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.