A Complete Guide to Creutzfeldt-Jakob Disease: What You Need to Know

Creutzfeldt-Jakob Disease

Creutzfeldt-Jakob disease (CJD) is a degenerative, very rare, and fatal brain disorder. According to the Centers for Disease Control, it impacts an estimated one in a million people every year in the U.S. This condition is the most common type of a rare group of fatal brain disorders called prion diseases. 

Prion diseases, such as CJD, occur when the “prior protein” —that is found throughout the human body — starts folding in an irregular 3D shape. Eventually, this deformed prion protein is replicated in the brain, which results in a type of dementia that progresses very fast. 

In contrast, Lewy body dementia, Alzheimer's, and frontotemporal dementia are more common types but tend to progress more slowly. Because of Creutzfeld-Jakob disease's relationship to dementia, the Long Island Alzheimer's and Dementia (LIAD) Center can offer programming and caregiver support for those impacted by this rare condition. Read on to learn more about CJD and how the LIAD Center can help impacted families. 

A Closer Look at Creutzfeldt-Jakob Disease 

Creutzfeldt-Jakob disease is a neurodegenerative disorder. While scientists do not understand the complete process, it's clear the irregular folds of the prion protein destroys brain cells. Once this happens, significant declines in an individual's ability to reason and think as well as involuntary muscle movements and mood changes typically occur. 

CJD typically appears later in an individual's life and tends to run a very rapid course. Many diagnosed individuals begin to experience symptoms around the age of 60. The symptoms individuals experience and the order in which they appear can vary. Some of the most common symptoms include:

  • Disorientation
  • Depression
  • Rapidly worsening confusion
  • Apathy, agitation, and mood swings
  • Vision problems, hallucinations, and double vision
  • Complications with thinking as well as memory, planning, and judgment.
  • Twitches, muscle stiffness, and involuntary jerky movements
  • Mobility issues, such as difficulty walking

Three Major Categories of Creutzfeldt-Jakob Disease 

While much research remains to be conducted on Creutzfeldt-Jakob disease, experts recognize three different groupings of the disorder. 

Sporadic CJD 

Sporadic CJD develops quickly for no understood reason. This type of CJD represents the majority of cases — an estimated 85%. On average, sporadic CJD begins to appear as an individual is between the ages of 60 and 65. 

Familial or Genetic CJD

Familial or genetic CJD is a single gene disorder caused by changes in the prion gene on chromosome 20. It's estimated anywhere from 10 to 15% of those who suffer from prion disease have the genetic form. In either case, those who are diagnosed with the condition have it because they inherited the genetic mutations from a parent. Genetic CJD tends to develop at an earlier age compared to sporadic CJD, and some genetic types can appear as early as 20. 

Acquired CJD

As the name suggests, acquired CJD is transmitted from exposure to nervous system tissue or brain tissue with the abnormal tissues. Acquired CJD is thought to account for only one percent of all cases. There are two common outside sources for this type of disease. 

Acquired CJD Via Medical Procedures

The most common source of acquired CJD transmission are medical procedures. However, the risk associated with getting acquired CJD from this mode has been significantly reduced due to: 

  • Modern neurosurgical instrumentation sterilization techniques, 
  • Synthetic sources of growth hormone, and 
  • One-time-use instruments

In either case, there is no evidence that suggests CJD is contagious through casual contact with someone suffering from the disease. 

Acquired vCJD

If someone eats cattle infected with "mad cow disease" —scientifically known as bovine spongiform encephalopathy (BSE) — they can develop CJD. In fact, BSE is a disease very similar to CJD. 

Scientists connected vCJD to the consumption of beef from cows who consumed feed that included brain tissue processed from other animals. Since the mid-90s when mad cow was connected to CJD, new cases of vCJD have significantly slowed primarily due to improved animal feeding habits. 

Contact the Long Island Alzheimer's & Dementia Center

Unfortunately, there is no treatment that has been proven to stop or slow the brain destruction caused by CJD and other types of prion diseases. Clinical studies of potential medical interventions are complicated by the rapid progression of the disease and its rarity. 

However, the Long Island Alzheimer's & Dementia Center offers support for individuals and caregivers living with all forms of dementia. We offer stage-specific services designed to focus on an individual’s abilities, not on disabilities. In addition, we offer various services for caregivers to receive the support, resources and care they need to provide the best for their loved one. 

If you or a loved one has been diagnosed with Creutzfeldt-Jakob disease, you are not alone. You have an entire team of support and dedicated solutions to help you navigate through your journey.

Contact the Long Island Alzheimer's & Dementia Center today to learn how we can help. Please call (516) 767-6856 or go to www.lidementia.org.

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