May 09, 2025
I am Nikki Bland, an infection preventionist at UHC, and a member of the national board of directors at the CJD Foundation. In 2007, I lost my mother to CJD. I am here to discuss this rare neurodegenerative disease.
1). What is CJD?
Creutzfeldt-Jakob disease or (CJD) is a rare, rapidly progressing brain disorder. It belongs to a family of diseases known as prion diseases, formerly known as transmissible spongiform encephalopathies (TSEs).
Spongiform refers to the way affected brains look. With prion diseases, the brain is filled with holes and looks like a sponge when the tissue is examined under a microscope.
Like other prion diseases, CJD causes problems with muscle coordination, thinking, and memory. There are about 600 cases per year in the United States. This disease tends to progress rapidly. About 70% of people with CJD die within one year of getting the disease.
2). What are symptoms of CJD?
The main symptoms of CJD are cognitive decline leading to dementia, involuntary muscle jerks (myoclonus), and lack of coordination of movements.
Early symptoms of the disease may include:
- Poor coordination
- Walking and balance problems
- Confusion, disorientation, and delusions
- Problems with thinking, memory, and judgment
- Behavior changes, such as depression, mood swings, and anxiety
- Speech difficulty
- Insomnia or changes in sleeping patterns
- Vision changes
- Hallucinations or distorted perception of the world
- Dizziness
- Tremor
As CJD progresses, other symptoms can appear, including:
- Weakness of the arms and legs
- Blindness
- Inability to move or speak
- Problems swallowing
- Coma
Some people with CJD have an increased risk of pneumonia and other infections. Also, some symptoms of CJD can look like symptoms of other progressive neurological disorders, such as Alzheimer’s disease and Huntington’s disease. These symptoms tend to worsen faster in CJD than in Alzheimer’s disease and most other neurodegenerative diseases.
3). Who is more likely to get CJD?
There are three different ways a person might develop CJD:
- Sporadic
- Hereditary
- Acquired
Sporadic: In these cases, the disease develops in a person with no known cause. The majority of cases of CJD are sporadic. Sporadic CJD and other prion diseases develop because some of a person’s normal prion proteins suddenly change into abnormal prions. Harmful prions are believed to be made from an error in the “quality control” part of the cell that makes sure proteins are made properly. These errors are more likely to happen with aging. Symptoms usually first appear in people between ages 60 and 70 and quickly get worse.
Hereditary: Gene mutations that affect normal prion protein production can run in families. If the prion protein gene is changed in a parent’s cells, there is a 50% chance that the changed gene will be passed on to their children. But not all people with mutations in the prion protein gene develop symptoms of CJD. About 10% to 15% of CJD cases in the U.S. are hereditary. Hereditary CJD can affect people at a younger age than sporadic CJD, often under age 55. In rare cases, symptoms of genetic CJD can begin in a person’s 20s. Other genetic prion diseases include Gerstmann-Straussler-Scheinker Disease, or GSS, and Fatal Familial Insomnia, or FFI.
Acquired: CJD and other prion diseases can’t transmit through the air or through touching or most other forms of casual contact. But they may transmit by exposure to infected brain or nervous system tissue. This can happen through certain medical procedures such as surgical grafts of dura mater (a tissue that covers the brain) or cornea transplantation. This is one of the most talked about types of prion disease, but fewer than 1% of documented CJD cases are acquired. Fortunately, most of the ways that CJD can be acquired have been identified and safety measures have been put in place to decrease the chances of this happening. However, human growth hormone injections from the 1970s and earlier can still lead to transmission today.
This content was originally posted on the WDTV News website here.
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