What is Creutzfeldt-Jakob Disease

What is Creutzfeldt-Jakob Disease

Creutzfeldt-Jakob disease (CJD) is a rare, fatal, neurodegenerative disorder that leads to dementia. It's the most common human form of prion diseases—a group of rare brain disorders that affect humans and animals.

Its symptoms are similar to Alzheimer's disease, but it progresses much more rapidly. In many instances, 70% of infected individuals die within one year of having the condition. This is a rare disorder, and annually, only about 1 in 1 million people worldwide acquire it. In the US, about 350 cases occur per year.

There are four types of Creutzfeldt-Jakob disease: Sporadic CJD, Variant CJD, Familial or inherited CJD, and Iatrogenic CJD. Of these, Sporadic CJD is the most common type and develops typically in people between 45 and 75.

What causes Creutzfeldt-Jakob disease

The condition is triggered when the prion protein present throughout the body starts to fold into an abnormal three-dimensional shape. Over time, this shape abnormality spreads to the brain and causes irreversible damage to nerve cells, leading to Creutzfeldt-Jakob disease.

An individual can contract this disease either from genetics or specific medical procedures, such as a cornea transplant. About 10% to 15% of CJD cases in the US are inherited. Also, the risk of transmission is very low as it can't be transferred through coughing, touching, or sexual contact.

Symptoms

The common symptoms of Creutzfeldt-Jakob disease are similar to other types of dementia and may include:

  • Problems with thinking, memory, judgment, and planning
  • Mood swings, indifference, agitation
  • Depression and insomnia
  • Rapidly worsening confusion
  • Muscle incoordination and loss of balance while walking
  • Slurred speech and difficulty in swallowing
  • Involuntary and sudden jerky movements, like twitches
  • Hallucinations and vision problems, such as blurred vision or blindness

Treatment

There's no cure for Creutzfeldt-Jakob disease. The rarity of the condition and its rapid progression makes it challenging for experts to conduct comprehensive clinical studies and learn more about it. Several drugs have been tested but failed to show any benefits. Painkillers and antiseizure drugs may help ease some of the symptoms.

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