• By Laurel Kelly

Consumer Health: Understanding Creutzfeldt-Jakob disease

April 21, 2022
an elderly white man with grey hair looking sad, worried, forgetful holding his head with his hands

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.

Connect with others caring for someone living with Creutzfeldt-Jakob disease in the Caregivers: Dementia support group on Mayo Clinic Connect, an online community moderated by Mayo Clinic.