Dementia > Creutzfeld-Jakob Disease (CJD)

What is Creutzfeldt-Jakob Disease (CJD)?
Creutzfeldt-Jakob disease (CJD) (pronounced CROYZ-felt YAH-cob) is a rare, rapidly fatal disorder affecting about 1 out of 1 million people per year worldwide. It usually affects individuals older than 60.

CJD is one of the prion (PREE-awn) diseases. These diseases occur when prion protein, which is present throughout the brain, begins to assume an abnormal three-dimensional shape. This shape gradually triggers the protein throughout the brain to fold into the same abnormal shape, leading to increasing damage and destruction of brain cells.

Recently, “variant Creutzfeldt-Jakob disease (vCJD)” was identified as the human disorder believed to be caused by eating meat from cattle affected by “mad cow disease.” It tends to occur in much younger individuals, in some cases as early as their teens.

Symptoms of Creutzfeldt-Jakob Disease (CJD)

• The first symptoms may involve impairment in memory, thinking and reasoning or changes in personality and behavior
  
  
• Depression or agitation also tend to be early symptoms
  
  
• Problems with movement may be present from the beginning or appear shortly after the other symptoms
  
  
• Symptoms progress rapidly and death typically occurs within a year
  
  
• There may be characteristic changes on an electroencephalogram (EEG, or “brain wave” test)
  
  
• There may also be a characteristic substance called “14-3-3 protein” in the spinal fluid