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CREUTZFELDT-JAKOB DISEASE (CJD)

Creutzfeldt-Jakob Disease (CJD) is a rare, fatal brain disorder caused by abnormal prion proteins. It leads to rapid brain damage, dementia, muscle jerks, and loss of coordination. Mostly affecting older adults, it has no cure and usually results in death within a year. Spread is rare and non-contagious.

Description

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Context:

Two people are believed to have died from Creutzfeldt-Jakob Disease (CJD) within a few months in the United States.

About Creutzfeldt-Jakob Disease (CJD)  

It is a very rare brain disease that causes the brain to break down quickly. It creates small holes in the brain, making it look like a sponge under a microscope. These holes damage the brain’s ability to function properly.

It is caused by abnormal proteins called prions. Once these prions start building up in the brain, they destroy nerve cells, which leads to severe neurological damage and death.

As the brain breaks down, people with CJD lose their ability to think, move, and function normally. 

This disease mostly affects older adults, usually between 50 and 75 years old. Around the world, only about 1 or 2 people out of every million get diagnosed with CJD each year. 

How Does CJD Spread?

CJD doesn’t spread like a cold or flu. It spreads in three main ways:

  1. Sporadically: Most cases (about 85%) happen randomly without any clear reason. Scientists aren’t sure why this happens, but it just occurs naturally.
  2. By Inheritance: About 15% of cases are passed down through families. If someone has a mutated gene, they can develop CJD.
  3. By Contamination: Though very rare, CJD can spread through contaminated surgical tools or infected tissue during medical procedures. Modern safety rules have made this less likely, but it’s still possible.

Symptoms of CJD

  • Cognitive Decline : People lose their ability to think clearly and develop dementia.
  • Muscle Jerks : They experience sudden, involuntary muscle movements called myoclonus.
  • Lack of Coordination : Their movements become clumsy and uncoordinated

These symptoms worsen over weeks or months until the person can no longer care for themselves.

Treatment

There is no cure for CJD. Once someone gets it, the disease will always lead to death. Doctors focus on

keeping patients comfortable and managing their symptoms. For example:

  • They give medications to reduce pain or anxiety.
  • They help with daily tasks like eating and moving around.
  • In advanced stages, full-time nursing care becomes necessary.

Most people with CJD die within a year after symptoms appear.

Source:

FIRST POST

PRACTICE QUESTION

Q. In the question given below, there are two statements marked as Assertion (A) and Reason (R). Mark your answer as per the codes provided:

Assertion (A): Creutzfeldt-Jakob Disease (CJD) results from misfolded prion proteins that induce neuronal degeneration.

Reason (R): Prions are unique infectious agents lacking nucleic acids, challenging traditional virology paradigms.

Which of the options given below is correct?

A) Both A and R are true, and R is the correct explanation for A.

B) Both A and R are true, but R is not the correct explanation for A.

C) A is true, but R is false.

D) A is false, but R is true.            

Answer: B

Explanation

Assertion (A): Creutzfeldt-Jakob Disease (CJD) is a fatal neurodegenerative disease caused by the accumulation of abnormally folded prion proteins in the brain, which leads to the death of neurons. This statement is true.

Reason (R): Prions are unique infectious agents that are composed solely of protein and lack any genetic material (nucleic acids like DNA or RNA). Their discovery challenged the conventional understanding of infectious agents, which were thought to require nucleic acids for replication. This statement is also true.

Reason (R) does not explain why the misfolded prion proteins cause neuronal degeneration. The lack of nucleic acids explains the unique biological nature of prions as infectious agents but does not elucidate the mechanism of neurotoxicity and degeneration described in Assertion (A). The neurodegeneration is caused by the misfolding and subsequent aggregation of the prion proteins, disrupting cellular function and leading to cell death.

Thus, both statements are true, but the reason is not the correct explanation for the assertion.

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