RESUMO
BACKGROUND: The detection of prenatal ventriculomegaly raises anxiety about possible neurological sequelae. A few studies have investigated possible neurodevelopmental sequelae in the first years after birth but no systematic assessment has been performed at school age. AIMS: The aim of this study was to assess minor neurological signs, perceptual and visual function in a cohort of children with isolated mild antenatal ventricular dilatation examined at school age. STUDY DESIGN: Seventeen children with evidence of mild antenatal ventriculomegaly in the second and third trimester of pregnancy were included in the study. OUTCOME MEASURES: Children were assessed at school age (range 5 years 3 months-11 years, 11 months) using a structured neurological examination for minor neurological signs and age specific tests assessing perceptual motor abilities (Developmental Test of Visual-Motor Integration; Movement Assessment Battery for Children). RESULTS: Only one of the 17 children had abnormal results. The remaining 16 had normal results on all the tests, irrespective of the magnitude and the symmetry of the dilatation or of its evolution on neonatal scan. CONCLUSIONS: Our results suggest that children who had mild isolated prenatal ventricular dilatation are unlikely to develop even minor motor or perceptual difficulties at school age.
Assuntos
Ventrículos do Coração/anormalidades , Desempenho Psicomotor , Acuidade Visual , Criança , Cognição , HumanosAssuntos
Encefalomielite Aguda Disseminada/líquido cefalorraquidiano , Encefalomielite Aguda Disseminada/virologia , Enterovirus/isolamento & purificação , Encéfalo/patologia , Encéfalo/virologia , Pré-Escolar , Enterovirus/genética , Humanos , Imageamento por Ressonância Magnética/métodos , Masculino , Reação em Cadeia da PolimeraseRESUMO
The aim of this study was to evaluate tolerability and efficacy of phenylbutyrate (PB) in patients with spinal muscular atrophy (SMA). Ten patients with SMA type II confirmed by DNA studies (age range 2.6-12.7 years, mean age 6.01) were started on oral PB (triButyrate) in powder or tablets. The dosage was 500 mg/kg per day (maximum dose 19 g/d), divided in five doses (every 4 h, skipping one night-dose) using an intermittent schedule (7 days on and 7 days off). Measures of efficacy were the change in motor function from baseline to 3 and 9 weeks, by means of the Hammersmith functional motor scale. In children older than 5 years, muscle strength, assessed by myometry, and forced vital capacity were also measured. We found a significant increase in the scores of the Hammersmith functional scale between the baseline and both 3-weeks (P < 0.012) and 9-weeks assessments (P < 0.004). Our results indicate that PB might be beneficial to SMA patients without producing any major side effect. Larger prospective randomised, double-blind, placebo controlled trials are needed to confirm these preliminary findings.
