Understanding Creutzfeldt-Jakob disease

Creutzfeldt-Jakob disease is a rare, degenerative, fatal brain disorder. It affects about 1 person in every 1 million per year worldwide, and about 350 cases are diagnosed per year in the U.S., according to the National Institute of Neurological Disorders and Stroke.

The cause of Creutzfeldt-Jakob disease appears to be abnormal versions of a type of protein called a prion. Normally these proteins are produced in the body and are harmless. But when they're misshapen, they become infectious and can harm normal biological processes.

Signs and symptoms of Creutzfeldt-Jakob disease can be similar to those of other dementialike brain disorders, such as Alzheimer's disease. But Creutzfeldt-Jakob disease usually progresses much more rapidly.

Early signs and symptoms typically include:

• Personality changes.
• Memory loss.
• Impaired thinking.
• Blurred vision or blindness.
• Insomnia.
• Incoordination.
• Difficulty speaking.
• Difficulty swallowing.
• Sudden, jerky movements.

As the disease progresses, mental symptoms worsen. Most people eventually fall into a coma. Heart failure, lung failure, pneumonia or other infections are generally the cause of death, which usually occurs within a year.

Treatment of Creutzfeldt-Jakob disease focuses on relieving pain and other symptoms.