Abstract

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.

Highlights

  • In 1992 and 1996 Shevell (1992) and Harper (1996) first described the role of Julius Hallervorden and Hugo Spatz in a preliminary manner during the Nazi regime, suggesting that the disease should be renamed for ethical reasons (cf. Fig. 1 for German publication on neurodegeneration with brain iron accumulation (NBIA) and photography of Hugo Spatz)

  • We found 305 autopsy index cards in the Archive of the Max Planck Society Berlin matching with 4 of the 30.146 patient files in the German Federal Archive Berlin

  • The following four victims of the National Socialist (NS) euthanasia program have been identified, from the Hugo Spatz brain specimens in the Max Planck Society Archive

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Summary

Introduction

In 1992 and 1996 Shevell (1992) and Harper (1996) first described the role of Julius Hallervorden and Hugo Spatz (cf. supplement “H. Spatz’s Biography”) in a preliminary manner during the Nazi regime, suggesting that the disease should be renamed for ethical reasons (cf. Fig. 1 for German publication on neurodegeneration with brain iron accumulation (NBIA) and photography of Hugo Spatz). In 1992 and 1996 Shevell (1992) and Harper (1996) first described the role of Julius Hallervorden and Hugo Spatz Spatz’s Biography”) in a preliminary manner during the Nazi regime, suggesting that the disease should be renamed for ethical reasons (cf Fig. 1 for German publication on neurodegeneration with brain iron accumulation (NBIA) and photography of Hugo Spatz). We looked at the potential involvement of Hugo Spatz in euthanasia activities during World War II (WWII). In this context, it should be noted that the German Neurological Society (DGN) named a well-known prize in honor of Hugo Spatz, which was changed to Adolf Wallenberg price in 1998. The present investigation aims to investigate the success of the renaming process of HSD to PKAN and NBIA between 1946 and 2019

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