ABSTRACT
Cognitive dysfunction is a well-known consequence of epilepsy in children. This review summarizes cognitive difficulties presenting in different types of childhood epilepsy. The possibility of screening and monitoring cognitive dysfunction is desirable to provide optimal support and treatment. The clinical test tool EpiTrack Junior is introduced. It was developed for screening and continuous monitoring of cognitive function in children with epilepsy.
Subject(s)
Cognitive Dysfunction , Epilepsy , Child , Humans , Anticonvulsants/therapeutic use , Neuropsychological Tests , Epilepsy/drug therapy , Cognitive Dysfunction/drug therapy , CognitionABSTRACT
Maple syrup urine disease (MSUD) is an autosomal recessive metabolic disorder causing accumulation of the branched amino acids valin, isoleucin, leucin and their toxic metabolites resulting in ketoacidosis, progressive neurological deterioration and cerebral oedema. The classical form presents in the first days of life. In contrast, the intermittent form of MSUD presents later in childhood and is difficult to diagnose biochemically. Clinical awareness is important due to high mortality if not treated. We here present two cases with late-onset intermittent MSUD.
Subject(s)
Maple Syrup Urine Disease/diagnosis , Adolescent , Child, Preschool , Humans , Magnetic Resonance Imaging , Male , Maple Syrup Urine Disease/therapyABSTRACT
AIM: Even short periods of hyperoxia may induce prolonged cerebral vasoconstriction in newborn infants, and this could theoretically lead to cerebral ischaemia even once normoxia is re-established. This study aimed to investigate the effect of brief hyperoxic exposures on regional cerebral tissue oxygen saturation (rStO2 ) and to evaluate whether any observed prolonged cerebral vasoconstriction was related to maturity. METHODS: The study included 30 infants with a postmenstrual age of more than 32 weeks, who were treated with nasal continuous positive airway pressure and a fraction of inspired oxygen of ≤0.3. The INVOS 5100C oximeter was used to measure rStO2 before, during and after two hyperoxic exposures. If hyperoxia induced a prolonged cerebral vasoconstriction, posthyperoxic rStO2 would be expected to decrease. RESULTS: rStO2 increased slightly after the first hyperoxic exposure, with a mean difference of 1.37% (95% CI 0.15, 2.6). After the second oxygen exposure, rStO2 remained unchanged with a mean difference of -0.4% (95% CI -1.6, 0.78). Differences in rStO2 were not related to gestational age in either of the two hyperoxic episodes. CONCLUSION: We found no evidence to support the theory that transient hyperoxia induces prolonged cerebral vasoconstriction in infants with a postmenstrual age above 32 weeks.
Subject(s)
Cerebrovascular Circulation/physiology , Hyperoxia/metabolism , Hyperoxia/therapy , Infant, Premature, Diseases/metabolism , Infant, Premature, Diseases/therapy , Oxygen Inhalation Therapy , Age Factors , Continuous Positive Airway Pressure , Female , Humans , Hyperoxia/physiopathology , Infant, Newborn , Infant, Premature , Infant, Premature, Diseases/physiopathology , Male , Oximetry , Oxygen Consumption/physiology , Vasoconstriction/physiologyABSTRACT
OBJECTIVE: The objective was to compare cerebral oxygenation in preterm newborns with that in healthy term newborns. METHODS: Forty-six preterm newborns with gestational ages of <33 weeks and 25 healthy term newborns were included. The cerebral tissue oxygenation index (c-TOI) was measured by using near-infrared spectroscopy in clinical steady state on the first day of life (median age: 19.2 hours). The mean gestational ages and birth weights in the 2 groups were 29.1 +/- 2.6 weeks versus 39.7 +/- 1.3 weeks and 1307 +/- 437 g versus 3484 +/- 346 g, respectively. Three preterm infants needed mechanical ventilation and 11 received inotropic drugs. Later, 3 preterm infants developed intraventricular hemorrhage and 2 infants died. All term infants were healthy newborns recruited in the maternity ward. RESULTS: There was a significant difference in c-TOI (preterm: 78.6% [95% confidence interval: 76.9%-80.3%]; term: 74.7% [95% confidence interval: 72.3%-77.1%]). Preterm newborns had significantly lower fractional tissue oxygen extraction, which suggests lower oxygen extraction in this group. There was no significant correlation between head size and c-TOI. The mean peripheral oxygen saturation was 95% in both groups. The median blood Pco(2) for the preterm infants was 6.1 kPa (range: 3.4-7.3 kPa). CONCLUSIONS: Cerebral oxygenation on the first day of life was higher in a group of relatively healthy, very preterm infants in stable condition, compared with healthy term newborns. Slightly elevated blood Pco(2) could be the explanation. Prematurity itself does not seem to dispose preterm infants to global cerebral hypoxia.
