Abstract Objectives To develop a checklist describing features of normal and abnormal general movements in order to guide General Movement Assessment novices through the assessment procedure, to provide a quantification of General Movement Assessment; and to demonstrate that normal and abnormal GMs can be distinguished on the basis of a metric checklist score. Methods Three examiners used General Movement Assessment and the newly developed GM checklist to assess 20 videos of 16 infants (seven males) recorded at 31-45 weeks postmenstrual age (writhing general movements). Inter- and intra-scorer agreement was determined for General Movement Assessment (nominal data; Kappa values) and the checklist score (metric scale ranging from 0 to 26; Intraclass Correlation values). The scorers’ satisfaction with the usefulness of the checklist was assessed by means of a short questionnaire (score 10 for maximum satisfaction). Results The scorers’ satisfaction ranged from 8.44 to 9.14, which indicates high satisfaction. The median checklist score of the nine videos showing normal general movements was significantly higher than that of the eleven videos showing abnormal general movements (26 vs. 11, p < 0.001). The checklist score also differentiated between poor-repertoire (median = 13) and cramped-synchronized general movements (median = 7; p = 0.002). Inter- and intra-scorer agreement on (i) normal vs. abnormal general movements was good to excellent (Kappa = 0.68-1.00); (ii) the distinction between the four general movement categories was considerable to excellent (Kappa = 0.56-0.93); (iii) the checklist was good to excellent (ICC = 0.77-0.96). Conclusion The general movement checklist proved an important tool for the evaluation of normal and abnormal general movements; its score may potentially document individual trajectories and the effect of therapeutic intervention.

ABSTRACT We report cognitive, language and motor neurodevelopment, assessed by the Bayley-III test, in 31 non-microcephalic children at age 3 with PCR-confirmed maternal Zika virus exposure (Rio de Janeiro, 2015–2016). Most children had average neurodevelopmental scores, however, 8 children (26%) presented delay in some domain. Language was the most affected: 7 children (22.6%) had a delay in this domain (2 presenting severe delay). Moderate delay was detected in the cognitive (3.2%) and motor (10%) domains. Maternal illness in the third trimester of pregnancy and later gestational age at birth were associated with higher Bayley-III scores. Zika-exposed children require long-term follow-up until school age.

ABSTRACT We report cognitive, language and motor neurodevelopment, assessed by the Bayley-III test, in 31 non-microcephalic children at age 3 with PCR-confirmed maternal Zika virus exposure (Rio de Janeiro, 2015–2016). Most children had average neurodevelopmental scores, however, 8 children (26%) presented delay in some domain. Language was the most affected: 7 children (22.6%) had a delay in this domain (2 presenting severe delay). Moderate delay was detected in the cognitive (3.2%) and motor (10%) domains. Maternal illness in the third trimester of pregnancy and later gestational age at birth were associated with higher Bayley-III scores. Zika-exposed children require long-term follow-up until school age.

RESUMO Função motora, cognição, independência funcional e qualidade de vida foram descritos em pacientes com mielomeningocele (MMC), mas sua correlação não foi investigada. Esse estudo investigou correlações entre função motora, cognição, independência funcional, qualidade de vida, idade, nível de lesão em pacientes com MMC e a influência da hidrocefalia nessas variáveis. Quarenta e sete pacientes foram avaliados com a Medida de Função Motora Grossa, Matrizes Progressivas de Raven, Inventário de Avaliação Pediátrica de Incapacidade e Autoquestionnaire Qualité de vie Enfant Imagé. Testes de Spearman investigaram as correlações. ANOVAS de Friedman investigaram a influência da hidrocefalia. A função motora correlacionou-se fortemente com mobilidade e nível de lesão e moderadamente com cognição, auto-cuidado e função social. Cognição e qualidade de vida correlacionaram-se moderadamante com independência funcional. Idade correlacionou moderadamente com independência funcional e qualidade de vida. Hidrocefalia associou-se a piores desfechos motores/cognitivos e menor independência funcional.

ABSTRACT Motor function, cognition, functional independence and quality of life have been described in myelomeningocele patients, but no study has investigated their relationships. We aimed to investigate the relationships between motor function, cognition, functional independence, quality of life, age, and lesion level in myelomeningocele patients, and investigate the influence of hydrocephalus on these variables. We assessed 47 patients with the Gross Motor Function Measure (motor function), Raven’s Colored Progressive Matrices (cognition), Pediatric Evaluation of Disability Inventory (functional independence) and the Autoquestionnaire Qualité de vie Enfant Imagé (quality of life). Spearman’s correlation tests determined relationships between the variables. The Friedman ANOVAs determined the influence of hydrocephalus. Motor function was strongly related to mobility and lesion level, and moderately related to cognition, self-care and social function. Cognition and quality of life were moderately related to functional independence. Age correlated moderately with functional independence and quality of life. Hydrocephalus resulted in poorer motor/cognitive outcomes and lower functional independence.

ObjetivoA relação entre qualidade de vida e função motora de pacientes com distrofia muscular de Duchenne (DMD) e sobrecarga e qualidade de vida (QV) dos cuidadores não está clara na literatura. Esse estudo investigou possíveis relações entre dependência funcional/QV de pacientes e sobrecarga/QV dos cuidadores em 35 meninos (6-17 anos) com DMD e respectivos cuidadores (acima de 21 anos).MétodoCada cuidador respondeu ao questionário de QV da Organização Mundial de Saúde e de sobrecarga de Zarit e o paciente foi avaliado com a medida de função motora e o Autoquestionnaire Qualité de vie Enfant Imagé. Correlações de Spearman e regressões lineares investigaram possíveis relações entre as variáveis.ResultadosA ocorrência de menor QV e maior sobrecarga entre cuidadores foi evidenciada, enquanto a dependência funcional dos pacientes não foi considerada fator determinante.ConclusãoCuidadores com maior sobrecarga apresentaram menor QV relacionada à maior idade dos pacientes.

ObjectiveThe relationship between functional dependence and quality of life (QOL) in Duchenne muscular dystrophy (DMD) patients and burden and QOL in caregivers is not clear. This study investigated possible relationships between functional dependence/QOL of DMD patients and QOL/burden of caregivers.MethodThis study included 35 boys (6-17 years) and respective caregivers (above 21 years). Caregivers answered to World Health Organization Quality of Life and Zarit Burden Interview questionnaires. Patients were assessed with the Motor Function Measure and the Autoquestionnaire Qualité de vie Enfant Imagé. Spearman correlations and linear regressions were run to investigate relationships between the variables.ResultsThe occurrence of lower QOL and higher burden among the caregivers of patients with Duchenne muscular dystrophy was evidenced. The functional dependence of patients was not considered a determinant factor. Higher caregivers’ burden was related to lower caregivers’ QOL and to higher patients’ ages.