical function, lapses into a coma, and usually dies from an infection.

When to Suspect CJD

CJD should be considered when an adult patient develops rapid dementia and involuntary muscle jerks. Traditional lab tests have not worked to diagnose the disease, unfortunately. MRIs and x-rays have not been helpful, and cerebrospinal fluid usually appears normal. The most helpful test is the electroencephalogram (EEG), which measures brain wave activity and often shows an abnormal pattern when CJD is present.

A definitive diagnosis requires a brain biopsy or autopsy that can detect the changes in brain tissue caused by the disease. Work is ongoing to develop new tests, including ways to identify the prion protein thought to cause the disease.

Like mad cow, there is no effective treatment or cure for CJD. It is 100 percent fatal.

A New Variation of CJD

Creutzfeld-Jakob was first diagnosed in the 1920s. During the past 10 years, a variation of the disease has been found in Great Britain. The average age of patients was 28 as opposed to 63, and the disease duration was 14 months as opposed to four to six months. There have been 10 confirmed cases, and the most likely explanation is a direct link to exposure to infected meat. There have been four cases of this variant of the disease in France, one in Ireland, and none to date in the United States.

book cover

Excerpted from The Complete Idiot's Guide to Dangerous Diseases and Epidemics © 2002 by David Perlin, Ph.D., and Ann Cohen. All rights reserved including the right of reproduction in whole or in part in any form. Used by arrangement with Alpha Books, a member of Penguin Group (USA) Inc.

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