- Creutzfeldt-Jakob Disease (CJD) is the most common human form of a group of rare, fatal brain disorders known as prion diseases.
- Prion diseases, such as CJD, occur when prion protein, which is found throughout the body but whose normal function isn't yet known, begins folding into an abnormal three-dimensional shape. This shape change gradually triggers prion protein in the brain to fold into the same abnormal shape. There are four types of CJD, one of which is genetic, another of which is blood-born. Caution urged when handling blood products as there is no cure and no way to properly sterilize implements.
Signs & Symptoms:
- Agitation, apathy and mood swings
- Rapidly worsening confusion, disorientation, and problems with memory, thinking, planning and judgment
- Difficulty walking
- Muscle stiffness, twitches and involuntary jerky movements
An estimated 90 percent of those diagnosed with sporadic CJD die within one year.
Tips for First Responders:
- Turn off lights and sirens as soon as possible
- Speak slowly and clearly
- Use "adult" words, but ask questions one at a time, rather than asking multiple part questions
- Ask the individual if s/he wears a medic alert device (this may tell you where they live)
- Maintain a calm manner; this may help the individual to calm down if s/he is anxious
Source: Henson, M., Ireton, M., & Mortensen, J. E., "Prions: A brief overview," (2013). AMT Events, 30 (2): 72-84. National Institute of Neurological Diseases & Stroke.