Creutzfeldt-Jakob disease differential diagnosis

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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

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

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

↑ ^1.0 ^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.

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[pmid23229042-1] 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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