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


Mikol, Jacqueline; Deslys, Jean-Philippe; Zou, Wen-Quan; Xiao, Wiangzhu; Brown, Paul; Budka, Herbert; Goutieres, Françoise (2012). Creutzfeldt-Jakob disease with unusually extensive neuropathology in a child treated with native human growth hormone. Clinical Neuropathology, 31(3):127-134.

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.

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.

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Additional indexing

Item Type:Journal Article, refereed, further contribution
Communities & Collections:04 Faculty of Medicine > University Hospital Zurich > Institute of Neuropathology
Dewey Decimal Classification:570 Life sciences; biology
610 Medicine & health
Language:English
Date:June 2012
Deposited On:18 Dec 2012 17:14
Last Modified:07 Dec 2017 17:27
Publisher:Dustri-Verlag Dr. Karl Feistle
ISSN:0722-5091
Publisher DOI:https://doi.org/10.5414/NP300441
Related URLs:http://www.dustri.com/index.php?id=98&artId=9468
PubMed ID:22551916

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