Proton magnetic resonance spectroscopy findings and clinical effects of montelukast sodium in a case with Sjogren-Larsson syndrome
Küçük Resim Yok
Tarih
2006
Yazarlar
Dergi Başlığı
Dergi ISSN
Cilt Başlığı
Yayıncı
SAGE PUBLICATIONS INC
Erişim Hakkı
info:eu-repo/semantics/closedAccess
Özet
Sjogren-Larsson syndrome is a rare hereditary metabolic disorder characterized by congenital ichthyosis, mental retardation, and spastic diplegia or tetraplegia. This genetic disease is caused by fatty acid aldehyde dehydrogenase deficiency, leading to an accumulation of long-chain alcohols. The role of enzyme in the degradation of leukotrienes paved the way to the development of a new therapeutic strategy for Sjogren-Larsson syndrome, leukotriene antagonists. We describe a 3-year-old boy with Sjogren-Larsson syndrome who had a lipid peak on proton magnetic resonance spectroscopy despite normal findings on cerebral magnetic resonance imaging. He benefited from treatment with montelukast sodium, especially with respect to the agonizing pruritus.
Açıklama
Anahtar Kelimeler
Kaynak
JOURNAL OF CHILD NEUROLOGY
WoS Q Değeri
Q2
Scopus Q Değeri
Q2
Cilt
21
Sayı
12
