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Creutzfeldt–Jakob disease (CJD) is a human transmissible spongiform encephalopathy or prion disease. Although CJD is most frequently sporadic, numerous acquired or iatrogenic CJD (iCJD) cases have been reported, about half of which are attributable to prion-contaminated human growth hormone (hGH) preparations.1 Cadaveric hGH was provided by public and commercial sources up to 1985, when recombinant GH became available. Incubation periods of hGH-iCJD peak at a median of 12 (range 5–30) years after exposure.2 3
We report the first Austrian case of hGH-associated autopsy-proven iCJD and discuss clinical features and serial magnetic resonance imaging (MRI) findings.
A 39-year-old man presented with right-sided clumsiness and dysaesthesia, which had started in his leg 3 weeks prior to admission and had spread to his right arm. No impairment of cognitive function and no involuntary movements were present. There was no family history of neurological disease. The patient had been healthy until the age of 11 years, when progressive obesity and growth impairment had been noticed and a diagnosis of Cushing syndrome had been made. The patient moved to Austria at the age of 15 years (1982) and was subsequently diagnosed with a hormone-producing pituitary adenoma, which was removed by transsphenoidal hypophysectomy. The frontal skull base defect was covered with autologous connective tissue (fascia lata). Due to persistent Cushing syndrome symptoms, bilateral adrenalectomy was performed. To promote body growth (height <3rd percentile), he received commercially manufactured cadaveric hGH (Crescormon®, Kabi Pharma, now discontinued) from September 1984 (2 IU IM three times per week, which was later reduced to 2 IU IM twice a week). The treatment was continued until November 1985 …