engleski

Glycosphingolipid expression in cerebrospinal fluid of infants with neurological abnormalities

The aim of this study was to analyse glycosphingolipids expression in cerebrospinal fluid (CSF) of infants with neurological abnormalities. Neurochemical understanding of the cell surface events that accompany neurogenesis is important for determining the pathogenesis of age-dependent diseases. Because they are adundant in the outer surface of neuronal plasma membrane, GSLs may play an important role in these events. CSF samples obtained from infants with neurological abnormalities (n=3) and from control group (n=7)were analyzed separately for GSL expression by highly sensitive thin-layer chromatography-immunostaining methods. Of the 3 infants aged 1-12 months with neurological abnormalities, one was diagnosed with idiopathic West syndrome (IWS), one with Reye like syndrome, and one with congenital hydrocephalus. We analysed these samples in comparison with CSF samples obtained from 7 infants admitted to our department for lumbar puncture due to seizures. Gangliotetraose-series gangliosides (acidic glycosphingolipids) were not detected in CSF of infant with idiopathic West syndrome and infant with congenital hydrocephalus. CSF of infant with IWS showed traces of neolacto-tetraose ganglioside fractions, which were absent in all other CSF examined. In addition, lactosylceramide fraction, and one ceramide fraction were highly expressed only in IWS CSF. Using HPTL-immunostaining chromatography, it was possible to detect CSF GSLs in as little as 10-30 microl of CSF. These results confirmed previously described lack of gangliotetraose-series gangliosides in IWS patient and for the first time is described increased expression of neolacto-series glycosphingolipids in IWS patient. Since follow up until the age of five years showed almost normal IWS patient psychomotor development, the discribed shift of glycosphingolipid expression may implicate on transient inhibition of specific glycosyl fransferases in the age of seven months.

idiopathic West syndrome; Reye like syndrome; congenital hydrocephalus; cerebrospinal fluid; glycosphingolipids

Sažetak je objavljen u časopisu citiranom u Neuroscience Citation Index i EMBASE/Excerpta Medica. Neurologia Croatica. 57 (2008) , Suppl. 1