Creutzfeldt–Jakob disease

Creutzfeldt-Jakob diseaseCJDcreutzfeldt-jakob syndromeclassic Creutzfeldt–Jakob diseaseCreutzfeldt–JakobCreutzfeldt–Jakob disease (CJD)variant Creutzfeldt–Jakob diseaseCreutzfeld-Jakob diseaseCreutzfeldt-Jacob diseaseCreutzfeldt–Jakob disorder
Creutzfeldt–Jakob disease (CJD), also known as classic Creutzfeldt–Jakob disease, is a fatal degenerative brain disorder.wikipedia
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senilesenilitysenile dementia
Later symptoms include dementia, involuntary movements, blindness, weakness, and coma.
Less common causes include normal pressure hydrocephalus, Parkinson's disease dementia, syphilis, HIV, and Creutzfeldt–Jakob disease.

Transmissible spongiform encephalopathy

prion diseasestransmissible spongiform encephalopathiesspongiform encephalopathy
It is classified as a type of transmissible spongiform encephalopathy.
TSEs of humans include Creutzfeldt–Jakob disease—which has four main forms, the sporadic (sCJD), the hereditary/familial (fCJD), the iatrogenic (iCJD) and the variant form (vCJD)—Gerstmann–Sträussler–Scheinker syndrome, fatal familial insomnia, kuru, and the recently discovered variably protease-sensitive prionopathy.

Variant Creutzfeldt–Jakob disease

vCJDvariant Creutzfeldt-Jakob diseaseVariant CJD
CJD is different from bovine spongiform encephalopathy (mad cow disease) and variant Creutzfeldt–Jakob disease (vCJD).
It is different from classic Creutzfeldt–Jakob disease, though both are due to prions.


myoclonicmyoclonic jerkmyoclonic jerks
Myoclonus (jerky movements) typically occurs in 90% of cases, but may be absent at initial onset.
Most often, myoclonus is one of several signs in a wide variety of nervous system disorders such as multiple sclerosis, Parkinson's disease, dystonia, Alzheimer's disease, Gaucher's disease, subacute sclerosing panencephalitis, Creutzfeldt–Jakob disease (CJD), serotonin toxicity, some cases of Huntington's disease, some forms of epilepsy, and occasionally in intracranial hypotension.

Bovine spongiform encephalopathy

mad cow diseaseBSEMad Cow
CJD is different from bovine spongiform encephalopathy (mad cow disease) and variant Creutzfeldt–Jakob disease (vCJD).
BSE is a transmissible disease that primarily affects the central nervous system; it is a form of transmissible spongiform encephalopathy, like Creutzfeldt–Jakob disease and kuru in humans and scrapie in sheep, and chronic wasting disease in deer.

Protein folding

Infectious prions are misfolded proteins that can cause normally folded proteins to become misfolded.
Aggregated proteins are associated with prion-related illnesses such as Creutzfeldt–Jakob disease, bovine spongiform encephalopathy (mad cow disease), amyloid-related illnesses such as Alzheimer's disease and familial amyloid cardiomyopathy or polyneuropathy, as well as intracellular aggregation diseases such as Huntington's and Parkinson's disease.


prion proteinPrPCD230
In the familial form, a mutation has occurred in the gene for PrP, PRNP, in that family.
The misfolded version PrP Sc is associated with a variety of cognitive disorders and neurodegenerative diseases such as in animals: ovine scrapie, bovine spongiform encephalopathy (BSE, mad cow disease), feline spongiform encephalopathy, transmissible mink encephalopathy (TME), exotic ungulate encephalopathy, chronic wasting disease (CWD) which affects cervids; and in humans: Creutzfeldt–Jakob disease (CJD), fatal familial insomnia (FFI), Gerstmann–Sträussler–Scheinker syndrome (GSS), kuru, and variant Creutzfeldt–Jakob disease (vCJD).

Kuru (disease)

kuruKuru diseasethe Kuru disease
Cannibalism has also been implicated as a transmission mechanism for abnormal prions, causing the disease known as kuru, once found primarily among women and children of the Fore people in Papua New Guinea.
The epidemic likely started when a villager developed sporadic Creutzfeldt–Jakob disease and died.


Prions, the infectious agent of CJD, may not be inactivated by means of routine surgical instrument sterilization procedures.
These abnormally folded proteins are found characteristically in some diseases such as scrapie, bovine spongiform encephalopathy (mad cow disease) and Creutzfeldt–Jakob disease.

14-3-3 protein

14-3-314-3-3 proteins14-3-3 binding
Elevated amounts of 14-3-3 proteins are found in the cerebrospinal fluid of patients with Creutzfeldt–Jakob disease.


prionsprion diseasePrion diseases
CJD is caused by a protein known as a prion.
Prion variants of the prion protein (PrP), whose specific function is uncertain, are hypothesized as the cause of transmissible spongiform encephalopathies (TSEs), including scrapie in sheep, chronic wasting disease (CWD) in deer, bovine spongiform encephalopathy (BSE) in cattle (commonly known as "mad cow disease") and Creutzfeldt–Jakob disease (CJD) in humans.

Alzheimer's disease

AlzheimerAlzheimer’s diseaseAlzheimer disease
Diffuse cortical vacuolization occurs in Alzheimer's disease, and superficial cortical vacuolization occurs in ischemia and frontotemporal dementia.
One receptor for A β oligomers may be the prion protein, the same protein that has been linked to mad cow disease and the related human condition, Creutzfeldt–Jakob disease, thus potentially linking the underlying mechanism of these neurodegenerative disorders with that of Alzheimer's disease.


Prions, the infectious agent of CJD, may not be inactivated by means of routine surgical instrument sterilization procedures.
However, prions, such as those associated with Creutzfeldt–Jakob disease, and some toxins released by certain bacteria, such as Cereulide, may not be destroyed by autoclaving at the typical 134 °C for three minutes or 121 °C for 15 minutes.

Hans Gerhard Creutzfeldt

CreutzfeldtHans-Gerhard Creutzfeldt
The disease was first described by German neurologist Hans Gerhard Creutzfeldt in 1920 and shortly afterward by Alfons Maria Jakob, giving it the name Creutzfeldt–Jakob.
Although he is typically credited as the physician to first describe the Creutzfeldt–Jakob disease, this has been disputed.

Real-Time Quaking-Induced Conversion

It samples cerebrospinal fluid (CSF) and so it is applicable to scrapie, to chronic wasting disease (CWD), to bovine spongiform encephalopathy (BSE) and to sporadic Creutzfeldt–Jakob disease, amongst others.≈


In the 1980s it was discovered that Lyodura, a dura mater transplant product was shown to transmit Creutzfeldt–Jakob disease from the donor to the recipient.
Lyodura was a medical product used in neurosurgery that has been shown to transmit Creutzfeldt–Jakob disease, a degenerative neurological disorder that is incurable, from affected donor cadavers to surgical recipients.

Dura mater

duraduralcovering of the spinal cord
The defective protein can be transmitted by contaminated harvested human brain products, corneal grafts, dural grafts, or electrode implants and human growth hormone.
The American Red Cross and some other agencies accepting blood donations consider dura mater transplants, along with receipt of pituitary-derived growth hormone, a risk factor due to concerns about Creutzfeldt–Jakob disease.

Chronic wasting disease

CWDwasting disease, chronic
TSEs are a family of diseases thought to be caused by misfolded proteins called prions and includes similar diseases such as BSE (mad cow disease) in cattle, Creutzfeldt-Jakob disease (CJD) in humans and scrapie in sheep.


In January 2007, she and her colleagues reported that they had found a virus-like particle in naturally and experimentally infected animals.
They can cause infections such as scrapie in sheep, bovine spongiform encephalopathy ("mad cow" disease) in cattle, and chronic wasting disease in deer; in humans, prionic diseases include Kuru, Creutzfeldt–Jakob disease, and Gerstmann–Sträussler–Scheinker syndrome.

Barbara Tarbuck

Barbara J. Tarbuck
American actress Barbara Tarbuck (General Hospital, American Horror Story) died of the disease on December 26, 2016.
Tarbuck had the neurodegenerative Creutzfeldt–Jakob disorder.

Stanley B. Prusiner

Stanley PrusinerPrusinerStanley Ben Prusiner
Stanley B. Prusiner of the University of California, San Francisco (UCSF) was awarded the Nobel Prize in physiology or medicine in 1997 "for his discovery of Prions—a new biological principle of infection".
Stanley Prusiner won the Nobel Prize in Physiology or Medicine in 1997 for his work in proposing an explanation for the cause of bovine spongiform encephalopathy ("mad cow disease") and its human equivalent, Creutzfeldt–Jakob disease.

Alfons Maria Jakob

The disease was first described by German neurologist Hans Gerhard Creutzfeldt in 1920 and shortly afterward by Alfons Maria Jakob, giving it the name Creutzfeldt–Jakob.
He first recognised and described Alper's disease and Creutzfeldt–Jakob disease (named along with Munich neuropathologist Hans Gerhard Creutzfeldt).

Rebecca D. Lockhart

Becky LockhartRebecca LockhartLockhart, Rebecca D.
In January 2015, former speaker of the Utah House of Representatives Rebecca D. Lockhart died of the disease within a few weeks of diagnosis.
Lockhart died at her home in Provo, Utah on January 17, 2015, from Creutzfeldt–Jakob disease, "an unrecoverable and extremely rare neurodegenerative brain disease".

Friedrich Meggendorfer

An early description of familial CJD stems from the German psychiatrist and neurologist Friedrich Meggendorfer (1880–1953).
In 1930 he provided an early description of familial Creutzfeldt–Jakob disease in the "Backer family" of northern Germany.


and full clinical trials of its use as a treatment for Creutzfeldt–Jakob disease are under way in the United Kingdom and the United States.