Creutzfeldt-Jakob disease: a review of current available evidence and the implications for prehospital emergency care.


creutzfeldt-jakob disease
transmissible spongiform encephalopathies
prion disease

How to Cite

Kirby HA. Creutzfeldt-Jakob disease: a review of current available evidence and the implications for prehospital emergency care. Australasian Journal of Paramedicine [Internet]. 2016Jul.31 [cited 2023Feb.6];13(3). Available from:


Introduction: Creutzfeldt-Jakob Disease effects 1 in 1 million people globally per year. It is an invariable fatal neurodegenerative disease belonging to the group of transmissible spongiform encephalopathies.  This review aims to examine the current clinical evidence surrounding CJD, as well as present management techniques to assist in minimizing the effect of symptoms. This review also highlights the important role of paramedics in reducing mortality and morbidity.

Methods: An electronic search was conducted using Medline (Via EBSCOHost), CINAHL, and the Cochrane Database of Systematic Reviews.
Results: This search resulted in 265 articles. Articles unavailable as full text, those not available in English, those articles not peer reviewed, and review articles were all excluded from analysis. The remaining 16 articles met the search criteria and were included in this report.

Conclusion: Scientific advancements are bringing a cure closer to reality, however further scientific research needs to be conducted. Training and education programs should be made available to prehospital workers to prevent further spread of infection.



Barnett F, McLean G. Care management of Creutzfeldt-Jakob disease within the United Kingdom. J Nurs Manag 2005;13:111–8.

Hsisch G, Kenney K, Gibbs C, Lee KH, Harrington MG. The 14-3-3 brain protein in cerebrospinal fluid as a marker for transmissible spongiform encephalopathies. N Engl J Med 1996;335:924–30.

Telfer JM. Creutzfeldt-Jakob disease - Implications for anaethetists in New Zealand. Anaesth Intensive Care 2009;37:386–91.

Millar CM, Makris M. Dealing with the uncertain risk of variant Creutzfeldt-Jakob disease transmission by coagulation replacement products. Br J Haematol 2012;158:442–52.

Millar CM, Connor N, Dolan G, et al. Risk reduction strategies for varient Creutzfeldt-Jakob disease transmission by UK plasma products and thier impact on patients with inherited bleeding disorders. Haemophilia 2010;16:305–15.

Relano-Genes A, Lehmann S, Bencsik A, Herva ME, Torres JM, Crozet CA. Stem cell therapy extends incubation and survival time in prion-infected mice in a time window-dependant manner. J Infect Dis 2011;1038–45.

Zerr I. Therapeutic trials in human transmissible spongiform encephalopathies: recent advances and problems to address. Infect Dis Drug Targets 2009;9:92–9.

Teruya K, Kawagoe K, Kimura T, Chen C-J, Sakasegawa Y, Doh-ura K. Amyloidophilic compounds for prion diseases. Infect Dis Drug Targets 2009;9:15–22.

Tsuboi Y, Doh-Ura K, Yamada T. Continious intraventricular infusion of pentosan polysulfaclinical trial against prion diseases. Neuropathology 2009;9:632–6.

Bone I, Belton L, Walker AS, Darbyshire J. Intraventricular infusion of pentosan polysulfate in human prion diseases: an observational study in the UK. Eur J Neurol 2008;15:458–64.

Newman PK, Todd NV, Scoones D, et al. Postmortem findings in a case of variant Creutzfeldt-Jakob disease treated with intraventricular pentosam polysulfate. Edinburgh: J Neurol Neurosurg Psychiatry 2014;85:921–4.

De Luigi A, Colombo L, Diomede L, et al. The efficacy of tetracyclines in peripheral and intracerebral prion infection. PLoS ONE. 2008;3:e1888.

Chapuis C, Casez O, Lagrange E, Bedouch P, Besson G. Hallucinatinos treated with rivastigmine in Creutzfeldt-Jakob disease. Fundam Clin Pharmacol 2011;2006:212–4.

Imperiale D, Bortolotto S, Cucatto A, Schiffer P, Cassano D, Buffa C. Levetiracetam control of myclonus in a pateint with Creutzfeldt-Jakob disease. Italy: Eur Neurol 2003;49:189–90.

Bird D, Robinson D. Torres and Ehrlich Modern Dental Assisting, 8th edn, 2005. Missouri: Elsevier Saunders.

Fraunhofer-Gesellschaft. Resistant prions: Can they be transmitted by environment as well as direct contact? Available at:

Imran M, Mahmood S. An overview of human prion diseases. Virol J 2011;8:559.