Creutzfeldt-Jakob disease classification
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
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 types of 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 | Most prevalent, idiopathic, average age of onset is 65 years |
| Familial CJD | Results when a person inherited the abnormal prion (rare) |
Parchi et al classified sporadic CJD (sCJD) based on molecular and phenotypic features.[1]
| Previous classification | sCJD variants according to Parchi et al | Clinical features | Neuropathological features |
| Myoclonic, Heidenhan variants |
MM1 or MV1 | Rapidly progressive dementia myoclonus Altered vision Unilateral signs in beginning Typical EEG findings |
Occipital cortex involvement Confluent vacuoles Perivacuolar PrP staining |
| Ataxic variant | VV2 | Ataxia in early stage Dementia in later stages Typical EEG findings not found |
Brainstem nuclei and subcortical areas are affected Perinuclear PrP staining Plaque like focal Prp deposits |
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)
- Kuru in humans
- Bovine spongiform encephalopathy (BSE) commonly known as mad cow disease
- Chronic wasting disease (CWD)
- Scrapie in sheep
- Clinical and Pathologic Characteristics:[2]
| 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
- ↑ Parchi, P.; Giese, A.; Capellari, S.; Brown, P.; Schulz-Schaeffer, W.; Windl, O.; Zerr, I.; Budka, H.; Kopp, N. (1999). "Classification of sporadic Creutzfeldt-Jakob disease based on molecular and phenotypic analysis of 300 subjects". Ann Neurol. 46 (2): 224–33. PMID 10443888. Unknown parameter
|month=ignored (help) - ↑ Belay ED, Schonberger LB (2002). "Variant Creutzfeldt-Jakob disease and bovine spongiform encephalopathy". Clin. Lab. Med. 22 (4): 849–62, v–vi. PMID 12489284.