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NEUROLOGY 1988;38:1131
© 1988 American Academy of Neurology

Creutzfeldt-Jakob disease following pituitary-derived human growth hormone therapy

A new American case

D. J. Marzewski, MD, J. Towfighi, MD, M. G. Harrington, MB, ChB, MRCP, C. R. Merril, MD and P. Brown, MD

From the Department of Neurology (Dr. Marzewski), Geisinger Medical Center, Danville, PA; the Division of Neuropathology (Dr. Towfighi), Department of Pathology, Milton S. Herahey Medical Center, Hershey, PA; and the Biochemical Genetics Section, Clinical Neurogenetics Branch (Drs. Harrington and Merril), and the Laboratory of CNS Studies (Dr. Brown), National Institute of Neurological and Communicative Disorders and Stroke, National Institutes of Health, Betheada, MD.

A fourth histologically-confirmed American case of Creutzfeldt-Jakob disease (CJD) related to human growth hormone (hGH) therapy is reported. Like kuru, the illness was dominated by cerebellar signs and relatively little mental deterioration. The diagnosis was strongly supported premortem by the presence of two abnormal 30 kDa proteins in the CSF that are seen almost exclusively in CJD. The characteristic clinical picture coupled with such biochemical data allow a reasonably accurate premortem diagnosis of hGH-related iatrogenic CJD to be made.

Address correspondence to Dr. Brown, Building 36, Room 5B21, NINCDS, NIH, Bethesda, MD 20892. Address reprint requests to Dr. Marzewski, Department of Neurology, Geisinger Medical Center, Danville, PA 17822.

Received September 2, 1987. Accepted for publication in final form November 25, 1987.




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