Creutzfeldt-Jakob disease (CJD) is a form of human prion diseases, fatal neurodegenerative conditions. They can be etiologically divided into sporadic, hereditary and acquired forms. Conformational change of the normal (cellular) form of prion protein (PrPc) to a pathological form (PrPSс) is a central event in the formation of an infectious agent. In this article, diagnostic criteria for sporadic CJD are summarized. Case report of probable sporadic CJD is presented. Many therapeutic strategies (based on cell cultures or animals) have been tested as potential treatments for prion diseases. However, only few clinical trials are in progress now or have been completed.
Sporadic Creutzfeldt-Jakob disease: clinical observation
- Authors: Peresedova A.V.1, Stoida N.I.1, Gnezditskiy V.V.1, Konovalov R.N.1, Korepina O.S.1, Zavalishin I.A.1
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Affiliations:
- Research Center of Neurology
- Issue: Vol 5, No 4 (2011)
- Pages: 52-56
- Section: Clinical analysis
- Submitted: 03.02.2017
- Published: 13.02.2017
- URL: https://annaly-nevrologii.com/journal/pathID/article/view/288
- DOI: https://doi.org/10.17816/psaic288
- ID: 288
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About the authors
A. V. Peresedova
Research Center of Neurology
Email: a.v.pesedova@mail.ru
Russian Federation, Moscow
N. I. Stoida
Research Center of Neurology
Email: a.v.pesedova@mail.ru
Russian Federation, Моscow
V. V. Gnezditskiy
Research Center of Neurology
Email: a.v.pesedova@mail.ru
Russian Federation, Moscow
Rodion N. Konovalov
Research Center of Neurology
Email: a.v.pesedova@mail.ru
ORCID iD: 0000-0001-5539-245X
Cand. Sci. (Med.), senior researcher, Neuroradiology department
Russian Federation, 125367 Moscow, Volokolamskoye shosse, 80Olga S. Korepina
Research Center of Neurology
Email: a.v.pesedova@mail.ru
Russian Federation, Moscow
I. A. Zavalishin
Research Center of Neurology
Author for correspondence.
Email: a.v.pesedova@mail.ru
Russian Federation, Moscow
References
- Chohan G., Pennington C., Mackenzie J.M. et al. The role of cerebrospinal fluid 14-3-3 and other proteins in the diagnosis of sporadic Creutzfeldt-Jakob disease in the UK: a 10-year review.J. Neurol. Neurosurg. Psychiatry 2010; 81: 1243–1248.
- Collinge G., Gorham M., Hudson F. et al. Safety and efficacy of quinacrine in human prion disease (PRION-1 study): a patient-preference trial. Lancet Neurol. 2009; 8: 334–344.
- Cuadrado-Corrales N., Jiménez-Huete A., Albo C. et al. Impact of the clinical context on the 14-3-3 test for the diagnosis of sporadic CJD. BMC Neurol. 2006; 6: 25.
- Geschwind M.D. Clinical trials for prion disease: difficult challenges, but hope for the future. Lancet Neurol. 2009; 8: 304–306.
- Hsich G., Kenney K., Gibbs C.J. et al. The 14-3-3 brain protein in cerebrospinal fluid as a marker for transmissible spongiform encephalopathies. N. Engl. J. Med. 1996; 335: 924–930.
- Kovacs G.G., Budka H. Molecular pathology of human prion diseases. Int. J. Mol. Sci. 2009; 10: 976–999.
- Kovacs G.G., Budka H. Prion diseases: from protein to cell pathology. Am. J. Pathol. 2008; 172: 555–565
- Ludewigs H., Zuber C., Vana K. Therapeutic approaches for prion disorders. Expert Rev. Anti. Infect. Ther. 2007; 5: 613–630.
- Otto M., Cepek L., Ratzka P. et al. Efficacy of flupirtine on cognitive function in patients with CJD: A double-blind study. Neurology 2004; 62: 714–718.
- Safar J., Wille H., Itri V. et al. Eight prion strains have PrP(Sc) molecules with different conformations. Nat. Med. 1998; 4: 1157–1165.
- Sanchez-Juan P., Bishop M.T., Croes E.A. et al. A polymorphism in the regulatory region of PRNP is associated with increased risk of sporadic Creutzfeldt-Jakob disease. BMC Med. Genet. 2011; 12: 73.
- Zerr I., Kallenberg K., Summers D.M. et al. Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease. Brain 2009; 132: 2659–2668.
- Zerr I., Pocchiari M., Collins S. et al. Analysis of EEG and CSF 14- 3-3 proteins as aids to the diagnosis of Creutzfeldt-Jakob disease. Neurology 2000; 55: 811–815.