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Embryonal tumor with multilayered rosettes (ETMR), C19MC-altered is a newly designated entity of the embryonal tumors of the central nervous system (CNS) according to the 2016 WHO classification system of CNS. Characteristically, these tumors are newly defined based on their specific molecular genetic amplification in chromosome 19q13.42 found at locus C19MC. To the best of our knowledge, we present the first reported case of ETMR in Saudi Arabian pediatric population. A 2-year-old boy presented to the hospital with generalized tonic-colonic seizure, vomiting, irritability, and inability to walk. Computed tomography (CT) scan showed a large left thalamic supratentorial brain tumor. The tumor measured 6.1 × 5.6 × 5.6 cm and was characterized by cystic changes, prominent vasculature, and calcifications. Histopathology, immunohistochemistry examination, and fluorescence in situ hybridization (FISH) analysis confirmed the diagnosis of ETMR. In addition to reporting this rare case, we provide a brief literature review, treatment options, patient outcome, and disease prognosis.

It is unclear how variations in operative duration affect outcomes after craniotomy for supratentorial brain tumor. We characterized three populations of patients with typical, shorter, and longer durations of craniotomy for supratentorial brain tumor using prospectively collected clinical data from 16,335 patients in the 2012–2018 ACS National Surgical Quality Improvement Program (NSQIP) database. We compared baseline characteristics including demographics, comorbidities, tumor type, and operative features. We used propensity score matching to attain covariate balance and logistic regression to assess odds of unfavorable outcomes. Patients with the shortest operation durations tended to be older, with fewer males, higher ASA class, more metastatic brain tumors, more medical comorbidities, and less use of intraoperative microscope or ultrasound. Patients with the longest operative durations tended to be younger, with more males, fewer non-white minorities, more obesity, lower ASA classes, more intrinsic brain tumors, fewer medical comorbidities, fewer emergency operations, and increased use of intraoperative microscope. For patients with the shortest operations, after matching, we observed significantly decreased odds of prolonged length-of-stay (LOS), major complication, any complication, reoperation, and discharge to a facility; however, there was a significantly increased risk of 30-day mortality. For patients with the longest operations, after matching, we observed significantly increased odds of prolonged LOS; minor, major, and any complication; discharge to facility; and 30-day reoperation. After matching to balance baseline characteristics, operative duration has implications for outcomes following craniotomy for supratentorial brain tumor.

Background: Hemorrhages in the surgical cavity post resection of tumor aren’t uncommon and neurosurgeons are well versed with this entity, however occurrence of an Extra Dural Hematoma(EDH), that too at a site remote to the surgical cavity, is rare. The study presents 3 cases of post operative remote site EDH after tumor resection with review of literature and an attempt to define and discuss the variables which play a role in determining the occurrence, extent and prognosis of the same. Methods: The study investigated 601 patients who underwent tumor resection in the Department of Neurosurgery, Dr D Y Patil Hospital, from January 2017 to December 2019. All patients had a normal coagulation profile preoperatively. Postoperative remote EDH occurred in 3 patients whose data was examined closely in terms of age, sex, location, final diagnosis and treatment. Results: Of the 601 patients who underwent tumor resection from Jan 2017 to Dec 2019, a total of 3 patients in our study were found to have a post-operative remote site EDH. Two of these patients were male and 1 female. The age range was 35 to 46 years with a mean of 41 years. Neither did any of these patients have a pre-existing hydrocephalus nor were they subjected to a CSF diversion procedure preoperatively. All 3 patients developed EDH on the ipsilateral side wherein 2 of our patients had a hematoma in the ipsilateral frontal region anterior to the surgical cavity while 1 patient developed hematoma in ipsilateral parietal region posterior to surgical cavity. Hematomas were unilateral with no extension to opposite side. Two patients had to be re-operated in an emergency setting while one patient was conservatively managed. Conclusion: In patients with an expected volume loss via large tumor size, excess blood and CSF loss and a large craniotomy, remote EDH may develop and the neurosurgeon must have a high index of suspicion for this entity

BACKGROUND AND PURPOSE: Synthetic MRI enables the generation of various contrast-weighted images and quantitative data in a reasonable scanning time. We aimed to use synthetic MRI to assess the detection and underlying tissue characteristics of focal areas of signal intensity and normal-appearing brain parenchyma and morphometric alterations in the brains of patients with neurofibromatosis type 1. MATERIALS AND METHODS: Conventional MR imaging and synthetic MRI were prospectively obtained from 19 patients with neurofibromatosis type 1 and 18 healthy controls. Two neuroradiologists independently evaluated focal areas of signal intensity on both conventional MR imaging and synthetic MRI. Additionally, automatically segmented volume calculations of the brain in both groups and quantitative analysis of myelin, including the focal areas of signal intensity and normal-appearing brain parenchyma, of patients with neurofibromatosis type 1 were performed using synthetic MRI. RESULTS: The comparison of conventional MR imaging and synthetic MRI showed good correlation in the supratentorial region of the brain (κ = 0.82–1). Automatically segmented brain parenchymal volume, intracranial volume, and GM volumes were significantly increased in the patients with neurofibromatosis type 1 (P

Cavernous angiomas of the brain (CCM) are being increasingly diagnosed, especially in the paediatric age group. Though classic presentations with haemorrhage or seizures are well recognised, presentation as a large lesion with mass effect is rare and creates difficulty in diagnosis as well as management. Our cases of paediatric giant CCMs that presented as a ‘mass lesion’ are reported here, and the PubMed database for giant CCMs in the paediatric population is reviewed. All articles where the size of the lesion was reported to be > 4 cm were selected for analysis to study the varying modes of presentation, treatment, and outcome; to gain a proper perspective on this distinct entity of ‘giant CCMs’. Analysis of a total of 53 cases (inclusive of our 3 cases) reported so far showed slight male preponderance (58.49%). The largest reported lesion was 14 cm in largest diameter. Most of the lesions (83.02%) occurred in the supratentorial region. In the infratentorial region, paediatric giant CCMs were more commonly seen in the cerebellum than in the brainstem. Seizures were observed in 47.17% at presentation. Features of mass effect were the mode of presentation in all our cases, and literature analysis has shown raised intracranial pressure in 37.74% (20 patients) and focal neurological deficit in 33.96% (18 patients) at presentation. Macrocephaly was seen in younger children up to the age of 7 years (16.98% or 9 patients). Gross total resection was carried out (with a good outcome) in all our cases and in 36 of the other 49 analysed patients who were operated on. About one-fourth of CCMs occur in paediatric patients. Giant CCMs are rare but can present in children even in the immediate post-natal period. Features of a mass lesion such as raised intracranial pressure, macrocephaly, and focal neurological deficit are much more common than their smaller counterparts. Their appearance on imaging also often causes diagnostic dilemmas with other intracranial mass lesions. Timely surgery with standard microsurgical principles leads to a favourable outcome in the majority. Giant CCMs, though rare, often present as a diagnostic challenge. Presentation with mass effect is common, and complete microsurgical excision remains the mainstay of treatment. Though transient neurological deficits may be encountered with this strategy, the long-term outcome remains favourable.

BACKGROUND Brain tumours represents 1.7 % of all cancers and contributes 1.8 % of all cancer deaths. Of all the brain tumours, 80 % are supratentorial.1 Magnetic resonance imaging (MRI) is an important modality, having higher sensitivity for detecting intracranial pathology. Multiplanar imaging is possible with MRI which helps in detection, localization and characterization of the lesion. MRI examination has helped in early diagnosis, accurate localization of the tumour, with prompt initiation of appropriate medical or surgical therapy. Recent advances like magnetic resonance (MR) spectroscopy, MR fluoroscopy with stereotactic guided biopsy have revolutionized the role of MRI in study of intracranial tumours. METHODS A cross sectional study of 75 patients was done by Siemens Essenza 1.5T MRI from June 2018 to June 2020 using dedicated head coil. RESULTS In our study, most of the patients were between 31 - 60 years of age (39 % of all patients) with the largest group between 31 - 40 years (20 % of all patients). Out of 75 cases of supratentorial neoplasms, 47 (72 %) were intra-axial lesions, 15 (23 %) were extra-axial lesions and 3 (5 %) were intra ventricular lesions. In this study, 69 % of the lesions were solitary and 31 % were multiple. Most of patients (89 %) showed some enhancement, most common being heterogeneous/inhomogeneous enhancement. Commonest type of supratentorial neoplasms were metastases comprising 29 % of this study. CONCLUSIONS Magnetic resonance imaging is an important modality, having higher sensitivity for detecting intracranial pathology. Multiplanar imaging is possible with MRI which helps in early detection, localization and characterization of the lesion. Metastasis is the most common supratentorial tumour in this study followed by meningioma. Intra-axial tumours are more common than extra-axial tumours in this study. KEYWORDS MRI, Metastasis, Glioma, Meningioma

Background. Linguistic impairment following cerebellar lesions is characterized by a marked cerebellocerebral diaschisis with decreased perfusion in the left cerebral hemisphere. Case. We report on a 60-year-old right-handed French chef who presented with linguistic deficits following a right cerebellar infarction. Neurolinguistic examinations in the acute phase showed impaired graphomotor planning, especially for kanji (Japanese morphograms). Despite the absence of any structural damage to the supratentorial brain regions, a quantitative 123I-IMP SPECT study revealed a relative hypoperfusion, mainly around the left posterior middle temporal gyrus, considered to be a crossed cerebellar-cerebral diaschisis. We performed functional near-infrared spectroscopy (fNIRS) and observed that a picture card task could increase blood perfusion in the affected area. This task was as follows: once he saw a picture card depicting a dish, the patient had to list the ingredients that make up the dish. For example, he had to name vegetables, meat, and spices upon seeing a “curry” picture card. We added this task to his daily speech-hearing therapy regimen. In the chronic phase, we confirmed symptom amelioration in linguistic performance-paralleled reduction in the level of hypoperfusion on SPECT study. Discussion. This case is the first report of an fNIRS approach used to evaluate evidence-based prospective speech-hearing tasks by observing blood flow to the hypoperfused area of the cerebral cortex surface.

OBJECTIVE Acute subdural hematoma (ASDH) is a major cause of mortality and morbidity after traumatic brain injury (TBI). Surgical evacuation is the mainstay of treatment in patients with altered neurological status or significant mass effect. Nevertheless, concerns regarding surgical indication still persist. Given that clinicians often make therapeutic decisions on the basis of their prognosis assessment, to accurately evaluate the prognosis is of great significance. Unfortunately, there is a lack of specific and reliable prognostic models. In addition, the interdependence of certain well-known predictive variables usually employed to guide surgical decision-making in ASDH has been proven. Because gray matter and white matter are highly susceptible to secondary insults during the early phase after TBI, the authors aimed to assess the extent of these secondary insults with a brain parenchyma densitometric quantitative CT analysis and to evaluate its prognostic capacity. METHODS The authors performed a retrospective analysis among their prospectively collected cohort of patients with moderate to severe TBI. Patients with surgically evacuated, isolated, unilateral ASDH admitted between 2010 and 2017 were selected. Thirty-nine patients were included. For each patient, brain parenchyma density in Hounsfield units (HUs) was measured in 10 selected slices from the supratentorial region. In each slice, different regions of interest (ROIs), including and excluding the cortical parenchyma, were defined. The injured hemisphere, the contralateral hemisphere, and the absolute differences between them were analyzed. The outcome was evaluated using the Glasgow Outcome Scale–Extended at 1 year after TBI. RESULTS Fifteen patients (38.5%) had a favorable outcome. Collected demographic, clinical, and radiographic data did not show significant differences between favorable and unfavorable outcomes. In contrast, the densitometric analysis demonstrated that greater absolute differences between both hemispheres were associated with poor outcome. These differences were detected along the supratentorial region, but were greater at the high convexity level. Moreover, these HU differences were far more marked at the cortical parenchyma. It was also detected that these differences were more prone to ischemic and/or edematous insults than to hyperemic changes. Age was significantly correlated with the side-to-side HU differences in patients with unfavorable outcome. CONCLUSIONS The densitometric analysis is a promising prognostic tool in patients diagnosed with ASDH. The supplementary prognostic information provided by the densitometric analysis should be evaluated in future studies.

Objectives This study aims to analyze lesions on computed tomography (CT) images and digital subtraction angiography (DSA) of ruptured cerebral arteriovenous malformation (AVM). Methods Cross-sectional description of 82 patients' cerebral bleeding due to rupture of AVM, determined by multislice computed tomography or cerebral DSA at Bach Mai Hospital. Results Patients with ruptured AVM are commonly under 40 years old (62.2%), average age: 35.1 ± 11.2. On CT or DSA images, it is common to see AVM rupture causing cerebral bleeding in the supratentorial region (91.5%), with 83% cerebral lobe bleeding, mainly small and moderate hematoma volume ( p p Conclusions On MSCT and DSA images, ruptured AVMs often cause lobar hemorrhage in young people. AVMs are usually small to moderate in size. The feeding arteries are mainly derived from middle and posterior cerebral arteries, and drained mainly by superficial veins. The Spetzler–Martin classification and the supplementary grading scales are used for ranking the severity of the lesion, as well as for choosing AVM treatment options and assessing the prognosis.

Primary CNS lymphoma (PCNSL) is rare malignant B cell lymphoid tumor of brain which predominantly occurs in supratentorial region in periventricular location. Majority of PCNSL are of DLBCL type and idiopathic in etiology. Here we are reporting a case of primary CNS lymphoma, DLBCL involving extremely uncommon intraventricular location. Central neurocytoma, subependymal giant cell astrocytoma, choroid plexus tumors and meningiomas are the common diagnosis at this site. Aim of reporting this case is to bring awareness of unusual intraventricular location of primary CNS lymphoma which should be kept in mind before considering gross total excision of lesion.

Intracranial extracerebral ectopic brain tissue, also called glioneuronal heterotopia, is a rare developmental disorder. These lesions are predominately seen at the base of the brain in the supratentorial region and are frequently associated with craniofacial anomalies. We report prenatal ultrasonographic and magnetic resonance imaging (MRI) findings of isolated extracerebellar ectopic brain tissue in the posterior fossa of a 22 weeks fetus.

Background Gender is a complex social determinant of health affected by both social and biological factors. There is a need to investigate the effect of gender on outcomes, in the absence of confounding characteristics, to mitigate disparities in care. Methods A total of 1970 consecutive patients at a university health system undergoing nonmeningioma supratentorial brain tumor resection over a 6-year period (June 9, 2013–April 26, 2019) were analyzed retrospectively. Coarsened exact matching was used to match patients on demographic factors including history of previous surgery, median household income, and race. Outcomes assessed included readmission, emergency department visit, unplanned reoperation, and mortality within 30 days of surgery. Regression analysis was performed among a prematched population and between the matched cohorts with significance set at a P value Results Within the matched population, no significant difference was observed between male and female patients in any of the recorded outcomes after nonmeningioma supratentorial brain tumor resection, including readmission, emergency department evaluation, unplanned reoperation, and mortality within 30 days of resection (P = 0.28–0.85). Similarly, no significant difference was found in any of the morbidity and mortality outcomes in the prematched regression analysis (P = 0.10–0.70). Conclusions When gender is isolated from race, household economics, and other key factors, it does not seem to independently predict morbidity or mortality in the short-term postoperative window after supratentorial brain tumor resection. Future studies should investigate the impact of gender in longer follow-up and its interrelation with other social determinants of health contributing to outcome disparity.

Fetal MRI is the modality of choice to study supratentorial brain malformations. To accurately interpret the MRI, the radiologist needs to understand the normal sequence of events that occurs during prenatal brain development; this includes familiarity with the processes of hemispheric cleavage, formation of interhemispheric commissures, neuro-glial proliferation and migration, and cortical folding. Disruption of these processes results in malformations observed on fetal MRI including holoprosencephaly, callosal agenesis, heterotopic gray matter, lissencephaly and other malformations of cortical development (focal cortical dysplasia, polymicrogyria). The radiologist should also be familiar with findings that have high association with specific conditions affecting the central nervous system or other organ systems. This review summarizes and illustrates common patterns of supratentorial brain malformations and emphasizes aspects that are important to patient care.

Brain tumours may cause disturbances of cerebral auto-regulation & the blood brain barrier (BBB). These effects could possibly have an unfavourable influence on brain condition. To compare the effects of the application of a bolus hypertonic saline with those of mannitol on intraoperative brain relaxation. Ninety patients of ASA grade II and III in the age group >18 years with supratentorial brain tumours posted for elective brain tumour surgery were studied in the current study. They were randomly allocated into two groups. Each group enrolled with 45 patients- Group HTS : receiving 160 ml of 3% hypertonic saline. Group M : receiving 150 ml of 20% mannitol. Patients from both groups were induced with injection fentanyl, propofol & rocuronium. Maintained with N2O: O2 in a ratio of 2:1 combined with isoflurane, fentanyl and vecuronium. End tidal Co2 pressure were maintained between 35-40 mm Hg. Arterial blood pressure were kept within baseline value ± 20. Hence application of hypertonic saline is a more effective method for brain relaxation in patients posted for elective supratentorial brain tumours surgery and can be a great value in routine practice.

Intraoperative cortical mapping provides functional information that permits the safe and maximal resection of supratentorial lesions infiltrating the so-called eloquent cortex or subcortical white matter tracts. Primary and secondary brain tumours located in eloquent cortex can render surgical treatment ineffective if it results in new or worsening neurology. A cohort of forty-six consecutive patients with supratentorial tumours of variable pathology involving eloquent cortical regions and aided with intraoperative neurophysiology were included for retrospective analysis at a single-centre tertiary institution. Intraoperative neurophysiological data has been related to immediate post-operative neurologic status as well as 3-month follow-up in patients that underwent awake or asleep surgical resection. Patients that experienced new or worsening neurologic symptoms post-operatively demonstrated a high incidence of recovery at 3-months. Those without new neurologic symptoms post-operatively demonstrated little to no worsening at 3-months. Our study explored the extent to which cortical mapping permitted safe surgical resection whilst preserving neurologic function. To the authors' knowledge this is the first documented case series in Singapore that has incorporated a systematic and individually tailored multimodal workflow to cortico-subcortical mapping and monitoring for the safe resection of infiltrative lesions of the supratentorial region.

Cryptococcal meningitis is the most common intracranial infectious fungal disease. After a period of antifungal treatment, as the number of cells in the cerebrospinal fluid decreases, the biochemical indexes improve and the number of cryptococcus reduces, the patient’s condition suddenly worsen. Most of the symptoms are severe headache, raised intracranial pressure, together with impaired clinical nerve function. These presentations are often mistaken for a failure of antifungal treatment. In fact it’s an encephalitis syndrome which is unrecognized by most clinicians: Immune reconstitution inflammatory syndrome (IRIS). To increase awareness we retrospectively analyzed clinical data of 100 cases of cryptococcal neoformans meningitis, among which 26 patients develop CM-IRIS. All patients have been divided into three groups: Group 1, patients who were not treated with glucocorticoid and didn’t experienced IRIS; Group 2, patients who were not treated with glucocorticoid although developed CM-IRIS; Group 3, patients started treatment with glucocorticoid for two weeks with new onset CM-IRIS. Compared with the group treated with glucocorticoid, treatment without glucocorticoid was subjected to a higher risk of incident IRIS. The difference was statistically significant (P

Background: In patients with lesions located around or within eloquent motor or speech cortex, surgical resection is a challenge; because of high risk of development of postoperative deficits and incomplete resection. intraoperative neurophysiological monitoring (IONM) paves the way to complete resection in these patients along with functional preservation postoperative. Aim of the study: To assess the utility and effectiveness of IONM techniques in resection of lesions located in eloquent motor speech cortex (intrinsic brain tumors and epileptic zones), regarding the extent of resection and functional preservation postoperative. Patients and Methods: we conducted prospective observationalstudy on 30 patients with different types of lesions located within eloquent motor or speech cortex, by the use of low frequency direct cortical and subcortical stimulation technique,12 patients were operated under general anaesthesia and 18 patients were operated by awake craniotomy technique.All patients were assessed clinically and do MRI brain with contrast and FLAIR study postoperatively, those who developed new onset neurological deficits were assessed at 3months postoperative. Results: complete resection was achieved in 17 patients (56.6%). Immediate postoperative new onset or worsened neurological deficits were encountered in 18 patients (60%), however 3 months postoperative the deficits were persistent only in 4 patients (13.3%). Conclusion: direct cortical stimulation is an easy safe and reliablemethod for detection of cortical and subcortical functionally eloquent structures during resection of brain tumors and epileptic lesions

Dysembryoplastic neuroepithelial tumor (DNT) considered as a benign cortical glioneuronal neoplasm of children or young adults, typically present with drug-resistant focal epilepsy. DNTs are usually located in the temporal lobe but can found in any part of the supratentorial brain cortex. Multifocal DNTs have rarely reported. Here we present an eight years old boy with two years follow up, having a somewhat stable diffuse multinodular DNT of the most significant spatial extent that may have reported, involving cortical and subcortical left temporo-occipital lobe, bilateral basal ganglia and thalamus, presenting with headache, short stature, and behavioral disorder.

Introduction: Extragonadal germinomas rarely emerge from the brain stem; however, proper diagnosis and treatment can result in favorable prognosis. Unfortunately, the preoperative diagnosis of medulla oblongata germinoma is difficult due to insufficient clinical signs and symptoms that are specific to this diagnosis. Case Representation: We present a 12-year-old male patient with an intra-fourth-ventricular germinoma, derived from the medulla oblongata, with no abnormalities in the supratentorial region. The germinoma was initially assessed by advance MRI sequences, including diffusion-weighted imaging, T1 perfusion, and spectroscopy. Conclusion: In summary, although existing imaging technologies cannot completely distinguish germinomas from other primary brain neoplasms in the fourth ventricle, in patients aged between 12 and 40 years, a small mass on the dorsal side of medulla oblongata that emerges into the fourth ventricle and is characterized by homogeneous contrast enhancement, the absence of calcification and hemorrhage, and the lack of hydrocephalus should be considered for a potential medulla oblongata germinoma diagnosis.

The most severe form of profound asphyxia in neonates is now known as “total brain injury,” which forms part of the clinical spectrum of hypoxic-ischemic encephalopathy (HIE). Although the magnetic resonance (MR) imaging features of total brain injury remain to be determined, a widespread hyperintensity of the supratentorial brain, known as the “white cerebrum sign,” has been reported in diffusion-weighted images (DWI). We examined four neonates who developed severe profound asphyxia. In the first week of life, all neonates showed the white cerebrum sign on DWI. A follow-up of these cases over a period of 1 month revealed diffuse bilateral multicystic encephalomalacia (MCE) as well as shrinkage of the basal ganglia and thalami (BG/T). These MR findings were common to all neonates, and all the neonates had severe adverse clinical outcomes. Neonates, who exhibit the white cerebrum sign on MR imaging due to profound asphyxia, develop major disabilities, and MCE with shrinkage of the BG/T suggests miserable outcomes.

Antibodies to myelin oligodendrocyte glycoprotein (MOG-ab) and antibodies to aquaporin-4 (AQP4-ab) have been suggested to play roles in commonly separated subsets of patients with neuromyelitis optica spectrum disorder (NMOSD) phenotypes. The aim of this study is to quantitatively delineate and compare the brain lesion distributions of AQP4-ab-positive and MOG-ab-positive patients. Fifty-seven and twenty-eight clinical MRI scans were collected from fifty-two AQP4-ab-positive and twenty-four MOG-ab-positive patients, respectively. T2 lesions were segmented manually on each axial FLAIR image. Probabilistic lesion distribution maps were created for each group after spatial normalization. Lobe-wise and voxel-wise quantitative comparisons of the two distributions were performed. A classification model based on the lesion distribution features was constructed to differentiate the two patient groups. Infratentorial and supratentorial brain lesions were found in both AQP4-ab-positive and MOG-ab-positive patients, with large inter-group overlap mainly in deep white matter (WM). In comparison with those in the AQP4 group, the brain lesions of the MOG-ab-positive patients had a larger size, dispersed distribution, and higher probabilities in the cerebellum, pons, midbrain, and GM and juxtacortical WM in temporal, sublobar, frontal, and parietal lobes. The area under the receiver operating characteristic curve of the lesion-distribution-based classification model was 0.951. MOG-ab-positive and AQP4-ab-positive groups showed similar but quantitatively different brain lesion distributions. These results may help clinicians in considering MOG versus AQP4 in initial diagnosis, and add rationale for sending corresponding serologic testing. • Brain lesion distributions of AQP-ab-positive and MOG-ab-positive NMOSD patients • Larger size, dispersed distribution, higher lesion probabilities in the cerebellum, pons, midbrain, and GM and juxtacortical WM in the MOG group • The lesion-distribution-based classification model differentiates the two groups with AUC = 0.951

Introducción: Los cavernomas son malformaciones vasculares de bajo flujo que ocurren principalmente en el cerebro y, menos frecuentemente, en la médula espinal. Son lesiones raras y representan alrededor del 5-13% de las malformaciones vasculares del sistema nervioso central. Objetivos: Evaluar el seguimiento de 30 casos de pacientes diagnosticados con cavernomas y describir los hallazgos clínicos, epidemiológicos, topográficos, evolutivos y la conducta terapéutica en un período de 22 años. Metodología: Se trata de un estudio observacional, transversal, de carácter cuantitativo, que incluye pacientes diagnosticados con cavernomas, a través de la recolección de datos en los Servicios de Neurocirugía de varios Hospitales de João Pessoa, Paraíba, y que requirieron abordaje clínico o quirúrgico durante el período comprendido entre 1995 y 2017. Resultados: Las manifestaciones clínicas más comunes fueron las crisis convulsivas en el 33,33% (n = 10), cefalea en un 20% (n = 6) y parálisis del nervio craneal en un 20% (n = 6). En cuanto al sexo, 63,33% (n = 19) fueron del sexo femenino y 36,66% (n = 11) del sexo masculino. En cuanto a la localización, predominó la región supratentorial (70,73%), siendo el lóbulo frontal el más acometido (41,46%). Las lesiones múltiples ocurrieron en tres casos, siendo dos de ellos confirmados de cavernoma familiar. De los 30 pacientes, 12 fueron sometidos a cirugía, por presencia de sintomatología importante o de complicaciones. Se produjo un fallecimiento debido a hematoma subdural agudo. Conclusión: Los cavernomas generalmente tienen evolución benigna, necesitando tratamiento quirúrgico sólo en los casos sintomáticos y ante la presencia de complicaciones. El seguimiento reveló una buena evolución del cuadro, siendo la mayoría de los pacientes asintomáticos o con síntomas controlados mediante medicación.

Background: Postoperative nausea and vomiting (PONV), and shivering are common general anesthesia complications. Hence, this study evaluates the effect of dexmedetomidine, given as a premedication, on PONV and shivering in patients with opium use who underwent elective supratentorial brain tumor surgeries. Materials and Methods: In a randomized clinical trial, 100 opium user patients who were candidates for elective supra-tentorial brain tumor surgery under general anesthesia were studied in two groups of 50 patients. The intervention group received dexmedetomidine (within 10 and 30 minutes infusion) before the anesthesia induction. Group placebo that received normal saline as a group. PONV and shivering rates were compared between the two groups. Results: Both groups did not differ in hemodynamic parameters during operation, including pulse rate, systolic and diastolic blood pressure, and anesthesia duration. In the dexmedetomidine group, patients suffered less from PONV and shivering rather than controls, and these differences were both significant (P=0.001 and P=0.027, respectively). Discussion: Dexmedetomidine administration before major surgeries might reduce post-operative nausea and vomiting and the occurrence of shivering, particularly in opium-addicted patients.[GMJ.2021;10:e2146]

Cerebral cavernous malformation (CCM) is a developmental abnormality of blood vessels that supply the brain. It is composed of large, adjacent capillaries which contain little or no neural tissue. They mostly occur in the supratentorial region. However, the occurrence of these vascular lesions can be seen at different sites of the central nervous system (CNS). The prevalence of CCM is estimated to be 0.4% in the general population and among the affected patients, 18.7% have multiple lesions. However, about 30-50% of CCM cases are asymptomatic and are found incidentally. Here we report a case of an eight-year-old girl with a massive hemorrhagic presentation of a left parietooccipital CCM.

ObjectivesTo test for cerebellar involvement in motor and nonmotor impairments in Parkinson disease (PD) and to determine patterns of metabolic correlations with supratentorial brain structures, we correlated clinical motor, cognitive, and psychiatric scales with cerebellar metabolism.MethodsWe included 90 patients with PD. Motor, cognitive, and psychiatric domains were assessed, and resting-state 18FDG-PET metabolic imaging was performed. The motor, cognitive, and psychiatric scores were entered separately into a principal component analysis. We looked for correlations between these 3 principal components and cerebellar metabolism. Furthermore, we extracted the mean glucose metabolism value for each significant cerebellar cluster and looked for patterns of cerebrum-cerebellum metabolic correlations.ResultsSeverity of impairment was correlated with increased metabolism in the anterior lobes and vermis (motor domain); the right crus I, crus II, and declive (cognitive domain); and the right crus I and crus II (psychiatric domain). No results survived multiple testing corrections regarding the psychiatric domain. Moreover, we found distributed and overlapping, but not identical, patterns of metabolic correlations for motor and cognitive domains. Specific supratentorial structures (cortical structures, basal ganglia, and thalamus) were strongly correlated with each of the cerebellar clusters.ConclusionsThese results confirm the role of the cerebellum in nonmotor domains of PD, with differential but overlapping patterns of metabolic correlations suggesting the involvement of cerebello-thalamo-striatal-cortical loops.

Purpose Gender is a known social determinant of health which has been linked disparities in medical care. This study intends to assess the impact of gender on 90-day and long-term morbidity and mortality outcomes following supratentorial brain tumor resection in a coarsened-exact matched population. Materials and methods A total of 1970 consecutive patients at a single, university-wide health system undergoing supratentorial brain tumor resection over a six-year period (09 June 2013 to 26 April 2019) were analyzed retrospectively. Coarsened Exact Matching was employed to match patients on key demographic factors including history of prior surgery, smoking status, median household income, American Society of Anesthesiologists (ASA) grade, and Charlson Comorbidity Index (CCI), amongst others. Primary outcomes assessed included readmission, ED visit, unplanned reoperation, and mortality within 90 days of surgery. Long-term outcomes such as mortality and unplanned return to surgery during the entire follow-up period were also recorded. Results Whole-population regression demonstrated significantly increased mortality throughout the entire follow-up period for the male cohort (p = 0.004, OR = 1.32, 95% CI = 1.09 - 1.59); however, no significant difference was found after coarsened exact matching was performed (p = 0.08). In both the whole-population regression and matched-cohort analysis, no significant difference was observed between gender and readmission, ED visit, unplanned reoperation, or mortality in the 90-day post-operative window, in addition to return to surgery after throughout the entire follow-up period. Conclusion After controlling for confounding variables, female birth gender did not significantly predict any difference in morbidity and mortality outcomes following supratentorial brain tumor resection. Difference between mortality outcomes in the pre-matched population versus the matched cohort suggests the need to better manage the underlying health conditions of male patients in order to prevent future disparities.

Hemangioblastomas (HBLs) are highly vascular and cystic benign neoplasms. They form very small part of intracranial tumours and are often localized in the posterior fossa. Although most of them are sporadic, a significant group is accompanied with von Hippel-Lindau (VHL) syndrome. This case report presents a 57-year-old woman treated with total resection using micro-surgical technique and was diagnosed as HBL based on histopathologic findings. Contrast-enhanced cranial MRI of the patient with the complaints of spasms in the right side of body showed a right paracentral mass that caused midline shift. In literature, the previously reported cases of supratentorial HBL unaccompanied with VHL syndrome were searched in PUBMED, compiled and presented. It should be borne in mind that rare HBLs manifesting with various neurological symptoms may occur in the supratentorial region, and may not accompany with VHL syndrome.

Object To evaluate the efficacy and safety of trans-sulcal or fissure approach in surgical treatment of supratentorial lesions Study design Retrospective clinical case series Methods This study included 42 patients. Age ranged from 4 to 78 years. Sulcal dissection was done in 26 patients at or near the eloquent areas, and in 16 patients, it was done in non-eloquent areas. Diffusion tensor tractography, neuronavigation, and intraoperative monitoring were applied for lesions at the motor areas. The follow-up period was 24 months. Results Gross total excision could be achieved in 52% of patients. Sulcal dissection was easy in 26 patients with the lax brain. The outcome, according to the Karnofsky scale, was 100 in 21/42 (50%). Complications were transient deterioration of function in three patients, and immediate post-operative hemorrhage in two patients. Three patients became seizure-free after surgery. Conclusion The trans-sulcal dissection is a safe, useful, and applicable approach. Through which it is possible to reach deeply seated lesions. It preserves the motor functions, provides wider exposure, minimizes the need of brain retraction during surgery, and preserves the gyral layers. Complications are usually transient, specially in the presence of preoperative diffusion tensor tractography, intraoperative neuronavigation, and monitoring.

Introduction: Information on epidemiological profile of supratentorial tumors pathology is scant in developing countries like India hence; more institutional data are needed to know the tumor burden in pediatric population, to undertake necessary research and to improve the therapeutic modalities in any given locality. Aim: To study the distribution of various supratentorial tumors in pediatric age group according to age, sex, anatomical location and histological type and correlate findings from our institute with other institutional studies. Materials and Methods: We conducted a retrospective study to analyze the data on archived surgical samples of 45 pediatric patients (0-18 years) with supratentorial brain tumors, operated over a period of 10 years (January 2008 to December 2017). Data regarding age, gender, site of the tumor and histopathology were considered for the analysis. The results obtained were compared with the available published literature data matching to our study on Indian population. Results: The most common pediatric primary brain tumors occupying supratentorial region were astrocytic tumors (33.3%) followed by craniopharyngiomas (20%) and meningiomas (13.4%). The most common astrocytic tumor was pilocytic astrocytoma. Pediatric brain tumors were more common in males (60%) as compared to females (40%) with male to female ratio of 1.5: 1. Conclusion: Astrocytomas and craniopharyngiomas were observed to be the common supratentorial tumors among pediatric age group at our institute consistent with other multicentric studies conducted in India. Keywords: Astrocytoma, craniopharyngioma, Pediatric tumors, Supratentorium.

Background Congenital intracranial tumors are very rare and account for less than 2% of all childhood brain tumors. Teratomas constitute about one third to one half of these, predominantly located midline in the supratentorial region. Posterior fossa location rarely occurs and, based on the cases reported in the literature, commonly has a poor prognosis. Case presentation A newborn female, diagnosed prenatally with hydrocephalus, is presented at birth with increasing head circumference and Parinaud’s syndrome. Magnetic resonance imaging scans demonstrated a huge posterior fossa tumor with obstructive hydrocephalus. At surgery, through a suboccipital craniotomy, complete excision was achieved of a histological-proven immature teratoma. The infant received adjuvant chemotherapy for 1 year. She had normal neurological development and remained tumor-free through her 20-year follow-up. Conclusion The authors report this rare case of congenital posterior fossa teratoma with long-term outcome, and the literature is reviewed.

Desmoplastic infantile ganglioglioma (DIG) is a rare supratentorial brain tumor, which is usually diagnosed before the age of two and has a favorable prognosis. To date, only a few cases have been reported in the literature. We report a case of DIG in a three-month-old boy who presented to our pediatric emergency department with decreased activity and excessive crying. This case report highlights the computed tomography (CT) and magnetic resonance imaging (MRI) findings of this rare tumor. Non-contrast CT scan findings in our case initially masqueraded as an extra-axial arachnoid cyst; however, the MRI findings after contrast agent administration were typical for the diagnosis of DIG.

Background: Surgical treatment of brain tumors were proved to be at highest risk of unplanned reoperation. Therefore, we decided to find possible predictors of complications leading to early unplanned reoperations among patients with supratentorial brain tumors. Methods: We retrospectively analysed 328 patients who underwent craniotomy due to supratentorial brain tumor. Early reoperation was defined as reoperation during the same hospital stay. To determine the potential predictors of early reoperation we used univariate and multivariate logistic regression analyses. Results: A total of 22 (6.51%) patients underwent unplanned reoperation. Those patients significantly more often were diagnosed with high grade glioma (81.82% vs. 55.70%; p = 0.02). They had lower Red Blood Cells count (4.16 ± 0.82 vs. 4.60 ± 0.56 103/μl; p

Ganglioglioma is a benign slow-growing neoplasm that most frequently occurs at the supratentorial region. Nevertheless, there are occasional reports of ganglioglioma occurring in the brainstem and spinal cord. Here we report a rare case of the craniocervical ganglioglioma. A 3.5-year-old male, presented with severe progressive quadriparesis, gait disturbance, and sphincter deficit. Physical examination demonstrated the quadriparesis, associated with positive Hoffman, Babinski, and clonus signs, and increased respond of deep tendon reflexes. Magnetic resonance imaging (MRI) demonstrated an ill-defined mass within medulla and upper cervical spinal cord, which was hypo to iso signal on T1, heterogeneous iso to hypersignal on T2 and demonstrated marked bright enhancement on T1 with gadolinium (Gad) injection. On surgery, the mass had a soft texture, ill-defined border, and grey to brown appearance. According to the frozen section report, and due to the absence of the tumour-neural parenchymal interference, only decompression of the tumour and expansile duraplasty were performed. The histopathology revealed ganglioglioma. On last follow-up 14 months after surgery, the patient was asymptomatic and neurological status was improved. The craniocervical MRI demonstrated the tumour that did not grow. Although it is rare, the ganglioglioma should be in the differentiated diagnoses of tumours with compatible clinical and radiologic features even in the unusual locations, especially in the pediatric and young patients. Safety surgical resection should be considered in these patients, whenever possible. In the case of partial resection, that is common in the tumours located within functionally critical structures, long close follow-up rather than radiation therapy is required.

Paragangliomas are tumours of extra-adrenal paraganglia. They may metastasize to the brain but primary paragangliomas are exceedingly rare in the supratentorial region and long-term outcomes after surgery is largely unknown. This description of an excellent outcome 13 years following surgery in a 40-year-old gentleman with a primary paraganglioma near the falx provides an important perspective on the value of gross total resection in these tumours. We also review the options for adjuvant therapy in tumours that cannot be excised completely.We describe a supratentorial paraganglioma in the parasagittal region in a 40-year-old gentleman who presented with clinical and radiological features suggestive of a right parafalcine meningioma. Histopathological examination following gross total excision of the tumour revealed histological and immunochemical features of a paraganglioma. A detailed search for a systemic primary was negative and the patient remains disease-free 13 years after the surgery.Differentiating between tumours arising primarily and those that are metastatic deposits in the central nervous system requires long-term follow-up and monitoring for the appearance of occult primary tumours. Gross total resection is likely to provide good long-term outcomes.

BACKGROUND AND PURPOSE: Acute cerebellitis is an acute neurologic condition attributable to a recent or concurrent infection or a recent vaccination or ingestion of medication, with MR imaging evidence of cerebellar edema. MR imaging can confirm an anatomic abnormality and may allow the radiologist to establish a differential diagnosis. The purpose of this research was to evaluate the MR imaging findings in children with acute cerebellitis due to infectious versus immune-related conditions, in particular whether MR imaging findings allow differentiation. MATERIALS AND METHODS: Electronic medical records were reviewed between 2003 and 2020 in our quaternary children’s hospital. Data included demographics and clinical records: presentation/symptoms, final diagnosis including acute cerebellitis and immune-related acute cerebellitis, length of stay, treatment, condition at discharge, and laboratory findings. Retrospective independent review of all brain MR imaging studies was performed. RESULTS: Forty-three patients (male/female ratio, 28:15) were included in this study. Average age at presentation was 7.08 years (range, 0.05–17.52 years). Thirty-five children had infectious and 8 children had immune-related acute cerebellitis. Significant differences in neuroimaging were the following: 1) T2-FLAIR hyperintense signal in the brainstem (37.50% versus 2.85%, P = .016); 2) T2-FLAIR hyperintense signal in the supratentorial brain higher in the immune-related group (37.50% versus 0.00%, P = .004); and 3) downward herniation, higher in the infectious acute cerebellitis group (42.85% versus 0.00%, P = .03). CONCLUSIONS: Acute cerebellitis is a rare condition, and MR imaging is helpful in the differential diagnosis. T2-FLAIR hyperintense signal in the brainstem and supratentorial brain may be indicative of immune-related acute cerebellitis, and downward herniation may be indicative of infectious acute cerebellitis.

Adult brain tumors mostly distinguish themselves from their pediatric counterparts. However, some typical pediatric brain tumors also occur in adults. The aim of this review is to describe the differences between classification, treatment, and outcome of medulloblastoma, pilocytic astrocytoma, and craniopharyngioma in adults and children. Medulloblastoma is a WHO IV posterior fossa tumor, divided into four different molecular subgroups, namely sonic hedgehog (SHH), wingless (WNT), Group 3, and Group 4. They show a different age-specific distribution, creating specific outcome patterns, with a 5-year overall survival of 25–83% in adults and 50–90% in children. Pilocytic astrocytoma, a WHO I tumor, mostly found in the supratentorial brain in adults, occurs in the cerebellum in children. Complete resection improves prognosis, and 5-year overall survival is around 85% in adults and >90% in children. Craniopharyngioma typically occurs in the sellar compartment leading to endocrine or visual field deficits by invasion of the surrounding structures. Treatment aims for a gross total resection in adults, while in children, preservation of the hypothalamus is of paramount importance to ensure endocrine development during puberty. Five-year overall survival is approximately 90%. Most treatment regimens for these tumors stem from pediatric trials and are translated to adults. Treatment is warranted in an interdisciplinary setting specialized in pediatric and adult brain tumors.

BACKGROUND AND PURPOSE: Definitions of fetal microcephaly and macrocephaly are debatable. A better understanding of their long-term prognoses would help guide parental education and counseling. This study aimed to explore the correlation between 2D and 3D fetal brain MR imaging biometry results and the long-term neurodevelopmental outcomes. MATERIALS AND METHODS: This analysis is a historical cohort study. Fetal brain biometry was measured on 2D and 3D MR imaging using a volumetric MR imaging semiautomated algorithm. We measured and assessed the following brain structures: the supratentorial brain volume and cerebellar volume and cerebellar volume/supratentorial brain volume ratio, in addition to commonly used 2D brain MR imaging biometric variables, including occipitofrontal diameter, biparietal diameter, and transcerebellar diameter. Microcephaly was defined as ≤ 3rd percentile; and macrocephaly, as ≥ 97th percentile, corresponding to −2 SDs and +2 SDs. The neurodevelopmental outcome of this study cohort was evaluated using the Vineland-II Adaptive Behavior Scales, and the measurements were correlated to the Vineland standard scores. RESULTS: A total of 70 fetuses were included. No significant correlation was observed between the Vineland scores and either the supratentorial brain volume, cerebellar volume, or supratentorial brain volume/cerebellar volume ratio in 3D or 2D MR imaging measurements, after correction for multiple comparisons. No differences were found among fetuses with macrocephaly, normocephaly, or microcephaly regarding the median Vineland standard scores. CONCLUSIONS: Provided there is normal brain structure on MR imaging, the developmental milestone achievements in early years are unrelated to 2D and 3D fetal brain MR imaging biometry, in the range of measurements depicted in this study.

BACKGROUND The authors investigated the incidence of ischemic lesions on immediate postoperative diffusion-weighted imaging (DWI) according to the findings of intraoperative motor evoked potential (MEP) and the correlation with postoperative motor function deficits. METHODS From January 2016 to June 2018, total 127 supratentorial brain tumor patients were enrolled in this study. The intraoperative MEP results were analyzed in the following three groups: no decline below 50 % of baseline (A), transient decline below 50 % of baseline (B), no recovery until end stage of surgery (C). Postoperative magnetic resonance imaging was performed within 48 hours after surgery. RESULTS Of the total 127 patients, MEP changes (group B&C) were observed in 25 patients (25/127, 19.7%), DWI positive findings were identified in 31 patients (31/127, 24.4%) and motor function deficits were observed in 19 patients (19/127, 15%), respectively. DWI positive finding rate was higher in gliomas (14/43, 32.6%) than meningiomas (8/51, 15.7%) or other tumors including metastasis (6/32, 18.8%), however, there was no statistical significance. In MEP changes, group B&C (16/25, 64%) showed higher DWI positive rate than group A (13/102, 12.7%). In DWI findings, the DWI positive cases (16/31, 51.6%) showed a higher motor function deficit rates than the DWI negative cases (3/96, 3%). These two results were all statistically significant (P CONCLUSIONS Both intraoperative MEP changes and postoperative DWI positive findings in supratentorial brain tumor surgery were significant related with postoperative motor function deficits. Even if intraoperative MEP changes occur during supratentorial brain tumor surgery, active and appropriate efforts to prevent irreversible MEP changes may reduce the occurrence of permanent postoperative motor function deficits.

The accurate recognition of multiple sclerosis (MS) lesions is challenged by the high sensitivity and imperfect specificity of MRI. To examine whether longitudinal changes in volume, surface area, 3-dimensional (3D) displacement (i.e. change in lesion position), and 3D deformation (i.e. change in lesion shape) could inform on the origin of supratentorial brain lesions, we prospectively enrolled 23 patients with MS and 11 patients with small vessel disease (SVD) and performed standardized 3-T 3D brain MRI studies. Bayesian linear mixed effects regression models were constructed to evaluate associations between changes in lesion morphology and disease state. A total of 248 MS and 157 SVD lesions were studied. Individual MS lesions demonstrated significant decreases in volume 3 (p = 0.04), greater shifts in 3D displacement by 23.4% with increasing duration between MRI time points (p = 0.007), and greater transitions to a more non-spherical shape (p 2.49 based on deviation from a perfect 3D sphere, a 92.7% in-sample and 91.2% out-of-sample accuracy was identified for the diagnosis of MS. Longitudinal 3D shape evolution and displacement characteristics may improve lesion classification, adding to MRI techniques aimed at improving lesion specificity.

Objective: Diagnosis of supratentorial space occupying lesion is a frequent dilemma in neurosurgical practice. MR spectroscopy that is an advanced MR imaging sequence can prove to be decisive in such cases. The objective of the study is to determine diagnostic accuracy of MRS in differentiating supra-tentorial brain space occupying lesions taking histopathology as gold standard Material and Methods: The cross sectional study was conducted at Department of Neurosurgery, Jinnah Postgraduate Medical Centre, Karachi from August 2018 to July 2019. A calculated sample size of 156 patients aged between 18-50 years after informed consent. All patients underwent MR Spectroscopy pre-operatively. Surgery was performed and histopathological findings were compared with spectroscopic findings. Results: The average age of the patients was 32.41 ± 10.02 years. There were 84 (53.84%) males and 72 (46.15%) females. The sensitivity of MRS was 90.69% with a specificity of 97.34%. Conclusion: MR Spectroscopy is an accurate diagnostic tool for establishing pre-operative differentiation between neoplastic and inflammatory lesion. It should be performed in all cases where contrast MRI is inconclusive in establishing the diagnosis. Keywords: , Brain Abscess, Supratentorial lesion, Brain Tumor

Developmental venous anomalies (DVAs) are extreme anatomical venous variations formed by multiple radiating medullary veins, which converge centripetally into a single collecting vein. Their coexistence with symptomatic cavernous malformations (CMs) has been reported in the literature. The aim of this study was to assess the characteristics of DVAs using MRI. A total of 6948 head MRIs of adult Caucasian patients were retrospectively analyzed to determine the number and locations of DVAs. We collected the data on the termination of the collecting vein, the prevalence of DVA-related CMs, and MRI FLAIR signal-hyperintensity corresponding to the location of the DVA. At least one DVA was identified in 7.46% of the patients. The prevalence decreased with age, with a Pearson correlation coefficient of − 0.7328. A total of 599 DVAs were identified. Multiple DVAs were found in 10.92% of the patients with DVAs. The DVAs were identified more often in the supratentorial region (73.12%, p

Background Childhood infratentorial tumor patients frequently suffer from long-term cognitive deficits. As each constituent of their treatment can lead to neurotoxicity, cascade effects can lead to profound reorganization of the underlying brain network, the so-called ‘connectome’. However, to date, few studies have assessed the relationship between brain network topology, the functional role of network hubs (i.e. highly connected regions), and neurocognitive outcomes in adult survivors of childhood infratentorial tumors. Methods In this cross-sectional study, childhood infratentorial tumor survivors (n = 21: pilocytic astrocytoma (n = 8), ependymoma (n = 1) and medulloblastoma (n = 12)) and healthy controls (n = 21) were recruited. Using multishell diffusion-weighted MRI, microstructural organization and topology of supratentorial white matter was investigated; using a voxel-based approach, a fixel-based analysis, and a graph theoretical approach. In addition, neurocognitive subscales of the WAIS-IV intelligence test, and their relationship with nodal strength and network efficiency metrics were assessed. Results Similar to earlier studies, we observed widespread decreases in fractional anisotropy (FA) in patients compared to controls, based on voxel-based analyses. In addition, the fixel-based analyses dissociated macro- from microstructural changes, which were encountered in in infratentorial versus supratentorial brain areas, respectively. Finally, regional reorganization (i.e. differences in local efficiency) occurred mainly in hubs, which suggests a specific vulnerability of these areas. These hubs were not only mostly affected, but also most strongly correlated with the intelligence subscales. Conclusion This study suggests that network hubs are functionally important for intellectual outcomes in infratentorial tumor survivors. Furthermore, these regions could be the primary targets of treatment toxicity. Validation of this specific hypothesis in larger samples is required.

In general, Glioblastomas multiforme frequently occurs in supratentorial region but in less than 4% occur in the posterior fossa predominantly in the cerebellum, and spinal cords tumors represents 6% to 8% of all central nervous system tumors and spinal cord glioblastomas occur only in 1.5% of all spinal cords tumors. We presents two cases a male whit posterior fossa glioblastoma and a girl with spinal cord glioblastoma in both cases, MRI is the study of choice with high sensitivity and specificity for diagnosis, after that they underwent biopsy and the results of pathology described GBM in both cases. The treatment of election is radiotherapy plus chemotherapy and surge if is possible, the prognosis is poor even with treatment, thats is why we need to identify new therapeutic strategies; We currently believe it is necessary to use genetic platforms to identify possible therapeutic targets.

Introduction: Very preterm infants are at risk for adverse neurodevelopmental outcome. To better identify children without brain injury at risk for developmental sequelae, we assessed predictive values of supratentorial brain metrics in relation to outcome. Methods: Very preterm infants underwent magnetic resonance imaging (MRI) at term-equivalent age. Infants with any grade of supra- or infratentorial brain injury according to Kidokoro et al. [Pediatrics 2014;134:e444–53] were excluded. Supratentorial brain metrics (biparietal width, extracerebral space, interhemispheric distance) were measured and categorised using existing cut-off values. The Psychomotor Developmental Index (PDI) and Mental Developmental Index (MDI) were assessed using the Bayley Scales of Infant Development, second and third edition, at 2 years of age. Developmental delay was defined as a score Results: A total of 237 very preterm infants were enrolled. Of all infants, 59 (21.2%) showed developmental delay. Infants with z-scores less than –0.5 for biparietal width had significantly lower PDI (p = 0.039) and MDI (p = 0.042) than infants with normal z-scores. Enlargement of extracerebral spaces was also related to lower PDI (p = 0.047) and MDI (p = 0.036). Negative predictive value was highest when all brain metrics were within the normal range (PDI Discussion: Supratentorial brain metrics are predictive for neurodevelopmental outcome in infants with ostensibly normal MRI. A combination of supratentorial brain metrics is most meaningful for identifying infants at risk for long-term sequelae.

Introduction Young age is an adverse prognostic factor in children with ependymomas. Treatment of these infants is challenging since beneficial therapeutic options are limited. As ependymomas are considered a biologically heterogeneous group, we aimed to characterize infant ependymomas with regard to their histological and genetic features. Materials and methods We analyzed 28 ependymomas occurring in children younger than 18 months at diagnosis enrolled into the HIT2000-E protocols with the aim to postpone irradiation until the age of 18 months if possible. All cases underwent neuropathological review, including immunohistochemical characterization. Genome-wide copy number alterations (CNA) were assessed by molecular inversion probe assays, and RELA and YAP1 fusions were detected by RT-PCR and sequencing. Results All infant ependymomas were anaplastic (WHO grade III). Twenty-one (75%) cases were located in the posterior fossa. Gross total resection was accomplished in 12 (57%) of these cases. All posterior fossa tumors showed loss of H3-K27me3 characteristic of PFA ependymomas. CNA analysis showed a stable genome in all cases with lack of chromosome 1q gain, an adverse prognostic marker in PFA ependymomas of older children. However, after a median follow-up of 5.4 years, 15 (71%) relapsed, and 9 (43%) died. Seven ependymomas (25%) occurred in the supratentorial region. Gross total resection could be achieved in only two of these cases. Four tumors carried C11orf95-RELA fusions, and two cases had typical YAP1-MAMLD1 fusions (one case was not analyzable). The RELA-fused cases did not display CDKN2A loss as an adverse indicator of prognosis in this disease entity. Although three infants (43%) with supratentorial ependymomas relapsed, all patients survived (median follow-up, 8.0 years). Conclusion Infant ependymomas seem to fall into three biological entities, with supratentorial tumors carrying RELA or YAP fusions and PFA posterior fossa ependymomas. The latter showed a poor outcome even though chromosome 1q gain was absent.

Utilization of intraoperative neurophysiology (ION) to map and assess various functions during supratentorial brain tumor and epilepsy surgery is well documented and commonplace in the adult setting. The applicability has yet to be established in the pediatric age group. All pediatric supratentorial surgery utilizing ION of the motor system, completed over a period of 10 years, was analyzed retrospectively for the following variables: preoperative and postoperative motor deficits, extent of resection, sensory-motor mappability and monitorability, location of lesion, patient age, and monitoring alarms. Intraoperative findings were correlated with antecedent symptomatology as well as short- and long-term postoperative clinical outcome. The monitoring impact on surgical course was evaluated on a per-case basis. Data were analyzed for 57 patients (ages 3–207 months (93 ± 58)). Deep lesions (in proximity to the pyramidal fibers) constituted 15.7% of the total group, superficial lesions 47.4%, lesions with both deep and superficial components 31.5%, and ventricular 5.2%. Mapping of the motor cortex was significantly more successful using the short-train technique than Penfield’s technique (84% vs. 25% of trials, respectively), particularly in younger children. The youngest age at which motor mapping was successfully achieved was 3 vs. 93 months for each method, respectively. Preoperative motor strength was not associated with monitorability. Direct cortial motor evoked potential (dcMEP) was more sensitive than transcranial (tcMEP) in predicting postoperative motor decline. dcMEP decline was not associated with tumor grade or extent of resection (EOR); however, it was associated with lesion location and more prone to decline in deep locations. ION actively affected surgical decisions in several aspects, such as altering the corticectomy location and alarming due to a MEP decline. ION is applicable in the pediatric population with certain limitations, depending mainly on age. When successful, ION has a positive impact on surgical decision-making, ultimately providing an added element of safety for these patients.