„Hallervorden-Spatz Syndrome“ (HSS) and „Seitelberger Disease“

Outdated terms with a Nazi past that should no longer be used.

Hoffnungsbaum e.V. is committed to ensuring that the correct, international and specific names, usually derived from the affected proteins or genes, are used for NBIA diseases. When using the two outdated eponyms discussed here in more detail, the problematic nature of the terms should always be pointed out, and at least a „formerly“ or „previously“ should be placed before the terms for identification.
The correct names and information on the individual NBIA variants can be found here: Info about NBIA

Background information on the eponym „Hallervorden-Spatz Syndrome“ (HSS)
Julius Hallervorden and Hugo Spatz were two prominent brain researchers who were involved in the so-called „euthanasia“ program during the Nazi era. In 1922, they were the first to describe an NBIA disease. Thus, „Hallervorden-Spatz Syndrome“ (HSS) was initially the term for what is now known as Pantothenate Kinase-Associated Neurodegeneration (PKAN) known NBIA variant and was later partially used as a synonym for other NBIA disorders. Today, at least ten NBIA variants are known, which can be attributed to different gene mutations and each has its own disease mechanism and course. Due to this ambiguity, but above all because of the far-reaching involvement of Julius Hallervorden and Hugo Spatz in the systematic killings of disabled people and patients by the Nazis, the eponym HSS should no longer be used today.

"Hallervorden-Spatz Syndrome" (HSS) in the ICD-10
Despite the problems described above, many NBIA patients are still diagnosed with "G23.0 Hallervorden-Spatz Syndrome" because the individual NBIA variants are not yet listed in the ICD-10 (International Statistical Classification of Diseases and Related Health Problems). The additional naming of the correct and, depending on the variant, specific names is appropriate and necessary.
For the future, Hoffnungsbaum e.V., together with the NBIA Disorders Association and leading NBIA researchers, has ensured that in the ICD-11 (successor to the ICD-10) all ten NBIA variants with their correct names and subforms will be listed instead of HSS.

Background information on the eponym "Seitelberger Disease"
The eponym "Seitelberger Disease" also offers insights into the abyss of National Socialist machinations. The namesake, Franz Seitelberger, habilitated with Hallervorden and used brains from Hallervorden's collection, which demonstrably came from three children who were murdered as part of the so-called "euthanasia" program.
The correct disease name today is Phospholipase A2-Associated Neurodegeneration (PLAN) or its early childhood subform Infantile Neuroaxonal Dystrophy (INAD).

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