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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Creutzfeldt-Jakob disease Microchapters |
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Differentiating Creutzfeldt-Jakob disease from other Diseases |
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Diagnosis |
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Treatment |
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Case Studies |
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Creutzfeldt-Jakob disease classification On the Web |
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American Roentgen Ray Society Images of Creutzfeldt-Jakob disease classification |
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Risk calculators and risk factors for Creutzfeldt-Jakob disease classification |
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 |