Ischemic cerebrovascular disease in childhood: cognitive assessment of 15 patients.

The aim of this study was to evaluate and to compare the cognitive function of children with ischemic cerebrovascular disease (ICVD). Fifteen children, 7 girls and 8 boys, aged 7.9 to 16.1 years, were evaluated by Piaget's clinical method. The control group was composed by fifteen children whose ages, sex and socioeconomic conditions were similar to those of the ICVD group. The cognitive function evaluation of the ICVD group showed that most of the children (10/15) were under their age group. The SPECT was performed on 14 children with ICVD and the results showed that 8/9 children with hypoperfusion presented a poor cognitive estimation and 3/5 children with normal perfusion demonstrated an adequate performance. We conclude that ICVD in childhood may compromise cognition; therefore, it needs a follow up of acquisitions during alI the stages of development.

Ischemic cerebrovascular disease in childhood: cognitive assessment of 15 patients

O objetivo deste estudo foi avaliar e comparar as funções cognitivas de crianças acometidas por doença cerebrovascular isquêmica (DCV-I). Quinze crianças com idade entre 7,9 e 16, 1 anos foram avaliadas pelo método clínico de Piaget, sendo 8 do sexo masculino. Outras 15 crianças, com idade, sexo e nível sócio-econômico similares aos do grupo propósito compuseram o grupo controle. A avaliação das funções cognitivas mostrou que a maioria das crianças (10/15) do grupo DCV-I apresentou defasagem para a faixa etária. Catorze crianças do Grupo DCV-I realizaram o SPECT. Comparando-se o resultado deste exame com a avaliação cognitiva, verificou-se que a maioria das crianças com hipoperfusão (8/9) apresentou déficit na avaliação cognitiva e 3/5 com perfusão normal tiveram desempenho adequado. Concluímos que a DCV-I na infância pode comprometer a cognição da criança, indicando a necessidade do acompanhamento evolutivo das aquisições em todas as etapas do desenvolvimento.

Fragile X syndrome: clinical, electroencephalographic and neuroimaging characteristics

We studied 11 patients (9 males) with cytogenetic diagnosis of fragile X syndrome (FXS) with the purpose of investigating the neural circuitry involved in this condition. The ages ranged from 8 to 19. All the individuals presented large ears, elongated faces and autistic features. Ten patients had severe mental retardation. Attention disorder was found in 10 individuals. Electroencephalographic recordings were abnormal in 6 of 10 patients examined, showing focal epileptiform discharges predominantly in frontal and parietal areas. All patients underwent magnetic resonance imaging studies which were abnormal in 8 of them. The most important abnormalities were reduction of the cerebellar vermis and enlargement of the IV ventricle. Single photon emission computerized tomography (SPECT) was performed in 7 patients and was abnormal in all of them, the most frequent finding being a hypoperfusion of the inferior portions of the frontal lobes. Based on the clinical picture, neuropsychological findings and functional and structural imaging studies we suggest that FXS presents with a dysfunction involving a large area of the central nervous system: cerebellum - basal frontal regions - parietal lobes. The literature points to a disturbance involving the same neural circuitry in patients with austin.