Background:Adult medulloblastoma(MB)represents less than 1%of central nervous system malignancies,lacking standardized therapeutic approaches due to its rarity.This retrospective single-center analysis aimed to assess...Background:Adult medulloblastoma(MB)represents less than 1%of central nervous system malignancies,lacking standardized therapeutic approaches due to its rarity.This retrospective single-center analysis aimed to assess survival outcomes and treatment-associated toxicities in adult MB patients managed with pediatric-derived protocols.Methods:Eighteen patients(≥18 years)with MB treated at Fondazione Policlinico Universitario Agostino Gemelli Istituto di Ricovero e Cura a Carattere Scientifico(IRCCS)(January 1997–January 2024)were analyzed.All received craniospinal radiotherapy with posterior fossa boost,followed by adjuvant chemotherapy utilizing pediatric regimens(PNET3,PNET4,PNET5,or high-risk protocols incorporating high-dose chemotherapy with autologous stem cell rescue).Primary outcomes included overall survival(OS)and progression-free survival(PFS).Secondary analyses focused on comprehensive toxicity assessment.Results:The cohort included 11 males and 7 females(median age:23 years).Metastatic disease was present in 6 patients(33%)at diagnosis.Histopathological distribution showed classic MB(55.5%),desmoplastic/nodular(39%),and large cell/anaplastic variants(5.5%).Molecular subgrouping(available in 6 patients)identified SHH subgroup in four cases and WNT subgroup in two.Three-year and fiveyear overall survival rates reached 94.5%and 88.8%,respectively.Treatment-related adverse events included grade 3–4 hematologic toxicities,clinically significant weight loss,and grade≥3 neurological and ototoxic complications.These toxicities necessitated treatment modifications including dose adjustments,cycle delays,and occasional early discontinuation.Conclusions:Adult MB patients treated with pediatric-adapted protocols demonstrated excellent long-termsurvival outcomes,comparable to or surpassing historical data.Despite frequent toxicity requiring treatment modifications,these regimens proved feasible with acceptable risk-benefit profiles.These results support implementing modified pediatric protocols for adult MB management.Future multicenter investigations with larger cohorts are essential for refining risk stratification,optimizing treatment intensity,and evaluating long-term outcomes in this rare malignancy.展开更多
Objective: Aim of the study was to assess the duration of preoperative external ventricular drain (EVD) as a predictor for permanent cerebrospinal fluid (CSF) diversion in pediatric posterior fossa tumors. Methods: Th...Objective: Aim of the study was to assess the duration of preoperative external ventricular drain (EVD) as a predictor for permanent cerebrospinal fluid (CSF) diversion in pediatric posterior fossa tumors. Methods: The study was conducted in the Department of Pediatric Neurosurgery, National Neuroscience Institute, King Fahad Medical City, Riyadh, Saudi Arabia from January 2010 to December 2013. The data were collected retrospectively. The patients who had emergency insertion of external ventricular drain (EVD) due to hydrocephalus before the definitive posterior fossa tumor surgery were included in the study. Results: The preoperative emergency insertion of EVD was done in 38 patients with pediatric posterior fossa tumors. The patients were divided into two groups. Group A consists of those patients who had duration of preoperative EVD equal or less than 7 days. Group B includes those patients who had duration of preoperative EVD more than 7 days. Group A has 16 patients while Group B has 22 patients. The predominant clinical feature was symptoms and signs of raised intracranial pressure (ICP) only. Infection related to EVD was seen in seven patients. Ventriculoperitoneal (VP) shunt was required in 31.25% of Group A patients and 18.18% of Group B patients (p value = 0.35). Overall shunt rate was 23.68%. Conclusion: There are well known number of factors that can determine the need of permanent CSF diversion in patients with posterior fossa tumors. However, there is no effect of preoperative duration of EVD in determining the requirement of postoperative VP shunt.展开更多
Background:This study aimed to evaluate the effectiveness of using animation as education material for family empowerment program on perioperative care for caregivers whose children were to undergo neurosurgery.Method...Background:This study aimed to evaluate the effectiveness of using animation as education material for family empowerment program on perioperative care for caregivers whose children were to undergo neurosurgery.Methods:A total of 204 caregivers were randomly assigned to either the face‐to‐face oral nursing educated group(Oral Group)or the animation‐assisted nursing educated group(Animated Group).The nursing education primarily focused on instructing caregivers about the manual vibration method.The primary outcome of interest in this study was participants'knowledge level,collected by a 10‐item questionnaire.Secondary outcomes included child patients'clinical data,including hospitalization days,treatments,and signs of pneumonia.Results:Participants in the Animated Group exhibited significantly higher accuracy in perioperative care knowledge assessment,and patients in this group had a lower chance of requiring atomization therapy compared to the Oral Group.Conclusions:The animation‐assisted nursing education program effectively enhances pediatric caregivers'knowledge,reduces respiratory complications after surgery,and offers valuable insights for future studies on the use of such programs to instruct caregivers.展开更多
Long-term levodopa administration can lead to the development of levodopa-induced dyskinesia.Gamma oscillations are a widely recognized hallmark of abnormal neural electrical activity in levodopa-induced dyskinesia.Cu...Long-term levodopa administration can lead to the development of levodopa-induced dyskinesia.Gamma oscillations are a widely recognized hallmark of abnormal neural electrical activity in levodopa-induced dyskinesia.Currently,studies have reported increased oscillation power in cases of levodopa-induced dyskinesia.However,little is known about how the other electrophysiological parameters of gamma oscillations are altered in levodopa-induced dyskinesia.Furthermore,the role of the dopamine D3 receptor,which is implicated in levodopa-induced dyskinesia,in movement disorder-related changes in neural oscillations is unclear.We found that the cortico-striatal functional connectivity of beta oscillations was enhanced in a model of Parkinson’s disease.Furthermore,levodopa application enhanced cortical gamma oscillations in cortico-striatal projections and cortical gamma aperiodic components,as well as bidirectional primary motor cortex(M1)↔dorsolateral striatum gamma flow.Administration of PD128907(a selective dopamine D3 receptor agonist)induced dyskinesia and excessive gamma oscillations with a bidirectional M1↔dorsolateral striatum flow.However,administration of PG01037(a selective dopamine D3 receptor antagonist)attenuated dyskinesia,suppressed gamma oscillations and cortical gamma aperiodic components,and decreased gamma causality in the M1→dorsolateral striatum direction.These findings suggest that the dopamine D3 receptor plays a role in dyskinesia-related oscillatory activity,and that it has potential as a therapeutic target for levodopa-induced dyskinesia.展开更多
Neural stem cell transplantation is a useful treatment for ischemic stroke, but apoptosis often occurs in the hypoxic-ischemic environment of the brain after cell transplantation. In this study, we determined if mild ...Neural stem cell transplantation is a useful treatment for ischemic stroke, but apoptosis often occurs in the hypoxic-ischemic environment of the brain after cell transplantation. In this study, we determined if mild hypothermia （27-28~C） can increase the survival rate of neural stem cells （1.0 x 105/~tL） transplanted into neonatal mice with hypoxic-ischemic encephalopathy. Long-term effects on neurological functioning of the mice were also examined. After mild hy- pothermia combined with neural stem cell transplantation, we observed decreased expression levels of inflammatory factor nuclear factor-kappa B and apoptotic factor caspase-3, reduced cerebral infarct volumes, increased survival rate of transplanted cells, and marked improvements in neurological function. Thus, the neuroprotective effects of mild hypothermia combined with neural stem cell transplantation are superior to those of monotherapy. Moreover, our findings suggest that the neuroprotective effects of mild hypothermia combined with neural stem cell transplantation on hypoxic-ischemic encephalopathy are achieved by anti-inflammatory and an- ti-apoptotic mechanisms.展开更多
Post traumatic dural sinus vein stenosis has been rarely described in pediatric population. We present a case of a 9-year-old child that had sustained a head injury after a fall from height causing an acute epidural h...Post traumatic dural sinus vein stenosis has been rarely described in pediatric population. We present a case of a 9-year-old child that had sustained a head injury after a fall from height causing an acute epidural hematoma compressing the dominant sigmoid sinus. The patient had developed sub acutely signs and symptoms of increased intracranial pressure. Prophylactic treatment with anticoagulants was initiated despite the presence of an intracranial bleeding. Clinical and radiological improvements were achieved. We had also reviewed the literature regarding this uncommon entity and discussed other existing diagnostic and therapeutic alternatives. Further gathering of information is essential in order to form a therapeutic protocol.展开更多
Perinatal brain injury（PBI）is one of the most important causes of lifelong deficits in cognition,behavior,social interaction and motor skills,as well as epilepsy.PBI encompasses,but is not limited to,hypoxic-ischemi...Perinatal brain injury（PBI）is one of the most important causes of lifelong deficits in cognition,behavior,social interaction and motor skills,as well as epilepsy.PBI encompasses,but is not limited to,hypoxic-ischemic encephalopathy（HIE）,intraventricular hemorrhage,periventricular leukomalacia and encephalopathy of prematurity.展开更多
Myelomeningocele(MMC)is a severe form of spinal dysraphism.Due to the failure of neural tube closure during early embryonic development,the affected part of the spinal cord is left open like a book at the back of the ...Myelomeningocele(MMC)is a severe form of spinal dysraphism.Due to the failure of neural tube closure during early embryonic development,the affected part of the spinal cord is left open like a book at the back of the affected child.This malformed part of the spinal cord is not covered by its protective mesodermal and ectodermal derived layers.Consequently,the exposed neural tissue(i.e.,the neural placode)is prone to injuryduring further intra-uterine development.展开更多
Introduction: teratomas are non-malignantgerm cell tumors commonly composed of cell types derived from all of the three germ layers. Intracerebral teratomas typically are midline or paraxial lesions located in the pit...Introduction: teratomas are non-malignantgerm cell tumors commonly composed of cell types derived from all of the three germ layers. Intracerebral teratomas typically are midline or paraxial lesions located in the pituitary stalk or the pineal region. We report our experience with mature teratomas of the posterior fossa. Case report: we present the case of an 11-year-old caucasian female with progressive headache that caused interrupted sleep. Cerebral magnetic resonance imaging showed a midline lesion in the posterior fossa with mass effect and without contrast enhancement. Anatomic pathology revealed a mature teratoma. Conclusion: differential diagnosis of midline lesions in pediatric patients must include teratomas in spite of being posterior fossa lesions.展开更多
Meningiomas occur in 1%-4% of primary intracranial tumors in the pediatric group, and is increasing in incidence with age. Some authors have reported that meningioma is more prevalent among adult males, but there is n...Meningiomas occur in 1%-4% of primary intracranial tumors in the pediatric group, and is increasing in incidence with age. Some authors have reported that meningioma is more prevalent among adult males, but there is no gender prevalence. The accepted origin of meningiomas is from the arachnoid ceils lining the meninges, or the choroid plexuses. Since Beckwith and Palmer introduced the term ＇rhabdoid tumor＇ in 1978 in reference to a subgroup of childhood malignant renal tumors, many tumors with a rhabdoid morphology have been reported in various sites, including the central nervous system. In 1998 Kepes et al.展开更多
Hypothalamic hamartoma(HH) is usually associated with refractory epilepsy, cognitive impairment, and behavioral disturbance. There is now increasing evidence that HH can be treated effectively with a variety of neuros...Hypothalamic hamartoma(HH) is usually associated with refractory epilepsy, cognitive impairment, and behavioral disturbance. There is now increasing evidence that HH can be treated effectively with a variety of neurosurgical approaches. Treatment options for intractable gelastic seizure in HH patients include direct open surgery with craniotomy, endoscopic surgery, radiosurgery with gamma knife and stereotactic radiofrequency thermocoagulation. Selection of treatment modalities depends on type and size of the HH and the surgeon's preference. Two surgical techniques, resection and disconnection, had been described with favorable outcomes. Pretreatmentevaluation, patient selection, surgical techniques, complications, and possible selection of treatment are discussed.展开更多
Patients with brain tumors,specifically,malignant forms such as glioblastoma,medulloblastoma and ependymoma,exhibit dismal survival rates despite advances in treatment strategies.Chemotherapeutics,the primary adjuvant...Patients with brain tumors,specifically,malignant forms such as glioblastoma,medulloblastoma and ependymoma,exhibit dismal survival rates despite advances in treatment strategies.Chemotherapeutics,the primary adjuvant treatment for human brain tumors following surgery,commonly lack efficacy due to either intrinsic or acquired drug resistance.New treatments targeting epigenetic factors are being explored.Post-translational histone modification provides a critical regulatory platform for processes such as chromosome condensation and segregation,apoptosis,gene transcription,and DNA replication and repair.This work reviews how aberrant histone modifications and alterations in histone-modifying enzymes can drive the acquisition of drug resistance in brain tumors.Elucidating these mechanisms should lead to new treatments for overcoming drug resistance.展开更多
Pantothenate kinase-associated neurodegeneration (PKAN)-causes a progressive generalized dystonia which remains pharmacologically intractable. We performed bilateral internal globus pallidus stimulation in six patient...Pantothenate kinase-associated neurodegeneration (PKAN)-causes a progressive generalized dystonia which remains pharmacologically intractable. We performed bilateral internal globus pallidus stimulation in six patients with genetically confirmed PKAN who obtained a major and long-lasting improvement of their painf ul spasms, dystonia, and functional autonomy. This study shows the benefits of p allidal DBS for the dystonia of PKAN patients.展开更多
Background:Current protein biomarkers are only moderately predictive at identifying individuals with mild traumatic brain injury or concussion.Therefore,more accurate diagnostic markers are needed for sport-related co...Background:Current protein biomarkers are only moderately predictive at identifying individuals with mild traumatic brain injury or concussion.Therefore,more accurate diagnostic markers are needed for sport-related concussion.Methods:This was a multicenter,prospective,case-control study of athletes who provided blood samples and were diagnosed with a concussion or were a matched non-concussed control within the National Collegiate Athletic Association-Department of Defense Concussion Assessment,Research,and Education Consortium conducted between 2015 and 2019.The blood was collected within 48 h of injury to identify protein abnormalities at the acute and subacute timepoints.Athletes with concussion were divided into 6 h post-injury(0-6 h post-injury)and after 6 h postinjury(7-48 h post-injury)groups.We applied a highly multiplexed proteomic technique that used a DNA aptamers assay to target 1305proteins in plasma samples from athletes with and without sport-related concussion.Results:A total of 140 athletes with concussion(79.3%males;aged 18.71±1.10 years,mean±SD)and 21 non-concussed athletes(76.2%males;19.14±1.10 years)were included in this study.We identified 338 plasma proteins that significantly differed in abundance(319 upregulated and 19 downregulated)in concussed athletes compared to non-concussed athletes.The top 20 most differentially abundant proteins discriminated concussed athletes from non-concussed athletes with an area under the curve(AUC)of 0.954(95%confidence interval:0.922-0.986).Specifically,after 6 h of injury,the individual AUC of plasma erythrocyte membrane protein band 4.1(EPB41)and alpha-synuclein(SNCA)were 0.956 and 0.875,respectively.The combination of EPB41 and SNCA provided the best AUC(1.000),which suggests this combination of candidate plasma biomarkers is the best for diagnosing concussion in athletes after 6 h of injury.Conclusion:Our data suggest that proteomic profiling may provide novel diagnostic protein markers and that a combination of EPB41 and SNCA is the most predictive biomarker of concussion after 6 h of injury.展开更多
Objective:There is a paucity of real-world evidence in the Indian context to address the outcome of primary brain tumors(PBTs)in children.This study aimed to describe the demographic profile,clinical characteristics,a...Objective:There is a paucity of real-world evidence in the Indian context to address the outcome of primary brain tumors(PBTs)in children.This study aimed to describe the demographic profile,clinical characteristics,and histological features of PBTs based on the 2016 World Health Organization classification,assess the efficacy of treatment methods,and identify the factors that influence the outcome.Methodology:This is a single-institution,hospital-based study.Data were collected for pediatric patients aged 0−19 years,from September 2001 to May 2023(22 years),who were diagnosed with malignant PBTs.Patients with radiologically or histologically proven tumors were included.Those with metastatic disease to the central nervous system were excluded.The overall survival(OS)and recurrence-free survival(RFS)were estimated using the Kaplan–Meier method.Results:A total of 251 patients with pediatric brain tumors were included in this analysis.The mean age was 9.10±5.54 years.The male-to-female ratio was 1.20:1.In this cohort,the most common histologies were medulloblastoma and astrocytoma.The mean survival of all patients with PBTs was 141.00±7.90 months with 1-,3-,and 8-year OS rates of 79.00%,67.00%,and 60.00%,respectively.Medulloblastoma had 1-,3-,and 8-year OS rates of 81.00%,72.00%,and 65.00%,respectively.The 1-year OS rates for glioblastoma and brainstem glioma were 46.00%and 45.00%,respectively.Complete tumoral resection showed longer survival than lesser degrees of resection(p=0.001).Embryonal tumors(ETs)had a better RFS of 133.60±12.70 months(p≤0.001).Conclusion:ETs have a better prognosis than glial tumors.With an improved OS,the surgical resection extent has a favorable outcome.As a chemosensitive tumor,medulloblastoma benefits most from systemic treatment and responds well to a multimodal approach.展开更多
Background:Meningiomas are rather uncommon tumors in the pediatric population,differing significantly from those found in adults by their atypical location,higher rate of more malignant types,consequently higher risk ...Background:Meningiomas are rather uncommon tumors in the pediatric population,differing significantly from those found in adults by their atypical location,higher rate of more malignant types,consequently higher risk of recurrence and a less favorable outcome.Even in children,suprasellar meningiomas without dural matrix are rare findings mimicking more common suprasellar lesions.Case presentation:Here we describe a case of a 12-year-old girl who presented with a rapidly progressing chiasmal syndrome and was diagnosed by MRI with an unusual suprasellar tumor that could not fit the diagnoses expected in a case of a parasellar mass in a child,similar to a craniopharyngioma or optic pathway glioma.After multiple clinical investigations,the tumor etiology was still unclear,so the preferred option of treatment was surgical resection.An endoscope-assisted gross total resection through a supraorbital keyhole approach was performed uneventfully,with total vision recovery in a short time.Benign meningiomas located in the skull base without dural attachment appear to be rare,even in pediatric patients.Conclusion:Differential diagnoses of suprasellar and para sellar tumor lesions in pediatric patients can be confusing.There are peculiar features of pediatric tumor diseases that should be considered while working out the management strategy.The main principle of meningioma treatment is the highest possible extent of resection minimally affecting the quality of life.展开更多
Background:Pediatric epidural hematomas(EDH)represent a neurosurgical emergency.Both surgical and conservative treatment can lead to a good clinical outcome.The aim of the study was to review our series of pediatric E...Background:Pediatric epidural hematomas(EDH)represent a neurosurgical emergency.Both surgical and conservative treatment can lead to a good clinical outcome.The aim of the study was to review our series of pediatric EDH and to determine the clinical and radiologic factors,which can influence the final outcome.Methods:All children aged from 0 to 16 that have been treated between 2013 and 2017 for cranial EDH have been selected.Results:Thirty children have been included in the study.Seventeen cases have been treated with surgical evacuation and 13 conservatively.Six months after the trauma,the outcome was excellent(mRS 0)in 25/30(83.3%)cases,mild deficits(mRS 1-2)were present in 4/30(13.3%),and severe deficits(mRS 3-5)in 1/30(3.3%)cases.Only a GCS(Glasgow Coma Scale)below 8 at admission was significantly related to the presence of a neurologic deficit at 6 months(p=0.048).Conclusions:EDH can be managed with excellent outcomes.Even in the presence of bad initial clinical and radiologic conditions,a correct treatment strategy can lead to a good recovery.In our series,only a GCS below 8 at admission was significantly related to the presence of neurological sequelae.展开更多
Gliomas are the most common primary neuroepithelial tumors of the central nervous system in adults,of which glioblastoma is the deadliest subtype.Apart from the intrinsically indestructible characteristics of glioma(s...Gliomas are the most common primary neuroepithelial tumors of the central nervous system in adults,of which glioblastoma is the deadliest subtype.Apart from the intrinsically indestructible characteristics of glioma(stem)cells,accumulating evidence suggests that the tumor microenvironment also plays a vital role in the refractoriness of glioblastoma.The primary functions of platelets are to stop bleeding and regulate thrombosis under physiological conditions.Furthermore,platelets are also active elements that participate in a variety of processes of tumor development,including tumor growth,invasion,and chemoresistance.Glioma cells recruit and activate resting platelets to become tumor-educated platelets(TEPs),which in turn can promote the proliferation,invasion,stemness,and chemoresistance of glioma cells.TEPs can be used to obtain genetic information about gliomas,which is helpful for early diagnosis and monitoring of therapeutic effects.Platelet membranes are intriguing biomimetic materials for developing efficacious drug carriers to enhance antiglioma activity.Herein,we review the recent research referring to the contribution of platelets to the malignant characteristics of gliomas and focusing on the molecular mechanisms mediating the interaction between TEPs and glioma(stem)cells,as well as present the challenges and opportunities in targeting platelets for glioma therapy.展开更多
Objective：This overview seeked to bring together the microRNA （miRNA） researches on biogenesis and bio-function in these areas of clinical diagnosis and therapy for malignant glioma.Data Sources：Using the keyword ...Objective：This overview seeked to bring together the microRNA （miRNA） researches on biogenesis and bio-function in these areas of clinical diagnosis and therapy for malignant glioma.Data Sources：Using the keyword terms ＂glioma＂ and ＂miRNA,＂ we performed the literature search in PubMed,Ovid,and web.metstr.com databases from their inception to October 2014.Study Selection：In screening out the quality of the articles,factors such as clinical setting of the study,the size of clinical samples were taken into consideration.Animal studied for verification and reviews article were also included in our data collection.Results：Despite many advance in miRNA for malignant glioma,further studies were still required to focus on the following aspects：（i） Improving the understanding about biogenesis of miRNA and up-down regulation;（ii） utilizing high-throughput miRNA expression analysis to screen out the core miRNA for glioma;（iii） Focusing related miRNAs on the signal transduction pathways that regulate the proliferation and growth of glioma.Conclusions：We discussed the most promising miRNA,correlative signaling pathway and their relation with gliomas in the way of prompting miRNA target into being a clinical therapeutic strategy.展开更多
基金approved by the Institutional Review Board of Università Cattolica del Sacro Cuore-Policlinico Gemelli IRCCS(number DIPUSVSP-21-03-252).
文摘Background:Adult medulloblastoma(MB)represents less than 1%of central nervous system malignancies,lacking standardized therapeutic approaches due to its rarity.This retrospective single-center analysis aimed to assess survival outcomes and treatment-associated toxicities in adult MB patients managed with pediatric-derived protocols.Methods:Eighteen patients(≥18 years)with MB treated at Fondazione Policlinico Universitario Agostino Gemelli Istituto di Ricovero e Cura a Carattere Scientifico(IRCCS)(January 1997–January 2024)were analyzed.All received craniospinal radiotherapy with posterior fossa boost,followed by adjuvant chemotherapy utilizing pediatric regimens(PNET3,PNET4,PNET5,or high-risk protocols incorporating high-dose chemotherapy with autologous stem cell rescue).Primary outcomes included overall survival(OS)and progression-free survival(PFS).Secondary analyses focused on comprehensive toxicity assessment.Results:The cohort included 11 males and 7 females(median age:23 years).Metastatic disease was present in 6 patients(33%)at diagnosis.Histopathological distribution showed classic MB(55.5%),desmoplastic/nodular(39%),and large cell/anaplastic variants(5.5%).Molecular subgrouping(available in 6 patients)identified SHH subgroup in four cases and WNT subgroup in two.Three-year and fiveyear overall survival rates reached 94.5%and 88.8%,respectively.Treatment-related adverse events included grade 3–4 hematologic toxicities,clinically significant weight loss,and grade≥3 neurological and ototoxic complications.These toxicities necessitated treatment modifications including dose adjustments,cycle delays,and occasional early discontinuation.Conclusions:Adult MB patients treated with pediatric-adapted protocols demonstrated excellent long-termsurvival outcomes,comparable to or surpassing historical data.Despite frequent toxicity requiring treatment modifications,these regimens proved feasible with acceptable risk-benefit profiles.These results support implementing modified pediatric protocols for adult MB management.Future multicenter investigations with larger cohorts are essential for refining risk stratification,optimizing treatment intensity,and evaluating long-term outcomes in this rare malignancy.
文摘Objective: Aim of the study was to assess the duration of preoperative external ventricular drain (EVD) as a predictor for permanent cerebrospinal fluid (CSF) diversion in pediatric posterior fossa tumors. Methods: The study was conducted in the Department of Pediatric Neurosurgery, National Neuroscience Institute, King Fahad Medical City, Riyadh, Saudi Arabia from January 2010 to December 2013. The data were collected retrospectively. The patients who had emergency insertion of external ventricular drain (EVD) due to hydrocephalus before the definitive posterior fossa tumor surgery were included in the study. Results: The preoperative emergency insertion of EVD was done in 38 patients with pediatric posterior fossa tumors. The patients were divided into two groups. Group A consists of those patients who had duration of preoperative EVD equal or less than 7 days. Group B includes those patients who had duration of preoperative EVD more than 7 days. Group A has 16 patients while Group B has 22 patients. The predominant clinical feature was symptoms and signs of raised intracranial pressure (ICP) only. Infection related to EVD was seen in seven patients. Ventriculoperitoneal (VP) shunt was required in 31.25% of Group A patients and 18.18% of Group B patients (p value = 0.35). Overall shunt rate was 23.68%. Conclusion: There are well known number of factors that can determine the need of permanent CSF diversion in patients with posterior fossa tumors. However, there is no effect of preoperative duration of EVD in determining the requirement of postoperative VP shunt.
基金Technology Foundation Project of Shanghai Jiaotong University School of Medicine,Grant/Award Number:Jyhz2015。
文摘Background:This study aimed to evaluate the effectiveness of using animation as education material for family empowerment program on perioperative care for caregivers whose children were to undergo neurosurgery.Methods:A total of 204 caregivers were randomly assigned to either the face‐to‐face oral nursing educated group(Oral Group)or the animation‐assisted nursing educated group(Animated Group).The nursing education primarily focused on instructing caregivers about the manual vibration method.The primary outcome of interest in this study was participants'knowledge level,collected by a 10‐item questionnaire.Secondary outcomes included child patients'clinical data,including hospitalization days,treatments,and signs of pneumonia.Results:Participants in the Animated Group exhibited significantly higher accuracy in perioperative care knowledge assessment,and patients in this group had a lower chance of requiring atomization therapy compared to the Oral Group.Conclusions:The animation‐assisted nursing education program effectively enhances pediatric caregivers'knowledge,reduces respiratory complications after surgery,and offers valuable insights for future studies on the use of such programs to instruct caregivers.
基金supported by the National Natural Science Foundation of China,No.82071254(to WZ).
文摘Long-term levodopa administration can lead to the development of levodopa-induced dyskinesia.Gamma oscillations are a widely recognized hallmark of abnormal neural electrical activity in levodopa-induced dyskinesia.Currently,studies have reported increased oscillation power in cases of levodopa-induced dyskinesia.However,little is known about how the other electrophysiological parameters of gamma oscillations are altered in levodopa-induced dyskinesia.Furthermore,the role of the dopamine D3 receptor,which is implicated in levodopa-induced dyskinesia,in movement disorder-related changes in neural oscillations is unclear.We found that the cortico-striatal functional connectivity of beta oscillations was enhanced in a model of Parkinson’s disease.Furthermore,levodopa application enhanced cortical gamma oscillations in cortico-striatal projections and cortical gamma aperiodic components,as well as bidirectional primary motor cortex(M1)↔dorsolateral striatum gamma flow.Administration of PD128907(a selective dopamine D3 receptor agonist)induced dyskinesia and excessive gamma oscillations with a bidirectional M1↔dorsolateral striatum flow.However,administration of PG01037(a selective dopamine D3 receptor antagonist)attenuated dyskinesia,suppressed gamma oscillations and cortical gamma aperiodic components,and decreased gamma causality in the M1→dorsolateral striatum direction.These findings suggest that the dopamine D3 receptor plays a role in dyskinesia-related oscillatory activity,and that it has potential as a therapeutic target for levodopa-induced dyskinesia.
基金supported by the National Natural Science Foundation of China,No.81271382
文摘Neural stem cell transplantation is a useful treatment for ischemic stroke, but apoptosis often occurs in the hypoxic-ischemic environment of the brain after cell transplantation. In this study, we determined if mild hypothermia （27-28~C） can increase the survival rate of neural stem cells （1.0 x 105/~tL） transplanted into neonatal mice with hypoxic-ischemic encephalopathy. Long-term effects on neurological functioning of the mice were also examined. After mild hy- pothermia combined with neural stem cell transplantation, we observed decreased expression levels of inflammatory factor nuclear factor-kappa B and apoptotic factor caspase-3, reduced cerebral infarct volumes, increased survival rate of transplanted cells, and marked improvements in neurological function. Thus, the neuroprotective effects of mild hypothermia combined with neural stem cell transplantation are superior to those of monotherapy. Moreover, our findings suggest that the neuroprotective effects of mild hypothermia combined with neural stem cell transplantation on hypoxic-ischemic encephalopathy are achieved by anti-inflammatory and an- ti-apoptotic mechanisms.
文摘Post traumatic dural sinus vein stenosis has been rarely described in pediatric population. We present a case of a 9-year-old child that had sustained a head injury after a fall from height causing an acute epidural hematoma compressing the dominant sigmoid sinus. The patient had developed sub acutely signs and symptoms of increased intracranial pressure. Prophylactic treatment with anticoagulants was initiated despite the presence of an intracranial bleeding. Clinical and radiological improvements were achieved. We had also reviewed the literature regarding this uncommon entity and discussed other existing diagnostic and therapeutic alternatives. Further gathering of information is essential in order to form a therapeutic protocol.
文摘Perinatal brain injury（PBI）is one of the most important causes of lifelong deficits in cognition,behavior,social interaction and motor skills,as well as epilepsy.PBI encompasses,but is not limited to,hypoxic-ischemic encephalopathy（HIE）,intraventricular hemorrhage,periventricular leukomalacia and encephalopathy of prematurity.
文摘Myelomeningocele(MMC)is a severe form of spinal dysraphism.Due to the failure of neural tube closure during early embryonic development,the affected part of the spinal cord is left open like a book at the back of the affected child.This malformed part of the spinal cord is not covered by its protective mesodermal and ectodermal derived layers.Consequently,the exposed neural tissue(i.e.,the neural placode)is prone to injuryduring further intra-uterine development.
文摘Introduction: teratomas are non-malignantgerm cell tumors commonly composed of cell types derived from all of the three germ layers. Intracerebral teratomas typically are midline or paraxial lesions located in the pituitary stalk or the pineal region. We report our experience with mature teratomas of the posterior fossa. Case report: we present the case of an 11-year-old caucasian female with progressive headache that caused interrupted sleep. Cerebral magnetic resonance imaging showed a midline lesion in the posterior fossa with mass effect and without contrast enhancement. Anatomic pathology revealed a mature teratoma. Conclusion: differential diagnosis of midline lesions in pediatric patients must include teratomas in spite of being posterior fossa lesions.
文摘Meningiomas occur in 1%-4% of primary intracranial tumors in the pediatric group, and is increasing in incidence with age. Some authors have reported that meningioma is more prevalent among adult males, but there is no gender prevalence. The accepted origin of meningiomas is from the arachnoid ceils lining the meninges, or the choroid plexuses. Since Beckwith and Palmer introduced the term ＇rhabdoid tumor＇ in 1978 in reference to a subgroup of childhood malignant renal tumors, many tumors with a rhabdoid morphology have been reported in various sites, including the central nervous system. In 1998 Kepes et al.
文摘Hypothalamic hamartoma(HH) is usually associated with refractory epilepsy, cognitive impairment, and behavioral disturbance. There is now increasing evidence that HH can be treated effectively with a variety of neurosurgical approaches. Treatment options for intractable gelastic seizure in HH patients include direct open surgery with craniotomy, endoscopic surgery, radiosurgery with gamma knife and stereotactic radiofrequency thermocoagulation. Selection of treatment modalities depends on type and size of the HH and the surgeon's preference. Two surgical techniques, resection and disconnection, had been described with favorable outcomes. Pretreatmentevaluation, patient selection, surgical techniques, complications, and possible selection of treatment are discussed.
基金Supported by the Rory David Deutsch Foundationthe Surgical Neuro-oncology Research Fund of Ann&Robert H Lurie Children’s Hospital(A&RLCH) of Chicagothe Dr.Ralph and Marian C.Falk Medical Research Trust
文摘Patients with brain tumors,specifically,malignant forms such as glioblastoma,medulloblastoma and ependymoma,exhibit dismal survival rates despite advances in treatment strategies.Chemotherapeutics,the primary adjuvant treatment for human brain tumors following surgery,commonly lack efficacy due to either intrinsic or acquired drug resistance.New treatments targeting epigenetic factors are being explored.Post-translational histone modification provides a critical regulatory platform for processes such as chromosome condensation and segregation,apoptosis,gene transcription,and DNA replication and repair.This work reviews how aberrant histone modifications and alterations in histone-modifying enzymes can drive the acquisition of drug resistance in brain tumors.Elucidating these mechanisms should lead to new treatments for overcoming drug resistance.
文摘Pantothenate kinase-associated neurodegeneration (PKAN)-causes a progressive generalized dystonia which remains pharmacologically intractable. We performed bilateral internal globus pallidus stimulation in six patients with genetically confirmed PKAN who obtained a major and long-lasting improvement of their painf ul spasms, dystonia, and functional autonomy. This study shows the benefits of p allidal DBS for the dystonia of PKAN patients.
基金supported by the Grand Alliance CARE Consortiumfunded in part by the National Collegiate Athletic Association(NCAA)+1 种基金the Department of Defense(DoD).supported by the Office of the Assistant Secretary of Defense for Health Affairs,through the Combat Casualty Care Research Program,endorsed by the Department of Defense,under Award No.W81XWH1420151。
文摘Background:Current protein biomarkers are only moderately predictive at identifying individuals with mild traumatic brain injury or concussion.Therefore,more accurate diagnostic markers are needed for sport-related concussion.Methods:This was a multicenter,prospective,case-control study of athletes who provided blood samples and were diagnosed with a concussion or were a matched non-concussed control within the National Collegiate Athletic Association-Department of Defense Concussion Assessment,Research,and Education Consortium conducted between 2015 and 2019.The blood was collected within 48 h of injury to identify protein abnormalities at the acute and subacute timepoints.Athletes with concussion were divided into 6 h post-injury(0-6 h post-injury)and after 6 h postinjury(7-48 h post-injury)groups.We applied a highly multiplexed proteomic technique that used a DNA aptamers assay to target 1305proteins in plasma samples from athletes with and without sport-related concussion.Results:A total of 140 athletes with concussion(79.3%males;aged 18.71±1.10 years,mean±SD)and 21 non-concussed athletes(76.2%males;19.14±1.10 years)were included in this study.We identified 338 plasma proteins that significantly differed in abundance(319 upregulated and 19 downregulated)in concussed athletes compared to non-concussed athletes.The top 20 most differentially abundant proteins discriminated concussed athletes from non-concussed athletes with an area under the curve(AUC)of 0.954(95%confidence interval:0.922-0.986).Specifically,after 6 h of injury,the individual AUC of plasma erythrocyte membrane protein band 4.1(EPB41)and alpha-synuclein(SNCA)were 0.956 and 0.875,respectively.The combination of EPB41 and SNCA provided the best AUC(1.000),which suggests this combination of candidate plasma biomarkers is the best for diagnosing concussion in athletes after 6 h of injury.Conclusion:Our data suggest that proteomic profiling may provide novel diagnostic protein markers and that a combination of EPB41 and SNCA is the most predictive biomarker of concussion after 6 h of injury.
文摘Objective:There is a paucity of real-world evidence in the Indian context to address the outcome of primary brain tumors(PBTs)in children.This study aimed to describe the demographic profile,clinical characteristics,and histological features of PBTs based on the 2016 World Health Organization classification,assess the efficacy of treatment methods,and identify the factors that influence the outcome.Methodology:This is a single-institution,hospital-based study.Data were collected for pediatric patients aged 0−19 years,from September 2001 to May 2023(22 years),who were diagnosed with malignant PBTs.Patients with radiologically or histologically proven tumors were included.Those with metastatic disease to the central nervous system were excluded.The overall survival(OS)and recurrence-free survival(RFS)were estimated using the Kaplan–Meier method.Results:A total of 251 patients with pediatric brain tumors were included in this analysis.The mean age was 9.10±5.54 years.The male-to-female ratio was 1.20:1.In this cohort,the most common histologies were medulloblastoma and astrocytoma.The mean survival of all patients with PBTs was 141.00±7.90 months with 1-,3-,and 8-year OS rates of 79.00%,67.00%,and 60.00%,respectively.Medulloblastoma had 1-,3-,and 8-year OS rates of 81.00%,72.00%,and 65.00%,respectively.The 1-year OS rates for glioblastoma and brainstem glioma were 46.00%and 45.00%,respectively.Complete tumoral resection showed longer survival than lesser degrees of resection(p=0.001).Embryonal tumors(ETs)had a better RFS of 133.60±12.70 months(p≤0.001).Conclusion:ETs have a better prognosis than glial tumors.With an improved OS,the surgical resection extent has a favorable outcome.As a chemosensitive tumor,medulloblastoma benefits most from systemic treatment and responds well to a multimodal approach.
文摘Background:Meningiomas are rather uncommon tumors in the pediatric population,differing significantly from those found in adults by their atypical location,higher rate of more malignant types,consequently higher risk of recurrence and a less favorable outcome.Even in children,suprasellar meningiomas without dural matrix are rare findings mimicking more common suprasellar lesions.Case presentation:Here we describe a case of a 12-year-old girl who presented with a rapidly progressing chiasmal syndrome and was diagnosed by MRI with an unusual suprasellar tumor that could not fit the diagnoses expected in a case of a parasellar mass in a child,similar to a craniopharyngioma or optic pathway glioma.After multiple clinical investigations,the tumor etiology was still unclear,so the preferred option of treatment was surgical resection.An endoscope-assisted gross total resection through a supraorbital keyhole approach was performed uneventfully,with total vision recovery in a short time.Benign meningiomas located in the skull base without dural attachment appear to be rare,even in pediatric patients.Conclusion:Differential diagnoses of suprasellar and para sellar tumor lesions in pediatric patients can be confusing.There are peculiar features of pediatric tumor diseases that should be considered while working out the management strategy.The main principle of meningioma treatment is the highest possible extent of resection minimally affecting the quality of life.
文摘Background:Pediatric epidural hematomas(EDH)represent a neurosurgical emergency.Both surgical and conservative treatment can lead to a good clinical outcome.The aim of the study was to review our series of pediatric EDH and to determine the clinical and radiologic factors,which can influence the final outcome.Methods:All children aged from 0 to 16 that have been treated between 2013 and 2017 for cranial EDH have been selected.Results:Thirty children have been included in the study.Seventeen cases have been treated with surgical evacuation and 13 conservatively.Six months after the trauma,the outcome was excellent(mRS 0)in 25/30(83.3%)cases,mild deficits(mRS 1-2)were present in 4/30(13.3%),and severe deficits(mRS 3-5)in 1/30(3.3%)cases.Only a GCS(Glasgow Coma Scale)below 8 at admission was significantly related to the presence of a neurologic deficit at 6 months(p=0.048).Conclusions:EDH can be managed with excellent outcomes.Even in the presence of bad initial clinical and radiologic conditions,a correct treatment strategy can lead to a good recovery.In our series,only a GCS below 8 at admission was significantly related to the presence of neurological sequelae.
基金supported by the Sichuan Science and Technology Program(No.2023YFQ0002)the internal research funds in West China Hospital of Sichuan University(No.19HXCX009)the Medical Science and Technology Project of Sichuan Health Commission(No.21PJ033).
文摘Gliomas are the most common primary neuroepithelial tumors of the central nervous system in adults,of which glioblastoma is the deadliest subtype.Apart from the intrinsically indestructible characteristics of glioma(stem)cells,accumulating evidence suggests that the tumor microenvironment also plays a vital role in the refractoriness of glioblastoma.The primary functions of platelets are to stop bleeding and regulate thrombosis under physiological conditions.Furthermore,platelets are also active elements that participate in a variety of processes of tumor development,including tumor growth,invasion,and chemoresistance.Glioma cells recruit and activate resting platelets to become tumor-educated platelets(TEPs),which in turn can promote the proliferation,invasion,stemness,and chemoresistance of glioma cells.TEPs can be used to obtain genetic information about gliomas,which is helpful for early diagnosis and monitoring of therapeutic effects.Platelet membranes are intriguing biomimetic materials for developing efficacious drug carriers to enhance antiglioma activity.Herein,we review the recent research referring to the contribution of platelets to the malignant characteristics of gliomas and focusing on the molecular mechanisms mediating the interaction between TEPs and glioma(stem)cells,as well as present the challenges and opportunities in targeting platelets for glioma therapy.
基金grants from the National Key Basic Research Program，the National Natural Science Foundation of China，the Program of Shanghai Subject Chief Scientist，Foundation of Shanghai Jiaotong University,School of Medicine，the Program of Shanghai Health and Family Planning Committee，Shanghai Municipal Natural Science Foundation （No.14ZR1427100）.Conflict of Interest：None declared
文摘Objective：This overview seeked to bring together the microRNA （miRNA） researches on biogenesis and bio-function in these areas of clinical diagnosis and therapy for malignant glioma.Data Sources：Using the keyword terms ＂glioma＂ and ＂miRNA,＂ we performed the literature search in PubMed,Ovid,and web.metstr.com databases from their inception to October 2014.Study Selection：In screening out the quality of the articles,factors such as clinical setting of the study,the size of clinical samples were taken into consideration.Animal studied for verification and reviews article were also included in our data collection.Results：Despite many advance in miRNA for malignant glioma,further studies were still required to focus on the following aspects：（i） Improving the understanding about biogenesis of miRNA and up-down regulation;（ii） utilizing high-throughput miRNA expression analysis to screen out the core miRNA for glioma;（iii） Focusing related miRNAs on the signal transduction pathways that regulate the proliferation and growth of glioma.Conclusions：We discussed the most promising miRNA,correlative signaling pathway and their relation with gliomas in the way of prompting miRNA target into being a clinical therapeutic strategy.
