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EDITORIALS:
Robert B. Daroff
Report from the Neurology Scientific Integrity Advisor: Year 1
Neurology 2005; 64: 588-589
[Full text][PDF]
Robert B. Daroff, M.D., Robert C. Griggs, MD - Editor-in-Chief, Neurology
(11 May 2005)
Report from the Neurology Scientific Integrity Advisor: Year 1
Roger L. Albin
(11 May 2005)
Reply to Albin
11 May 2005
Robert B. Daroff, M.D., Scientific Integrity Advisor, Neurology CASE School of Medicine, 10900 Euclid Ave. - T101, Cleveland, OH 44106-4994, Robert C. Griggs, MD - Editor-in-Chief, Neurology
Robert.Daroff{at}case.edu Robert B. Daroff, M.D., et al.
I (RBD) did not state that Hallervorden-Spatz was a universally accepted eponym, but since PubMed listed 146 publications citing Hallervorden between 1994 and March 2004, it certainly has not been universally expunged.
The Neurology Editorial Board in 1993 decided not to eliminate the eponym and we thought it appropriate for the current Editorial Board to reconsider the issue, and it was discussed at the April 12, 2005 Neurology Editorial Board meeting in Miami. For a variety of reasons, the Board decided to continue permitting the Hallervorden-Spatz eponym.
Robert B. Daroff, MD, Scientific Integrity Advisor
Robert C. Griggs, MD, Editor-in-Chief
Report from the Neurology Scientific Integrity Advisor: Year 1
11 May 2005
Roger L. Albin, Ann Arbor VAMC GRECC; Dept. of Neurology, University of Michigan 4412D Kresge III, 200 Zina Pitcher Place, Ann Arbor, MI, 48109-0585
Dr. Daroff’s comments to the contrary, Hallervorden-Spatz syndrome
(HSS) is no longer a universally accepted eponym. Hayflick et al [1], the
discovers of the pantothenate kinase 2 (PANK2) mutations responsible for
most cases of this syndrome, recommend that cases of this syndrome caused
by PANK2 mutations be termed Pantothenate Kinase Associated
Neurodegeneration (PKAN). Phenotypically similar cases not associated
with PANK2 mutations should be termed Neurodegeneration with Brain Iron
Accumulation (NBIA). This recommendation is followed in the authoritative
Online Mendelian Inheritence in Man (OMIM) which no longer has a separate
heading for HSS but instead lists it under the more accurate descriptor
PKAN.
The excellent GeneReviews section of the Gene Tests website
(www.genetests.org) lists PKAN and Neuronal Brain Iron Accumulation
(NBIA), and has no separate listing for HSS. In these reviews, it is
pointed out that PKAN and NBIA were formerly referred to as HSS, clearly
discouraging the use of the HSS eponym.
The adoption of this alternative, and more accurate nomenclature, is
driven primarily by Michael Shevell’s writings on Hallervorden’s
complicity in the Nazi euthanasia program. [2,3] Shevell argues strongly
that eponyms exist as honorifics and that use of the HSS eponym is
honoring an accessory to mass murder.
Given the existence of more
accurate and accepted nomenclature, Neurology should discourage the use of
the HSS eponym.
References
1. Hayflick SJ, Westaway SK, Levinson B, et al. Clinical, genetic, and radiologic delineation of
Hallervorden-Spatz syndrome. N Engl J Med 2003;348:33-40.
2. Shevell M. Racial hygiene, active euthanasia, and Julius Hallervorden.
Neurology 1992;42:2214-2219.
3. Shevell M. Hallervorden and history. N Engl J Med 2003;348:3-4.