Creutzfeldt-Jakob Disease

Overview

CJD is characterized by rapid deterioration of brain function, leading to severe cognitive impairment, motor dysfunction, and ultimately death. Although it shares some symptoms with Alzheimer’s disease, CJD progresses much more rapidly and has a devastating impact on affected individuals.

Types of Creutzfeldt-Jakob Disease

1. Sporadic CJD: This is the most common type, accounting for approximately 85% of cases. Sporadic CJD occurs spontaneously without any known risk factors. It typically affects adults aged 45 to 75, with symptoms developing between 60 and 65 years of age.

2. Variant CJD (vCJD): Linked to consuming meat from cattle infected with bovine spongiform encephalopathy (BSE or “mad cow” disease), vCJD has a longer incubation period. Strict controls have minimized its occurrence, but vigilance remains crucial.

3. Familial or Inherited CJD: A rare genetic condition caused by mutations in the prion protein gene inherited from parents. Prions accumulate in the brain during adulthood, triggering CJD symptoms.

4. Iatrogenic CJD: Rarely transmitted through medical procedures (e.g., contaminated surgical instruments or blood transfusions).

Causes of Creutzfeldt-Jakob Disease

Prion Proteins: CJD results from abnormal infectious proteins called prions. These prions accumulate in the brain, causing irreversible damage to nerve cells. Unlike viruses and bacteria, prions are resistant to heat, radiation, and standard sterilization methods.

Risk Factors of Creutzfeldt-Jakob Disease

1. Age: Sporadic CJD typically affects adults aged 45 to 75, with symptoms developing between 60 and 65 years of age.
2. Genetics: Familial or inherited CJD occurs due to mutations in the prion protein gene inherited from parents.
3. Consumption of Contaminated Meat: Variant CJD (vCJD) is linked to consuming meat from cattle infected with bovine spongiform encephalopathy (BSE or “mad cow” disease).
4. Medical Procedures: Iatrogenic CJD can rarely be transmitted through contaminated surgical instruments or blood transfusions.

Symptoms of Creutzfeldt-Jakob Disease

Preventions of Creutzfeldt-Jakob Disease

While complete prevention is challenging, several measures help minimize the risk of CJD:

• Food Chain Protection: Strict controls prevent BSE-infected meat from entering the human food chain.
• Blood Transfusion Safety: Screening donors and removing white blood cells reduce the risk of transmitting vCJD through blood transfusions.
• Sterilization Guidelines: Adherence to guidelines prevents iatrogenic CJD transmission via surgical instruments.

Diagnosis of Creutzfeldt-Jakob Disease

• No specific test confirms CJD, but a combination of medical history, neurological exams, EEG, MRI, and CSF analysis aids diagnosis.
• Brain autopsy and biopsy confirm CJD postmortem.

Treatment for Creutzfeldt-Jakob Disease

• No cure exists for CJD, so treatment focuses on symptom relief.
• Medications like morphine and methadone help manage pain.
• Self-care strategies improve quality of life.

When to seek medical attention ?

If you or a loved one experience any of the following symptoms, seek medical attention promptly:

• • Personality changes
  • Memory loss
  • Impaired thinking
  • Blurry vision or blindness
  • Insomnia
  • Problems with coordination
  • Trouble speaking and swallowing
  • Sudden, jerky movements