The Clinical Neurology and Epidemiology of Creutzfeldt-Jakob Disease, with Special Reference to Iatrogenic Cases
Paul Brown
Nervous System Studies Laboratory, NINCDS, Building 36, Room 5B 21, National Institutes of Health, Bethesda, Maryland 20892, USA
Search for more papers by this authorPaul Brown
Nervous System Studies Laboratory, NINCDS, Building 36, Room 5B 21, National Institutes of Health, Bethesda, Maryland 20892, USA
Search for more papers by this authorJoan Marsh
Search for more papers by this authorSummary
The clinical characteristics of Creutzfeldt-Jakob disease (CJD) in a newly analysed group of 223 cases transmitted to primates at the NIH are compared to a recent large series of neuropathologically verified cases in France, and the limited conclusions from worldwide epidemiological studies are briefly summarized. Discussion then focuses on iatrogenic CJD, with special attention to the interplay of clinical, laboratory and epidemiological features of the current outbreak of CJD in hypopituitary dwarfs treated with growth hormone extracted from pools of human pituitary glands.
References
- Bernoulli C, Siegfried J, Baumgartner G et al 1977 Danger of accidental person-to-person transmission of Creutzfeldt-Jakob disease by surgery. Lancet 1: 478–479.
- Bernoulli CC, Masters CL, Gajdusek DC et al 1979 Early clinical features of Creutzfeldt-Jakob disease (subacute spongiform encephalopathy). In: SB Prusiner, WJ Hadlow (eds) Slow transmissible diseases of the nervous system. Academic Press, New York, vol 1: 229–251.
- Brown P 1980 An epidemiologic critique of Creutzfeldt-Jakob disease. Epidemiol Rev 2: 113–135.
- Brown P 1988 Human growth hormone therapy and Creutzfeldt-Jakob disease: a drama in three acts. Pediatrics, in press.
- Brown P, Rodgers-Johnson P, Cathala F et al 1984 Creutzfeldt-Jakob disease of long duration: clinicopathological characteristics, transmissibility, and differential diagnosis. Ann Neurol 16: 295–304.
- Brown P, Gajdusek DC, Gibbs CJ Jr, Asher DM 1985 Potential epidemic of Creutzfeldt-Jakob disease from human growth hormone therapy. N Engl J Med 313: 728–731.
- Brown P, Cathala F, Castaigne P, Gajdusek DC 1986 Creutzfeldt-Jakob disease: clinical analysis of a consecutive series of 230 neuropathologically-verified cases. Ann Neurol 20: 597–602.
- Brown P, Cathala F, Raubertas RF et al 1987 The epidemiology of Creutzfeldt-Jakob disease: conclusion of a 15-year investigation in France and review of the world literature. Neurology 37: 895–904.
- Bruce EM, Dickinson AG 1987 Biological evidence that scrapie agent has an independent genome. J Gen Virol 68: 79–89.
- Duffy P, Wolf J, Collins G et al 1974 Possible person-to-person transmission of Creutzfeldt-Jakob disease. N Engl J Med: 290: 692–693.
- Foncin J, Gaches J, Cathala F, et al, 1980 Transmission iatrogène interhumaine possible de maladie de Creutzfeldt-Jakob avec atteinte des grains du cervelet. Rev Neurol (Paris) 136: 280.
- German TL, Marsh RF 1983 The scrapie agent: a unique self-replicating pathogen. Prog Mol Subcell Biol 8: 111–121.
- Gibbs CJ Jr, Gajdusek DC, Asher DM et al 1968 Creutzfeldt-Jakob disease (subacute spongiform encephalopathy): transmission to the chimpanzee. Science (Wash DC) 161: 388–389.
- Gibbs CJ Jr, Gajdusek DC, Amyx H 1979 Strain variation in the viruses of Creutzfeldt-Jakob disease and kuru. In: SB Prusiner, WJ Hadlow (eds) Slow transmissible diseases of the nervous system. Academic Press, New York, vol 2: 87–110.
- Hunter GD, Kimberlin RH, Gibbons RA 1968 Scrapie: a modified membrane hypothesis. J Theor Biol 20: 355–357.
- Jakob A 1921 Uber eigenartige erkrankung des zentralnervensystems mit bemerkenswertem anatomischen befunde (spastische pseudosklerose-encephalomyelopathie mit disseminierten degenerationsherden). Dtsch Z Nervenheilkd 70: 132–146.
- Masters CL, Gajdusek DC, Gibbs CJ Jr 1981 Creutzfeldt-Jakob disease virus isolations from the Gerstmann-Sträussler syndrome, with an analysis of the various forms of amyloid plaque deposition in the virus-induced spongiform encephalopathies. Brain 104: 559–588.
- Prichard J, Thadani V, Kalb R, Manuelidis E 1987 Rapidly progressive dementia in a patient who received a cadaveric dura mater graft. Morb Mortal Wkly Rep 36: 49–55.
- Prusiner SB 1984 Prions. Sci Am 251: 50–59.
- Roos R, Gajdusek DC, Gibbs CJ Jr 1973 The clinical characteristics of transmissible Creutzfeldt-Jakob disease. Brain 96: 1–20.
- Salazar AM, Masters CL, Gajdusek DC, Gibbs CJ Jr 1983 Syndromes of amyotrophic lateral sclerosis and dementia: relation to transmissible Creutzfeldt-Jakob disease. Ann Neurol 14: 17–26.
- Tateishi J, Sato Y, Nagara H, Boellard JW 1984 Experimental transmission of human spongiform encephalopathy to small rodents. IV. Positive transmission from a typical case of Gerstmann-Straüssler-Scheinker disease. Acta Neuropathol 64: 85–88.
- Traub R, Gajdusek DC, Gibbs CJ Jr 1977 Transmissible virus dementia: the relation of transmissible spongiform encephalopathy to Creutzfeldt-Jakob disease. In: NL Smith, M Kinsbourne (eds) Aging and dementia. Spectrum, New York, p 91–154.
- Will RG, Matthews WB 1982 Evidence for case-to-case transmission of Creutzfeldt-Jakob disease. J Neurol Neurosurg Psychiatry 45: 235–238.
- Will RG, Matthews WB 1984 A retrospective study of Creutzfeldt-Jakob disease in England and Wales 1970–79. I: clinical features. J Neurol Neurosurg Psychiatry 47: 134–140.
- Wills PR 1986 Scrapie, ribosomal proteins and biological information. J Theor Biol 122: 157–178.
- Dickinson AG, Fraser H, Outram GW 1975 Scrapie incubation time can exceed natural lifespan. Nature (Lond) 256: 732–733.
- Fraser H, Dickinson AG 1985 Targeting of scrapie lesions and spread of agent via the retino-tectal projection. Brain Res 346: 32–41.
- Johnson RT, Mims CA 1968 Pathogenesis of viral infections of the nervous system. N Engl J Med 278: 23–30, 84–92.
- Kim YS, Carp RI, Callahan SM, Wisniewski HM 1987 Incubation periods and survival times for mice injected stereotaxically with three scrapie strains in different brain regions. J Gen Virol 68: 695–702.
- Manuelidis EE, Manuelidis L 1979 Observations on Creutzfeldt-Jakob disease propagated in small rodents. In: S Prusiner, WJ Hadlow (eds) Slow transmissible diseases of the nervous system. Academic Press, New York, 2: 147–173.
- Manuelidis EE, Gorgacz EJ, Manuelidis L 1978a Viremia in experimental Creutzfeldt-Jakob disease. Science (Wash DC) 200: 1069–1071.
- Manuelidis EE, Manuelidis L, Pincus JH, Collins WF 1978b Transmission from man to hamster of Creutzfeldt-Jakob disease with clinical recovery. Lancet 2: 40–42.
- Manuelidis EE, Kim JH, Mericangas JR, Manuelidis L 1985 Transmission of Creutzfeldt-Jakob disease from human blood. Lancet 2: 896–897.