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We present a 16 year-old right-handed case who underwent a left temporo-occipital resection to treat intractable epilepsy. Pre- and post-surgical evaluations showed an average intellectual quotient, preserved abilities in language and visuo-spatial functions and increased reading and spelling deficits (difficulties with irregular words, homophones and phonologically valid spelling errors of irregularly spelled words, associated with preserved performances in non-words). This pattern of characteristic lexical route deficits highlights the major role of the temporo-occipital areas in reading acquisition. We discussed the consequences of temporo-occipital dysfunction on reading. [ABSTRACT FROM AUTHOR]

We followed the neuropsychological development of five children who underwent unilateral neurosurgery of the occipitoparietal lobes as a treatment for epilepsy caused by a developmental lesion (cortical dysplasia). The follow-up period ranged from 3-7 years postsurgery. Two participants had a verbal intelligence quotient (IQ) >100 and three had a verbal IQ between 65 and 80. All five children had abnormal nonverbal IQ and exhibited deficits related to visual attention, object recognition, and praxis. Nevertheless, our results suggest that brain plasticity after parietooccipital epilepsy surgery in young children allows for a schooling level of cognitive skills such as reading and arithmetic. Although recovery for visual perceptual cognition was more limited than for verbal functions, long-term neuropsychological outcomes showed that early surgery for epilepsy offers the possibility of optimizing cognitive outcomes in children with posterior intractable epilepsies. [ABSTRACT FROM AUTHOR]

Epilepsy is a chronic neurological condition that affects the quality of life (QoL) of patients and their families. In this study, we compare two sets of reports on QoL that were completed by two samples of parents whose children have epilepsy. Parents of children with various types of epilepsy from Spain (N = 196) and France (N = 219) completed the same QoL questionnaire. Medical variables were recorded from the referred specialist doctor for each patient. The factors associated with parental reports on QoL were similar in both countries. Parents of children with nonidiopathic generalized or unclassified epilepsy reported poorer QoL and the highest proportion of learning and behavioral problems. However, the intensity of difficulties varied between the two samples. This questionnaire made it possible to detect comorbidities and daily life difficulties in children with epilepsy and their families. The type of epilepsy had the same influence on Spanish and French families' ratings of QoL. Families shared the same comorbidities in terms of hyperactivity/attention/sociability problems. Nevertheless, the intensity of reported difficulties varied in both countries, possibly because of differences in cultural and educational environments. This aspect should be further explored in future research. • Childhood epilepsy affects the quality of life of patients and their families. • The poor quality of life in childhood epilepsy is a cross-cultural phenomenon. • Psychopedagogical and behavioral intervention is essential in children with epilepsy. [ABSTRACT FROM AUTHOR]

The reason why some children and adolescent with epilepsy (CAWE) still challenge the "inclusive" educative policy needs to be explored. We conducted a transversal study in French medical, social, and educative rehab centers (MSERCs) dedicated to CAWE to describe the profile of 263 centers-involved (CI)-CAWE. Centers-involved CAWE were prospectively followed from September 2012 to August 2013. Medical, social, and educative rehab centers were dichotomized according to their care-provider agreement (i.e., CAWE of "moderate" (M) vs. "severe" (S) conditions). Clinical factors known to impact clinical outcome and quality of life (QoL) in epilepsy and four disabling conditions at risk to impact school life (i.e., cognitive and psychiatric/behavioral disorders, risk of physical hazards (i.e., refractory seizures with unpredictable loss of tone and/or awareness), and one or more seizure/week) were evaluated. The electronic chart of the French collaborative database (namely GRENAT) was used for data collection allowing comparison with the profile of 731 "normally integrated and schooled" (NIS)-CAWE extracted from GRENAT and matching for generation (i.e., born between 1988 and 2006). Centers-involved CAWE's profile was found, after adjustment, to be associated with clinical factors and disabling conditions reflecting the poorest clinical outcome and health-related quality of life (HR-QoL) (all p < 0.001). A cutoff of two disabilities/child highly discriminated NIS-CAWE vs. CI-CAWE. Centers-involved CAWE of S-MSERCs were the most severe (all p < 0.001), and the type of cognitive disability (i.e., intellectual disability (ID) vs. specific learning disorders (SLD)) highly paralleled the types of MSERCs (S vs. M). Using a parent-informant questionnaire, the number of disabilities/child was found to correlate with both the evaluation of the impact of epilepsy (r = 0.47, p < 0.001) and the HR-QoL (r = 0.37, p < 0.001). A satisfactory social life was reported (83.8%) even after S vs. M dichotomization (77.2% vs. 94.7%; p < 0.001). Multiple disabilities rather than epilepsy per se challenge the inclusive educative policy. Evaluation of disabilities could be the missing bridge to optimize this policy and understand its limits. • More than 20 millions of children and adolescents (CA) suffer from epilepsy worldwide. • Epilepsy, more than any other chronic disease, disables CA's mental, behavioral, emotional, social and physical life. • International laws recommend moving to inclusive education whenever possible or to develop ethical alternative strategies. • We herein show that CA with epilepsy and multiple disabilities highly challenge the mainstream/educational system. • Evaluation of school-life disabilities could optimize the inclusive policy and understand its limits. [ABSTRACT FROM AUTHOR]

Objectives: Hypothalamic hamartomas (HHs) are rare developmental brain lesions associated with drug-resistant epilepsy and often subjected to epilepsy surgery. Brain somatic variants in genes affecting the Sonic hedgehog (Shh) and primary cilia signaling pathways have been implicated in approximately 50% of nonsyndromic HH cases. This study aims to characterize a new cohort of 9 HH cases and elucidate their genetic etiology., Methods: We recruited 9 HH cases including 8 nonsyndromic cases of which 4 were type IV HH. Genomic DNA was extracted from peripheral blood and surgical brain tissues, and somatic variants were investigated using high-depth whole-exome sequencing., Results: Pathogenic somatic variants in known HH genes ( GLI3 , OFD1 , and PRKACA) were identified in 7 of the 9 cases. In addition, a 2-hit mutational event comprising a germline variant (predicted to impair kinase activity) and a somatic loss-of-heterozygosity was identified in TNK2 , a gene encoding a brain-expressed tyrosine kinase., Discussion: Our findings reinforce the role of somatic variants in Shh and cilia genes in HH cases while also shedding light on TNK2 as a potential novel disease-causing gene. This study emphasizes the increasing importance of brain mosaicism in epilepsy disorders and underscores the critical role of genetic diagnosis derived from resected brain tissue., Competing Interests: The authors report no relevant disclosures. Go to Neurology.org/NG for full disclosures., (Copyright © 2024 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.)

Rasmussen's encephalitis is a rare neurological pathology affecting one cerebral hemisphere, therefore, posing unique challenges. Patients may undergo hemispherectomy, a surgical procedure after which cognitive development occurs in the isolated contralateral hemisphere. This rare situation provides an excellent opportunity to evaluate brain plasticity and cognitive recovery at a hemispheric level. This literature review synthesizes the existing body of research on cognitive recovery following hemispherectomy in Rasmussen patients, considering cognitive domains and modulatory factors that influence cognitive outcomes. While language function has traditionally been the focus of postoperative assessments, there is a growing acknowledgment of the need to broaden the scope of language investigation in interaction with other cognitive domains and to consider cognitive scaffolding in development and recovery. By synthesizing findings reported in the literature, we delineate how language functions may find support from the right hemisphere after left hemispherectomy, but also how, beyond language, global cognitive functioning is affected. We highlight the critical influence of several factors on postoperative cognitive outcomes, including the timing of hemispherectomy and the baseline preoperative cognitive status, pointing to early surgical intervention as predictive of better cognitive outcomes. However, further specific studies are needed to confirm this correlation. This review aims to emphasize a better understanding of mechanisms underlying hemispheric specialization and plasticity in humans, which are particularly important for both clinical and research advancements. This narrative review underscores the need for an integrative approach based on cognitive scaffolding to provide a comprehensive understanding of mechanisms underlying the reorganization in Rasmussen patients after hemispherectomy., (© 2024 the author(s), published by De Gruyter, Berlin/Boston.)

Objective: Lennox-Gastaut syndrome (LGS) is a severe form of epileptic encephalopathy, presenting during the first years of life, and is very resistant to treatment. Once medical therapy has failed, palliative surgeries such as vagus nerve stimulation (VNS) or corpus callosotomy (CC) are considered. Although CC is more effective than VNS as the primary neurosurgical treatment for LGS-associated drop attacks, there are limited data regarding the added value of CC following VNS. This study aimed to assess the effectiveness of CC preceded by VNS., Methods: This multinational, multicenter retrospective study focuses on LGS children who underwent CC before the age of 18 years, following prior VNS, which failed to achieve satisfactory seizure control. Collected data included epilepsy characteristics, surgical details, epilepsy outcomes, and complications. The primary outcome of this study was a 50% reduction in drop attacks., Results: A total of 127 cases were reviewed (80 males). The median age at epilepsy onset was 6 months (interquartile range [IQR] = 3.12-22.75). The median age at VNS surgery was 7 years (IQR = 4-10), and CC was performed at a median age of 11 years (IQR = 8.76-15). The dominant seizure type was drop attacks (tonic or atonic) in 102 patients. Eighty-six patients underwent a single-stage complete CC, and 41 an anterior callosotomy. Ten patients who did not initially have a complete CC underwent a second surgery for completion of CC due to seizure persistence. Overall, there was at least a 50% reduction in drop attacks and other seizures in 83% and 60%, respectively. Permanent morbidity occurred in 1.5%, with no mortality., Significance: CC is vital in seizure control in children with LGS in whom VNS has failed. Surgical risks are low. A complete CC has a tendency toward better effectiveness than anterior CC for some seizure types., (© 2023 The Authors. Epilepsia published by Wiley Periodicals LLC on behalf of International League Against Epilepsy.)

Functional hemispherectomy results in good outcomes in cases of refractory epilepsy and constitutes a unique situation in which to study cerebral plasticity and the reorganization of lateralized functions of the brain, especially in cases of infancy or childhood surgery. Previous studies have highlighted the remarkable ability of the brain to recover language after left hemispherectomy. This leads to a reorganization of language networks toward right hemisphere, causing limitation in the development of visuo-spatial abilities, known as a crowding effect in the right hemisphere. Deficits in nonverbal functions have also been described as a more direct consequence of right hemipherectomy, but the results from case studies have sometimes been contradictory. We conducted a group study which may effectively compare patients with left and right hemispherectomy and address the effects of the age of seizure onset and surgery. We analyzed the general visuo-spatial and visuo-perceptive abilities, including face and emotional facial expression processing, in a group of 40 patients aged 7-16 years with left (n = 24) or right (n = 16) functional hemispherectomy. Although the groups did not differ, on average, in general visuo-spatial and visuo-perceptive skills, patients with right hemispherectomy were more impaired in the processing of faces and emotional facial expressions compared with patients with left hemispherectomy. This may reflect a specific deficit in the perceptual processing of faces after right hemispherectomy. Results are discussed in terms of limited plasticity of the left hemisphere for facial and configural processing., (Copyright © 2022. Published by Elsevier Inc.)

Purpose: Corpus callosotomy is a palliative surgical procedure for patients with drug-resistant epilepsy and suffering from drop attacks, which are a source of major deterioration in quality of life and can be responsible for severe traumatic injury. The objective of this study is to identify clinical markers that would predict a better outcome in terms of drop attacks and other types of epileptic seizures., Methods: We reviewed a retrospective series of children who underwent complete corpus callosotomy at our institution, between January 1998 and February 2019. We analyzed the neurological and cognitive pre- and postoperative status, radiological datas, and electroencephalography (EEG) monitoring data., Results: Fifty children underwent a complete callosotomy at a mean age of 7.5 years. The median postoperative follow-up was 42.5 months. Forty-one patients (82%) had a favorable outcome, 29 (58%) of them becoming totally free of drop attacks. Statistical analysis of correlation between outcome of drop attacks and the characteristics of the patients did not find any trend in terms of age, etiology or developmental level. Regarding seizure types, the probability of being drop attack-free was significantly higher in case of tonic seizures (p = 0.017). Neurological complications occurred in two patients. A transient disconnection syndrome was observed in one child with good preoperative cognitive level. The mean hospital stay was short (5 -10 days)., Conclusion: The results of this large monocentric case series with a long follow-up indicate that total callosotomy is a safe and effective treatment for children with drug-resistant epileptic drop attacks. Aside from a better surgical outcome for children with tonic seizures causing the falls, the lack of any other significant prognostic factor implies that no patient should a priori be excluded from this palliative surgical indication., (Copyright © 2022. Published by Elsevier Ltd.)

Vertical parasagittal hemispherotomy (VPH) is a well-established surgical treatment which is proposed for children with widespread unilateral onset of intractable epileptic seizures. VPH allows to disconnect from a vertical transventricular approach all white matter fibers of the hemisphere around a central core including the thalamus. We present the case of a girl who underwent VPH for hemimegalencephaly in early infancy. Postoperatively, she developed unexpected seizures of mesio-temporal origin. Stereo-EEG provided arguments for an amygdalar origin. High-resolution MRI with tractography confirmed the presence of the amygdalo-fugal pathway to be responsible of epileptic discharges propagation. She became seizure-free after temporal resection., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Austria, part of Springer Nature.)

Objective: Report of the contribution of invasive EEG (iEEG) and epileptogenicity mappings (EM) in a pediatric cohort of patients with epilepsy associated with focal polymicrogyria (PMG) and candidates for resective surgery., Method: Retrospective pediatric case series of patients presenting focal PMG-related refractory epilepsy undergoing an invasive exploration (iEEG) at Fondation Rothschild Hospital. We reviewed clinical data, structural MRI, and visual analysis of iEEG recordings. Moreover, time-frequency analysis of SEEG signals with a neuroimaging approach (epileptogenicity maps) was used to support visual analysis., Results: Between 2012 and 2019, eight patients were selected. Five patients were explored with stereoelectroencephalography (SEEG) only, one patient with subdural exploration (SDE) only and two patients first underwent SEEG and then SDE. The mean age at seizure onset was 40.3 months (range 3-120), and the mean age for the iEEG 10.8 years (range 7-15). The epileptogenic zone (EZ) appeared concordant to the PMG lesion in only one case, was larger in three cases, smaller in two cases and different in one case. Four cases were selected for tailored resective surgery and one for total callosotomy. Two patients remained seizure-free at their last follow-up (mean 32.6 months, range 7-98). Epileptogenicity mapping (EM) refined the qualitative analysis, showing in four patients an EZ larger than visually defined., Conclusion: This study is the first pediatric study to analyze the value of iEEG and EM as well as operability in focal PMG-related refractory epilepsy. The results illustrate the complexity of this pathology with variable concordance between the EZ and the lesion and mixed response to surgery., (Copyright © 2021 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.)

The concept of vulnerability of the immature brain is multifactorial by definition. Newer scientific work in this area has shifted and enlarged the concept from theoretical frameworks to the multiple levels (molecular, cellular, anatomic, network, behavioral) of the organization of the growing brain. The concept of vulnerability was first introduced by Donald O. Hebb in the 1950s and referred to the inability of the immature brain to completely recover normal development after a brain insult. The concept of vulnerability was further extended to the limitations of the brain in the development of specific skills in neuronal substrates originally used for other functions. We present an overview of some neurodevelopmental processes that characterize the immature brain and that can predict vulnerability in the case of disturbances: Hebb's principle, synaptic homeostasis, selective vulnerability of immature cells in mammals, and inherited constraint networks. A better understanding of the vulnerability mechanisms may help in early detection and prevention and further proposed individualized therapeutic approaches to enhance children's developmental outcomes., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Epilepsy surgery raises hopes, but still remains reserved for a small number of cases of epilepsy resistant to medical treatments. It requires the involvement of multidisciplinary medical and allied health teams with expertise in this field. From the patient's admission through to their discharge, the nurse and the electroencephalogram technician have an essential role to play., (Copyright © 2015 Elsevier Masson SAS. All rights reserved.)

Objective: Hemispherotomy techniques have been developed to reduce complication rates and achieve the best possible seizure control. We present the results of our pediatric patients who underwent vertical parasagittal hemispherotomy and evaluate the safety and global long-term outcome of this technique., Methods: Eighty-three patients underwent vertical parasagittal hemispherotomy by the same neurosurgeon (OD) between 1990 and 2000. We reviewed all patients between 2001 and 2003 for a standard global evaluation. The general principle is to achieve, through a posterior frontal cortical window, the same line of disconnection as performed with the classic hemispherectomy, while leaving the majority of the hemisphere intact along with its afferent and efferent vascular supply., Methods: Seventy-four percent of the patients were seizure-free; among them, 77% were seizure-free without further drug treatment. Twelve percent rarely had seizures (Engel Class II) and 14% continued to have seizures (Engel Class III or IV). The results varied according to the etiology, but this variation was not statistically significant. The early postoperative course was uneventful for 94% of the children, and shunt placement was necessary in 15%. We found a correlation between the preoperative delay and the Vineland Adaptive Behavior score: children with a longer duration of seizures had lower performances., Conclusion: Vertical parasagittal hemispherotomy is an effective surgical technique for hemispheric disconnection. It allows complete disconnection of the hemisphere through a cortical window with good results in terms of seizure outcome and a comparably low complication rate.

Objective: A small percentage of tuberous sclerosis patients will develop a subependymal giant-cell astrocytoma. Given the morbidity and mortality when such a lesion is left undiagnosed, successive follow-up imaging in pediatric patients has been recommended. Surgical removal of the lesion has become the procedure of choice; however, the timing of this surgery is still a controversial subject. By analyzing our own series of data, as well as other published series, we have attempted to reach a consensus on the benefits of early versus late surgery., Methods: We retrospectively reviewed 19 patients treated surgically for intraventricular tumors in Foch Hospital and at the Fondation Adolphe de Rothschild in Paris, France, and we analyzed published pediatric reports from 1980 to 2006., Results: The results from our own population, as well as from other published pediatric series (15 series), indicate that subependymal giant-cell astrocytomas have a good prognosis when a macroscopically total resection has been performed. In our series, residual lesions tended to enlarge, but residual tumors remaining stable have been reported. Careful follow-up examination should be undertaken because late recurrences do occur. Larger or symptomatic lesions tend to have a higher morbidity., Conclusion: We think that any lesion fulfilling the criteria for a subependymal giant-cell astrocytoma as previously described in the literature (lesion around the foramen of Monro, greater than 5 mm, with incomplete calcifications) should be removed as soon as clear evidence of growth has been confirmed.

Objective: Hypothalamic hamartomas (HHs) require surgical treatment in patients presenting with refractory epilepsy., Methods: The authors report on a single-center series of 33 patients (24 males, 9 females) who underwent surgery between January 1997 and April 2004. They experienced several types of seizure (gelastic, tonic, partial, atonic, generalized tonic-clonic, dacrystic, infantile spasm, mental retardation, and behavioral and endocrinological abnormalities). Forty-nine interventions were carried out. Every patient, with the exception of the first, underwent hamartoma disconnection (pterional approach, six patients; endoscopy, 15 patients; both, 11 patients). The endoscopic approach was carried out with a frameless stereotactic system to enhance feasibility and efficacy of the disconnecting procedure., Results: Surgery-related neurological complications occurred in two patients, both after a pterional microsurgical approach. Furthermore, two patients experienced panhypopituitarism and one patient experienced transitory central insipid diabetes. All patients but one showed recovery or considerable improvement of their epilepsy (Engel Class 1, 48.5%; Engel Class 2, 3%; Engel Class 3, 45.5%; mean follow-up duration, 1 yr 7 mo)., Conclusion: According to the proposed classification of sessile HH into four types, the best candidates for endoscopic disconnection are Type 2 and Type 3 HHs. In the present series, 90% of patients affected by Type 2 HH became seizure free and the remaining 10% improved; of those with Type 3 HH at presentation, 35.3% recovered and 60% improved. Neuropsychological and endocrinological test results showed improvement in many patients. Data from our series demonstrate that frameless stereotactic endoscopic disconnection should be considered as the treatment of choice in the presence of favorable anatomic conditions.