Sporadic Creutzfeldt-Jakob disease in Northern Tasmania

Creutzfeldt-Jakob disease is a rare and incurable form of rapidly progressive neurodegenerative disease. The disease is fatal, and most patients die within 1 year of diagnosis. Clinical features include progressive cognitive dysfunction, delusions or hallucinations, cerebellar ataxia, myoclonus, vis...

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Bibliographic Details
Published in:The Journal of the Royal College of Physicians of Edinburgh 2023-03, Vol.53 (1), p.35-39
Main Authors: Rajalingam, Priyanka, de Souza, Aaron, Lee-Archer, Matthew, Dhakal, Mahesh
Format: Article
Language:English
Subjects:
Biomarkers
Creutzfeldt-Jakob Syndrome - diagnostic imaging
Humans
Neurodegenerative Diseases
Tasmania
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Summary:Creutzfeldt-Jakob disease is a rare and incurable form of rapidly progressive neurodegenerative disease. The disease is fatal, and most patients die within 1 year of diagnosis. Clinical features include progressive cognitive dysfunction, delusions or hallucinations, cerebellar ataxia, myoclonus, visual disturbances, extrapyramidal signs and eventually akinetic mutism. Most patients present with varied clinical presentation, hence making it difficult to diagnose at an early stage. We report five cases of sporadic Creutzfeldt-Jakob disease presenting to a Tasmanian hospital in Australia over a period of 52 months. We highlight significant clinical features in all our patients including few atypical presentations, emphasise on relevant clinical biomarkers and illustrate characteristic abnormalities on electroencephalogram and neuroimaging.
ISSN:1478-2715
2042-8189
DOI:10.1177/14782715221150580