Creutzfeldt-Jakob disease differential diagnosis: Difference between revisions
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Revision as of 17:29, 18 September 2017
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Creutzfeldt-Jakob disease Microchapters |
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Differentiating Creutzfeldt-Jakob disease from other Diseases |
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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.[1]
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
- 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
- Adverse drug reaction
- 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.