[Creutzfeldt-Jacob disease in continental France. Retrospective study from 1968 to 1977]

During the decade 1968-1977, 170 cases of Creutzfeldt-Jakob disease were found in France. The clinical features of 124 neuropathologically-verified cases are summarized and a multi-factor analysis performed to obtain the most frequent combinations of signs and symptoms. Certain exceptional features are discussed. The annual mortality rate during this period was 0.32 cases per million for the whole of France, and 0.69 cases per million for the Paris metropolitan area. Preliminary totals for 1978 suggest that these figures may possibly be doubled, with 30-40 new cases occurring annually. No temporal-spatial clustering of cases was observed, and no contact between patients could be established, except for familial cases (9% of the total). No risk factors were identified; in particular, iatrogenic surgical or neurosurgical transmission did not occur, nor was any relationship apparent between CJD in man, and scrapie in sheep or goats. These data furnish a base of comparison for prospective studies in France, with a view towards discovering the natural mode of transmission of the virus of CJD.