title: [Non-familial Creutzfeldt-Jakob disease: a study of 53 cases].
abstract: Creutzfeldt-Jakob disease (CJD) is the most frequent human spongiform encephalopathy. We have analyzed 53 cases of definite non familial CJD over a study period of 28 years. All were autopsied in the same neuropathological unit. Clinical and epidemiological data were in accordance with previous studies: low incidence of CJD under the age of 40, high incidence around the sixty years of age (26%) a sex ratio at 0.65 and high frequency of myoclonus, dementia and cerebellar ataxia during evolution (78%). This study highlights the rapidity at the onset of the disease (within 24 hours) in 16% of the cases. EEG disclosed typical pseudoperiodic activity in only 53% of cases and cerebral MRI showed high T2 signal intensity in basal nuclei in 15%. This regional study is the second of its nature to be carried out in France, the first one covering the area of Paris.
authors.affiliation: Service de Neurologie D, Hôpital Neurologique Pierre Wertheimer, Lyon. glap@wanadoo.fr
authors.forename: P
authors.initials: P
authors.lastname: Giraud
authors.forename: A
authors.initials: A
authors.lastname: Perret-Liaudet
authors.forename: A G
authors.initials: AG
authors.lastname: Biacabe
authors.forename: J P
authors.initials: JP
authors.lastname: Deslys
authors.forename: J L
authors.initials: JL
authors.lastname: Laplanche
authors.forename: G
authors.initials: G
authors.lastname: Chazot
authors.forename: N
authors.initials: N
authors.lastname: Kop
mesh_terms: D000328:Adult; D001921:Brain / Q000473:pathology; D007562:Creutzfeldt-Jakob Syndrome / Q000175:diagnosis / Q000453:epidemiology / Q000473:pathology*; D004569:Electroencephalography; D005260:Female; D005602:France / Q000453:epidemiology; D006801:Humans; D015994:Incidence; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D012307:Risk Factors