A novel technique to treat acquired Chiari I malformation after supratentorial shunting

PURPOSE: The acquired Chiari I malformation with abnormal cranial vault thickening is a rare late complication of supratentorial shunting. It poses a difficult clinical problem, and there is debate about the optimal surgical strategy. Some authors advocate supratentorial skull enlarging procedures while others prefer a normal Chiari decompression consisting of a suboccipital craniectomy, with or without C1 laminectomy and dural patch grafting. METHODS: We illustrate three cases of symptomatic acquired Chiari I malformation due to inward cranial vault thickening. RESULTS: We describe a new surgical approach that appears to be effective in these patients. This approach includes the standard Chiari decompression combined with posterior fossa augmentation by thinning the occipital planum. CONCLUSION: Internal volume re-expansion of the posterior fossa by thinning the occipital planum appears to be an effective novel surgical strategy in conjunction with the standard surgical therapy of Chiari decompression

Surgical intervention for hydrocephalus in infancy; etiology, age and treatment data in a Dutch cohort

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Insights from the supplementary motor area syndrome in balancing movement initiation and inhibition

The supplementary motor area syndrome is a characteristic neurosurgical syndrome that can occur after unilateral resection of the supplementary motor area. Clinical symptoms may vary from none to a global akinesia, predominantly on the contralateral side, with preserved muscle strength, and mutism. A remarkable feature is that these symptoms completely resolve within weeks to months, leaving only a disturbance in alternating bimanual movements. In this review we give an overview of the old and new insights from the supplementary motor area syndrome and extrapolate these findings to seemingly unrelated diseases and symptoms such as Parkinson’s disease and tics. Furthermore, we integrate findings from lesion, stimulation and functional imaging studies to provide insight in the motor function of the supplementary motor area

Enhancement of drug oxidation and conjugation by carcinogens in different rat tissues

OBJECTIVE After endoscopic third ventriculostomy (ETV), some patients develop recurrent symptoms of hydrocephalus. The optimal treatment for these patients is not clear: repeat ETV (re-ETV) or CSF shunting. The goals of the study were to assess the effectiveness of re-ETV relative to initial ETV in pediatric patients and validate the ETV success score (ETVSS) for re-ETV. METHODS Retrospective data of 624 ETV and 93 re-ETV procedures were collected from 6 neurosurgical centers in the Netherlands (1998-2015). Multivariable Cox proportional hazards modeling was used to provide an adjusted estimate of the hazard ratio for re-ETV failure relative to ETV failure. The correlation coefficient between ETVSS and the chance of re-ETV success was calculated using Kendall's tau coefficient. Model discrimination was quantified using the c-statistic. The effects of intraoperative findings and management on re-ETV success were also analyzed. RESULTS The hazard ratio for re-ETV failure relative to ETV failure was 1.23 (95% CI 0.90-1.69; p = 0.20). At 6 months, the success rates for both ETV and re-ETV were 68%. ETVSS was significantly related to the chances of re-ETV success (tau = 0.37; 95% bias corrected and accelerated CI 0.21-0.52; p < 0.001). The c-statistic was 0.74 (95% CI 0.64-0.85). The presence of prepontine arachnoid membranes and use of an external ventricular drain (EVD) were negatively associated with treatment success, with ORs of 4.0 (95% CI 1.5-10.5) and 9.7 (95% CI 3.4-27.8), respectively. CONCLUSIONS Re-ETV seems to be as safe and effective as initial ETV. ETVSS adequately predicts the chance of successful re-ETV. The presence of prepontine arachnoid membranes and the use of EVD negatively influence the chance of success

Resting energy expenditure in children at risk of hypothalamic dysfunction

Objective: Children with suprasellar brain damage are at risk of hypothalamic dysfunction (HD). HD may lead to decreased resting energy expenditure (REE). Decreased REE, however, is not present in all children with HD. Our aim was to assess which children suspect for HD have low REE, and its association with clinical severity of HD or radiological hypothalamic damage. Patients and methods: A retrospective cohort study was performed. Measured REE (mREE) of children at risk of HD was compared to predicted REE (pREE). Low REE was defined as mREE <90% of predicted. The mREE/pREE quotient was associated to a clinical score for HD symptoms and to radiological hypothalamic damage. Results: In total, 67 children at risk of HD (96% brain tumor diagnosis) with a mean BMI SDS of +2.3 ± 1.0 were included. Of these, 45 (67.2%) had low mREE. Children with severe HD had a significant lower mean mREE/pREE quotient compared to children with no, mild, or moderate HD. Mean mREE/pREE quotient of children with posterior hypothalamic damage was significantly lower compared to children with no or anterior damage. Tumor progression or tumor recurrence, severe clinical HD, and panhypopituitarism with diabetes insipidus (DI) were significant risk factors for reduced REE. Conclusion: REE may be lowered in children with hypothalamic damage and is associated to the degree of clinical HD. REE is, however, not lowered in all children suspect for HD. For children with mild or moderate clinical HD symptoms, REE measurements may be useful to distinguish between those who may benefit from obesity treatment that increases REE from those who would be better helped using other obesity interventions

Chromosomal Instability Characterizes Pediatric Medulloblastoma but Is Not Tolerated in the Developing Cerebellum

Medulloblastoma is a pediatric brain malignancy that consists of four transcriptional subgroups. Structural and numerical aneuploidy are common in all subgroups, although they are particularly profound in Group 3 and Group 4 medulloblastoma and in a subtype of SHH medulloblastoma termed SHH alpha. This suggests that chromosomal instability (CIN), the process leading to aneuploidy, is an important player in medulloblastoma pathophysiology. However, it is not known if there is ongoing CIN in medulloblastoma or if CIN affects the developing cerebellum and promotes tumor formation. To investigate this, we performed karyotyping of single medulloblastoma cells and demonstrated the presence of distinct tumor cell clones harboring unique copy number alterations, which is suggestive of ongoing CIN. We also found enrichment for processes related to DNA replication, repair, and mitosis in both SHH medulloblastoma and in the highly proliferative compartment of the presumed tumor cell lineage-of-origin, the latter also being sensitive to genotoxic stress. However, when challenging these tumor cells-of-origin with genetic lesions inducing CIN using transgenic mouse modeling, we found no evidence for large chromosomal aberrations in the cerebellum or for medulloblastoma formation. We therefore conclude that without a background of specific genetic mutations, CIN is not tolerated in the developing cerebellum in vivo and, thus, by itself is not sufficient to initiate medulloblastoma

Comparing the influence of mixed reality, a 3D viewer, and MRI on the spatial understanding of brain tumours

Background: Multiple 3D visualization techniques are available that obviates the need for the surgeon to mentally transform the 2D planes from MRI to the 3D anatomy of the patient. We assessed the spatial understanding of a brain tumour when visualized with MRI, 3D models on a monitor or 3D models in mixed reality.Methods: Medical students, neurosurgical residents and neurosurgeons were divided into three groups based on the imaging modality used for preparation: MRI, 3D viewer and mixed reality. After preparation, the participants needed to position, scale, and rotate a virtual tumour inside a virtual head of the patient in the same orientation as the original tumour would be. Primary outcome was the amount of overlap between the placed tumour and the original tumour to evaluate accuracy. Secondary outcomes were the position, volume and rotation deviation compared to the original tumour.Results: A total of 12 medical students, 12 neurosurgical residents, and 12 neurosurgeons were included. For medical students, the mean amount of overlap for the MRI, 3D viewer and mixed reality group was 0.26 (0.22), 0.38 (0.20) and 0.48 (0.20) respectively. For residents 0.45 (0.23), 0.45 (0.19) and 0.68 (0.11) and for neurosurgeons 0.39 (0.20), 0.50 (0.27) and 0.67 (0.14). The amount of overlap for mixed reality was significantly higher on all expertise levels compared to MRI and on resident and neurosurgeon level also compared to the 3D viewer. Furthermore, mixed reality showed the lowest deviations in position, volume and rotation on all expertise levels.Conclusion: Mixed reality enhances the spatial understanding of brain tumours compared to MRI and 3D models on a monitor. The preoperative use of mixed reality may therefore support the surgeon to improve spatial 3D related surgical tasks such as patient positioning and planning surgical trajectories

Advanced intraoperative MRI in pediatric brain tumor surgery

Introduction: In the pediatric brain tumor surgery setting, intraoperative MRI (ioMRI) provides “real-time” imaging, allowing for evaluation of the extent of resection and detection of complications. The use of advanced MRI sequences could potentially provide additional physiological information that may aid in the preservation of healthy brain regions. This review aims to determine the added value of advanced imaging in ioMRI for pediatric brain tumor surgery compared to conventional imaging.Methods: Our systematic literature search identified relevant articles on PubMed using keywords associated with pediatrics, ioMRI, and brain tumors. The literature search was extended using the snowball technique to gather more information on advanced MRI techniques, their technical background, their use in adult ioMRI, and their use in routine pediatric brain tumor care.Results: The available literature was sparse and demonstrated that advanced sequences were used to reconstruct fibers to prevent damage to important structures, provide information on relative cerebral blood flow or abnormal metabolites, or to indicate the onset of hemorrhage or ischemic infarcts. The explorative literature search revealed developments within each advanced MRI field, such as multi-shell diffusion MRI, arterial spin labeling, and amide-proton transfer-weighted imaging, that have been studied in adult ioMRI but have not yet been applied in pediatrics. These techniques could have the potential to provide more accurate fiber tractography, information on intraoperative cerebral perfusion, and to match gadolinium-based T1w images without using a contrast agent.Conclusion: The potential added value of advanced MRI in the intraoperative setting for pediatric brain tumors is to prevent damage to important structures, to provide additional physiological or metabolic information, or to indicate the onset of postoperative changes. Current developments within various advanced ioMRI sequences are promising with regard to providing in-depth tissue information

Identification of Two Protein-Signaling States Delineating Transcriptionally Heterogeneous Human Medulloblastoma

Summary: The brain cancer medulloblastoma consists of different transcriptional subgroups. To characterize medulloblastoma at the phosphoprotein-signaling level, we performed high-throughput peptide phosphorylation profiling on a large cohort of SHH (Sonic Hedgehog), group 3, and group 4 medulloblastomas. We identified two major protein-signaling profiles. One profile was associated with rapid death post-recurrence and resembled MYC-like signaling for which MYC lesions are sufficient but not necessary. The second profile showed enrichment for DNA damage, as well as apoptotic and neuronal signaling. Integrative analysis demonstrated that heterogeneous transcriptional input converges on these protein-signaling profiles: all SHH and a subset of group 3 patients exhibited the MYC-like protein-signaling profile; the majority of the other group 3 subset and group 4 patients displayed the DNA damage/apoptotic/neuronal signaling profile. Functional analysis of enriched pathways highlighted cell-cycle progression and protein synthesis as therapeutic targets for MYC-like medulloblastoma. : Using peptide phosphorylation profiling, Zomerman et al. identify two medulloblastoma phosphoprotein-signaling profiles that have prognostic value and are potentially targetable. They find that these profiles extend across transcriptome-based subgroup borders. This suggests that diverse genetic information converges on common protein-signaling pathways and highlights protein-signaling as a unique information layer. Keywords: medulloblastoma, protein-signaling, protein synthesis, MYC, TP53, proteome, phosphoproteom

Clinical characteristics of subsequent histologically confirmed meningiomas in long-term childhood cancer survivors:A Dutch LATER study

Background: Meningiomas are the most frequent brain tumours occurring after pediatric cranial radiotherapy (CrRT). Data on course of disease, to inform clinical management of meningiomas, are sparse. This study reports the clinical characteristics of histologically confirmed meningiomas in childhood cancer survivors (CCS) in the Netherlands.& nbsp; Methods: In total, 6015 CCS from the Dutch Long-Term Effects After Childhood Cancer (LATER) cohort were eligible, including 1551 with prior CrRT. These CCS were diagnosed with cancer ag