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Neuropediatrics 2000; 31(1): 1-3
DOI: 10.1055/s-2000-15288
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Georg Thieme Verlag Stuttgart · New York

5-Lipoxygenase Inhibition: A New Treatment Strategy for Sjögren-Larsson Syndrome

M. A.A.P. Willemsen1 , J. J. Rotteveel1 , P. M. Steijlen2 , A. Heerschap3 , E. Mayatepek4
  • Departments of1 Pediatric Neurology,
  • 2 Dermatology, and
  • 3 Radiology, University Hospital Nijmegen, Nijmegen, The Netherlands
  • 4 Department of General Pediatrics, University Children's Hospital, Heidelberg, Germany
Further Information

Publication History

August 30, 1999

November 8, 1999

Publication Date:
31 December 2000 (online)

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The Sjögren-Larsson syndrome (SLS) is a severe neurocutaneous disorder due to fatty aldehyde dehydrogenase (FALDH) deficiency. The recent discovery of the role of FALDH in the degradation of leukotriene 84 (LTB4) opened the way to the development of a new therapeutic strategy for SLS, i.e. 5-lipoxygenase inhibition. We treated one SLS patient with zileuton during five weeks. During the treatment period we found decreased values of LTB4 and (ο-OH-LTB4. The severity of the pruritus diminished, and favorable changes in the child's behavior were observed. The height of the prominent “lipid peak” of cerebral white matter (that is characteristically found on proton magnetic resonance spectroscopy in SLS patients) decreased during treatment, and increased again when treatment was stopped. In conclusion, the benefidal effects of 5-lipoxygenase inhibition in SLS are very promising and encourage further research.

Key words

Sjögren-Larsson syndrome - Fatty aldehyde dehydrogenase deficiency - Leukotriene B4 - 5-Lipoxygenase inhibition - Pruritus