Creutzfeldt-Jakob disease classification: Difference between revisions

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There are two typed CJD, classic and variant form. Classic CJD characteristics, as compared to variant CJD, are presented in the table below.
There are two typed CJD, classic and variant form. Classic CJD characteristics, as compared to variant CJD, are presented in the table below.
The classic types of CJD are:
:*Sporadic CJD makes up the majority of cases. It occurs for no known reason. Average age at onset is 65
:*Familial CJD results when a person inherited the abnormal prion (inherited CJD is rare)
:*Classic CJD is not related to mad cow disease (bovine spongiform encephalitis).


Transmissible spongiform encephalopathy diseases are caused by prions. The diseases are thus sometimes called prion diseases.
Transmissible spongiform encephalopathy diseases are caused by prions. The diseases are thus sometimes called prion diseases.

Revision as of 18:53, 26 December 2012

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Classification

The prion that is believed to cause Creutzfeldt-Jakob exhibits at least two stable conformations. One, the native state, is water-soluble and present in healthy cells. As of 2006, its biological function is unknown. The other conformational state is very poorly water-soluble and readily forms protein aggregates.

There are two typed CJD, classic and variant form. Classic CJD characteristics, as compared to variant CJD, are presented in the table below. The classic types of CJD are:

  • Sporadic CJD makes up the majority of cases. It occurs for no known reason. Average age at onset is 65
  • Familial CJD results when a person inherited the abnormal prion (inherited CJD is rare)
  • Classic CJD is not related to mad cow disease (bovine spongiform encephalitis).

Transmissible spongiform encephalopathy diseases are caused by prions. The diseases are thus sometimes called prion diseases. Other prion diseases include Gerstmann-Sträussler-Scheinker syndrome (GSS), fatal familial insomnia (FFI) and kuru in humans, as well as bovine spongiform encephalopathy (BSE) commonly known as mad cow disease, chronic wasting disease (CWD), and scrapie in sheep.

  • Clinical and Pathologic Characteristics:[1]
Characteristic Classic CJD Variant CJD
Median age at death 68 years 28 years
Median duration of illness 4-5 months 13-14 months
Clinical signs and symptoms Dementia; early neurologic signs Prominent psychiatric/behavioral symptoms; painful dysesthesias; delayed neurologic signs
Periodic sharp waves on electroencephalogram Often present Often absent
Signal hyperintensity in the caudate nucleus and putamen on diffusion-weighted and FLAIR MRI Often present Often absent
Pulvinar sign on MRI Not reported Present in >75% of cases
Immunohistochemical analysis of brain tissue Variable accumulation. Marked accumulation of protease-resistant prion protein
Presence of agent in lymphoid tissue Not readily detected Readily detected
Increased glycoform ratio on immunoblot analysis of protease-resistant prion protein Not reported Marked accumulation of protease-resistant prion protein
Presence of amyloid plaques in brain tissue May be present May be present

References

  1. Belay ED, Schonberger LB (2002). "Variant Creutzfeldt-Jakob disease and bovine spongiform encephalopathy". Clin. Lab. Med. 22 (4): 849–62, v–vi. PMID 12489284.


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