We report the case of a 76 year old woman who presented with a clinical picture initially typical of a vascular dementia. The diagnosis was revised to Creutzfeldt-Jacob Disease in the terminal stages. While Creutzfeldt-Jacob Disease is rare, one must consider it in the differential diagnosis as it can mimic other dementias particularly in the chronic form which we describe here.
Get full access to this article
View all access options for this article.
References
1.
PrusinerS.B.HsiaoK.K.Human prion diseases. Annals of Neurology. 1994; 35: 385–395.
2.
PrusinerS B. Shattuck lecture-Neurodegenerative diseases and prions. The New Erg J Med, Vol. 344, No. 20, May 17, 2001.
3.
BrownPCathalaFRaubertasR FGajdusekD CCastaigneP. The epidemiology of Creutzfeldt-Jakob disease; conclusion of a 15-year investigation in France and review of the world literature. Neurology1987. 37: 895–904.
4.
WillR.G.IronsideJ WZeldlerM.. A new variant of Creutzfeldt-Jacob disease in the UK. Lancet. 1996; 347: 921–925.
5.
YarnadaSAibaTEndoYHaraMKitamotoTTateishiJ. Creutzfeldt-Jacob disease transmitted by a cadaveric dura mater graft. Neurosurgery. 1994; 34, 740744.
6.
BernoulliQSiegfriedJBaumgartnerG. Danger of accidental person-toperson transmission of Creutzfeldt-Jacob disease by surgery. Lancet. 1977, 1, 478479.
7.
BrownPGajdusekD CGibbsC JAsherD M. Potential epidemic of Creutzfeldt Jacob disease from human growth hormone therapy. N Engl J Med. 1985, 313: 728731.
8.
MendezWSelwoodA. Frey WH Clinical characteristics of chronic Creutzfeldt-Jacob disease. Journal of Geriatric Psychiatry&Neurology. 1994, 7(4): 206–208.
9.
Report of the WHO consultation on the global surveillance, diagnosis and therapy of human transmissible spongiform encephalopathies. Geneva: WHO, 1998.
10.
BrownPRodgers-JohnsonPCathalaFGibbsC JJr.GajdusekD C. Creutsfeldt-Jacob disease of long duration: Clinicopathological characteristics, transmissibility, and differential diagnosis. Annals of Neurology. 16(3)295–304, 1984 Sep.
11.
ZeidlerMStewartG EBarracloughC R. New variant Creutzfeldt-Jacob disease: Neurological features and diagnostic tests. Lancet. 350(9082): 903–7, 1997 Sep 27.
12.
FinkenstaedtMSzudraAZerrI. MR imaging of Creutzfeldt-Jakob disease. Radiology1996, 199: 793–8,.
13.
ZerrIBodemerMGefellerO. Detection of 14-3-3 protein in the cerebrospinal fluid supports the diagnosis of Creutzfeldt-Jacob disease. Ann Neurol1998; 43: 32–40.
14.
KraemerD E. Diagnosis and reporting of Creutzfeldt-Jakob Disease (letter). JAMA, 285(6): 733–734. 2001, Feb 14.
15.
MajeedALehmannPKirbyLKnightRColemanM. Extent of misclassification of deathfrom Creutzfeldi-Jakob disease in England 1979–96: Retrospective examination of clinical records. BMJ, Vol 320(7228) 145–147 Jan 15, 2000.
