Creutzfeldt-Jakob disease classification: Difference between revisions
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==Overview== | ==Overview== | ||
Creutzfeldt-Jakob disease | Creutzfeldt-Jakob disease may be classified into either [[sporadic]], [[familial]] and [[iatrogenic]] forms. The sporadic form may be further categorized into either classic or variant Creutzfeldt-Jakob disease. | ||
==Classification== | ==Classification== | ||
The | *Creutzfeldt-Jakob disease may be classified into either [[sporadic]], [[familial]] and [[iatrogenic]] forms. | ||
* The sporadic form may be further categorized into either classic or variant Creutzfeldt-Jakob disease. | |||
===Sporadic CJD=== | |||
*Most common, idiopathic | |||
*Average age of onset is approximately 65 years | |||
===Familial CJD=== | |||
*Inheritance of abnormal prion | |||
*Exceptionally rare | |||
===Iatrogenic CJD=== | |||
*Associated with the use of contaminated human growth hormone, dura mater and corneal grafts, or neurosurgical equipment.<ref name="www.cdc.gov">{{Cite web | last = | first = | title = http://www.cdc.gov/ncidod/dvrd/cjd/qa_cjd_infection_control.htm | url = http://www.cdc.gov/ncidod/dvrd/cjd/qa_cjd_infection_control.htm | publisher = | date = | accessdate = 14 February 2014 }}</ref> | |||
Sporadic CJD (sCJD) may be classified based on molecular and phenotypic features into the following subtypes<ref name="Parchi-1999">{{Cite journal | last1 = Parchi | first1 = P. | last2 = Giese | first2 = A. | last3 = Capellari | first3 = S. | last4 = Brown | first4 = P. | last5 = Schulz-Schaeffer | first5 = W. | last6 = Windl | first6 = O. | last7 = Zerr | first7 = I. | last8 = Budka | first8 = H. | last9 = Kopp | first9 = N. | title = Classification of sporadic Creutzfeldt-Jakob disease based on molecular and phenotypic analysis of 300 subjects. | journal = Ann Neurol | volume = 46 | issue = 2 | pages = 224-33 | month = Aug | year = 1999 | doi = | PMID = 10443888 }}</ref> | |||
{|Class="wikitable" | {|Class="wikitable" | ||
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|'''Previous classification'''||'''sCJD variants | |'''Previous classification'''||'''sCJD variants'''||'''Clinical features'''||'''Neuropathological Features''' | ||
|- | |- | ||
|Myoclonic,<br> Heidenhan variants ||MM1 or MV1 ||Rapidly progressive [[dementia]]<br>[[Myoclonus]]<br>Altered vision<br>Unilateral signs in beginning<br>Typical [[EEG]] findings ||[[Occipital lobe|Occipital cortex]] involvement<br>Confluent vacuoles<br>Perivacuolar PrP staining | |Myoclonic,<br> Heidenhan variants ||MM1 or MV1 ||Rapidly progressive [[dementia]]<br>[[Myoclonus]]<br>Altered vision<br>Unilateral signs in beginning<br>Typical [[EEG]] findings ||[[Occipital lobe|Occipital cortex]] involvement<br>Confluent vacuoles<br>Perivacuolar PrP staining | ||
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<SMALL>Abbreviations: PrP=Prion protein | |||
* MM, VV and MV are genotypes of PrP. | *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 | *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 | *Type 1 and type 2 are based on the molecular mass of PrP, type 1: 19 kd, type 2: 21kd | ||
</SMALL> | |||
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==References== | ==References== | ||
{{Reflist|2}} | {{Reflist|2}} | ||
{{WikiDoc Help Menu}} | {{WikiDoc Help Menu}} | ||
{{WikiDoc Sources}} | {{WikiDoc Sources}} | ||
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[[Category:Disease]] | [[Category:Disease]] | ||
[[Category:Transmissible spongiform encephalopathies]] | [[Category:Transmissible spongiform encephalopathies]] | ||
Revision as of 19:43, 17 March 2016
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Creutzfeldt-Jakob disease Microchapters |
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Creutzfeldt-Jakob disease classification On the Web |
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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]
Overview
Creutzfeldt-Jakob disease may be classified into either sporadic, familial and iatrogenic forms. The sporadic form may be further categorized into either classic or variant Creutzfeldt-Jakob disease.
Classification
- Creutzfeldt-Jakob disease may be classified into either sporadic, familial and iatrogenic forms.
- The sporadic form may be further categorized into either classic or variant Creutzfeldt-Jakob disease.
Sporadic CJD
- Most common, idiopathic
- Average age of onset is approximately 65 years
Familial CJD
- Inheritance of abnormal prion
- Exceptionally rare
Iatrogenic CJD
- Associated with the use of contaminated human growth hormone, dura mater and corneal grafts, or neurosurgical equipment.[1]
Sporadic CJD (sCJD) may be classified based on molecular and phenotypic features into the following subtypes[2]
| Previous classification | sCJD variants | 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 |
Brain-stem 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 |
Abbreviations: 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
References
- ↑ "http://www.cdc.gov/ncidod/dvrd/cjd/qa_cjd_infection_control.htm". Retrieved 14 February 2014. External link in
|title=(help) - ↑ 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)