Creutzfeldt-Jakob Disease

Creutzfeldt-Jakob disease (CJD) is a rare, degenerative, invariably fatal brain disorder. (Read about "The Brain") According to the Centers for Disease Control and Prevention (CDC), it strikes approximately one in a million people. However, the disease has become more widely known because a variant form of CJD seems to have developed in people who may have consumed meat tainted with so-called "Mad Cow Disease." (Read about "Mad Cow Disease") The variant is called vCJD, has a long incubation period and it is always fatal.

Like the variant form, prognosis for all forms of CJD is bleak. According to the National Institute of Neurological Disorders and Stroke (NINDS) there is no cure for CJD and about 90 percent of CJD patients die within a year.

In the early stages of the disease, patients may have the following symptoms:

• failing memory
• behavioral changes
• lack of coordination
• visual disturbances

As the illness progresses, mental deterioration becomes pronounced and involuntary movements, blindness, weakness of extremities, and coma may occur. According to the Creutzfeldt-Jakob Disease Foundation (CJF), the disease can afflict anyone. It affects both men and women, usually between 50 to 75 years of age.

CJD develops in any of three different ways. It can happen sporadically; it can be passed down through families, or it can be acquired. However, we don't know exactly what causes CJD to develop, though there has been some progress. NINDS says scientists are focusing on a type of protein called a prion. CJF says prions are thought to transform normal, benign protein molecules into infectious, deadly ones by altering the shape of the healthy molecules.

One problem with tracking down a cause is the fact that there is no diagnostic test for CJD. Doctors first rule out treatable forms of dementia. (Read about "Dementia") After that, the only way to confirm a diagnosis of CJD is by brain biopsy (Read about "Biopsy") or autopsy. However, NINDS says that biopsy is usually discouraged, since even a correct diagnosis of CJD does not help the patient. CJF says treatment is aimed at alleviating symptoms and making the patient as comfortable as possible. Painkillers can help to relieve pain; drugs may also be used to help calm the involuntary muscle movements.