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.
Highlights
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 between the ages of 9 months and 7 years
In their review on “iatrogenic CreutzfeldtJakob disease at the millennium”, Brown et al [1] emphasized some peculiarities according to the country of origin and showed that more than half of all growth hormone cases have occurred in France. 115 cases have been listed by 2010 [2]
We found several histopathological hallmarks of the panencephalopathic form of CJD (PE-CJD) described by Mizutani [6, 7] principally seen in Japanese dura mater-associated iatrogenic CreutzfeldtJakob disease (iCJD) [8] but in our case the hemispheric white matter lesions were less prominent
Summary
In their review on “iatrogenic CreutzfeldtJakob disease (iCJD) at the millennium”, Brown et al [1] emphasized some peculiarities according to the country of origin and showed that more than half of all growth hormone cases have occurred in France. 115 cases have been listed by 2010 [2]. 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. 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.
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