Cystic spinal dysraphism of the cervical and upper thoracic region.

BACKGROUND: Cystic dysraphic lesions of the cervical and upper thoracic region are rare and only a few series have been published about the topic. These malformations can be divided into categories that include both myelocystoceles and the so-called cervical meningoceles or myelomeningoceles. METHODS: A retrospective study of 18 patients was conducted. RESULTS: In 17 patients a squamous or a cicatricial epithelium of variable thickness covered the dome of the lesions, while the base was covered with full-thickness skin. In one case the skin was entirely normal. Four patients displayed associated CNS malformations and three more had systemic congenital anomalies. All patients underwent surgical exploration and the length of time between birth and surgery ranged from 6 h to 9 months. The most frequent surgical finding, seen in 14 patients, was a stalk connecting the dorsal surface of the spinal cord to the cyst. In three patients the findings were consistent with myelocystocele. Only in one case was a true meningocele found. Hydrocephalus and Chiari II malformation were not as consistently associated as in myelomeningoceles. Neurological signs and symptoms were not so marked as in myelomeningoceles and were found in the follow-up of four patients. In two of them there was a non-progressive deficit, probably expressing an imperceptible involvement of the nervous system in the first year of life. The histopathological findings were of three types: neuroglial stalks, fibrovascular stalks and myelocystoceles. CONCLUSIONS: Cystic dysraphisms of the cervical and upper thoracic region differ clinically and structurally from meningomyelocele and have a more favorable outcome. We believe that these malformations have not been properly labeled and propose a classification based on the structures found inside the cyst.

Positive reaction for cysticercosis and multicentric anaplastic oligoastrocytoma.

INTRODUCTION: An unusual case of positive immunological testing for cysticercosis in the cystic fluid obtained from an anaplastic oligoastrocytoma is presented. CASE REPORT: A 15-year-old boy was admitted with multiple brain lesions. The biggest was a cyst with a mural node and neurocysticercosis was suspected. In order to relieve intracranial pressure, the cyst was punctured and the immunological testing for cysticercosis was positive, reinforcing the clinical suspicion and leading to a clinical trial with albendazole and steroids. As the patient deteriorated the cystic lesion was removed and the diagnosis of anaplastic oligoastrocytoma was established. A second lesion was eventually approached and the histopathological diagnosis of both specimens concurred. DISCUSSION: Although some authors believe that chronic inflammatory changes following neurocysticercosis could induce the formation of brain tumors, this association may be a mere coincidence. In our case no clinical evidence of a prior infestation by Cysticercus was found. In fact, an exhaustive examination of the specimens did not reveal any areas of inflammatory reaction. We believe that the similarity of the glioma and cysticercosis antigens may be the cause of the positive reactions in the cystic fluid.

Early infantile form of galactosialidosis in a female baby with a prenatal diagnosis of fetal ascites - First case in Brazil

Apresentamos o primeiro caso de galactosialidose do tipo infantil precoce identificado entre a populaçäo brasileira, uma grave e rara doença de depósito lisossomal, com apenas 12 casos claramente descritos mundialmente. Estudos clínicos, patológicos e bioquímicos realizados foram consistentes com os dados já publicados na literatura científica. Detectamos a doença em uma menina de 7 meses de idade, com diagnóstico de ascite no período pré-natal e evoluçäo compatível com doença de depósito, através da cromatografia em camada fina para oligossacarídeos, que é parte integrante do programa de triagem para erros inatos do metabolismo (EIM) em crianças de alto risco, realizado no Estado do Rio de Janeiro.

Tuberculose nao-reativa: um estudo clinico-patologico / Non-reactive tuberculosis: a clinicopathological study

Os autores relatam um caso de tuberculose nao-reativa diagnosticada por autopsia em um lactente de 11 meses. Esta forma da tuberculose e muito rara e usualmente esta associada com imunossupressao e anormalidades grosseiras de hematopoiese