Creutzfeldt-Jakob disease (CJD): Symptoms, causes, and treatment

Creutzfeldt-Jakob disease is a rare neurodegenerative disease that rapidly, progressively, and severely affects the brain.

Creutzfeldt-Jakob Disease (CJD) gradually destroys brain cells, and it causes tiny holes in the brain. People with CJD will have ataxia, or difficulty controlling body movements, abnormal gait, speech, and dementia.

It is always fatal, and there is no cure.

CJD affects one person in every million globally each year, including the United States (U.S.).

Causes can be sporadic, inherited, or acquired. It mostly affects people over the age of 60 years, and it is rare in people under 30 years old.

A person with CJD is unlikely to survive longer than one year after symptoms appear.

What is CJD?

CJD is a rare but fatal disease.

CJD is a transmissible spongiform encephalopathy (TSE) that destroys the brain over time. It is caused by an infectious agent, called a prion. A prion is not a virus or bactera.

Other types of TSE include Gerstmann-Sträussler-Scheinker (GSS) syndrome, fatal familial insomnia, and kuru in humans. Other examples are scrapie in sheep and goats, and bovine spongiform encephalopathy (BSE), or “mad-cow disease,” in cattle.

Similar encephalopathies and wasting syndromes are found in other species, and they have been shown to be transmissible in laboratory animals.

The Centers for Disease Control and Prevention (CDC) note that classic CJD is not related to BSE or other variants.

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Sporadic CJD

In 85 percent of cases, CJD is sporadic. There are no apparent risk factors.

Inherited CJD

Between 5 percent and 10 percent of cases are inherited. They happen when a change occurs in the gene that controls the formation of prion proteins. There may be a family history of CJD, or a mutation may occur in the egg or sperm cells, putting offspring at risk of developing the disease.

Prions do not contain genetic information, and they do not need genes to reproduce themselves, but a mutation in the gene for the body’s normal prion protein can cause prions to act abnormally.

Several different mutations in the prion gene have been identified. The particular mutation found in each family affects how frequently the disease appears, and which symptoms are most noticeable.

Not everyone with mutations in the prion protein gene develop CJD.

Acquired CJD

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