Creutzfeldt-Jakob disease with unusually extensive neuropathology in a child treated with native human growth hormone.

Abstract

We report a case of iatrogenic Creutzfeldt-Jakob disease(iCJD) in a child with a neonatal growth hormone (GH) deficiency that was treated with native human growth hormone (hGH) between the ages of 9 months and 7 years. Three years after the end of treatment a progressive neurological syndrome consistent with Creutzfeldt-Jakob disease (CJD) developed, leading to death within a year, at age 11. Neuropathological examination showed an unusual widespread form of CJD, notably characterized by (i) involvement of the cerebellar white matter, (ii) cortico-spinal degeneration and (iii) ballooned neurons. A transitional form of the disease between common iatrogenic and panencephalopathic CJD is suggested.

• Authors: Jacqueline Mikol, Jean-Philippe Deslys, Wen-Quan Zou, Wiangzhu Xiao, Paul Brown, Herbert Budka, Françoise Goutieres
• Journal: Clinical neuropathology (Clin Neuropathol) 2012 May-Jun Vol. 31 Issue 3 Pg. 127-34 ISSN: 0722-5091 [Print] Germany
• PMID: 22551916 (Publication Type: Case Reports, Journal Article, Research Support, N.I.H., Extramural, Research Support, Non-U.S. Gov't)

Chemical References

• Human Growth Hormone

Topics

• Brain (pathology)
• Child
• Creutzfeldt-Jakob Syndrome (drug therapy, pathology)
• Fatal Outcome
• Human Growth Hormone (deficiency, therapeutic use)
• Humans
• Male