Adult-onset Alexander disease with brainstem and cervical cord enhancing lesions.

Leukodystrophies are a group of genetic diseases with diverse clinical features and prominent involvement of the central nervous system white matter. We describe a 27-year-old man who presented with a progressive neurological disease, and striking involvement of the brainstem and symmetrical white matter lesions on MR scanning. Having excluded several other causes of leukodystrophy, we confirmed Alexander disease when a genetic panel showed a probable pathogenic variant in GFAP: p.Leu359Pro. Clinicians should suspect Alexander disease in people with a progressive neurological motor decline who has pyramidal and bulbar signs and compatible neuroimaging.

Complementary feeding patterns in the first year of life in the city of Rio de Janeiro, Brazil: time trends from 1998 to 2008.

This study aimed to analyze time trends in complementary feeding practices among children under one year of age in the city of Rio de Janeiro, Brazil, from 1998 to 2008. Based on comparable questions on food consumption in five surveys conducted in representative randomized samples of children in 1998 (n=3,762), 2000 (n=3,670), 2003 (n=4,305), 2006 (n=3,686), and 2008 (n=2,621), 16 complementary feeding indicators were constructed. The percentage frequency of all indicators was estimated for each year of the study. Linear trend analyses were also conducted by means of multivariate regression models. All indicators of early complementary feeding decreased from 1998 to 2008. In the 6-11.9 month age group, there was a significant downward trend in the proportion of children receiving food with adequate consistency for their age, consumption of iron-rich foods, and overall adequacy of the diet. A significant upward trend in fruit consumption was observed. There was a decrease in the early complementary feeding, and generally a worsening of complementary feeding practices in children aged 6-11.9 months in Rio de Janeiro during the period studied.

Complementary feeding patterns in the first year of life in the city of Rio de Janeiro, Brazil: time trends from 1998 to 2008 / Padrões de alimentação complementar no primeiro ano de vida no Município do Rio de Janeiro, Brasil: tendência temporal (1998-2008) / Patrones de alimentación complementaria durante el primer año de vida en la ciudad de Río de Janeiro, Brasil: tendencias temporales (1998-2008)

This study aimed to analyze time trends in complementary feeding practices among children under one year of age in the city of Rio de Janeiro, Brazil, from 1998 to 2008. Based on comparable questions on food consumption in five surveys conducted in representative randomized samples of children in 1998 (n = 3,762), 2000 (n = 3,670), 2003 (n = 4,305), 2006 (n = 3,686), and 2008 (n = 2,621), 16 complementary feeding indicators were constructed. The percentage frequency of all indicators was estimated for each year of the study. Linear trend analyses were also conducted by means of multivariate regression models. All indicators of early complementary feeding decreased from 1998 to 2008. In the 6-11.9 month age group, there was a significant downward trend in the proportion of children receiving food with adequate consistency for their age, consumption of iron-rich foods, and overall adequacy of the diet. A significant upward trend in fruit consumption was observed. There was a decrease in the early complementary feeding, and generally a worsening of complementary feeding practices in children aged 6-11.9 months in Rio de Janeiro during the period studied.

Este estudo objetivou analisar as tendências temporais das práticas de alimentação complementar entre crianças < 1 ano de idade na cidade do Rio de Janeiro, Brasil, de 1998 a 2008. Com base em questões comparáveis sobre o consumo de alimentos de cinco inquéritos realizados em amostras probabilísticas de crianças em 1998 (n = 3.762), 2000 (n = 3.670), 2003 (n = 4.305), 2006 (n = 3.686) e 2008 (n = 2.621), foram construídos 16 indicadores de alimentação complementar. A frequência percentual dos indicadores foi estimada para cada ano. Análises de tendências lineares foram realizadas por meio de modelos de regressão multivariada. Os indicadores de alimentação complementar precoce diminuíram de 1998 a 2008. No grupo de 6 a 11,9 meses de idade, houve tendência significativa do aumento no consumo de frutas e de queda na proporção de crianças que recebiam preparações com consistência adequada para sua idade, que consumiam alimentos ricos em ferro e que recebiam alimentação adequada. Durante o período estudado, houve queda da alimentação complementar precoce e, em geral, pioraram as práticas de alimentação complementar em crianças de 6 a 11,9 meses no Rio de Janeiro.

Se analizaron las tendencias temporales de las prácticas de alimentación complementaria en niños < 1 año de edad en la ciudad de Río de Janeiro, Brasil, de 1998 a 2008. En base a preguntas comparables acerca del consumo de alimentos en cinco encuestas, basadas en muestras aleatorias de niños en 1998 (n = 3.762), 2000 (n = 3.670), 2003 (n = 4.305), 2006 (n = 3.686) y 2008 (n = 2.621), se construyeron 16 indicadores de alimentación complementaria. Se calculó para cada año la frecuencia porcentual de los indicadores. Los análisis de tendencia lineal se realizaron por medio de modelos de regresión multivariable. Los indicadores de alimentación complementaria precoz disminuyeron. En niños con 6 a 11,9 meses de edad, hubo una tendencia significativa de aumento del consumo de frutas y una caída en la proporción de los que recibieron papillas con una consistencia adecuada para su edad, que consumieron alimentos ricos en hierro y que recibieron una alimentación adecuada. Durante el período de estudio, hubo una disminución de la alimentación complementaria precoz y, en general, un deterioro de las prácticas de alimentación complementaria en niños de 6 a 11,9 meses de edad en Río de Janeiro.