NR AEPI
AU Giraud,P.; Perret-Liaudet,A.; Biacabe,A.G.; Deslys,J.P.; Laplanche,J.L.; Chazot,G.; Kop,N.
TI [Non-familial Creutzfeldt-Jakob disease: a study of 53 cases]
OT Maladie de Creutzfeldt-Jakob non familiale certaine: etude de 53 observations.
QU Revue Neurologique 2000 Jul; 156(6-7): 616-21
PT journal article; review; review, multicase
AB 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.
ZR 40
MH Adult; Brain/pathology; Creutzfeldt-Jakob Syndrome/diagnosis/epidemiology/*pathology; Electroencephalography; English Abstract; Female; France/epidemiology; Human; Incidence; Magnetic Resonance Imaging; Male; Middle Age; Retrospective Studies; Risk Factors
AD Service de Neurologie D, Hopital Neurologique Pierre Wertheimer, Lyon. glap@wanadoo.fr
SP französisch
PO Frankreich