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What is Creutzfeldt-Jakob Disease?

What is Creutzfeldt-Jakob Disease?

  • Creutzfeldt-Jakob Disease: A very rare degenerative brain disease that can be inherited, transmitted (eg in surgical transplants using infected tissue) or as a result of genetic mutations. The condition is fatal.
  • Creutzfeldt-Jakob Disease: A rare transmissible encephalopathy most prevalent between the ages of 50 and 70 years. Affected individuals may present with sleep disturbances, personality changes, ATAXIA; APHASIA, visual loss, weakness, muscle atrophy, MYOCLONUS, progressive dementia, and death within one year of disease onset. A familial form exhibiting autosomal dominant inheritance and a new variant CJD (potentially associated with ENCEPHALOPATHY, BOVINE SPONGIFORM) have been described. Pathological features include prominent cerebellar and cerebral cortical spongiform degeneration and the presence of PRIONS. (From N Engl J Med, 1998 Dec 31;339(27))
    Source - Diseases Database
  • Creutzfeldt-Jakob Disease: rare (usually fatal) brain disease (usually in middle age) caused by an unidentified slow virus; characterized by progressive dementia and gradual loss of muscle control.
    Source - WordNet 2.1

Creutzfeldt-Jakob Disease is listed as a "rare disease" by the Office of Rare Diseases (ORD) of the National Institutes of Health (NIH). This means that Creutzfeldt-Jakob Disease, or a subtype of Creutzfeldt-Jakob Disease, affects less than 200,000 people in the US population.
Source - National Institutes of Health (NIH)

Ophanet, a consortium of European partners, currently defines a condition rare when it affects 1 person per 2,000. They list Creutzfeldt-Jakob Disease as a "rare disease".
Source - Orphanet

Creutzfeldt-Jakob Disease: Introduction

Types of Creutzfeldt-Jakob Disease:

Types of Creutzfeldt-Jakob Disease:

  • Sporadic CJD - no family history, about 85% of cases
  • Hereditary CJD - genetic family history, 5-10%
  • Variant CJD - believed to be related to BSE
  • Acquired CJD - exposure to brain products, less than 1% of cases
  • more types...»

Broader types of Creutzfeldt-Jakob Disease:

How many people get Creutzfeldt-Jakob Disease?

Incidence (annual) of Creutzfeldt-Jakob Disease: 1-per-million
Incidence Rate of Creutzfeldt-Jakob Disease: approx 1 in 1,000,000 or 0.00% or 271 people in USA [about data]
Prevalance of Creutzfeldt-Jakob Disease: It affects about one person in every one million people per year worldwide; in the United States there are about 200 cases per year. (Source: excerpt from Creutzfeldt-Jakob Disease Fact Sheet: NINDS) ... On the basis of mortality surveillance from 1979 to 1994, the annual incidence of CJD remained stable at approximately 1 case per million persons in the United States. (Source: excerpt from Facts About Creutzfeldt-Jakob Disease: CDC-OC) ... The usual type of CJD occurs worldwide and strikes about one in a million people per year. (Source: excerpt from Update Follow-Up Study of NHPP Growth Hormone Recipients: NIDDK)

Who gets Creutzfeldt-Jakob Disease?

Profile for Creutzfeldt-Jakob Disease: Typically, onset of symptoms occurs at about age 60. (Source: excerpt from NINDS Creutzfeldt-Jakob Disease Information Page: NINDS) ... The disease is found most frequently in patients 55-65 years of age, but cases can occur in persons older than 90 years and younger than 55 years of age. (Source: excerpt from Facts About Creutzfeldt-Jakob Disease: CDC-OC) ... Most people with CJD are between 55 and 79 years old. (Source: excerpt from Update Follow-Up Study of NHPP Growth Hormone Recipients: NIDDK)

How serious is Creutzfeldt-Jakob Disease?

Prognosis of Creutzfeldt-Jakob Disease: Poor. Typically death within 12 months.
Complications of Creutzfeldt-Jakob Disease: see complications of Creutzfeldt-Jakob Disease
Prognosis of Creutzfeldt-Jakob Disease: About 90 percent of patients die within 1 year. In the early stages of disease, patients may have failing memory, behavioral changes, lack of coordination and visual disturbances. As the illness progresses, mental deterioration becomes pronounced and involuntary movements, blindness, weakness of extremities, and coma may occur. (Source: excerpt from NINDS Creutzfeldt-Jakob Disease Information Page: NINDS)

What causes Creutzfeldt-Jakob Disease?

Causes of Creutzfeldt-Jakob Disease: see causes of Creutzfeldt-Jakob Disease
Risk factors for Creutzfeldt-Jakob Disease: see risk factors for Creutzfeldt-Jakob Disease

What are the symptoms of Creutzfeldt-Jakob Disease?

Symptoms of Creutzfeldt-Jakob Disease: see symptoms of Creutzfeldt-Jakob Disease

Complications of Creutzfeldt-Jakob Disease: see complications of Creutzfeldt-Jakob Disease

Duration of Creutzfeldt-Jakob Disease: In more than 85 percent of cases, the duration of CJD is less than 1 year (median: 4 months) after onset of symptoms. (Source: excerpt from Facts About Creutzfeldt-Jakob Disease: CDC-OC)

Can anyone else get Creutzfeldt-Jakob Disease?

Contagion of Creutzfeldt-Jakob Disease: Not generally contagious. Not even by blood transfusion. Very rarely by direct brain contagion such as in brain surgery or organ transplants; see contagion of prion diseases.
More information: see contagiousness of Creutzfeldt-Jakob Disease
Inheritance: see inheritance of Creutzfeldt-Jakob Disease

Creutzfeldt-Jakob Disease: Testing

Diagnostic testing: see tests for Creutzfeldt-Jakob Disease.

Misdiagnosis: see misdiagnosis and Creutzfeldt-Jakob Disease.

How is it treated?

Treatments for Creutzfeldt-Jakob Disease: see treatments for Creutzfeldt-Jakob Disease
Research for Creutzfeldt-Jakob Disease: see research for Creutzfeldt-Jakob Disease

Organs Affected by Creutzfeldt-Jakob Disease:

Organs and body systems related to Creutzfeldt-Jakob Disease include:

Name and Aliases of Creutzfeldt-Jakob Disease

Main name of condition: Creutzfeldt-Jakob Disease

Class of Condition for Creutzfeldt-Jakob Disease: prion

Other names or spellings for Creutzfeldt-Jakob Disease:

CJD, Jakob's disease, Jakob-Creutzfeldt disease, atrophia spongiforme cerebrale, Brownell-Oppenheimer, corticostriatal-spinal degeneration, Creutzfeldt Jacob disease, Hereditary CJD (subtype), Acquired CJD (subtype), Sporadic CJD (subtype)

CJD, Jakob-Creutzfeldt Disease Source - Diseases Database

Creutzfeldt-Jakob disease, CJD, Jakob-Creutzfeldt disease
Source - WordNet 2.1

Hereditary CJD (subtype), Sporadic CJD (subtype), VCJD, Creutzfeldt Jacob disease, Acquired CJD (subtype)
Source - Office of Rare Diseases (ORD) of the National Institutes of Health (NIH)

Research the causes of these diseases that are similar to, or related to, Creutzfeldt-Jakob Disease:


 » Next page: Online Medical Textbooks for Creutzfeldt-Jakob Disease

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