The diagnosis of rapidly progressive dementias (RPDs), particularly sporadic Jakob–Creutzfeldt disease (sCJD), can prove challenging. Treatable RPDs can mimic sCJD, which is currently untreatable. A recent review of a large cohort of patients with suspected CJD highlights the extent of misdiagnosis and possible sources of error.
This is a preview of subscription content, access via your institution
Relevant articles
Open Access articles citing this article.
-
Creutzfeldt-Jakob disease mimicking autoimmune encephalitis with CASPR2 antibodies
BMC Neurology Open Access 30 November 2014
Access options
Subscribe to this journal
Receive 12 print issues and online access
$209.00 per year
only $17.42 per issue
Buy this article
- Purchase on Springer Link
- Instant access to full article PDF
Prices may be subject to local taxes which are calculated during checkout
References
Geschwind, M. D., Shu, H., Haman, A. Seyvar, J. J. & Miller, B. L. Rapidly progressive dementia. Ann. Neurol. 64, 97–108 (2008).
Tschampa, H. J. et al. Patients with Alzheimer's disease and dementia with Lewy bodies mistaken for Creutzfeldt–Jakob disease. J. Neurol. Neurosurg. Psychiatry 71, 33–39 (2001).
Poser, S. et al. How to improve the clinical diagnosis of Creutzfeldt–Jakob disease. Brain 122, 2345–2351 (1999).
Chitravas, N. et al. Treatable neurological disorders misdiagnosed as Creutzfeldt–Jakob disease. Ann. Neurol. doi: 10.1002/ana.22454.
World Health Organization. Global surveillance, diagnosis and therapy of human transmissible spongiform encephalopathies: Report of a WHO consultation. World Health Organization [online], (1998).
Vitali, P. et al. Diffusion-weighted MRI hyperintensity patterns differentiate CJD from other rapid dementias. Neurology 76, 1711–1719 (2011).
Zerr, I. et al. Updated clinical diagnostic criteria for sporadic Creutzfeldt–Jakob disease. Brain 132, 2659–2668 (2009).
Geschwind, M. D., Josephs, K. A., Parisi, J. E. & Keegan, B. M. A 54-year-old man with slowness of movement and confusion. Neurology 69, 1881–1887 (2007).
Papageorgiou, S. G. et al. Rapidly progressive dementia: causes found in a Greek tertiary referral center in Athens. Alzheimer Dis. Assoc. Disord. 23, 337–346 (2009).
Atarashi, R. et al. Ultrasensitive human prion detection in cerebrospinal fluid by real-time quaking-induced conversion. Nat. Med. 17, 175–178 (2011).
Acknowledgements
The authors are supported by NIH National Institute on Aging grants R01-AG031189 (M. D. Geschwind) and T32-AG23481 (D. C. Perry). M. D. Geschwind also receives research support from the Michael J. Homer Family Fund.
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Competing interests
M. D. Geschwind serves on a scientific advisory board for Lundbeck and has served as a consultant for MedaCorp, Gerson-Lehman Group, and The Council of Advisors. D. C. Perry declares no competing interests.
Rights and permissions
About this article
Cite this article
Perry, D., Geschwind, M. Thorough work-up and new diagnostic criteria needed for CJD. Nat Rev Neurol 7, 479–480 (2011). https://doi.org/10.1038/nrneurol.2011.118
Published:
Issue Date:
DOI: https://doi.org/10.1038/nrneurol.2011.118
This article is cited by
-
Creutzfeldt-Jakob disease mimicking autoimmune encephalitis with CASPR2 antibodies
BMC Neurology (2014)
-
Role of magnetic resonance imaging, cerebrospinal fluid, and electroencephalogram in diagnosis of sporadic Creutzfeldt-Jakob disease
Journal of Neurology (2013)
-
Voltage-gated potassium channel complex antibodies in Creutzfeldt-Jakob disease
Journal of Neurology (2012)
-
Allosteric function and dysfunction of the prion protein
Cellular and Molecular Life Sciences (2012)