Non familial Creutzfeldt-Jakob disease: a study of 53 cases.

Creutzfeldt-Jakob disease (CJD) is the most frequent human spongiform encep halopathy. We have analyzed 53 cases of definite non familial CJD over a st udy period of 28 years. Ail were autopsied in the same neuropathological un it. Clinical and epidemiological data were in accordance with previous stud ies: low incidence of CJD under the age of 40 high incidence around the six ty years of age (26 p.100), a sex ratio at 0.65 and high frequency of myocl onus, dementia and cerebellar ataxia during evolution (78 p.100 of cases). This study highlights the rapidity at the onset of the disease (within 24 h ours) in 16p.100 of the case. EEG disclosed typical pseudoperiodic activity in only 53 p.100 of cases and cerebral MRI showed high T2 signal intensity in basal nuclei in 15p.100. This regional study is the second of its natur e to be carried out in France, the first one covering the area of Paris.