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Spongiform Encephalopathy of Long Duration: A Family Study

Published online by Cambridge University Press:  18 September 2015

G.P.A. Rice
Affiliation:
University Hospital and St. Joseph's Hospital, London University of Western Ontario, Homewood Sanitarium, Guelph, Ontario
D.W. Paty
Affiliation:
University Hospital and St. Joseph's Hospital, London University of Western Ontario, Homewood Sanitarium, Guelph, Ontario
M.J. Ball*
Affiliation:
University Hospital and St. Joseph's Hospital, London University of Western Ontario, Homewood Sanitarium, Guelph, Ontario
R. Tatham
Affiliation:
University Hospital and St. Joseph's Hospital, London University of Western Ontario, Homewood Sanitarium, Guelph, Ontario
A. Kertesz
Affiliation:
University Hospital and St. Joseph's Hospital, London University of Western Ontario, Homewood Sanitarium, Guelph, Ontario
*
University Hospital, 339 Windermere Road, London, Ontario, N6A 5A5, Canada
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Summary:

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Seven members of a family succumbed to prolonged dementing illnesses. In two, whose dementia lasted 10 years, spongiform encephalopathy was found at autopsy. This duration of illness has not been a feature in other instances of familial Creutzfeldt-Jakob disease.

Type
Research Article
Copyright
Copyright © Canadian Neurological Sciences Federation 1980

References

REFERENCES

Bignami, A. and Forno, L.S., (1970) Status spongiosus in Jakob-Creutzfeldt disease: Electron-microscopic study of a cortical biopsy. Brain 93: 8994.CrossRefGoogle ScholarPubMed
Bubis, J.J., Goldhammer, Y, and Braham, J., (1972) Subacute spongiform encephalopathy. Electron microscopic studies. J. Neurol. Neurosurg., Psychiat. 35: 881887.CrossRefGoogle ScholarPubMed
Daniel, P.M (1970) Creutzfeldt-Jakob disease: J. Clin. Path. 25, Supp.20 6: 97101.CrossRefGoogle Scholar
Davison, C. and Rabiner, A.M., (1940) Spastic pseudosclerosis. Arch. Neurol. Psychiat. 44: 578598.CrossRefGoogle Scholar
Gajdusek, D.C., (1972) Spongiform virus encephalopathies, J. Clin. Path., 25: Supp.6, 7883.CrossRefGoogle Scholar
Gajdusek, D.C., (1977) Unconventional viruses and the origin and disappearance of Kuru. Science 197: 943960.CrossRefGoogle ScholarPubMed
Kidd, M., (1967) Some electron-microscopal observations on status spongiosus. Acta. Neuropath. Supp. 3: 137144.Google Scholar
Kirschbaum, S.R., (1968) Jakob-Creutzfeldt disease. New York, American Elsevier Publishing Co. Inc.Google Scholar
Masters, C.L., Harris, J.D., Gajdusek, D.C. et al., (1979) Creutzfeldt-Jakob disease: Patterns of worldwide occurrence and the significance of familial and sporadic clustering. Ann. Neurol. 5: 177188.CrossRefGoogle ScholarPubMed
Masters, C.L. and Richardson, E.P., (1978) Subacute spongiform encephalopathy (Creutzfeldt-Jakob disease). Brain 101: 333344.CrossRefGoogle ScholarPubMed
May, W.W., Itabashi, H.H.. Dejong, R.N., (1968) Creutzfeldt-Jakob disease. Arch. Neurol. 19: 137149.CrossRefGoogle ScholarPubMed
Mayer, V., Orolin, D. and Mitrova, E., (1977) Cluster of Creutzfeldt-Jakob disease and Presenile Dementia. Lancet 2: 256.CrossRefGoogle ScholarPubMed
Rewcastle, N.B., Gibbs, C.J., Gajdusek, D.C., (1978) Transmission of Alzheimer’s disease to primates. VIII International Congress of Neuropathology, Washington, DC.Google Scholar
Rosenthal, N.P., Keesey, J., Crandall, B., Et AL, , (1976) Familial Neurological Disease Associated with Spongiform Encephalopathy. Arch. Neurol. 33: 252259.CrossRefGoogle ScholarPubMed
Traub, R., Gajdusek, DC, Gibbs, C.J., (1977) Aging and Dementia. Kinsbourne, and Smith, , Eds. (Spectrum, Flushing, N.Y.) pp. 91146.Google Scholar