"Hallervorden-Spatz syndrome" (HSS) and "Seitelberger's 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Outdated terms with a Nazi past that should no longer be used.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 are used for NBIA diseases, usually derived from the affected proteins or genes. When using the two outdated eponyms discussed in more detail here, the problematic nature of the names should always be pointed out and at least a "formerly" or "ehemals" should be placed in front of the names for identification purposes.
The correct designations and information on the individual NBIA variants can be found here: Info about NBIA
Background to 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 National Socialist era. In 1922, they were the first to describe an NBIA disease. Thus, "Hallervorden-Spatz syndrome" (HSS) was initially used as a term for what is now known as Pantothenate kinase-associated neurodegeneration (PKAN) known NBIA variant and was later sometimes used as a synonym for other NBIA diseases. Today, at least ten NBIA variants are known, which can be traced back to different gene mutations and each have their own disease mechanism and course. Due to this lack of clarity, but above all because of the far-reaching involvement of Julius Hallervorden and Hugo Spatz in the systematic murder of the disabled and sick 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 people with NBIA are still diagnosed with "G23.0 Hallervorden-Spatz syndrome", as 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 designations is appropriate and necessary.
For the future Hoffnungsbaum e.V. has worked with the NBIA Disorders Association and leading NBIA researchers to ensure that the ICD-11 (successor to the ICD-10) lists all ten NBIA variants with their correct names and subtypes instead of HSS.
Background to the eponym "Seitelberger's disease"
The eponym "Seitelberger disease" also provides a glimpse into the abyss of National Socialist machinations. The eponym Franz Seitelberger habilitated under Hallervorden and used brains from Hallervorden's collection, which were proven to come from three children who were murdered as part of the so-called "euthanasia" program.
The correct name of the disease today is Phospholipase A2-Associated Neurodegeneration (PLAN) or its early childhood subform Infantile neuroaxial dystrophy (INAD).