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: Mohamadmostafa Jahansouz M.D.[2] Twinkle Singh, M.B.B.S. [3]
Overview
Creutzfeldt-Jakob disease must be differentiated from other causes of rapidly progressive dementia, such as variant Creutzfeldt-Jakob disease, Mad Cow disease, Alzheimer disease, Dementia with Lewy bodies, Frontotemporal dementia, Corticobasal degeneration, Normal pressure hydrocephalus, parkinsonian disorder, Paraneoplastic syndrome, Vasculitis, Neurosarcoidosis,Encephalitis, Whipple disease, Rabies, Herpes simplex virus, postinfectious syndrome, Vitamin B12 deficiency, Hypothyroidism, Wernicke-Korsakoff syndrome and urinary tract infections.
Differential Diagnosis of Creutzfeldt-Jakob Disease
- CJD should be differentiated from other diseases presenting with dementia, ataxia, pyramidal/extrapyramidal signs, myoclonus, rigidity and visual hallucinations. The major differentials of CJD include the following:
| Disease | Signs and Symptoms | Imaging Findings | Laboratory Findings | |||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Dementia | Rigidity | Myoclonus | Visual Hallucinations | Parkinsonism | Speech Impairment | Fever | Cerebellar Signs (Ataxia, imbalance, dysdiadochokinesia) | Hyperkinetic Signs | Pyramidal Signs (Spasticity, Hyperreflexia, loss of fine motor skills, upgoing plantars) | |||
| Creutzfeldt-Jakob Disease | +
(Rapidly progressive) |
+ | + | + | - | + | - | + | + | + | On MRI:
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On CSF exam:
On EEG:
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| Lewy Body Dementia | + | + | + | + | + | + | - | - | - | - | ||
| Alzheimer's disease | + (Slow progression) | + | + | - | - | -/+ | - | - | - | - | On CSF exam:
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| Herpes Simplex Encephalitis | -/+ | - | - | - | - | + | + | -/+ | - | -/+ | On MRI:
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On CSF exam:
On EEG:
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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:[5]
| 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. PMC 4401069. PMID 23229042.
- ↑ Kojima G, Tatsuno BK, Inaba M, Velligas S, Masaki K, Liow KK (2013). "Creutzfeldt-Jakob disease: a case report and differential diagnoses". Hawaii J Med Public Health. 72 (4): 136–9. PMC 3689509. PMID 23795314.
- ↑ Poser S, Zerr I, Schroeter A, Otto M, Giese A, Steinhoff BJ; et al. (2000). "Clinical and differential diagnosis of Creutzfeldt-Jakob disease". Arch Virol Suppl (16): 153–9. PMID 11214918.
- ↑ Zerr I, Poser S (2002). "Clinical diagnosis and differential diagnosis of CJD and vCJD. With special emphasis on laboratory tests". APMIS. 110 (1): 88–98. PMID 12064260.
- ↑ Belay ED, Schonberger LB (2002). "Variant Creutzfeldt-Jakob disease and bovine spongiform encephalopathy". Clin. Lab. Med. 22 (4): 849–62, v–vi. PMID 12489284.