5-lipoxygenase inhibition: A new treatment strategy for Sjogren-Larsson syndrome

Citation
Maap. Willemsen et al., 5-lipoxygenase inhibition: A new treatment strategy for Sjogren-Larsson syndrome, NEUROPEDIAT, 31(1), 2000, pp. 1-3
Citations number
10
Categorie Soggetti
Neurology,"Neurosciences & Behavoir
Journal title
NEUROPEDIATRICS
ISSN journal
0174304X → ACNP
Volume
31
Issue
1
Year of publication
2000
Pages
1 - 3
Database
ISI
SICI code
0174-304X(200002)31:1<1:5IANTS>2.0.ZU;2-E
Abstract
The Sjogren-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 B-4 (LTB4) opened the way to the development of a new therapeutic strategy for SLS, i.e. 5-lipoxy genase inhibition. We treated one SLS patient with zileuton during five wee ks. During the treatment period we found decreased values of LTB4 and omega -OH-LTB4. The severity of the pruritus diminished, and favorable changes in the child's behavior were observed. The height of the prominent "lipid pea k" of cerebral white matter (that is characteristically found on proton mag netic resonance spectroscopy in SLS patients) decreased during treatment, a nd increased again when treatment was stopped. In conclusion, the beneficia l effects of 5-lipoxygenase inhibition in SLS are very promising and encour age further research.