Objective The aim investigate neurodevelopment outcome in children with isolated umbilical'cord stragulation at age of 3. Also investigate neurodevelopment outcome health children and two groups ...related between. Methods Investigation provide during period from 2005. to 2013. On Ginecological and Opstetric department Cantonal – Hospital Dr Irfan Ljubičić Bihać and Hospital Sanski Most and Ginecological Obstretical outpatients Dr Karabeg. We studied prospectively 56 term infants with umbilical'cord strangulation excluded other malformation and chromosomotaphies. Umbilical cord anomaly had been diagnosed by a gynecologist and pathologist according to Fox' classification. Longitudinal neurodevelopment was prospectively followed multidisciplinary to age three. Study included evaluated neurological, psychological, language and cognitive function. Results At age three 36 children (ADHD – 4, visual disturbances – 6, ear disorder – 10, epilepsy – 4, cerebral palsy – 2, hemiparetic form – 2, milder derogation – 8) of 56 indicate different neurodevelopment disability. ADHD 4, It was statistical significant regarding health children. Conclusion It is very important that term infant with umbilical'cord strangulation include in longitudinal prospectively neurodevelopment follow, because early detection deviation and early include in habilitation succeed better outcome.
Novorođenče ima ograničeni repertoar iskazivanja teškoća, pa su konvulzije često jedina neurološka manifestacija ozbiljne bolesti središnjeg živčanog sustava ili pratećega podliježećeg poremećaja. ...Incidencija neonatalnih konvulzija kreće se od 3,5-4,4/1000 živorođene djece s tendencijom povećanja smanjenjem životne dobi. Konvulzije koje se dogode nezrelom mozgu mogu uzrokovati ireverzibilne promjene neuronskih sinapsa i tako mogu biti odgovorne za razvojne poremećaje mozga, što čini podlogu za nastavak konvulzija. S obzirom na kliničku sliku, EEG promjene kao i kasniju prognozu dijelimo ih na dobroćudne i teške.epileptičke sindrome. One mogu bit uzrokovane genetskim poremećajima kao što su konvulzije ovisne o piridoksinu, neketotična hiperglicinemia, nedostatak sulfitne oksidaze, konvulzije koje reagiraju na folnu kiselinu i nedostatak protein- transportera glukoze (GLUT-1 deficiency) te benigne obiteljske konvulzije zbog poremećaja kalijevih kanala. Konvulzije izazvane ovim poremećajima često su refrakterne na uobičajenu antiepileptičku terapiju. Rana dijagnoza novorođenačkih konvulzija je važna, jer često otkriva prisutnost teških strukturalnih poremećaja mozga koji u podlozi imaju genetski poremećaj i zahtijevaju specifično liječenje.
The purpose of this study was to question the correlation of different grades of periventricular leukomalacia (PVL) and subsequent neurodevelopmental outcome. In a prospective study we followed 52 ...preterm infants. Infants were divided into three groups according to their cranial ultrasound findings of PVL (De Vries classification). Seventeen children had PVL 1, 20 children had PVL 2, and 15 children had PVL 3. All 15 (100%) children with PVL 3 developed cerebral palsy with additional visual perceptual dysfunctions and epilepsy. Children with PVL 1 had high frequency of mild neuromotoric delay and visual impairment. PVL 2 and 3 have great predictive value for subsequent severe neurodevelopmental disorder which refers to cerebral palsy, different cognitive deficits, vision impairment and epilepsy. We have determined that due to high frequency of visual impairment and epilepsy we need to include neurophysiologic examinations very early in children with PVL lesions.
The aim of the study was to evaluate magnetic resonance imaging (MRI) findings in infants with periventricular leukomalacia (PVL) and West syndrome (WS) and determine the neurodevelopmental outcome ...in children with West syndrome and PVL. Ultrasound and brain MRI were performed in 37 infants with recognized PVL. PVL was categorized according to De Vries, whereas West syndrome was categorized according to International League Against Epilepsy 1989. West syndrome in our patients developed during the first 2 years of life. The most common interictal abnormality was hypsarrhythmia. All, except two patients had delayed development and various degrees of mental retardation. The most characteristic neuroimaging findings were major reduction in cerebral cortical gray matter volume, reduction in the volume of brain myelin, and delayed myelination. These findings may explain the anatomical association between the West syndrome onset and PVL and intellectual and cognitive deficit in premature infants with PVL.
The aim of this study was to analyse glycosphingolipid expression in cerebrospinal fluid (CSF) from one idiopathic West syndrome (IWS) infant, one with Reye like syndrome, and one with congenital ...hydrocephalus, in comparison to control group (n=7) using highly sensitive thin-layer chromatography-immunostaining methods. 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 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 transferases in the age of seven months.
