تعريف

Creutzfeldt-Jakob disease (CJD) is a rare, fatal, degenerative brain disorder characterized by rapidly progressive dementia.

CJD can be categorized into different subtypes:

  • Sporadic CJD—also called classical CJD; most common type; usually affects people aged 50 years and older
  • Familial CJD—an inherited form of the disease
  • Iatrogenic CJD—contracted through medical procedures, such as injections of growth hormone or receipt of corneal transplants or dura mater implants from affected donors
  • New variant CJD (vCJD) —caused by eating contaminated beef products, which may cause bovine spongiform encephalopathy (BSE)
    • BSE is commonly known as mad cow disease. vCJD differs from other forms of CJD because it affects younger people and has a longer average time course.

أسباب

There has been a lot of scientific research about the cause of CJD. Today, it is generally believed that most noninherited cases are caused by infectious proteins called prions. Prions can transform normal protein molecules into abnormal, disease-causing molecules.

عوامل الخطر

Factors that can increase your chance of developing CJD include:

  • Age: 50-75 years
  • Use of cadaveric growth hormone
  • Cornea transplants
  • Dura mater grafts
  • Family members with CJD—Approximately 10%-15% of cases are inherited
  • Eating beef products produced by countries with an epidemic of BSE
  • Healthcare workers who work with brain tissues
  • Blood transfusion from someone with CJD

الأعراض

Initially, there are no symptoms. As CJD progresses, symptoms that may occur include:

  • Memory lapses
  • Difficulty concentrating
  • Impaired judgment
  • Difficulty with speech
  • Loss of coordination
  • عدم وضوح الرؤية
  • Behavior and mood changes
  • تشنجات عضلية
  • النوبات
  • Loss of mental and physical function

Depending on the type of CJD, the disease may last from 3-36 months or longer. It is almost always fatal.

التشخيص

الطبيب سوف يسأل عن الأعراض والتاريخ الطبي. الفحص السريري سيتم القيام به.

CJD is a difficult disease to diagnose. There is no single test to detect it. The following tests may be used to help make a diagnosis:

  • You may need to have your brain activity recorded. This can be done withelectroencephalogram (EEG).
  • Images may need to be taken of your brain. This can be done with:
    • التصوير المقطعي المحوسب (CT).
    • التصوير بالرنين المغناطيسي (MRI).
  • You may need to have your brain’s use of glucose checked. This can be done with a brain positron emission tomography (PET) scan.
  • Your bodily fluids may need to be tested. This can be done with: removal of a sample of brain tissue for testing
    • خزعة الدماغ
    • تحاليل الدم
    • البزل القطني

Creutzfeldt Jakob Disease

العلاج

There is no cure for CJD. The aim of treatment is to relieve pain and alleviate symptoms.

Drug therapy may include:

  • Opiates to treat pain
  • Anticonvulsive drugs to help decrease neuromuscular problems

الوقاية

To avoid the new variant form of this condition, it is recommended that you avoid eating beef produced in areas that may have BSE. There are no known ways to prevent other forms of CJD.

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