CLASSIC CREUTZFELDT-JAKOB DISEASE (CJD, CLASSIC)

Classic Creutzfeldt-Jakob disease (CJD, Classic) is a fatal neurodegenerative illness that affects both the nervous system and the brain. It is a specific kind of prion illness, which means that prion protein in an aberrant form is what causes it. 

Rapidly progressing dementia, including memory loss, behavioral abnormalities, and problems with coordination and movement, are hallmarks of CJD. Patients may endure muscle stiffness, uncontrollable movements, and blindness as the illness worsens. Moreover, patients eventually become immobile and may experience respiratory failure, which results in death.

TYPES

The brain and nervous system are both impacted by Classical Creutzfeldt-Jakob Disease (CJD, classic). Based on several variables, including the disease’s cause, symptoms, and course, it is divided into distinct categories. These are some examples of classic CJD:

  • Sporadic CJD: Making up 85% of cases, sporadic CJD is the most prevalent form of classic CJD.
  • Familial CJD: Inherited genetic alterations that impair the prion protein are the basis for familial CJD.
  • Iatrogenic CJD: Iatrogenic CJD is contracted during medical operations, such as using contaminated surgical equipment, ingesting hormones originating from the human pituitary, or receiving corneal grafts. 
  • Variant CJD: In the 1990s, mainly in the United Kingdom, exposure to tainted meat products was the main cause of variant CJD. 
  • Sporadic Fatal Insomnia (SFI): Less than 1% of people with conventional CJD have SFI, which is an extremely rare variant of the disease. 

SYMPTOMS

The most common symptoms of classic CJD include the following:

  • rapidly progressive dementia
  • coordination and balance problems
  • visual disturbances
  • behavioral changes
  • sleep disturbances
  • speech and language problems
  • seizures

DIAGNOSIS

Here are some of the diagnostic tests used to diagnose classic CJD:

  • Clinical evaluation
  • Electroencephalogram (EEG)
  • Magnetic Resonance Imaging (MRI)
  • Cerebrospinal Fluid (CSF) analysis
  • Brain biopsy

TREATMENT

The major goal of CJD treatment is to provide supportive care to manage symptoms and enhance the affected person’s quality of life. Supportive care may consist of the following, depending on the patient’s particular symptoms and needs:

  • Medication. This is used to treat symptoms like depression, convulsions, and muscle stiffness.
  • Nutritional support. This ensures the person gets enough food and water, which can be challenging due to swallowing issues.
  • Physical therapy. This helps to maintain flexibility and lessen stiffness in the muscles.
  • Hospice care. When a patient’s condition has advanced, this type of care can aid with symptom management and emotional support for both the patient and their family.

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