Creutzfeldt-Jakob disease from contaminated growth hormone extracts in France

We diagnosed Creutzfeldt-Jakob disease in 34 patients (16 definite, 18 probable) who had received human growth hormone extract for various periods of time (mean +/- SD, 6.4 +/- 2.9 years), but particularly during the period between January 1984 and July 1985, a potential high-risk factor. Disease duration for deceased patients (n = 30) was 17 +/- 9 months. The clinical picture was homogeneous, starting with cerebellar ataxia and ocular motor disorders in about 90% of the patients. Neurologic deterioration, including dementia and myoclonic jerks, occurred within months. The high number of cases (1.5% of those treated between 1959 and 1988, 3% of those treated during the putative high-risk period) is still unexplained. We discuss the possibility that new cases will be detected, the risk of contaminating the general public, and the sanitary measures undertaken to prevent this. NEUROLOGY 1996;47: 690-695

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