[Epidemiologic study of Creutzfeldt-Jakob disease from autopsy-confirmed cases]

We investigated epidemiologic data from 61 cases of autopsy-confirmed sporadic Creutzfeldt-Jakob disease (CJD). Dura mater-associated CJD cases and familial CJD cases were excluded. There were 34 male and 27 female cases, with an average age at onset of 66.0 +/- 10.5 years (range 27 to 89). At onset of CJD, 1 case was aged in the 20's and 1 in 30's, but there were no cases aged in the 40's. In 6 cases, age at onset was in the 80's. There was no significant difference in relation to the age at onset between males and females, averaging 66.2 +/- 12.4 years (range 27 to 89), and 65.9 7.5 years (range 53 to 82), respectively. Two cases had been employed as medical workers (a medical technologist and a nurses' aide) but neither had an apparent history of contact with CJD patients. No cases in the study had either family history of CJD or apparent contact with CJD patients. Ten cases had a history of hypertension, 5 cases had a history of diabetes mellitus, 2 cases had a history of malaria and 1 case had suffered from atomic bomb exposure. Twenty-one cases had operation history, including 2 cases of an operation involving the central nervous system. One case had a skull-base fracture operation 19 years before the onset of CJD and the other case had an atlantoaxial subluxation operation 11 years before the onset of CJD; there was no transplantation of dura mater graft in either operation. There was one case with a history of conservative treatment for cerebral hemorrhage; this case had symptomatic secondary epilepsy as a coexisting disease at the onset of CJD. Two cases had Parkinson's disease as a coexisting disease at the onset of CJD. The source hospitals enforcing medical treatment were located in the Aichi (n = 42), Gifu (n = 12) and Mie (n = 7) prefectures of the Tokai region of Japan. Regarding patients' place of residence, 22 cases resided in Nagoya-city. Nagoya University Hospital performed the autopsy in 12 cases, but 10 cases of those were transported after death from the source hospital. Departments of neurology provided clinical treatment in 54 cases. Other departments that provided treatment were Internal Medicine (n = 3), Psychiatry (n = 2), Geriatrics (n = 1), and Neurosurgery (n = 1). In 39 cases, prion protein gene analyses using peripheral blood leukocyte or cryopreserved brain tissue were performed. As for polymorphic codon 129, 36 cases (92%) showed Met/Met, 3 cases showed Met/Val (8%) and no case showed Val/Val polymorphism. Polymorphic codon 219 showed Glu/Glu homozygosity in all of the examined cases. Active autopsy performance of CJD in the Tokai region was suggested from the present study. We estimated that the CJD autopsy rate of the district was more than 50% over the past 7 years.