Prion disease facts for kids

A prion disease is a sickness caused by tiny things called prions. Prions are like normal proteins in your body, but they have changed their shape. Unlike bacteria, prions are not considered alive. They don't have their own way to make energy or reproduce by themselves.

When a prion touches a normal protein, it can make the normal protein change its shape too. This is how prion diseases spread inside the body. These diseases are very rare, and doctors don't have a cure for most of them yet.

Most prion diseases affect the brain and nervous system. Two main signs of these diseases are:

• Ataxia: This means a person has trouble standing or walking steadily. It's like losing your balance often because of a problem in the part of the brain called the cerebellum.
• Dementia: This is when a person slowly loses their ability to think clearly, remember things, or solve problems.

Types of Prion Diseases

There are a few types of prion diseases that doctors know about. The most important ones include:

Creutzfeldt-Jakob Disease

Creutzfeldt-Jakob disease is a serious prion disease. People can get it in different ways, such as by eating beef infected with a similar disease called BSE (mad cow disease). It can also spread through blood tranfusions from an infected person, or from certain medical treatments using human growth hormone, or even through infected surgical tools. Patients with this disease often have trouble walking and thinking clearly. Sadly, they usually pass away within about a year.

Kuru Disease

Kuru disease was mainly seen in people living in New Guinea. It spread when people ate the brains of dead people as part of a funeral ritual. Patients with Kuru also have trouble walking, thinking, and moving their eyes. They usually die within two years.

Gerstmann-Straussler-Scheinker Syndrome

Gerstmann-Straussler-Scheinker syndrome is a very rare disease. People with this syndrome also experience problems with balance and thinking. Patients usually pass away within about a year.

How Rare Are Prion Diseases?

Prion diseases are very uncommon. For example, Creutzfeldt-Jakob disease, which is the most common type, affects only about 1 to 1.5 people out of every one million people each year.

Related Pages

• Prion
• Encephalopathy

Images for kids

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  Prion protein (stained in red) revealed in a photomicrograph of neural tissue from a scrapie-infected mouse.

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  Fibril model of prion propagation.

See also

In Spanish: Encefalopatía espongiforme transmisible para niños