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

Abstract

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.

Early cognitive decline in Creutzfeldt-Jakob disease associated with human growth hormone treatment

“…Similarly, Marzewski et al described a 37 year old man who presented with a one month of progressive imbalance. 13 Neuropsychology testing revealed ''borderline cognitive impairment with possibly slightly reduced learning efficiency.'' The authors, however, remarked on the relative preservation of mental function at presentation.…”

“…[11][12][13][14] The first published case, of a 22 year old British woman, is, however, the only report to provide formal neuropsychology data, aquired seven months from the onset of the cerebellar syndrome. 5 15 This showed evidence of intellectual decline, with a verbal intelligence quotient (VIQ) of 80.…”

Status Report on the US Human Growth Hormone Recipient Follow-Up Study

“…Thirteen families were not contacted at the request of the treating physician, usually for psychologi cal reasons. Finally, the 5 known (US) cases of CJD [7,8] were not given the standard interview because data were already available on them and the study investigators did not want to risk upsetting family members.…”

Untoward Events in Patients Treated with Growth Hormone in the USA

“…There have now been a total of ten cases of Creutzfeldt-Jakob disease iden tified in patients treated with pituitary GH prepared in the United States. Of these, eight occurred in the United States [7][8][9][10], while one patient in Brazil [11] and one in New Zealand [12] were associated with the use of pitu itary GH prepared in the United States. It soon became apparent that this problem was not limited to the GH pre pared from pituitary glands in the United States [ 13.…”