Hostname: page-component-78c5997874-v9fdk Total loading time: 0 Render date: 2024-11-07T23:24:59.842Z Has data issue: false hasContentIssue false
  • English
  • Français

Sporadic Creutzfeldt-Jakob disease in older people

Part of: Bespoke shallow archive Weizmann

Published online by Cambridge University Press:  11 December 2009

N Shah ,
A Agbobu ,
I Costello ,
A Beri  and
K Minhas
N Shah*
Affiliation:
Department of Medicine for the Elderly, Leicester Royal Infirmary, UK
A Agbobu
Affiliation:
Department of Medicine for the Elderly, Leicester Royal Infirmary, UK
I Costello
Affiliation:
Department of Medicine for the Elderly, Leicester Royal Infirmary, UK
A Beri
Affiliation:
Department of Medicine for the Elderly, Leicester Royal Infirmary, UK
K Minhas
Affiliation:
Department of Medicine for the Elderly, Leicester Royal Infirmary, UK
*
Address for correspondence: Dr Nainal Shah, Department of Medicine for the Elderly, University Hospitals of Leicester NHS Trust, Leicester Royal Infirmary, Leicester LE1 5WW, UK. E-mail: shahnainal@hotmail.com
Get access Rights & Permissions [Opens in a new window]

Summary

Prion diseases are rare degenerative disorders of the nervous system caused by abnormal accumulation and/or metabolism of prion proteins. They are invariably fatal. Creutzfeldt-Jakob disease (CJD) is the most common human prion disease and the sporadic form accounts for 85% of cases, whilst about 15% are genetic and 1% is iatrogenic. Variant CJD (vCJD) was mostly seen in the UK and France and mainly affected young people. Sporadic CJD (sCJD) occurs throughout the world with mean age of onset in the seventh decade. It typically presents with rapidly declining cognition that may get confused with other forms of dementia. Thus identification of sCJD in older people depends on a judicious awareness of the clinical features. Here we present a clinical, pathological, therapeutic and diagnostic review of sCJD.

Keywords

Creutzfeldt-Jakob syndromeprion diseasesspongiform encephalopathyolder people
Type
Neuropsychiatry of old age
Information
Reviews in Clinical Gerontology , Volume 19 , Issue 3 , August 2009 , pp. 207 - 215
Copyright
Copyright © Cambridge University Press 2009

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1Johnson, RT. Prion diseases. Lancet Neurol 2005; 4: 635–42.CrossRefGoogle ScholarPubMed
2Brown, P, Gibbs, CJ, Rodgers-Johnson, P, Asher, DM, Sulima, MP, Bacote, A et al. Human spongiform encephalopathy: the National Institutes of Health series of 300 cases of experimentally transmitted disease. Ann Neurol 1994; 35: 513–29.CrossRefGoogle ScholarPubMed
3Will, RG, Matthews, WB. A retrospective study of Creutzfeldt-Jakob disease in England and Wales 1970–79. I: Clinical features. J Neurol Neurosurg Psychiatry 1984; 47: 134–40.CrossRefGoogle ScholarPubMed
4Buganza, M, Ferrari, S, Cecchini, ME, Orrico, D, Monaco, S, Zanusso, G. The oldest old Creutzfeldt-Jakob disease case. J Neurol Neurosurg Psychiatry 2009; 80: 1140–42.CrossRefGoogle ScholarPubMed
5de Silva, R, Findlay, C, Awad, I, Harries-Jones, R, Knight, R, Will, R. Creutzfeldt-Jakob disease in the elderly. Postgrad Med J 1997; 73: 557–59.CrossRefGoogle ScholarPubMed
6Kretzschmar, HA. Human prion diseases (spongiform encephalopathies). Arch Virol Suppl 1993; 7: 261–93.CrossRefGoogle ScholarPubMed
7Ravilochan, K, Tyler, KL. Human transmissible neurodegenerative diseases (prion diseases). Semin Neurol 1992; 12: 178–92.CrossRefGoogle ScholarPubMed
8Knight, RS, Will, RG. Prion diseases. J Neurol Neurosurg Psychiatry 2004; 75 suppl 1: i36–42.CrossRefGoogle ScholarPubMed
9Bobowick, AR, Brody, JA, Matthews, MR, Roos, R, Gajdusek, DC. Creutzfeldt-Jakob disease: a case-control study. Am J Epidemiol 1973; 98: 381–94.CrossRefGoogle ScholarPubMed
10Davanipour, Z, Alter, M, Sobel, E, Asher, DM, Gajdusek, DC. A case-control study of Creutzfeldt-Jakob disease. Dietary risk factors. Am J Epidemiol 1985; 122: 443–51.CrossRefGoogle ScholarPubMed
11Matthews, WB, Will, RG. Creutzfeldt-Jakob disease in a lifelong vegetarian. Lancet 1981; 2: 937.Google Scholar
12Davanipour, Z, Alter, M, Sobel, E, Asher, D, Gajdusek, DC. Creutzfeldt-Jakob disease: possible medical risk factors. Neurology 1985; 35: 1483–86.CrossRefGoogle ScholarPubMed
13Castellani, RJ, Colucci, M, Xie, Z, Zou, W, Li, C, Parchi, P et al. Sensitivity of 14-3-3 protein test varies in subtypes of sporadic Creutzfeldt-Jakob disease. Neurology 2004; 63: 436–42.CrossRefGoogle ScholarPubMed
14Appleby, BS, Appleby, KK, Rabins, PV. Does the presentation of Creutzfeldt-Jakob disease vary by age or presumed etiology? A meta-analysis of the past 10 years. J Neuropsychiatry Clin Neurosci 2007; 19: 428–35.CrossRefGoogle ScholarPubMed
15Hill, AF, Joiner, S, Wadsworth, JD, Sidle, KC, Bell, JE, Budka, H et al. Molecular classification of sporadic Creutzfeldt-Jakob disease. Brain 2003; 126: 1333–46.CrossRefGoogle ScholarPubMed
16Appleby, BS, Appleby, KK, Crain, BJ, Onyike, CU, Wallin, MT, Rabins, PV. Characteristics of established and proposed sporadic Creutzfeldt-Jakob disease variants. Arch Neurol 2009; 66: 208–15.Google ScholarPubMed
17Parchi, P, Giese, A, Capellari, S, Brown, P, Schulz-Schaeffer, W, Windl, O et al. Classification of sporadic Creutzfeldt-Jakob disease based on molecular and phenotypic analysis of 300 subjects. Ann Neurol 1999; 46: 224–33.3.0.CO;2-W>CrossRefGoogle ScholarPubMed
18Prusiner, SB. Genetic and infectious prion diseases. Arch Neurol 1993; 50: 1129–53.CrossRefGoogle ScholarPubMed
19Brown, P, Gajdusek, DC. The human spongiform encephalopathies: kuru, Creutzfeldt-Jakob disease, and the Gerstmann-Straussler-Scheinker syndrome. Curr Top Microbiol Immunol 1991; 172: 1–20.Google ScholarPubMed
20Brown, P, Cathala, F, Castaigne, P, Gajdusek, DC. Creutzfeldt-Jakob disease: clinical analysis of a consecutive series of 230 neuropathologically verified cases. Ann Neurol 1986; 20: 597–602.CrossRefGoogle ScholarPubMed
21Cohn, DA, Crimmins, DS, Heard, R, Rose, M. Creutzfeldt-Jakob disease: the need for vigilance. J Clin Neurosci 2000; 7: 277–79.CrossRefGoogle ScholarPubMed
22Collins, SJ, Sanchez-Juan, P, Masters, CL, Klug, GM, van Duijn, C, Poleggi, A et al. Determinants of diagnostic investigation sensitivities across the clinical spectrum of sporadic Creutzfeldt-Jakob disease. Brain 2006; 129: 2278–87.CrossRefGoogle ScholarPubMed
23Geschwind, MD, Josephs, KA, Parisi, JE, Keegan, BM. A 54-year-old man with slowness of movement and confusion. Neurology 2007; 69: 1881–87.CrossRefGoogle ScholarPubMed
24Shiga, Y, Miyazawa, K, Sato, S, Fukushima, R, Shibuya, S, Sato, Y et al. Diffusion-weighted MRI abnormalities as an early diagnostic marker for Creutzfeldt-Jakob disease. Neurology 2004; 63: 443–49.CrossRefGoogle ScholarPubMed
25Young, GS, Geschwind, MD, Fischbein, NJ, Martindale, JL, Henry, RG, Liu, S et al. Diffusion-weighted and fluid-attenuated inversion recovery imaging in Creutzfeldt-Jakob disease: high sensitivity and specificity for diagnosis. Am J Neuroradiol 2005; 26: 1551–62.Google ScholarPubMed
26Zerr, I, Kallenberg, K, Summers, DM, Romero, C, Taratuto, A, Heinemann, U et al. Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease. Brain 2009; 132: 2659–68.CrossRefGoogle ScholarPubMed
27Galves, S, Carter, L. CT findings in 15 cases of Creutzfeldt-Jakob disease with its histological verification. J Neurosurg Psychiatry 1984; 47: 1244–46.CrossRefGoogle Scholar
28Steinhoff, BJ, Racker, S, Herrendorf, G, Poser, S, Grosche, S, Zerr, I et al. Accuracy and reliability of periodic sharp wave complexes in Creutzfeldt-Jakob disease. Arch Neurol 1996; 53: 162–66.CrossRefGoogle ScholarPubMed
29Schroter, A, Zerr, I, Henkel, K, Tschampa, HJ, Finkenstaedt, M, Poser, S. Magnetic resonance imaging in the clinical diagnosis of Creutzfeldt-Jakob disease. Arch Neurol 2000; 57: 1751–57.CrossRefGoogle ScholarPubMed
30Onofrj, M, Fulgente, T, Gambi, D, Macchi, G. Early MRI findings in Creutzfeldt-Jakob disease. J Neurol 1993; 240: 423–26.CrossRefGoogle ScholarPubMed
31Tartaro, A, Fulgente, T, Delli Pizzi, C, Bonomo, L, Bocola, V, Onofrj, M. MRI alterations as an early finding in Creutzfeld-Jakob disease. Eur J Radiol 1993; 17: 155–58.CrossRefGoogle ScholarPubMed
32Collie, DA, Sellar, RJ, Zeidler, M, Colchester, AC, Knight, R, Will, RG. MRI of Creutzfeldt-Jakob disease: imaging features and recommended MRI protocol. Clin Radiol 2001; 56: 726–39.CrossRefGoogle ScholarPubMed
33Korth, C, Peters, PJ. Emerging pharmacotherapies for Creutzfeldt-Jakob disease. Arch Neurol 2006; 63: 497–501.CrossRefGoogle ScholarPubMed
34Pruisner, SB. Prions. Proc Natl Acad Sci 1998; 95: 13363–83.CrossRefGoogle Scholar
35Mallucci, G, Dickinson, A, Linehan, J, Klohn, PC, Brandner, S, Collinge, J. Depleting neuronal PrP in prion infection prevents disease and reverses spongiosis. Science 2003; 302: 871–74.CrossRefGoogle ScholarPubMed
36White, AR, Enever, P, Tayebi, M, Mushens, R, Linehan, J, Brandner, S et al. Monoclonal antibodies inhibit prion replication and delay the development of prion disease. Nature 2003; 422: 80–83.CrossRefGoogle ScholarPubMed
37Stewart, LA, Rydzewska, LH, Keogh, GF, Knight, RS. Systematic review of therapeutic interventions in human prion disease. Neurology 2008; 70: 1272–81.CrossRefGoogle ScholarPubMed
38Otto, M, Cepek, L, Ratzka, P, Doehlinger, S, Boekhoff, I, Wiltfang, J et al. Efficacy of flupirtine on cognitive function in patients with CJD: A double-blind study. Neurology 2004; 62: 714–18.CrossRefGoogle ScholarPubMed
39Shaked, GM, Shaked, Y, Kariv-Inbal, Z, Halimi, M, Avraham, I, Gabizon, R. A protease-resistant prion protein isoform is present in urine of animals and humans affected with prion diseases. J Biol Chem 2001; 276: 31479–82.CrossRefGoogle ScholarPubMed
40Head, MW, Kouverianou, E, Taylor, L, Green, A, Knight, R. Evaluation of urinary PrPSc as a diagnostic test for sporadic, variant, and familial CJD. Neurology 2005; 64: 1794–96.CrossRefGoogle ScholarPubMed
41Ritchie, DL, Head, MW, Ironside, JW. Advances in the detection of prion protein in peripheral tissues of variant Creutzfeldt-Jakob disease patients using paraffin-embedded tissue blotting. Neuropathol Appl Neurobiol 2004; 30: 360–68.CrossRefGoogle ScholarPubMed
42Zanusso, G, Ferrari, S, Cardone, F, Zampieri, P, Gelati, M, Fiorini, M et al. Detection of pathologic prion protein in the olfactory epithelium in sporadic Creutzfeldt-Jakob disease. N Engl J Med 2003; 348: 711–19.CrossRefGoogle ScholarPubMed
43Tyler, KL. Creutzfeldt-Jakob disease. N Engl J Med 2003; 348: 681–82.CrossRefGoogle ScholarPubMed
44Cervenakova, L, Brown, P. Advances in screening test development for transmissible spongiform encephalopathies. Expert Review of Anti-Infective Therapy 2004; 2: 873–80.CrossRefGoogle ScholarPubMed