Brain & development
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Brain & development · Oct 2011
Case ReportsLeukoencephalopathy with brain stem and spinal cord involvement and lactate elevation in the first Polish patient.
Leukoencephalopathy with brain stem and spinal cord involvement and elevated white matter lactate (LBSL) is a very rare autosomal recessive mitochondrial disorder. Clinically patients have slowly progressive ataxia, pyramidal syndrome and dorsal column dysfunction. The disease is defined on the basis of characteristic abnormalities observed on magnetic resonance imaging such as inhomogeneous, spotty involvement of the cerebral white matter, selective involvement of brain stem and spinal cord tracts as well as lactate elevation in the affected white matter on spectroscopy. We present the first identified Polish patient suffering from LBSL confirmed molecularly.
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Dysplasia of the cerebellar dentate nucleus is a state of apparent maturational arrest that involves the cerebellar dentate nucleus. Origins include Joubert syndrome, other disorders of axon guidance and dentato-olivary dysplasia. An overview is given, linking the diverse etiologies.
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Brain & development · Sep 2011
ReviewErythropoietin in neonatal brain protection: the past, the present and the future.
Over the last decade, neuroprotective effects of erythropoietin (Epo) and its underlying mechanisms in terms of signal transduction pathways have been defined and there is a growing interest in the potential therapeutic use of Epo for neuroprotection. Several mechanisms by which Epo provides neuroprotection are recognized. In this review, we focused on the neuroprotective mechanisms of Epo and provide a short overview on both experimental and clinical studies, testing Epo as a neuroprotective agent in the neonatal brain injury, and the safety concerns with the clinical use of Epo treatment in neonates.
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Brain & development · May 2011
Sleep cyclic alternating pattern analysis in healthy children during the first year of life: a daytime polysomnographic study.
We evaluated the cyclic alternating pattern (CAP) during the first year of life in order to obtain information on the maturation of arousal mechanisms during NREM sleep and to provide normative data for CAP parameters in this age range (5-16months). Eleven healthy children (mean age 7.9±3.3months, seven boys) were studied while they slept in the morning. They underwent a 3-h video-EEG-polysomnographic recording at the Pediatric Sleep Unit of Sant'Andrea Hospital in Rome, Italy. ⋯ Our results confirm the trend toward an increase in CAP rate during the first year of life. In addition, we observed a progressive increase in CAP rate with deepness of sleep, and with age, reflecting maturation of slow-wave activity. The decreased percentage of A1 subtypes may reflect the maturation of arousability.
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Brain & development · Mar 2011
Pharmacokinetics and clinical application of intravenous valproate in Thai epileptic children.
Roles of intravenous administration of valproate in status epilepticus and serial seizures are documented in adults and children. Pharmacokinetic parameters are necessary to predict the optimum therapeutic level after administration. A cross-sectional study to determine the pharmacokinetic parameters and safety of intravenous valproate for future application was conducted in Thai children from January to December 2008. ⋯ Seizure recurred in two children at 4th and 5th hour. Asymptomatic transient elevation of serum ammonia was observed in two children. Volume of distribution of 0.20 L/kg could be applied for initial intravenous administration with a favorable efficacy.