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Neuropathologic verification of Creutzfeldt-Jakob disease in the exhumed American recipient of human pituitary growth hormone

Epidemiologic and pathogenetic implications

Department of Neurology (Dr. Tintner), The University of Texas Health Science Center, Dallas, TX; Laboratory of CNS Studies (Drs. Brown, Piccardo, and Gajdusek), National Institute of Neurological and Communicative Disorders and Stroke, National Institutes of Health, Bethesda, MD; Laboratory for Neuropathology (Dr. Hedley-Whyte), Massachusetts General Hospital, Boston, MA; and the Division of Metabolism and Endocrine Drug Products (Dr. Rappaport), Food and Drug Administration, Rockville, MD.

Beginning at age 12, a boy with idiopathic hypopituitarism was treated with cadaver pituitary-derived human growth hormone during the period from 1963 to 1969. Fifteen years after the last treatment, the then 32-year-old man developed Creutzfeldt-Jakob disease (CJD). The illness was atypical in showing predominantly cerebellar signs, little mental deterioration, and no abnormal movements or periodic EEG activity. Examination of the embalmed brain, 7 months after interment, revealed the characteristic changes of CJD mainly in the cerebellum and basal ganglia. This case establishes the contamination of at least two American lots of human growth hormone and, together with other cases of iatrogenic disease, suggests that virus enters the brain from the blood, rather than along neural pathways.

Address correspondence and reprint requests to Dr. Brown, Building 36, Room 5B05, National Institutes of Health, Bethesda, MD 20205.

Accepted for publication April 11, 1986.

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