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.

-Pilocytic astrocytoma (PA) is a benign tumor that rarely spread along the neuraxis. At the moment there are no more than five cases of leptomeningeal dissemination (LD) from PA at diagnosis described in the literature. Different patterns of presentation or recurrence may be noted: local recurrence, malignant transformation, multicentric disease or metastatic disease. LD and multicentric disease can be distinct pathological entities. We report two cases and analyse literature, emphasizing leptomeningeal spread at presentation. Hydrocephalus, biopsy and parcial ressection are likely to be favorable factors to the occurrence of LD. Otherwise, LD may be part of natural history of PA, as evidenced by its ocurrence in non-treated cases.KEY WORDS: pilocytic astrocytoma, leptomeningeal dissemination, multicentric disease, cerebrospinal fluid, metastasis, radiation therapy, chemotherapy, hydrocephalus.Disseminação leptomeníngea de astrocitoma pilocítico ao diagnóstico: relato de dois casos. Disseminação leptomeníngea de astrocitoma pilocítico ao diagnóstico: relato de dois casos. Disseminação leptomeníngea de astrocitoma pilocítico ao diagnóstico: relato de dois casos. Disseminação leptomeníngea de astrocitoma pilocítico ao diagnóstico: relato de dois casos. Disseminação leptomeníngea de astrocitoma pilocítico ao diagnóstico: relato de dois casos.RESUMO -Astrocitoma pilocítico (AP) é tumor benigno que raramente se dissemina ao longo do neuroeixo. Até o momento não há mais que cinco casos de AP que se tenham apresentado com disseminação leptomeníngea (DL) descritos na literatura. Diferentes padrões de apresentação ou recorrência podem ser observados: recorrência local, transformação maligna, doença multicêntrica ou doença metastática. DL e doença multicêntrica podem ser entidades diferentes. Relatamos dois casos e analisamos a literatura. Hidrocefalia, biópsia e ressecção parcial são provavelmente fatores predisponentes à DL. Por outro lado, DL pode ser parte da história natural de AP, como pode ser evidenciado pela sua ocorrência em casos não tratados.

Background/Aims: To present a protocol of immediate surgical repair of myelomeningocele (MMC) after birth (‘time zero’) and compare this surgical outcome with the surgery performed after the newborn’s admission to the nursery before the operation. Methods: Data from the medical files of 31 patients with MMC that underwent surgery after birth and after admission at the nursery (group I) were compared with a group of 23 patients with MMC admitted and prospectively followed, who underwent surgery immediately after birth – ‘at time zero’ (group II). Results: The preoperative rupture of the MMC occurred more frequently in group I (67 vs. 39%, p < 0.05). The need for ventriculoperitoneal shunt was 84% in group I and 65% in group II and 4 of them were performed during the same anesthetic time as the immediate MMC repair, with no statistically significant difference. Group I had a higher incidence of small dehiscences when compared to group II (29 vs. 13%, p < 0.05); however, there was no statistically significant difference regarding infections. After 1 year of follow-up, 61% of group I showed neurodevelopmental delay, whereas only 35% of group II showed it. Conclusions: The surgical intervention carried out immediately after the birth showed benefits regarding a lower incidence of preoperative rupture of the MMC, postoperative dehiscences and lower incidence of neurodevelopmental delay 1 year after birth.

-Objective: To evaluate the diagnostic usefulness of cerebral spinal fluid (CSF) cellularity, protein, neutrophils, glucose and lactate for detection of postoperative bacterial meningitis. Method: This p rospective study was conducted in 28 postoperative neuro s u rgical patients from 2002 to 2005 at University of São Paulo. The CSF markers were plotted in a receiver operating characteristic (ROC) curve to evaluate their accuracy. Results: Based on the area under ROC curve CSF glucose, cellularity, and lactate were cons i d e red good tests. Polymorphonuclear and protein did not achieve enough accuracy to be used clinicall y. Conclusion: The CSF glucose, lactate, and cellularity can be used for the diagnosis of bacterial meningitis. Moreover, it can be helpful to differentiate bacterial from aseptic meningitis.KEY WORDS: CSF, bacterial meningitis, neurosurgery, ROC analysis, postoperative infection. Baseadas na área sob a curva ROC, glicorraquia, celularidade e concentração de lactato foram considerados bons testes. A contagem de polimorf o n u c l e a res e a pro t e í n o rraquia não atingiram acurácia suficiente para serem utilizadas clinicamente. Conclusão: A glicorraquia, a concentração de lactato e a celularidade podem ser utilizadas clinicamente para o diagnóstico da meningite bacteriana. Esses marc a d o res também podem ser úteis na diferenciação entre meningite bacteriana e asséptica. PALAVRAS-CHAVE: meningite bacteriana, LCR, neurocirurgia, curva ROC, infecção pós-operatória.

Gain-of-function mutations in FGFR2 cause Apert syndrome (AS), a disease characterized by craniosynostosis and limb bone defects both due to abnormalities in bone differentiation and remodeling. Although the periosteum is an important cell source for bone remodeling, its role in craniosynostosis remains poorly characterized. We hypothesized that periosteal mesenchymal stem cells (MSCs) and fibroblasts from AS patients have abnormal cell phenotypes that contribute to the recurrent fusion of the coronal sutures. MSCs and fibroblasts were obtained from the periostea of 3 AS patients (S252W) and 3 control individuals (WT). We evaluated the proliferation, migration, and osteogenic differentiation of these cells. Interestingly, S252W mutation had opposite effects on different cell types: S252W MSCs proliferated less than WT MSCs, while S252W fibroblasts proliferated more than WT fibroblasts. Under restrictive media conditions, only S252W fibroblasts showed enhanced migration. The presence of S252W mutation increased in vitro and in vivo osteogenic differentiation in both studied cell types, though the difference compared to WT cells was more pronounced in S252W fibroblasts. This osteogenic differentiation was reversed through inhibition of JNK. We demonstrated that S252W fibroblasts can induce osteogenic differentiation in periosteal MSCs but not in MSCs from another tissue. MSCs and fibroblasts responded differently to the pathogenic effects of the FGFR2S252W mutation. We propose that cells from the periosteum have a more important role in the premature fusion of cranial sutures than previously thought and that molecules in JNK pathway are strong candidates for the treatment of AS patients.Electronic supplementary materialThe online version of this article (doi:10.1007/s12015-011-9327-6) contains supplementary material, which is available to authorized users.

Humans with CC are incidental intermediate hosts, which replace the pig in the life cycle of the T. solium. Children are more frequently affected by parenchyma infestation of cysticercus, of which the main clinical manifestation is epilepsy. Hydrocephalus is more common in adults and is caused by cerebrospinal fluid blockage by ventricular cysts and inflammatory reactions (ependimitis/arachnoiditis). Treatment should be individualized based on clinical presentation, degree of infestation, location and viability of cysticercus, and host response. Hydrocephalus can be controlled only by removal of obstructive intraventricular cysts or associated with either ventriculoperitoneal shunt or endoscopic third ventriculostomy. The degree of infestation and complications related to the shunt represents the most important prognostic factors in the outcome of NCC.

Birth-related traumatic brain injury may cause serious complications in newborn infants. Its successful management includes special training, teamwork, and an individual approach.

OBJECTIVES:We investigated four components of the Wnt signaling pathway in medulloblastomas. Medulloblastoma is the most common type of malignant pediatric brain tumor, and the Wnt signaling pathway has been shown to be activated in this type of tumor.METHODS:Sixty-one medulloblastoma cases were analyzed for β-catenin gene (CTNNB1) mutations, β-catenin protein expression via immunostaining and Wnt signaling pathway-related gene expression. All data were correlated with histological subtypes and patient clinical information.RESULTS:CTNNB1 sequencing analysis revealed that 11 out of 61 medulloblastomas harbored missense mutations in residues 32, 33, 34 and 37, which are located in exon 3. These mutations alter the glycogen synthase kinase-3β phosphorylation sites, which participate in β-catenin degradation. No significant differences were observed between mutation status and histological medulloblastoma type, patient age and overall or progression-free survival times. Nuclear β-catenin accumulation, which was observed in 27.9% of the cases, was not associated with the histological type, CTNNB1 mutation status or tumor cell dissemination. The relative expression levels of genes that code for proteins involved in the Wnt signaling pathway (CTNNB1, APC, AXIN1 and WNT1) were also analyzed, but no significant correlations were found. In addition, large-cell variant medulloblastomas presented lower relative CTNNB1 expression as compared to the other tumor variants.CONCLUSIONS:A small subset of medulloblastomas carry CTNNB1 mutations with consequent nuclear accumulation of β-catenin. The Wnt signaling pathway plays a role in classic, desmoplastic and extensive nodularity medulloblastoma variants but not in large-cell medulloblastomas.