title: 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.affiliation: Denis Diderot University. Jacqueline.mikol@wanadoo.fr
authors.forename: Jacqueline
authors.initials: J
authors.lastname: Mikol
authors.forename: Jean-Philippe
authors.initials: JP
authors.lastname: Deslys
authors.forename: Wen-Quan
authors.initials: WQ
authors.lastname: Zou
authors.forename: Wiangzhu
authors.initials: W
authors.lastname: Xiao
authors.forename: Paul
authors.initials: P
authors.lastname: Brown
authors.forename: Herbert
authors.initials: H
authors.lastname: Budka
authors.forename: Françoise
authors.initials: F
authors.lastname: Goutieres
mesh_terms: D001921:Brain / Q000473:pathology*; D002648:Child; D007562:Creutzfeldt-Jakob Syndrome / Q000188:drug therapy / Q000473:pathology*; D017809:Fatal Outcome; D019382:Human Growth Hormone / Q000172:deficiency* / Q000627:therapeutic use*; D006801:Humans; D008297:Male
chemical_list: D019382:Human Growth Hormone