MOSMotor optimality score MOL Motor Optimality List AIM To determine whether motor development at 3 months of age is associated with cognitive, motor, and behavioural outcomes in healthy children at early school age. METHODIn this cohort study, we included 74 term-born, healthy children (44 males, 30females; median gestational age 40.1wks, range 38.0-42.6wks). From video recordings (median 12.9wks, range 9.3-18.6wks), we assessed the quality of fidgety movements, and calculated a motor optimality score. At school age (median 5y 11mo, range 5y 8mo-7y 6mo), we performed detailed cognitive, motor, and behavioural assessments. We examined whether aspects of motor development were associated with functional outcomes.RESULTS An age-adequate motor repertoire, in particular the presence of antigravity, midline leg, and manipulation movements, was related to poorer cognition, whereas variable finger postures was related to better cognition. Children with a monotonous concurrent motor repertoire had better ball skills but experienced more behavioural problems. The presence of antigravity movements tended to be associated with abnormal recognition (odds ratio INTERPRETATION Detailed aspects of motor development at 3 months of age are associated with cognition and behaviour, but not with motor outcome, in healthy children at early school age. Our findings suggest that early motor development may be the basis for later cognitive and behavioural performance. Since the associations were only moderate, possible environmental influences should be acknowledged.[

Tyrosinemia type 1 (HT1) is an inborn error of tyrosine catabolism caused by fumarylacetoacetase deficiency. Biochemically, this results in accumulation of toxic metabolites including succinylacetone. Clinically, HT1 is characterized by severe liver, kidney, and neurological problems. Treatment with NTBC and dietary restriction of tyrosine and phenylalanine have strongly improved outcome, but impaired neurocognitive development has been reported. Whether impaired neurocognitive outcome results from high blood tyrosine or low blood phenylalanine concentrations is currently unknown. In this report, two HT1 newborns, diagnosed by neonatal screening, are presented. The first patient showed low phenylalanine concentrations, growth retardation, neurological impairments, and skin problems, clearly improving after institution of phenylalanine supplementation (~30 mg/kg/ day) at age 6 months, while both blood phenylalanine and tyrosine concentrations increased. In the second patient, phenylalanine supplementation (~20 mg/kg/day) was initiated as soon as low phenylalanine concentrations were observed at age 19 days. On this regimen, blood phenylalanine concentrations increased, and hypophenylalaninemia was less frequently observed than in the first patient, whereas blood tyrosine concentrations tended to increase. Clinically, no growth, neurological, or skin problems have been observed. The combination of knowledge obtained from these cases suggests that hypophenylalaninemia rather than hypertyrosinemia during the first months of life may impair neurocognitive development in young HT1 infants. Phenylalanine supplementation should really be considered in HT1 patients with consistently low blood phenylalanine concentrations during the first months of life. However, the minimal phenylalanine concentrations acceptable and the optimal phenylalanine supplementation regimen require further investigation.

ASQAges and Stage Questionnaire BOTMP Bruininks-Oseretsky Test of Motor Proficiency MABC Movement Assessment Battery for ChildrenFine motor skills are related to functioning in daily life and at school. We reviewed the status of knowledge, in preterm children, on the development of fine motor skills, the relation with gross motor skills, and risk factors for impaired fine motor skills. We searched the past 15 years in PubMed, using ['motor skills' or 'fine motor function' and 'preterm infant'] as the search string. Impaired gross and fine motor skills are among the most frequently occurring problems encountered by preterm children who do not develop cerebral palsy. The prevalence is around 40% for mild to moderate impairment and 20% for moderate impairment. Fine motor skill scores on the Movement Assessment Battery for Children are about 0.62 of a standard deviation lower compared with term children. Risk factors for fine motor impairments include moderately preterm birth (odds ratio [OR] 2.0) and, among very preterm children (<32wk gestation), intra-uterine growth restriction (ORs 2-3), inflammatory conditions (late-onset sepsis and necrotizing enterocolitis, ORs 3-5), and dexamethasone therapy for bronchopulmonary dysplasia (OR 2.7).A better understanding of factors that play a role in the development of and recovery from brain injury could guide future intervention attempts aimed at improving fine motor skills of preterm children.Survival rates of preterm infants have improved during recent decades, and this tendency is continuing. During the same period, the prevalence of major impairments has remained relatively constant or even decreased, 1 but the prevalence of milder dysfunctions is high, 2,3 with a tendency to increase. 4,5 This mainly appears to be the result of the increased survival of very preterm infants (gestational age <32wk), although more sensitive testing may also play a role. 5 Cognitive, behavioural, and mild motor problems without major motor deficits are by now the most dominant neurodevelopmental sequelae in children born preterm, with prevalences reported up to 50 to 70%.5 These 'high prevalence, low severity' impairments often do not occur in isolation and may considerably hamper the children's functional abilities in daily life. 6,7 Among the most frequently occurring problems encountered by preterm children who do not develop cerebral palsy (CP), are impaired gross and fine motor skills.2,4 Because particularly fine motor skills are related to adequate functioning in daily life and at school, our aim was to review the current status of knowledge, in preterm children, on the development of fine motor skills, the relation with gross motor skills, and risk factors for impaired fine motor skills. To this end, we searched PubMed for relevant articles.Impairments in fine motor skills may hamper various aspects of daily functioning such as getting dressed, lacing shoes, eating, and writing. In preterm children without CP, it has been shown that poor fine motor skills affect the learning of w...