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Renaming of Hallervorden–Spatz disease: the second man behind the name of the disease

Hallervorden–Spatz disease (HSD) has been recently renamed to pantothenate kinase-associated neurodegeneration (PKAN) and neurodegeneration with brain iron accumulation (NBIA), mainly due to the unethical behavior of Julius Hallervorden in the National Socialist (NS) euthanasia program of the Nazi T...

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Autores principales: Voges, Luca, Kupsch, Andreas
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Springer Vienna 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8536572/
https://www.ncbi.nlm.nih.gov/pubmed/34655340
http://dx.doi.org/10.1007/s00702-021-02408-x
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author Voges, Luca
Kupsch, Andreas
author_facet Voges, Luca
Kupsch, Andreas
author_sort Voges, Luca
collection PubMed
description Hallervorden–Spatz disease (HSD) has been recently renamed to pantothenate kinase-associated neurodegeneration (PKAN) and neurodegeneration with brain iron accumulation (NBIA), mainly due to the unethical behavior of Julius Hallervorden in the National Socialist (NS) euthanasia program of the Nazi Third Reich. The role of the second name giver in the NS euthanasia program is less clear. Hugo Spatz was the director of the Kaiser Wilhelm Institute for Brain Research in Berlin-Buch during World War II (WWII), renamed to Max Planck Institute after 1945. After the war, he headed the Max Planck Institute for Brain Research in Frankfurt am Main. The present study investigates the potential involvement of Hugo Spatz in the NS euthanasia program. In the present study, we compared a list of euthanasia victims from the German Federal Archive Berlin (30.146 cases published after the reunification of Germany, BArch R179) with the files of the collection of specimens from 1940 until 1945 of Hugo Spatz as listed in the Archive of the Max Planck Society Berlin-Dahlem (n = 305). Furthermore, the old term HSD and the new terms PKAN and NBIA were systematically searched in PubMed from 1946, through January 2019 to evaluate the renaming process from HSD to PKAN/NBIA. Following Hugo Spatz’s death in 1969 growing evidence indicated that he may have taken part in the NS euthanasia program. This study identifies 4 euthanized victims in the patient files of Hugo Spatz from 1940 to 1945, suggesting involvement of Hugo Spatz in the NS euthanasia program. This further strengthens the argument that the former HSD should be exclusively referred to as PKAN or NBIA. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s00702-021-02408-x.
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spelling pubmed-85365722021-11-04 Renaming of Hallervorden–Spatz disease: the second man behind the name of the disease Voges, Luca Kupsch, Andreas J Neural Transm (Vienna) Neurology and Preclinical Neurological Studies - Original Article Hallervorden–Spatz disease (HSD) has been recently renamed to pantothenate kinase-associated neurodegeneration (PKAN) and neurodegeneration with brain iron accumulation (NBIA), mainly due to the unethical behavior of Julius Hallervorden in the National Socialist (NS) euthanasia program of the Nazi Third Reich. The role of the second name giver in the NS euthanasia program is less clear. Hugo Spatz was the director of the Kaiser Wilhelm Institute for Brain Research in Berlin-Buch during World War II (WWII), renamed to Max Planck Institute after 1945. After the war, he headed the Max Planck Institute for Brain Research in Frankfurt am Main. The present study investigates the potential involvement of Hugo Spatz in the NS euthanasia program. In the present study, we compared a list of euthanasia victims from the German Federal Archive Berlin (30.146 cases published after the reunification of Germany, BArch R179) with the files of the collection of specimens from 1940 until 1945 of Hugo Spatz as listed in the Archive of the Max Planck Society Berlin-Dahlem (n = 305). Furthermore, the old term HSD and the new terms PKAN and NBIA were systematically searched in PubMed from 1946, through January 2019 to evaluate the renaming process from HSD to PKAN/NBIA. Following Hugo Spatz’s death in 1969 growing evidence indicated that he may have taken part in the NS euthanasia program. This study identifies 4 euthanized victims in the patient files of Hugo Spatz from 1940 to 1945, suggesting involvement of Hugo Spatz in the NS euthanasia program. This further strengthens the argument that the former HSD should be exclusively referred to as PKAN or NBIA. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s00702-021-02408-x. Springer Vienna 2021-10-16 2021 /pmc/articles/PMC8536572/ /pubmed/34655340 http://dx.doi.org/10.1007/s00702-021-02408-x Text en © The Author(s) 2021 https://creativecommons.org/licenses/by/4.0/Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) .
spellingShingle Neurology and Preclinical Neurological Studies - Original Article
Voges, Luca
Kupsch, Andreas
Renaming of Hallervorden–Spatz disease: the second man behind the name of the disease
title Renaming of Hallervorden–Spatz disease: the second man behind the name of the disease
title_full Renaming of Hallervorden–Spatz disease: the second man behind the name of the disease
title_fullStr Renaming of Hallervorden–Spatz disease: the second man behind the name of the disease
title_full_unstemmed Renaming of Hallervorden–Spatz disease: the second man behind the name of the disease
title_short Renaming of Hallervorden–Spatz disease: the second man behind the name of the disease
title_sort renaming of hallervorden–spatz disease: the second man behind the name of the disease
topic Neurology and Preclinical Neurological Studies - Original Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8536572/
https://www.ncbi.nlm.nih.gov/pubmed/34655340
http://dx.doi.org/10.1007/s00702-021-02408-x
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