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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. |
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