Creutzfeldt-Jakob disease differential diagnosis: Difference between revisions
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* [[Wernicke-Korsakoff syndrome]] | * [[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> | * [[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> | ||
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"> | |||
<tr> | |||
<td>'''Characteristic''' </td> | |||
<td>'''Classic CJD'''</td> | |||
<td>'''Variant CJD'''</td> | |||
</tr> | |||
<tr> | |||
<td>Median age at death</td> | |||
<td>68 years</td> | |||
<td>28 years </td> | |||
</tr> | |||
<tr> | |||
<td>Median duration of illness</td> | |||
<td>4-5 months</td> | |||
<td>13-14 months </td> | |||
</tr> | |||
<tr> | |||
<td>Clinical signs and symptoms</td> | |||
<td>Dementia; early neurologic signs</td> | |||
<td>Prominent psychiatric/behavioral symptoms; painful [[dysesthesias]]; | |||
delayed neurologic signs </td> | |||
</tr> | |||
<tr> | |||
<td>Periodic sharp waves on [[electroencephalogram]]</td> | |||
<td>Often present</td> | |||
<td>Often absent</td> | |||
</tr> | |||
<tr> | |||
<td>Signal hyperintensity in the [[caudate nucleus]] and [[putamen]] on diffusion-weighted and FLAIR MRI</td> | |||
<td>Often present</td> | |||
<td>Often absent</td> | |||
</tr> | |||
<tr> | |||
<td>[[Pulvinar]] sign on MRI</td> | |||
<td>Not reported</td> | |||
<td>Present in >75% of cases</td> | |||
</tr> | |||
<tr> | |||
<td>[[Immunohistochemical staining|Immunohistochemical analysis]] of brain tissue</td> | |||
<td>Variable accumulation.</td> | |||
<td>Marked accumulation of protease-resistant prion protein</td> | |||
</tr> | |||
<tr> | |||
<td>Presence of agent in [[lymphoid]] tissue</td> | |||
<td>Not readily detected</td> | |||
<td>Readily detected </td> | |||
</tr> | |||
<tr> | |||
<td>Increased [[glycoform]] ratio on immunoblot analysis of | |||
protease-resistant prion protein</td> | |||
<td>Not reported </td> | |||
<td>Marked accumulation of protease-resistant prion protein </td> | |||
<tr> | |||
<td>Presence of amyloid plaques in brain tissue</td> | |||
<td>May be present</td> | |||
<td>May be present</td> | |||
</tr> | |||
</table> | |||
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Overview
Early signs of Creutzfeldt-Jakob disease(CJD) are highly variable, due to which it is often misdiagnosed or diagnosis is delayed. It should be differentiated from other causes of rapidly progressive dementia such as other neurodegenerative diseases, infections and dementia due to metabolic or toxic etiology.[1]
Differential Diagnosis of Creutzfeldt-Jakob Disease
CJD should be differentiated from variant Creutzfeldt-Jakob disease and Mad Cow disease. Other diseases it should be differentiated from are:
Neurodegenerative Diseases
- Alzheimer disease
- Dementia with Lewy bodies
- Frontotemporal dementia
- Corticobasal degeneration
- Normal pressure hydrocephalus
- Parkinsonian disorder
Autoimmune diseases
Infections
- Encephalitis
- Whipple disease
- Rabies
- Herpes simplex virus
- Postinfectious syndrome
Toxic or metabolic causes
- Vitamin B12 deficiency
- Hypothyroidism
- Side effects of drugs
- Wernicke-Korsakoff syndrome
- Urinary tract infection[1]
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
- ↑ 1.0 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.
- ↑ Belay ED, Schonberger LB (2002). "Variant Creutzfeldt-Jakob disease and bovine spongiform encephalopathy". Clin. Lab. Med. 22 (4): 849–62, v–vi. PMID 12489284.