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Variant Creutzfeldt-Jakob disease
Jean-Philippe Brandel1, Richard Knight2
1Cellule nationale de référence des MCJ, Hôpital de la Salpêtrière, Paris, France.
Handbook of Clinical Neurology
|June 12, 2018
Summary
Variant Creutzfeldt-Jakob disease (vCJD) is a rare, fatal prion disease linked to BSE-infected cattle. While extremely rare now due to protective measures, concerns persist regarding asymptomatic infections and potential medical transmission.
Area of Science:
- Neurology
- Epidemiology
- Zoonotic Diseases
Background:
- Variant Creutzfeldt-Jakob disease (vCJD) emerged in the UK in 1996 as a zoonotic prion disease.
- vCJD results from dietary exposure to bovine spongiform encephalopathy (BSE)-infected cattle material.
- It presents distinct epidemiological, clinical, and neuropathological features compared to other human prion diseases.
Purpose of the Study:
- To summarize the key characteristics of variant CJD.
- To highlight diagnostic approaches and ongoing concerns.
- To provide an overview of vCJD's epidemiology and transmission.
Main Methods:
- Literature review of vCJD cases and research.
- Analysis of epidemiological data and clinical presentations.
- Evaluation of diagnostic methods, including neuropathology and imaging.
Main Results:
- vCJD cases have predominantly occurred in the UK and France.
- BSE decline and protective measures have made vCJD extremely rare.
- Concerns include asymptomatic infections and iatrogenic transmission risks.
Conclusions:
- Definitive vCJD diagnosis relies on neuropathology.
- Clinical diagnosis is possible using specific signs like the pulvinar sign.
- Emerging diagnostic tests show promise, but no cure currently exists for this progressive, fatal illness.
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