Creutzfeldt-Jakob disease classification: Difference between revisions
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Clinical and pathologic characteristics:<ref>{{cite journal |author=Belay ED, Schonberger LB |title=Variant Creutzfeldt-Jakob disease and bovine spongiform encephalopathy |journal=Clin. Lab. Med. |volume=22 |issue=4 |pages=849-62, v-vi |year=2002 |pmid=12489284 |doi=}}</ref> | |||
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Revision as of 21:14, 14 February 2014
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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 absent |
Brainstem nuclei and subcortical areas are affected Perinuclear PrP staining Plaque like focal Prp deposits |
| Kuru-plaques variant | MV2 | Ataxia Dementia Typical EEG findings absent Longer duration (>2 yrs) compared to other variants |
Amyloid-kuru plaques in cerebellum Plaque like focal PrP deposits |
| Thalamic variant | MM2 (thalamic) | Insomnia Hyperactivity Ataxia Cognitive impairment Typical EEG findings absent |
Thalamic and inferior olive atrpohy Spongiosis could be absent Lower amount of PrP staining |
| MM2 (cortical) | Dementia Typical EEG findings are absent |
Large confluent vacuoles Perivacuolar PrP staining All layers of cortex are affected | |
| VV1 | Dementia Typical EEG finding are absent |
Diffuse cortical involvement along with straitum Cerebellum is spared No large confluent vacuoles are present Lower amount of PrP staining |
- PrP: Prion protein
- MM, VV and MV are genotypes of PrP.
- MM1: MM genotype type 1 (M:Methionine;V:Valine), MV1:MV genotype type 1, VV2:VV genotype type 2, MV2:MV genotype type 2
- Type 1 and type 2 are based on the molecular mass of PrP, type 1: 19 kd, type 2: 21kd
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.