Variant Creutzfeldt-Jakob Disease (cjd)

What is cjd?

Creutzfeldt-Jakob disease (CJD) is a rare, degenerative, invariably fatal brain disorder. It affects about one person in every one million people per year worldwide; in the United States there are about 300 cases per year. CJD usually appears in later life and runs a rapid course. Typically, onset of symptoms occurs about age 60, and about 90 percent of individuals die within 1 year.

DISCOVERY

  • CJD was discovered by Hans Gerhard Creutzfeldt and Alfons Maria Jakob in Germany in 1920.
  • The neurologists discovered the disease after observing a female patient with neurological abnormalities, formerly diagnosed with dementia.
Hans Gerhard Creutzfeldt and Alfons Maria Jakob

symptoms of cjd

  • amnesia, mental confusion, dementia, delusion, disorientation, blurred vision, insomnia, inability to speak or understand, or a lack of concentration
  • rhythmic muscle contractions, slow bodily movements, jerking muscle spasms, and problems with coordination, or overactive reflexes
  • anxiety or apathy, possible personality change
  • depression or hallucination
CJD prion

pathogen transmission

  • CJD is transmitted sporadically-or for no apparent reason
  • By inheritance- in the U.S., about 5-10% of people with CJD have a family history of the disease
  • By contamination- during a medical procedure, when a person is exposed to infected human tissue or when medical tools aren't properly sterilized
  • By exposure to BSE-

treatment options

Currently there is no cure for CJD. Treatment is aimed at alleviating symptoms and making the patient as comfortable as possible. Drugs can help relieve pain if it occurs.

contributions

For research, scientists are seeking biopsy and autopsy tissue, blood, and cerebrospinal fluid from patients with CJD and related diseases.

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