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Hemispherotomy is an effective disconnection technique for the treatment of different forms of drug-resistant epilepsy due to encephalopathies with unilateral hemispheric involvement.1-8 We describe the case of a 6-year-old child affected by Rasmussen encephalitis who underwent right vertical parasagittal hemispherotomy.9 The goal of the procedure was to isolate the basal ganglia region by interrupting the interhemispheric and intrahemispheric white matter connectivity. The main surgical steps include the transcortical approach to the lateral ventricle, the posterior callosotomy, the fimbria-fornix incision, the laterothalamic vertical incision, the anterior callosotomy, the frontobasal disconnection, and the transcaudate lateral incision to the anterior temporal horn.7,10 At 10-month follow-up, the patient was seizure free with a stable left hemiparesis. The antiepileptic therapy was progressively interrupted. The video describes the main surgical steps, using both intraoperative videos and advanced three-dimensional modeling of neuroimaging pictures. Patient' parents consented to the procedure. The participants and any identifiable individuals consented to publication of his/her image. Approval from the ethics committee was acquired.
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The post-surgical outcome for Hypothalamic Hamartoma (HH) related epilepsy in terms of seizure freedom (SF) has been extensively studied, while cognitive and psychiatric outcome has been less frequently reported and defined. This is a systematic review of English language papers, analyzing the post-surgical outcome in series of patients with HH-related epilepsy (≥5 patients, at least 6 months follow-up), published within January 2002-December 2022. SF was measured using Engel scale/equivalent scales. We looked at the outcome related to different surgical techniques, and HH types according to Delalande classification. We evaluated the neuropsychological and neuropsychiatric status after surgery, and the occurrence of post-surgical complications. Forty-six articles reporting 1318 patients were included, of which ten pediatric series. SF was reported in 686/1222 patients (56,1%). Delalande classification was reported in 663 patients from 24 studies, of which 70 were type I HH (10%), 320 were type II HH (48%), 189 were type III HH (29%) and 84 were type IV HH (13%). The outcome in term of SF was reported in 243 out of 663 patients. SF was reported in 12 of 24 type I HH (50%), 80 of 132 type II HH (60,6%), 32 of 59 type III HH (54,2%) and 12 of 28 type IV HH (42,9%). SF was reached in 129/262 (49,2%) after microsurgery, 102/199 (51,3%) after endoscopic surgery, 46/114 (40,6%) after gamma knife surgery, 245/353 (69,4%) after radiofrequency thermocoagulation, and 107/152 (70,4%) after MRI-guided laser interstitial thermal therapy. Hyperphagia/weight gain were the most reported surgical complications. Others were electrolyte alterations, diabetes insipidus, hypotiroidism, transient hyperthermia/poikilothermia. The highest percentage of memory deficits was reported after microsurgery, while hemiparesis and cranial nerves palsy were reported after microsurgery or endoscopic surgery. Thirty studies reported developmental delay/intellectual disability in 424/819 (51,7%) patients. 248/346 patients obtained a global improvement (72%), 70/346 were stable (20%), 28/346 got worse (8%). 22 studies reported psychiatric disorders in 257/465 patients (55,3%). 78/98 patients improved (80%), 13/98 remained stable (13%), 7/98 got worse (7%). Most of the patients had non-structured cognitive/psychiatric assessments. Based on the available data, the surgical management in patients with HH related epilepsy should be individualized, aiming to reach not only the best epilepsy result, but also the optimal cognitive and psychiatric outcome.
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Epilepsia , Hamartoma , Doenças Hipotalâmicas , Humanos , Doenças Hipotalâmicas/cirurgia , Doenças Hipotalâmicas/complicações , Hamartoma/cirurgia , Hamartoma/complicações , Epilepsia/cirurgia , Epilepsia/psicologia , Resultado do Tratamento , Procedimentos Neurocirúrgicos/efeitos adversos , Testes Neuropsicológicos , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/psicologia , Transtornos Mentais/etiologiaRESUMO
Epilepsy surgery is effective for patients with medication-resistant seizures, however 20-40% of them are not seizure free after surgery. Aim of this study is to evaluate the role of linear and non-linear EEG features to predict post-surgical outcome. We included 123 paediatric patients who underwent epilepsy surgery at Bambino Gesù Children Hospital (January 2009-April 2020). All patients had long term video-EEG monitoring. We analysed 1-min scalp interictal EEG (wakefulness and sleep) and extracted 13 linear and non-linear EEG features (power spectral density (PSD), Hjorth, approximate entropy, permutation entropy, Lyapunov and Hurst value). We used a logistic regression (LR) as feature selection process. To quantify the correlation between EEG features and surgical outcome we used an artificial neural network (ANN) model with 18 architectures. LR revealed a significant correlation between PSD of alpha band (sleep), Mobility index (sleep) and the Hurst value (sleep and awake) with outcome. The fifty-four ANN models gave a range of accuracy (46-65%) in predicting outcome. Within the fifty-four ANN models, we found a higher accuracy (64.8% ± 7.6%) in seizure outcome prediction, using features selected by LR. The combination of PSD of alpha band, mobility and the Hurst value positively correlate with good surgical outcome.
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Eletroencefalografia , Aprendizado de Máquina , Humanos , Eletroencefalografia/métodos , Criança , Feminino , Masculino , Pré-Escolar , Adolescente , Epilepsia/cirurgia , Epilepsia/fisiopatologia , Epilepsia/diagnóstico , Redes Neurais de Computação , Resultado do Tratamento , Lactente , Sono/fisiologiaRESUMO
Modern neuroscience agrees that neurological processing emerges from the multimodal interaction among multiple cortical and subcortical neuronal hubs, connected at short and long distance by white matter, to form a largely integrated and dynamic network, called the brain "connectome." The final architecture of these circuits results from a complex, continuous, and highly protracted development process of several axonal pathways that constitute the anatomical substrate of neuronal interactions. Awareness of the network organization of the central nervous system is crucial not only to understand the basis of children's neurological development, but also it may be of special interest to improve the quality of neurosurgical treatments of many pediatric diseases. Although there are a flourishing number of neuroimaging studies of the connectome, a comprehensive vision linking this research to neurosurgical practice is still lacking in the current pediatric literature. The goal of this review is to contribute to bridging this gap. In the first part, we summarize the main current knowledge concerning brain network maturation and its involvement in different aspects of normal neurocognitive development as well as in the pathophysiology of specific diseases. The final section is devoted to identifying possible implications of this knowledge in the neurosurgical field, especially in epilepsy and tumor surgery, and to discuss promising perspectives for future investigations.
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Modern epilepsy science has overcome the traditional interpretation of a strict region-specific origin of epilepsy, highlighting the involvement of wider patterns of altered neuronal circuits. In selected cases, surgery may constitute a valuable option to achieve both seizure freedom and neurocognitive improvement. Although epilepsy is now considered as a brain network disease, the most relevant literature concerning the "connectome-based" epilepsy surgery mainly refers to adults, with a limited number of studies dedicated to the pediatric population. In this review, the Authors summarized the main current available knowledge on the relevance of WM surgical anatomy in epilepsy surgery, the post-surgical modifications of brain structural connectivity and the related clinical impact of such modifications within the pediatric context. In the last part, possible implications and future perspectives of this approach have been discussed, especially concerning the optimization of surgical strategies and the predictive value of the epilepsy network analysis for planning tailored approaches, with the final aim of improving case selection, presurgical planning, intraoperative management, and postoperative results.
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Encefalopatias , Conectoma , Epilepsia , Adulto , Criança , Humanos , Resultado do Tratamento , Epilepsia/diagnóstico por imagem , Epilepsia/cirurgia , Encéfalo/diagnóstico por imagem , Encéfalo/cirurgiaRESUMO
Craniopagus is a rare condition in which the skulls of conjoined twins, and the neurovascular structures in their skulls, are incompletely separated. Separation surgery is complex and requires a range of skills and expertise, including from all the nursing teams involved. A multidisciplinary group of professionals in a tertiary paediatric care setting in Italy developed a comprehensive protocol for the nursing care of craniopagus twins in hospital, informed by the findings of a literature review and the expertise of its members. The protocol was applied to the management of a pair of craniopagus twins who successfully underwent separation surgery at the authors' hospital. The protocol describes the crucial role of nurses in the care of craniopagus twins and their families before, during and after separation surgery.
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BACKGROUND: Human cystic echinococcosis (CE) is a zoonotic parasitic infection caused by the larval stage of the species belonging to the Echinococcus granulosus sensu lato (s.l.) complex. Parasitic cysts causing human CE are mainly localized in the liver and in the lungs. In a smaller number of cases, larvae may establish in any organ or tissue, including the central nervous system (CNS). Cerebral CE (CCE) is rare but poses serious clinical challenges. METHODS: This study presents a case of CCE in a child living in the countryside near Rome (Italy), along with a comparative molecular analysis of the isolated cyst specimens from the patient and sheep of local farms. We also systematically searched the literature to summarize the most relevant epidemiological and clinical aspects of this uncommon localization. FINDINGS: The comparative molecular analysis confirmed that the infection was caused by E. granulosus sensu stricto (s.s.) (G3 genotype), and most likely acquired in the family farm. The literature search identified 2,238 cases of CCE. In 80.51% of cases, brain was the only localization and single CCE cysts were present in 84.07% of cases. Mean patients' age was 20 years and 70.46% were children. Cyst rupture was reported in 12.96% and recurrence of CCE after treatment in 9.61% of cases. Permanent disability was reported in 7.86% of cases, while death occurred in 6.21%. In case series reporting all CE localization, CCE represented 1.5% of all CE cases. In the few reports that identified at molecular level the CCE cyst, E. granulosus s.s. was found in 40% and E. canadensis in 60% of cases. CONCLUSIONS: We report a rare case of CCE and evidenced the probable local origin of infection. The proportions of CE cases with uncommon localizations and with high impact on patients' lives have been globally neglected and should be included in the computation of the global burden of CE.
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Infecções Parasitárias do Sistema Nervoso Central , Cisticercose , Cistos , Equinococose , Humanos , Criança , Animais , Ovinos , Adulto Jovem , Adulto , Zoonoses , LarvaRESUMO
Craniopagus separation requires careful planning and a multi-staged surgical approach, managed by a multidisciplinary team. Despite growing experience on surgical management, the long-term restoration of the cranial defect has been rarely discussed so far. Several alloplastic materials have been proposed as effective and safe solutions for cranioplasty even for pediatric patients, allowing for bone generation, good aesthetic results, and low complication rates. We report, for the first time, on a separated craniopagus child who underwent successful implant of a tailor-made system based on the combination of calcium phosphate ceramic reinforced with a titanium mesh.
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Procedimentos de Cirurgia Plástica , Titânio , Humanos , Criança , Crânio/cirurgia , Próteses e Implantes , Fosfatos de CálcioRESUMO
Anorectal malformation (ARM) is often associated to other congenital malformations, requiring a tailored management. Hypospadias' treatment in ARM is poorly described. Aim of study is to describe our experience in ARM-hypospadias patients especially in relation to occult spinal dysraphism (OSD). ARM patients treated from 1999 to 2022 were retrospectively reviewed, selecting male with hypospadias. Clinical data, hypospadias's severity, ARM sub-type (Group A: perineal fistula; Group B: urethral fistula, bladder fistula, no fistula), OSD, other associated malformations, NLUTD were evaluated. Exclusion criteria: uncomplete data. Among 395 ARMs, 222 were males, 22 (10%) had hypospadias. Two patients were excluded. Group A: 8 patients, Group-B: 12. Hypospadias: proximal 9 patients, distal 11. Neuro-urological evaluation was performed before hypospadias repair. Eleven patients (55%) had OSD. Four OSD patients presented NLUTD and underwent detethering and CIC (two via cystostomy button, two via appendicostomy); two of them had hypospadias repaired. All proximal hypospadias underwent two stages of surgery. Distal hypospadias was corrected in 4/11 cases. Hypospadias is quite common in ARM patients and its surgical management must be scheduled considering the possible OSD and NLUTD, with the possible need for intermittent catheterization. Complexity of ARM and hypospadias appears to be related to each other.
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Malformações Anorretais , Hipospadia , Defeitos do Tubo Neural , Fístula Urinária , Humanos , Criança , Masculino , Feminino , Hipospadia/complicações , Hipospadia/cirurgia , Malformações Anorretais/complicações , Malformações Anorretais/cirurgia , Estudos Retrospectivos , Uretra/cirurgia , Defeitos do Tubo Neural/cirurgiaRESUMO
AIM: To evaluate clinical phenotype and molecular findings of 157 cases with GNAO1 pathogenic or likely pathogenic variants delineating the clinical spectrum, course, and response to treatments. METHOD: Clinical phenotype, genetic data, and pharmacological and surgical treatment history of 11 novel cases and 146 previously published patients were analyzed. RESULTS: Complex hyperkinetic movement disorder (MD) characterizes 88% of GNAO1 patients. Severe hypotonia and prominent disturbance of postural control seem to be hallmarks in the early stages preceding the hyperkinetic MD. In a subgroup of patients, paroxysmal exacerbations became so severe as to require admission to intensive care units (ICU). Almost all patients had a good response to deep brain stimulation (DBS). Milder phenotypes with late-onset focal/segmental dystonia, mild to moderate intellectual disability, and other minor neurological signs (i.e., parkinsonism and myoclonus) are emerging. MRI, previously considered noncontributory to a diagnosis, can show recurrent findings (i.e., cerebral atrophy, myelination and/or basal ganglia abnormalities). Fifty-eight GNAO1 pathogenic variants, including missense changes and a few recurrent splice site defects, have been reported. Substitutions at residues Gly203, Arg209 and Glu246, together with the intronic c.724-8G > A change, account for more than 50% of cases. INTERPRETATION: Infantile or childhood-onset complex hyperkinetic MD (chorea and/or dystonia) with or without paroxysmal exacerbations, associated hypotonia, and developmental disorders should prompt research for GNAO1 mutations. DBS effectively controls and prevents severe exacerbations and should be considered early in patients with specific GNAO1 variants and refractory MD. Prospective and natural history studies are necessary to define genotype-phenotype correlations further and clarify neurological outcomes.
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Transtornos dos Movimentos , Humanos , Masculino , Feminino , Criança , Transtornos dos Movimentos/tratamento farmacológico , Transtornos dos Movimentos/patologia , Transtornos dos Movimentos/fisiopatologia , Transtornos dos Movimentos/cirurgia , Hipotonia Muscular , Deficiências do Desenvolvimento , Relatos de Casos como AssuntoRESUMO
Background: Tumors of the pre-sacral and sacral spaces are a rare occurrence in children. Total tumor excision is required due to the significant risk of relapse in the event of partial surgery, but the surgical procedure may lead to postoperative problems such as urinary, sexual, and anorectal dysfunctions. Intraoperative neuromonitoring (IONM) has gained popularity in recent years as a strategy for preventing the onset of neurologic impairments by combining several neurophysiological techniques. The aim of our study is to describe the experience of Bambino Gesù Children's Hospital in the use of IONM in pediatric pelvic surgery. Materials and Methods: The data of patients treated for pelvic malignancies at Bambino Gesù Children's Hospital from 2015 to 2019 were retrospectively collected. All patients were assessed from a neurologic and neuro-urologic point of view at different time-points (before and immediately after surgery, after 6 months, and 1-year follow-up). They were all monitored during a surgical procedure using multimodal IONM including transcranial motor evoked potentials (TcMEP), triggered-EMG (t-EMG), pudendal somatosensory evoked potentials (PSSEP), and bulbocavernosus reflex (BCR). Results: During the study period, ten children underwent pelvic tumor removal at our Institution. In all cases, intraoperative neurophysiological recordings were stable and feasible. The preservation of neurophysiological response at the same intensity during surgical procedures correlated with no new deficits for all neurophysiological techniques. Discussion: Although the impact of the IONM on surgical strategies and clinical follow-up is unknown, this preliminary experience suggests that the appropriate use of several neurophysiological techniques can influence both the radicality of pelvic tumor removal and the neurological and urological outcome at clinical follow-up. Finally, because of the highly complex anatomy and inter-individual variances, this is especially useful in this type of surgery.
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PURPOSE: Caudal regression syndrome (CRS) is a rare congenital abnormality including orthopedic deformities, urological, anorectal, and cardiac malformations. The clinical spectrum of CRS varies in severity, therefore multiple surgeries and complex medical care may be required with the efforts and support of a multidisciplinary team to ensure the most accurate treatment and the best outcome. The aim of our retrospective study was to evaluate the role of a multidisciplinary treatment and the long-term outcome in patients with severe CRS. METHODS: Clinical, surgical and psychological data were collected for all patients with diagnosis of CRS, treated at our Pediatric Hospital from January 1995 to December 2020. Patients with a severe form of CRS according to Pang's classification were included in the study. All patients have been followed by a multidisciplinary team composed by urologists, neurosurgeons, neonatal and digestive surgeons, orthopedic surgeon, physiatrists and psychologists. On admission, patients were screened for CRS with sacrum, spine and legs X-ray, spine MRI, renal ultrasound, voiding cystography and urodynamic, and renography. Clinical data about bowel function were evaluated. RESULTS: During the study period, 55 patients with CRS were treated at our Institution. Six out of 55 (10.9%), presented with severe form of CRS (5 pts with type 1; 1 pt, with type 2) and represent our study group. Diagnosis of severe CRS was made at birth because of the typical deformities of the pelvis (fusion of the iliac wings), and of the lower extremities (undeveloped legs with flexion of the knees, clubfoot). All patients presented with neurogenic bladder, 4/6 (66.6%) with vesicoureteral reflux (VUR) and 2/6 (33.3%) with renal agenesia and contralateral ectopic hypoplastic kidney. Bowel symptoms occurred in 5/6 pts (83.3%). All patients were started with an earlier clean intermittent catheterization (CIC) regimen. In 3 patients oxybutynin has been effective or well tolerated, while in other 3 onabotulinumtoxin A has been used. Vesicoureteral reimplantation has been performed in 1 patient, 2 required endoscopic treatment of VUR. On long-term, 2 patients required bladder augmentation with ileum and appendicostomy (Mitrofanoff). Low adherences in CIC have been observed in three patients, mainly at puberty. Two patients presented with chronic renal failure. One patient reported suicide proposal. Regarding social life, only one is regularly performing sport activity. CONCLUSION: CRS is characterized by maldevelopment of the caudal half of the body with variable involvement of the gastrointestinal, genitourinary, skeletal, and nervous systems. Management of CRS includes a wide variety of interventions to address the full spectrum of possible anatomical abnormalities. Hence, a multidisciplinary team is also mandatory for a correct bladder and bowel management, in order to maintain continence and preserve renal function, improve quality of life and increase self-esteem.
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Anormalidades Múltiplas , Bexiga Urinaria Neurogênica , Refluxo Vesicoureteral , Criança , Seguimentos , Humanos , Recém-Nascido , Equipe de Assistência ao Paciente , Qualidade de Vida , Estudos Retrospectivos , Bexiga Urinaria Neurogênica/etiologiaRESUMO
PURPOSE: Urethral duplication (UD) is a rare malformation, which can be associated with other anomalies, like anorectal malformations (ARM). ARM has been described with occult spinal dysraphism (OSD). No ARM-UD-OSD combination has been reported. AIM: To share our experience and to discuss the management of ARM-UD-OSD association. METHODS: We retrospectively reviewed records of five boys with UD. Four of these had ARM-UD-OSD association. ARM was the first diagnosis in all; OSD and UD was detected during screening for associated malformation. RESULTS: All patients underwent ARM correction, 3 after colostomy. All reached fecal continence, 3 are performing bowel management. Three patients underwent UD surgical correction. Because of symptoms' worsening, 2 children had detethering surgery. At a mean follow-up of 9.5 years, all patients have normal renal function, 3 are on clean intermittent catheterization (CIC) for neurogenic bladder (1 has a cystostomy, another one an appendicostomy). CONCLUSIONS: UD and OSD should be considered in patients with ARM. Children with these conditions associated must be centralized in a third-level Center and management carefully planned; in particular, urethral reconstruction should be weighed, considering CIC could be required. Suspicion of neurogenic bladder must be present in OSD patient.
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Malformações Anorretais , Defeitos do Tubo Neural , Bexiga Urinaria Neurogênica , Malformações Anorretais/complicações , Malformações Anorretais/diagnóstico , Malformações Anorretais/cirurgia , Criança , Humanos , Masculino , Defeitos do Tubo Neural/complicações , Estudos RetrospectivosRESUMO
Subacute sclerosing panencephalitis (SSPE) is a late complication of measles virus infection that occurs in previously healthy children. This disease has no specific cure and is associated with a high degree of disability and mortality. In recent years, there has been an increase in its incidence in relation to a reduction in vaccination adherence, accentuated by the COVID-19 pandemic. In this article, we take stock of the current evidence on SSPE and report our personal clinical experience. We emphasise that, to date, the only effective protection strategy against this disease is vaccination against the measles virus.
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COVID-19 , Sarampo , Panencefalite Esclerosante Subaguda , COVID-19/prevenção & controle , Criança , Humanos , Sarampo/epidemiologia , Sarampo/prevenção & controle , Vírus do Sarampo , Pandemias , Panencefalite Esclerosante Subaguda/epidemiologia , Panencefalite Esclerosante Subaguda/etiologia , Panencefalite Esclerosante Subaguda/prevenção & controle , Vacinação/efeitos adversosRESUMO
Tuberous sclerosis complex (TSC) is a rare multisystem, autosomal dominant neurocutaneous syndrome in which epilepsy is the most common of several neurological and psychiatric manifestations. Around two thirds of patients develop drug-resistant epilepsy for whom surgical resection of epileptogenic foci is indicated when seizures remain inadequately controlled following trial of two antiseizure medications. The challenge with presurgical and surgical approaches with patients with TSC is overcoming the complexity from the number of tubers and the multiplex epileptogenic network forming the epileptogenic zone. Data suggest that seizure freedom is achieved by 55%-60% of patients, but predictive factors for success have remained elusive, which makes for unconfident selection of surgical candidates. This article presents three different cases as illustrations of the potential challenges faced when assessing the suitability of TSC patients for epilepsy surgery.
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De novo arterio-venous malformations (AVMs) of the brain have been rarely previously reported, especially in the pediatric population. Although AVMs have possible connections with other diseases, the association with congenital portosystemic shunt (CPSS) has never been reported before. A child was followed for CPSS and cutaneous and hepatic angiomas. Brain MRI and angiography revealed an AVM within the left temporal region that was not present at a previous MRI. The patient underwent successful resection of the AVM. This case adds new evidence on the complex variety of diseases associated with multisystemic vascular malformations corroborating the hypothesis of a multifactorial origin of de novo cerebral AVMs, under a possible common genetic substrate.
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Hemangioma , Malformações Arteriovenosas Intracranianas , Malformações do Sistema Nervoso , Malformações Vasculares , Encéfalo , Criança , Hemangioma/complicações , Humanos , Malformações Arteriovenosas Intracranianas/complicações , Malformações Arteriovenosas Intracranianas/diagnóstico por imagem , Malformações Arteriovenosas Intracranianas/cirurgia , Imageamento por Ressonância MagnéticaRESUMO
The current study, conceived with the contribution of the Commission for Epilepsy Surgery of the Italian League Against Epilepsy (LICE) and the Epilepsy Study Group of the Italian Neurological Society (SIN), aimed to assess potential physician-related barriers to refer subjects for epilepsy surgery. All the members of SIN and LICE were invited by email to complete a 28-item online questionnaire. The survey items included: (1) individual and medical practice characteristics, (2) knowledge of current indications to select candidates for epilepsy surgery, (3) factors potentially affecting the attitude toward epilepsy surgery. Overall, 210 physicians completed the survey. More than half (63.3%) of the participants showed proper knowledge of the ILAE drug-resistance. Definition and almost two-thirds of them (71.9%) considered themselves adequately informed about indications, risks, and benefits of epilepsy surgery. Surgery was regarded as a valid option to be used as early as possible by 84.8% of the interviewees, and 71% of them estimated its complication rate to be low. However, more than half (63%) of the respondents reportedly referred patients for surgery only after the failure of 3-5 antiseizure medications. Overestimation of risks/complications of surgery and inadequate healthcare resources were identified as the main factor contrasting the patient referral for surgery by 43% and 40.5% of the participants, respectively. In conclusion, this survey confirms the existence of knowledge gap within both physicians and the healthcare system, as well as an educational need regarding epilepsy surgery. Further researches are warranted to define learning outcomes and optimize educational tools.
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Epilepsia , Médicos , Epilepsia/tratamento farmacológico , Epilepsia/cirurgia , Conhecimentos, Atitudes e Prática em Saúde , Humanos , Neurologistas , Inquéritos e QuestionáriosRESUMO
In the past, the cerebellum was considered to be substantially involved in sensory-motor coordination. However, a growing number of neuroanatomical, neuroimaging, clinical and lesion studies have now provided converging evidence on the implication of the cerebellum in a variety of cognitive, affective, social, and behavioral processes as well. These findings suggest a complex anatomo-functional organization of the cerebellum, involving a dense network of cortical territories and reciprocal connections with many supra-tentorial association areas. The final architecture of cerebellar networks results from a complex, highly protracted, and continuous development from childhood to adulthood, leading to integration between short-distance connections and long-range extra-cerebellar circuits. In this review, we summarize the current evidence on the anatomo-functional organization of the cerebellar connectome. We will focus on the maturation process of afferent and efferent neuronal circuitry, and the involvement of these networks in different aspects of neurocognitive processing. The final section will be devoted to identifying possible implications of this knowledge in neurosurgical practice, especially in the case of posterior fossa tumor resection, and to discuss reliable strategies to improve the quality of approaches while reducing postsurgical morbidity.
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OBJECTIVES: Hemispherotomy (HT) is a surgical option for treatment of drug-resistant seizures due to hemispheric structural lesions. Factors affecting seizure outcome have not been fully clarified. In our study, we used a brain Machine Learning (ML) approach to evaluate the possible role of Inter-hemispheric EEG Connectivity (IC) in predicting post-surgical seizure outcome. METHODS: We collected 21 pediatric patients with drug-resistant epilepsy; who underwent HT in our center from 2009 to 2020; with a follow-up of at least two years. We selected 5-s windows of wakefulness and sleep pre-surgical EEG and we trained Artificial Neuronal Network (ANN) to estimate epilepsy outcome. We extracted EEG features as input data and selected the ANN with best accuracy. RESULTS: Among 21 patients, 15 (71%) were seizure and drug-free at last follow-up. ANN showed 73.3% of accuracy, with 85% of seizure free and 40% of non-seizure free patients appropriately classified. CONCLUSIONS: The accuracy level that we reached supports the hypothesis that pre-surgical EEG features may have the potential to predict epilepsy outcome after HT. SIGNIFICANCE: The role of pre-surgical EEG data in influencing seizure outcome after HT is still debated. We proposed a computational predictive model, with an ML approach, with a high accuracy level.