The current study examined how individual differences in children's temperament and motivation relate to attention skills in children from economically disadvantaged families. A total of 73 mother-child dyads participated in this study. Children were between the ages of 3 and 5, and all attended a Head Start program. Using multiple hierarchical regression analyses, the hypotheses that effortful control (an aspect of temperament) and motivation would individually and collectively relate to attention skills were supported. The current study supports the importance of understanding attention as a multifaceted concept (Posner & Petersen, 1990) that relates to cognitive, socioemotional, and motivational systems. The utility of applying a systems approach to school readiness issues in children of poverty is discussed.
ADCY5 mutations have been reported as a cause of early onset hyperkinetic movements associated with delayed motor milestones, hypotonia, and exacerbation during sleep. The movement disorder may be continuous or episodic, and can vary considerably in severity within families and in individuals. The authors report a case series of 3 patients with ADCY5 mutations treated with deep brain stimulation after unsuccessful medication trials. All had extensive imaging, metabolic, and genetic testing prior to confirmation of their ADCY5 mutation. Two of the patients had the c.1252C>T; p.R418W mutation, while the youngest and most severely affected had a de novo c.2080_2088del; p.K694_M696 mutation. All had variable and incomplete, but positive responses to deep brain stimulation. The authors conclude that deep brain stimulation may provide benefit in ADCY5-related movement disorders. Long-term efficacy remains to be confirmed by longitudinal observation. ADCY5 should be considered in the differential diagnosis of early onset hyperkinetic movement disorders, and may respond to deep brain stimulation.
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