Autoimmune myasthenia gravis, immunotherapy and thymectomy in children

Autoimmune myasthenia gravis is a rare condition in children. Identifying antibodies directed against the acetylcholine receptor is helpful in making the diagnosis. However, seronegative cases do exist and need to be distinguished from congenital forms of myasthenia. There is little published experience to inform the judicious management of autoimmune myasthenia gravis in children. In this article, we report our experience in the management of 12 cases of autoimmune myasthenia gravis in children in the modern era of medical immunotherapy and thymectomy.     

Liver X receptors regulate cholesterol homeostasis in oligodendrocytes

Cholesterol synthesis and transport in oligodendrocytes are essential for optimal myelination and remyelination in pathological conditions such as multiple sclerosis. However, little is known about cholesterol homeostasis in the myelin-forming oligodendrocytes. Liver X receptors (LXRs) are nuclear oxysterol receptors that regulate genes involved in cholesterol homeostasis and may therefore play an important role in de- and remyelination. We investigated whether LXRs regulate cholesterol homeostasis in oligodendrocytes. mRNA expression of genes encoding LXR-α and LXR-β and their target genes (ABCA1, ABCG1, ABCG4, apoE, and LDLR) was detected in oligodendrocytes derived from both neonatal and adult rats using quantitative real-time PCR. The expression of LXR-β and several target genes was increased during oligodendrocyte differentiation. We further demonstrated that treatment of primary neonatal rat oligodendrocytes with the synthetic LXR agonist T0901317 induced the expression of several established LXR target genes, including ABCA1, ABCG1, apoE, and LDLR. Treatment of oligodendrocytes with T0901317 resulted in an enhanced cholesterol efflux in the presence of apolipoprotein A-I or high-density lipoprotein particles. These data show that LXRs are involved in regulating cholesterol homeostasis in oligodendrocytes.     

The assessment of an inhibited, anxiety-prone temperament in a Dutch multi-ethnic population of preschool children

The Behavioral Inhibition Questionnaire-Short Form (BIQ-SF) is a 14-item parent-rating scale for assessing an inhibited, anxiety-prone temperament in preschool children. This study examined the psychometric properties of the BIQ-SF scores in a multi-ethnic community population of Dutch boys and girls aged 2.5–6?years (total N?=?2,343, from which various subsamples were derived). Results revealed that the factor structure of the BIQ-SF was as hypothesized: a model with six correlated factors representing children’s inhibited behaviors in various social and non-social contexts provided a good fit for the data. The internal consistency of the BIQ-SF was generally satisfactory and scores on the scale were found to be fairly stable over a time period of up to 2?years. Parent-teacher agreement was acceptable, and relations between the BIQ-SF and observations of an inhibited temperament were moderate. Finally, BIQ-SF scores were positively associated with measures of anxiety and internalizing symptoms, whereas no significant links were found with externalizing symptoms. Altogether, these results provide support for the reliability and validity of the BIQ-SF as an economical method for assessing behavioral inhibition and anxiety proneness in young children.     

Demyelinating prenatal and infantile developmental neuropathies

The prenatal and infantile neuropathies are an uncommon and complex group of conditions, most of which are genetic. Despite advances in diagnostic techniques, approximately half of children presenting in infancy remain without a specific diagnosis. This review focuses on inherited demyelinating neuropathies presenting in the first year of life. We clarify the nomenclature used in these disorders, review the clinical features of demyelinating forms of Charcot-Marie-Tooth disease with early onset, and discuss the demyelinating infantile neuropathies associated with central nervous system involvement. Useful clinical, neurophysiologic, and neuropathologic features in the diagnostic work-up of these conditions are also presented.     

Attention biases,anxiety, and development: toward or away from threats or rewards?

Research on attention provides a promising framework for studying anxiety pathophysiology and treatment. The study of attention biases appears particularly pertinent to developmental research, as attention affects learning and has down-stream effects on behavior. This review summarizes recent findings about attention orienting in anxiety, drawing on findings in recent developmental psychopathology and affective neuroscience research. These findings generate specific insights about both development and therapeutics. The review goes beyond a traditional focus on biased processing of threats and considers biased processing of rewards. Building on this work, we then turn to the treatment of pediatric anxiety, where manipulation of attention to threat and/or reward may serve a therapeutic role as a component of Attention Bias Modification Therapy.