RESUMO
Angiomas cavernosos ocupam lugar de destaque dentre as manifestaçöes vasculares do sistema nervoso. Apresenta-se o caso de um paciente adulto com quadro neurológico iniciado subitamente (sindrome de tronco encefálico), após procedimento cirúrgico abdominal. Evoluiu favoravelmente com melhora espontânea, permanecendo com sequelas distúrbios da sensibilidade superficial da hemiface direita e membro inferior esquerdo e amiotrofia segmentar. Os exames de neuroimagem revelaram malformaçäo vascular (angioma cavernoso) do tronco encefálico (ponte). Os autores discutem a importância dos métodos de neuroimagem para determinaçäo diagnóstica e, principalmente, debatem aspectos da terapêutica conservadora e indicaçöes cirúrgicas
Assuntos
Humanos , Masculino , Pessoa de Meia-Idade , Hemangioma Cavernoso , Ponte/anormalidades , Espectroscopia de Ressonância Magnética , Tomografia Computadorizada por Raios XRESUMO
We report a neonate who presented with marked hypotonia and absent suck reflex. MR demonstrated complete absence of the pons as well as absence of a basilar artery flow void. Our case exhibits features similar to those described in previous reports of pontoneocerebellar hypoplasia, but with a more severe degree of pontine involvement. The associated vascular findings suggest a vascular insult to the brain stem as the cause.
Assuntos
Tronco Encefálico/anormalidades , Cerebelo/anormalidades , Ponte/anormalidades , Artéria Basilar/patologia , Artéria Basilar/fisiopatologia , Tronco Encefálico/irrigação sanguínea , Tronco Encefálico/patologia , Cerebelo/patologia , Circulação Cerebrovascular/fisiologia , Evolução Fatal , Feminino , Humanos , Recém-Nascido , Imageamento por Ressonância Magnética , Hipotonia Muscular/fisiopatologia , Ponte/patologia , Comportamento de Sucção/fisiologia , Artéria Vertebral/patologia , Artéria Vertebral/fisiopatologiaRESUMO
A case of pontoneocerebellar hypoplasia (PNCH) in a 9-day-old child--the youngest in the literature--is described. Right after birth the child showed serious respiratory insufficiency and developed frequent episodes of apnea and cardiorespiratory arrests, dying on the 9th day. Postmortem examination showed significant alterations only in the brain. There was intense neuron reduction in the pontine, arcuate, inferior olivary and dentate nuclei, reduction of Purkinje cells and of the granular layer of the cerebellum and the disappearance of the pontocerebellar fibers, together with dense fibrillary astrocytosis, which seems to have first appeared in the pontine nuclei. This case is compared with others in the literature and attention is drawn to the more intense, and probably initial, involvement of the pontine nuclei. The time of appearance of pontoneocerebellar hypoplasia is commented upon, based on data concerning the ontogenesis of the structure and on the presence or absence of gliosis in the pontine nuclei and persistence of the fetal pattern in the inferior olivary and dentate nuclei. It is suggested that the process may start in the 2nd or 3rd month of fetal life, or even later, as probably occurred in the present case, during the second or third trimester of intrauterine life.