Author Contributions S.J. and C.L.K. designed and coordinated analysis of single-cell data. S.J. and A.B.-C. performed the majority of the scRNA-seq analyses and visualizations. J.M. and G.B. contributed to the CNA analysis. Y.H. contributed to the algorithm for marker gene discovery. F.M.G.C., M.C., A.B.-C. and S.J. analyzed transcription factor activity in the scRNA-seq data. N.D.J., S.H. and S.J. contributed to the analysis of the bulk RNA-seq data and the data availability submission. M.V. contributed to timed mating and tissue isolation in developing mouse embryos. D.F., M.V. and L.K.D. and contributed to primary tissue isolation, preparation and production of scRNA-seq libraries. B.K. performed all experiments in cellular models. L.G., S.J., W.T.F. and K.K.M. contributed to literature review and cell cluster annotations. L.G. provided expert advice on identification of developing pre-cerebellar populations. M.K.M. and L.G.M. contributed to the clinical annotation of tumor samples. P.-E.L. and G.T. provided bulk adult human brain RNA-seq samples. M.R., B.P. and A.A. provided human fetal brain samples.
Thirty-eight patients underwent surgery for an intramedullary spinal cord ependymoma. In 37 patients, postoperative magnetic resonance imaging confirmed that the tumor was totally removed. The morbidity of surgery was directly related to the preoperative neurological condition. Patients who were normal or nearly normal preoperatively were rarely worse after surgery, and those who had significant disability preoperatively were at greatest risk of being more impaired after surgery. There has been no tumor recurrence in any patient after a mean follow-up period of 24 months, and radiation therapy has not been employed as a surgical adjunct.
Summary:Objective: Patients with intractable epilepsy due to extensive lesions involving the posterior quadrant (temporal, parietal, and occipital lobes) form a small subset of epilepsy surgery. This study was done with a view to analyze our experience with this group of patients and to define the changes in the surgical technique over the last 15 years. We also describe the microsurgical technique of the different surgical variants used, along with their functional neuroanatomy.Methods: In this series there were 13 patients with a median age of 17 years. All patients had extensive presurgical evaluation that provided concordant evidence localizing the lesion and seizure focus to the posterior quadrant. The objective of the surgery was to eliminate the effect of the epileptogenic tissue and preserve motor and sensory functions.Results: During the course of this study period of 15 years, the surgical procedure performed evolved toward incorporating more techniques of disconnection and minimizing resection. Three technical variants were thus utilized in this series, namely, (i) anatomical posterior quadrantectomy (APQ), (ii) functional posterior quadrantectomy (FPQ), and (iii) periinsular posterior quadrantectomy (PIPQ). After a median follow-up period of 6 years, 12/13 patients had Engel's Class I seizure outcome.Conclusion: The results of surgery for posterior quadrantic epilepsy have yielded excellent seizure outcomes in 92% of the patients in the series with no mortality or major morbidity. The incorporation of disconnective techniques in multilobar surgery has maintained the excellent results obtained earlier with resective surgery.
In this series, 25 adult patients with intramedullary astrocytomas were treated by radical excision alone. Six patients proved to have anaplastic astrocytoma; five of them died within approximately 2 years and the sixth has demonstrated disease progression. The other 19 patients were diagnosed as having low-grade astrocytoma (16 cases) or ganglioglioma (three cases); two of these had advanced preoperative neurological disability and died of medical complications. Fifteen of the remaining 17 patients have no clinical evidence of tumor recurrence after a mean follow-up period of 50.2 months; the other two have a small residual neoplasm that demonstrates no progression. Of these 17 patients, seven had previously received radiation therapy, but had clear evidence of tumor growth subsequently. This experience suggests that surgery is not beneficial for anaplastic spinal astrocytoma. However, in cases of low-grade tumor, radical excision is associated with minimal morbidity and an excellent long-term prognosis when carried out before significant disability occurs.
During the last decade, several authors have reported that certain brain-stem gliomas may be associated with a better prognosis than others. In this paper, retrospective correlations between the pathological findings and the magnetic resonance (MR) imaging appearance of 88 brain-stem gliomas are established. The authors propose an anatomical hypothesis that helps identify glioma growth patterns in general and that clarifies why cervicomedullary, dorsally exophytic, and focal tumors have a more favorable prognosis. According to this hypothesis, growth of benign gliomas of the brain stem is guided by secondary structures such as the pia, fiber tracts, and the ependyma, which in turn leads to stereotypical growth patterns that are clearly identified on MR images. The authors believe that this hypothesis, in conjunction with clinical data, may help establish selection criteria for the surgical treatment of specific brain-stem lesions.
Trametinib appears to be a suitable option for refractory pediatric low-grade glioma and warrants further investigations in case of progression.
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