"Hallervorden-Spatz Syndrome" (HSS) and "Seitelberger Disease"

Outdated terms with a Nazi past that should no longer be used.

Hoffnungsbaum e.V. advocates the use of the correct, international and specific names for NBIA disorders, mostly derived from the affected proteins or genes. When using the two obsolete eponyms discussed in more detail here, the problem of the designations should always be pointed out and a "formerly" or "ehemals" should be placed in front of the designations at least for identification purposes.
The correct designations and information on the individual NBIA variants can be found here: Info about NBIA

Background 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 NBIA disease. Thus, "Hallervorden-Spatz syndrome" (HSS) was initially used as a name for what is now known as the Pantothenate kinase-associated neurodegeneration (PKAN). known NBIA variant and was later partly used as a synonym for other NBIA disorders. Today, at least ten NBIA variants are known, which are due to different gene mutations and each have their own disease mechanism and course. Because of this vagueness, but especially because of the far-reaching involvement of Julius Hallervorden and Hugo Spatz in the systematic murders 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 NBIA sufferers still receive the diagnosis "G23.0 Hallervorden-Spatz syndrome" because the ICD-10 (International Statistical Classification of Diseases and Related Health Problems) does not yet list the individual NBIA variants. The additional mention of the correct and, depending on the variant, specific designations is appropriate and necessary.
For the future Hoffnungsbaum e.V. together with the NBIA Disorders Association and leading NBIA researchers, has ensured that ICD-11 (successor to ICD-10) lists all ten NBIA variants with their correct names and subtypes instead of HSS.

Eponym backgrounds "Seitelberg disease"
The eponym "Seitelberger disease" also provides a glimpse into the abysses of National Socialist machinations. The eponym 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".
The correct disease name today is Phospholipase A2-Associated Neurodegeneration (PLAN). or its early childhood subtype Infantile Neuroaxional Dystrophy (INAD).



Subscribe to our Hoffnungsbaum newsletter!

With our newsletter emails you will receive valuable information about NBIA. If you would like to subscribe to additional info on individual NBIA variants or special info for researchers/clinicians besides the general newsletter, please click here: Subscribe additional info

Privacy policy

You have successfully registered!