English (United Kingdom)

https://curated-unify.zendy.io/wp-json/zendy-region/v1/featured_content/oa?rat=en

https://curated-unify.zendy.io/wp-json/zendy-region/v1/highlighted_journal/

Global: Zendy Plus annual

Presents the access of premium content as premium feature

Premium Content

Presents the keyphrase highlighting as premium feature

Keyphrase Highlighting

Presents the summarisation as premium feature

Summarisation

Insights

Presents the pdf analysis as premium feature

PDF Analysis

Presents the zaia usage as premium feature

ZAIA

Global: Zendy Tools annual

Global: Zendy Free Plan

Global: Zendy Tools monthly

Global: Zendy Plus monthly

OBJETIVO: Correlacionar o quadro clínico de um grupo de crianças com diagnóstico tomográfico de esquizencefalia com a extensão e localização das fendas. MATERIAIS E MÉTODOS: Estudo retrospectivo de prontuários do arquivo dos serviços de Neurologia e Genética Médica do Instituto Fernandes Figueira e Hospital Municipal Jesus, Rio de Janeiro, RJ, Brasil, no período de 2000 a 2003. Foram incluídos 16 pacientes, nove do sexo feminino e sete do sexo masculino, com diagnóstico tomográfico de esquizencefalia e analisados quanto a aspectos da tomografia computadorizada, desenvolvimento neuropsicomotor, déficit motor e cognitivo e epilepsia. RESULTADOS: Predominaram as fendas bilaterais em 10:16 pacientes, lábios abertos em 23:27 fendas e pequenas em 11:27 fendas. Das anomalias associadas à esquizencefalia, a ausência de septo pelúcido foi a mais freqüente (10:16 pacientes). Dos aspectos clínicos, 15 pacientes apresentaram atraso do desenvolvimento e déficit motor; seis apresentaram déficit cognitivo e dez apresentaram epilepsia. Em três pacientes observamos discordância entre o quadro clínico e o tamanho das fendas: embora as fendas fossem pequenas, o quadro clínico foi intenso, em virtude de presença de outras anomalias cerebrais. CONCLUSÃO: O quadro clínico guarda relação com o tamanho das fendas, independentemente da lateralidade, sendo mais intenso quando há associação com outras anomalias cerebrais. OBJECTIVE: To establish a correlation between clinical features in a group of children with tomographic diagnosis of schizencephaly and clefts extent and localization. MATERIALS AND METHODS: Retrospective study of dossiers from the archives of Neurology and Medical Genetics Services at Instituto Fernandes Figueira/Fiocruz and Hospital Municipal Jesus, Rio de Janeiro, RJ, Brazil, in the period between 2000 and 2003. The study included 16 patients, nine female and seven male, with tomographic diagnosis of schizencephaly investigated for clinical findings, psychomotor development, motor/cognitive deficits and epilepsy. RESULTS: Predominance of bilateral clefts in 10:16 patients, open-lip schizencephaly type in 23:27 patients, and small lips in 11:27 patients. As regards anomalies associated with schizencephaly, pellucid septum absence was the most frequent one (10:16 patients). As regards clinical findings, 15 patients presented with developmental delay and motor deficit, six patients with cognitive deficit and ten with epilepsy. In three patients, we observed discordant clinical findings and cleft sizes, although the clefts were small, the clinical features severity was high because of other cerebral anomalies. CONCLUSION: The clinical features of schizencephaly are related to the size of the clefts, regardless laterality, presenting higher severity when associated with other cerebral anomalies

Aspectos clínicos em 16 pacientes com diagnóstico tomográfico de esquizencefalia