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==Overview==
==Overview==
Creutzfeldt-Jakob disease must be differentiated from other causes of rapidly progressive [[dementia]], such as other neurodegenerative diseases, infections, Alzheimer's disease, vascular dementia, and dementia due to metabolic or toxic etiology.<ref name="pmid23229042">{{cite journal| author=Paterson RW, Torres-Chae CC, Kuo AL, Ando T, Nguyen EA, Wong K et al.| title=Differential diagnosis of jakob-creutzfeldt disease. | journal=Arch Neurol | year= 2012 | volume= 69 | issue= 12 | pages= 1578-82 | pmid=23229042 | doi=10.1001/2013.jamaneurol.79 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23229042  }} </ref>
Creutzfeldt-Jakob disease must be differentiated from other causes of rapidly progressive [[dementia]], such as other neurodegenerative diseases, infections, Alzheimer's disease, vascular dementia, and dementia due to metabolic or toxic etiology.  
*CJD should be differentiated from variant Creutzfeldt-Jakob disease and Mad Cow disease.
*Differential diagnosis of Creutzfeldt-Jakob disease includes the following:
===Neurodegenerative Diseases===
* [[Alzheimer disease]]
* [[Dementia with Lewy bodies]]
* [[Frontotemporal dementia]]
* [[Corticobasal degeneration]]
* [[Normal pressure hydrocephalus]]
* [[Parkinsonism|Parkinsonian disorder]]
===Autoimmune diseases===
* [[Paraneoplastic syndrome]]
* [[Vasculitis]]
* [[Neurosarcoidosis]]
===Infections===
* [[Encephalitis]]
* [[Whipple disease]]
* [[Rabies]]
* [[Herpes simplex virus]]
* Postinfectious syndrome
===Toxic or metabolic causes===
* [[Vitamin B12 deficiency]]
* [[Hypothyroidism]]
* [[Adverse drug reaction]]
* [[Wernicke-Korsakoff syndrome]]
* [[Urinary tract infection]]<ref name="pmid23229042">{{cite journal| author=Paterson RW, Torres-Chae CC, Kuo AL, Ando T, Nguyen EA, Wong K et al.| title=Differential diagnosis of jakob-creutzfeldt disease. | journal=Arch Neurol | year= 2012 | volume= 69 | issue= 12 | pages= 1578-82 | pmid=23229042 | doi=10.1001/2013.jamaneurol.79 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23229042  }} </ref>


==Differential Diagnosis of Creutzfeldt-Jakob Disease==
==Differential Diagnosis of Creutzfeldt-Jakob Disease==
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Clinical and pathologic characteristics of classic CJD and variant CJD:<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>
Clinical and pathologic characteristics of classic CJD and variant CJD:<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>
<table border="0" width="80%" id="table1" class="wikitable">
<table class="wikitable" id="table1" width="80%" border="0">
  <tr>
  <tr>
  <td>'''Characteristic''' </td>
  <td>'''Characteristic''' </td>
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[[Category:Neurology]]
[[Category:Neurology]]
[[Category:Disease]]
[[Category:Disease]]
[[Category:Transmissible spongiform encephalopathies]]
[[Category:Transmissible spongiform encephalopathies]]

Revision as of 13:43, 22 October 2018

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Twinkle Singh, M.B.B.S. [2]

Overview

Creutzfeldt-Jakob disease must be differentiated from other causes of rapidly progressive dementia, such as other neurodegenerative diseases, infections, Alzheimer's disease, vascular dementia, and dementia due to metabolic or toxic etiology.

  • CJD should be differentiated from variant Creutzfeldt-Jakob disease and Mad Cow disease.
  • Differential diagnosis of Creutzfeldt-Jakob disease includes the following:

Neurodegenerative Diseases

Autoimmune diseases

Infections

Toxic or metabolic causes

Differential Diagnosis of Creutzfeldt-Jakob Disease

  • CJD should be differentiated from variant Creutzfeldt-Jakob disease and Mad Cow disease.
  • Differential diagnosis of Creutzfeldt-Jakob disease includes the following:

Neurodegenerative Diseases

Autoimmune diseases

Infections

Toxic or metabolic causes

Clinical and pathologic characteristics of classic CJD and variant CJD:[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

  1. ↑ 1.0 1.1 Paterson RW, Torres-Chae CC, Kuo AL, Ando T, Nguyen EA, Wong K; et al. (2012). "Differential diagnosis of jakob-creutzfeldt disease". Arch Neurol. 69 (12): 1578–82. doi:10.1001/2013.jamaneurol.79. PMID 23229042.
  2. ↑ 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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