A Retrospective Study of Creutzfeldt-Jakob Disease in Belgium

• Published in: European journal of epidemiology Vol. 15; no. 6; pp. 517 - 519
• Main Authors: Pals, P., Van Everbroeck, B., Sciot, R., Godfraind, C., Robberecht, W., Dom, R., Laterre, C., J.-J. Martin, Cras, P.
• Format: Journal Article
• Language: English
• Published: Dordrecht Kluwer Academic Publishers 01.07.1999; Springer; Springer Nature B.V
• Subjects: Adult; Age; Aged; Aged, 80 and over; Archives; Archives & records; Belgium; Belgium - epidemiology; Biological and medical sciences; Creutzfeldt Jakob syndrome; Creutzfeldt-Jakob disease; Creutzfeldt-Jakob Syndrome - epidemiology; Creutzfeldt-Jakob Syndrome - mortality; Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases; Dementia; Epidemiology; Female; Females; Humans; Iatrogenesis; Laboratories; Male; Medical records; Medical sciences; Middle Aged; Myoclonus; Nervous system diseases; Neurology; Neuropathology; Patients; Prion diseases; Retrospective Studies; Symptoms; Belgium; Europe; Human; Nervous system diseases; Electroencephalography; Epidemiology; Retrospective; Cerebral disorder; Incidence; Infection; Electrodiagnosis; Register; Central nervous system disease; Degenerative disease; Prion; Creutzfeldt Jakob disease; Public health
• ISSN: 0393-2990; 1573-7284
• DOI: 10.1023/a:1007581313067

Using data from Belgian neuropathological archives, completed with the results of a comprehensive study of available medical records, we found 100 patients who fulfilled diagnostic criteria for probable or definite Creutzfeldt-Jakob d1551isease (CJD). Mean age at death was 63 years. The median disease duration was 9 months. Progressive mental deterioration was present in all cases, whereas signs of cerebellar dysfunction and myoclonus were found in approximately 80% of the patients. In 50% of the population, the EEG revealed characteristic abnormalities. Ninety-six patients suffered from the sporadic type of CJD, while 4 suffered from a hereditary form. In our series, we could find no evidence for the new variant, neither for an iatrogenic cause.