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Journal of Neurology, Neurosurgery, and Psychiatry logoLink to Journal of Neurology, Neurosurgery, and Psychiatry
. 1998 Dec;65(6):836–841. doi: 10.1136/jnnp.65.6.836

Creutzfeldt-Jakob disease in Sweden

P O Lundberg 1
PMCID: PMC2170389  PMID: 9854958

Abstract

OBJECTIVES—To find and investigate, retrospectively, as many cases as possible of Creutzfeldt-Jakob disease (CJD) in Sweden dying during the period 1 January 1985 to 31 December 1996 and to detect any possible case(s) of new variant CJD.
METHODS—The patients were found through computer search of all death certificates in Sweden on which CJD was mentioned, through information from the Swedish neuropathologists, and spontaneous reports from Swedish doctors and hospitals. Data concerning the patients were then collected from patients' case records and from brain histopathology reports.
RESULTS—In total 72 cases of spongiform encephalopathy were confirmed as definite by neuropathology, one of them with Gerstmann-Stäussler-Scheinker disease. In 51 further cases there were no brain pathology data but the diagnosis "probable" (37 patients) or "possible" (14 patients) CJD according to WHO criteria could be made on clinical grounds. There was a variation in number of deaths/year, from a minimum of five (1985) to a maximum of 16 (1990). Sixty patients died during the period 1985-90 and 62 during 1991-6. The sex ratio was nearly 1:1. Calculated for a population of 8.6 million (mean of 12 years) in Sweden this gives 1.18/million/year. Age at the time of the presenting symptoms ranged from 34 to 84 years. Only one patient was under 40 at the onset of symptoms. He had a spongiform encephalopathy but prion protein staining was negative. The duration of symptoms that could be attributed to CJD was 6 months or less in 75 cases, 7-12 months in 16 cases, 1 to 2 years in 15 cases, and more than 2 years in 16 patients. By definition all patients were demented. Other more common symptoms and signs were aphasia, dysphasia, dysathria, ataxia, myoclonus, pareses of the extremities, rigidity or spasticity, different types of hyperkinesias, and other psychiatric symptoms (depression, anxiety, and aggressiveness). Less common symptoms were hallucinations (mainly visual), visual defects, sensory symptoms (paraesthesias, itching, or pain), apraxia of swallowing, and disorders of eye movements.
CONCLUSIONS—The incidence, the symptomatology, the age distribution (age in years at onset and at death), and the duration of illness were similar to those of other countries except for the cases of new variant CJD in the United Kingdom. There is so far no indication of any cases of new variant CJD in Sweden.



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Selected References

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  1. Brown P., Cathala F., Castaigne P., Gajdusek D. C. Creutzfeldt-Jakob disease: clinical analysis of a consecutive series of 230 neuropathologically verified cases. Ann Neurol. 1986 Nov;20(5):597–602. doi: 10.1002/ana.410200507. [DOI] [PubMed] [Google Scholar]
  2. Brown P., Cathala F., Gajdusek D. C. Creutzfeldt-Jakob disease in France: III. Epidemiological study of 170 patients dying during the decade 1968--1977. Ann Neurol. 1979 Nov;6(5):438–446. doi: 10.1002/ana.410060511. [DOI] [PubMed] [Google Scholar]
  3. Brown P., Cathala F., Labauge R., Pages M., Alary J. C., Baron H. Epidemiologic implications of Creutzfeldt-Jakob disease in a 19 year-old girl. Eur J Epidemiol. 1985 Mar;1(1):42–47. doi: 10.1007/BF00162311. [DOI] [PubMed] [Google Scholar]
  4. Brown P., Cathala F., Sadowsky D., Gajdusek D. C. Creutzfeldt-Jakob disease in France: II. Clinical characteristics of 124 consecutive verified cases during the decade 1968--1977. Ann Neurol. 1979 Nov;6(5):430–437. doi: 10.1002/ana.410060510. [DOI] [PubMed] [Google Scholar]
  5. Brown P., Gibbs C. J., Jr, Rodgers-Johnson P., Asher D. M., Sulima M. P., Bacote A., Goldfarb L. G., Gajdusek D. C. Human spongiform encephalopathy: the National Institutes of Health series of 300 cases of experimentally transmitted disease. Ann Neurol. 1994 May;35(5):513–529. doi: 10.1002/ana.410350504. [DOI] [PubMed] [Google Scholar]
  6. Brown P., Rodgers-Johnson P., Cathala F., Gibbs C. J., Jr, Gajdusek D. C. Creutzfeldt-Jakob disease of long duration: clinicopathological characteristics, transmissibility, and differential diagnosis. Ann Neurol. 1984 Sep;16(3):295–304. doi: 10.1002/ana.410160305. [DOI] [PubMed] [Google Scholar]
  7. Bruce M. E., Will R. G., Ironside J. W., McConnell I., Drummond D., Suttie A., McCardle L., Chree A., Hope J., Birkett C. Transmissions to mice indicate that 'new variant' CJD is caused by the BSE agent. Nature. 1997 Oct 2;389(6650):498–501. doi: 10.1038/39057. [DOI] [PubMed] [Google Scholar]
  8. Brun A., Gottfries C. G., Roos B. E. Studies of the monoamine metabolism in the central nervous system in Jakob-Creutzfeldt disease. Acta Neurol Scand. 1971;47(5):642–645. doi: 10.1111/j.1600-0404.1971.tb07515.x. [DOI] [PubMed] [Google Scholar]
  9. Chazot G., Broussolle E., Lapras C. l., Blättler T., Aguzzi A., Kopp N. New variant of Creutzfeldt-Jakob disease in a 26-year-old French man. Lancet. 1996 Apr 27;347(9009):1181–1181. doi: 10.1016/s0140-6736(96)90638-8. [DOI] [PubMed] [Google Scholar]
  10. Collinge J., Sidle K. C., Meads J., Ironside J., Hill A. F. Molecular analysis of prion strain variation and the aetiology of 'new variant' CJD. Nature. 1996 Oct 24;383(6602):685–690. doi: 10.1038/383685a0. [DOI] [PubMed] [Google Scholar]
  11. Cousens S. N., Zeidler M., Esmonde T. F., De Silva R., Wilesmith J. W., Smith P. G., Will R. G. Sporadic Creutzfeldt-Jakob disease in the United Kingdom: analysis of epidemiological surveillance data for 1970-96. BMJ. 1997 Aug 16;315(7105):389–395. doi: 10.1136/bmj.315.7105.389. [DOI] [PMC free article] [PubMed] [Google Scholar]
  12. Hainfellner J. A., Jellinger K., Diringer H., Guentchev M., Kleinert R., Pilz P., Maier H., Budka H. Creutzfeldt-Jakob disease in Austria. J Neurol Neurosurg Psychiatry. 1996 Aug;61(2):139–142. doi: 10.1136/jnnp.61.2.139. [DOI] [PMC free article] [PubMed] [Google Scholar]
  13. Holman R. C., Khan A. S., Kent J., Strine T. W., Schonberger L. B. Epidemiology of Creutzfeldt-Jakob disease in the United States, 1979-1990: analysis of national mortality data. Neuroepidemiology. 1995;14(4):174–181. doi: 10.1159/000109793. [DOI] [PubMed] [Google Scholar]
  14. Kretzschmar H. A., Ironside J. W., DeArmond S. J., Tateishi J. Diagnostic criteria for sporadic Creutzfeldt-Jakob disease. Arch Neurol. 1996 Sep;53(9):913–920. doi: 10.1001/archneur.1996.00550090125018. [DOI] [PubMed] [Google Scholar]
  15. Monreal J., Collins G. H., Masters C. L., Fisher C. M., Kim R. C., Gibbs C. J., Jr, Gajdusek D. C. Creutzfeldt-Jakob disease in an adolescent. J Neurol Sci. 1981 Nov-Dec;52(2-3):341–350. doi: 10.1016/0022-510x(81)90015-0. [DOI] [PubMed] [Google Scholar]
  16. Poser S., Zerr I., Schulz-Schaeffer W. J., Kretzschmar H. A., Felgenhauer K. Die Creutzfeldt-Jakob-Krankheit. Eine Sphinx der heutingen Neurobiologie. Dtsch Med Wochenschr. 1997 Sep 12;122(37):1099–1105. doi: 10.1055/s-2008-1047733. [DOI] [PubMed] [Google Scholar]
  17. Raverdy P., Hauw J. J., Cathala F., Rémy A., Brown P., Ernest A. M., Périé G. Maladie de Creutzfeldt-Jakob ayant évolué 34 mois chez une femme de 26 ans. Rev Neurol (Paris) 1983;139(5):381–383. [PubMed] [Google Scholar]
  18. Ruffié A., Delasnerie-Lauprêtre N., Brandel J. P., Jaussent I., Dormont D., Laplanche J. L., Hauw J. J., Richardson S., Alpérovitch A. Incidence de la maladie de Creutzfeldt-Jakob en France, 1992-1995. Rev Epidemiol Sante Publique. 1997 Dec;45(6):448–453. [PubMed] [Google Scholar]
  19. Steinhoff B. J., Räcker S., Herrendorf G., Poser S., Grosche S., Zerr I., Kretzschmar H., Weber T. Accuracy and reliability of periodic sharp wave complexes in Creutzfeldt-Jakob disease. Arch Neurol. 1996 Feb;53(2):162–166. doi: 10.1001/archneur.1996.00550020074017. [DOI] [PubMed] [Google Scholar]
  20. Will R. G., Ironside J. W., Zeidler M., Cousens S. N., Estibeiro K., Alperovitch A., Poser S., Pocchiari M., Hofman A., Smith P. G. A new variant of Creutzfeldt-Jakob disease in the UK. Lancet. 1996 Apr 6;347(9006):921–925. doi: 10.1016/s0140-6736(96)91412-9. [DOI] [PubMed] [Google Scholar]
  21. Zeidler M., Johnstone E. C., Bamber R. W., Dickens C. M., Fisher C. J., Francis A. F., Goldbeck R., Higgo R., Johnson-Sabine E. C., Lodge G. J. New variant Creutzfeldt-Jakob disease: psychiatric features. Lancet. 1997 Sep 27;350(9082):908–910. doi: 10.1016/s0140-6736(97)03148-6. [DOI] [PubMed] [Google Scholar]
  22. Zeidler M., Stewart G. E., Barraclough C. R., Bateman D. E., Bates D., Burn D. J., Colchester A. C., Durward W., Fletcher N. A., Hawkins S. A. New variant Creutzfeldt-Jakob disease: neurological features and diagnostic tests. Lancet. 1997 Sep 27;350(9082):903–907. doi: 10.1016/s0140-6736(97)07472-2. [DOI] [PubMed] [Google Scholar]

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