[Non-familial Creutzfeldt-Jakob disease: a study of 53 cases].
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.
Giraud
P
P
Service de Neurologie D, Hôpital Neurologique Pierre Wertheimer, Lyon. glap@wanadoo.fr
Perret-Liaudet
A
A
Biacabe
A G
AG
Deslys
J P
JP
Laplanche
J L
JL
Chazot
G
G
Kop
N
N
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