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Creutzfeldt-Jakob Disease

Overview

Creutzfeldt-Jakob disease (CJD) is a rare, quickly progressing degenerative brain disorder that is invariably fatal. About 300 to 400 cases occur each year in the United States. Nearly 75 percent of Creutzfeldt-Jakob disease patients die within one year. CJD belongs to a family of human and animal diseases known as transmissible spongiform encephalopathies (also known as prion diseases).

There are three major categories of Creutzfeldt-Jakob disease based on etiology:

  • Sporadic

    (approximately 85 percent of CJD cases)

    The disease occurs sporadically or spontaneously, with no apparent cause and is due to a modification of the normal prion protein.

  • Genetic

    (approximately 10 to 15 percent of CJD cases)

    The disease occurs by genetic mutation. Genetic prion diseases include genetic CJD, fatal familial insomnia (FFI), and Gerstmann-Straussler-Scheinker disease (GSS). Affected individuals often have a family history of prion disease.

  • Acquired

    (less than 1 percent of CJD cases)

    CJD can be transmitted through medical procedures with contaminated equipment or tissue (e.g., cadaveric human growth hormone, pituitary derived gonadotropins, dura mater grafts, corneal transplants); this is called iatrogenic CJD. Another form of the disease — variant CJD — is transmitted through eating beef that is infected with bovine spongiform encephalopathy, or mad cow disease.

Symptoms

The onset of symptoms typically occurs at about age 60 often with signs and symptoms of dementia, imbalance, incoordination, and/or visual symptoms.

In diagnosing Creutzfeldt-Jakob disease, the first concern is to rule out treatable forms of dementia, such as encephalitis.

Treatment

There is no treatment that can cure or control CJD; treatment is aimed at alleviating symptoms and making the patient as comfortable as possible in addition to providing the family with adequate support and resources. Our staff works closely with the National Prion Disease Pathology Surveillance Center and the CJD Foundation to assist with the diagnosis and support of individuals affected by prion diseases.

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