Insights on cognitive reorganization after hemispherectomy in Rasmussen's encephalitis. A narrative review.

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.

Determinants of Functional Outcome after Pediatric Hemispherotomy.

Objective: We aimed to evaluate determinants of functional outcome after pediatric hemispherotomy in a large and recent multicenter cohort.

Methods: We retrospectively investigated the functional outcomes of 455 children who underwent hemispherotomy at 5 epilepsy centers in 2000-2016. We identified determinants of unaided walking, voluntary grasping with the hemiplegic hand, and speaking through Bayesian multivariable regression modeling using missing data imputation.

Detection of brain somatic mutations in focal cortical dysplasia during epilepsy presurgical workup.

Brain-restricted somatic variants in genes of the mechanistic target of rapamycin signalling pathway cause focal epilepsies associated with focal cortical dysplasia type II. We hypothesized that somatic variants could be identified from trace tissue adherent to explanted stereoelectroencephalography electrodes used in the presurgical epilepsy workup to localize the epileptogenic zone. We investigated three paediatric patients with drug-resistant focal epilepsy subjected to neurosurgery.

Not surgical technique, but etiology, contralateral MRI, prior surgery, and side of surgery determine seizure outcome after pediatric hemispherotomy.

Objective: We aimed to assess determinants of seizure outcome following pediatric hemispherotomy in a contemporary cohort.

Methods: We retrospectively analyzed the seizure outcomes of 457 children who underwent hemispheric surgery in five European epilepsy centers between 2000 and 2016. We identified variables related to seizure outcome through multivariable regression modeling with missing data imputation and optimal group matching, and we further investigated the role of surgical technique by Bayes factor (BF) analysis.

Cognitive outcome after left functional hemispherectomy on dominant hemisphere in patients with Rasmussen encephalitis: beyond the myth of aphasia. Patient series.

Background: Rasmussen encephalitis is a rare chronic neurological pathology frequently treated with functional hemispherectomy (or hemispherotomy). This surgical procedure frees patients of their severe epilepsy associated with the disease but may induce cognitive disorders and notably language alterations after disconnection of the left hemisphere.

Observations: The authors describe longitudinally 3 cases of female patients with Rasmussen encephalitis who underwent left hemispherotomy in childhood and benefited from a favorable cognitive outcome.

Complete callosotomy in children with drop attacks; A retrospective monocentric study of 50 patients.

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.

Vertical parasagittal hemispherotomy: a case report of postoperative mesio-temporal seizures via amygdalofugal pathway.

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.

Epilepsy surgery in infants up to 3 months of age: Safety, feasibility, and outcomes: A multicenter, multinational study.

Objective: Drug-resistant epilepsy (DRE) during the first few months of life is challenging and necessitates aggressive treatment, including surgery. Because the most common causes of DRE in infancy are related to extensive developmental anomalies, surgery often entails extensive tissue resections or disconnection. The literature on "ultra-early" epilepsy surgery is sparse, with limited data concerning efficacy controlling the seizures, and safety.

Focal polymicrogyria in children: Contribution of invasive explorations and epileptogenicity mapping in the surgical decision.

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.

Memory dysfunctions.

Since the seminal work on the patient HM, who in his adulthood presented an acquired amnesic syndrome following the resection of the bilateral temporal lobe, other research has described several cases of isolated memory dysfunction in children. This chapter presents developmental and long-lasting memory disorders emerging from an organic or neurologic cause at birth or in infancy. More notably, we focus on developmental amnesic syndrome caused by neonatal bihippocampal damage and memory dysfunction caused by medial temporal developmental epilepsy.

The vulnerability of the immature brain.

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.

Parental report of quality of life in children with epilepsy: A Spanish/French comparison.

Background: 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.

Method: Parents of children with various types of epilepsy from Spain (N = 196) and France (N = 219) completed the same QoL questionnaire.

Children and adolescents with epilepsy in rehabilitation centers: A French prospective transversal study.

Introduction: The reason why some children and adolescent with epilepsy (CAWE) still challenge the "inclusive" educative policy needs to be explored.

Methods/patients: 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.

Seizure outcome and prognostic factors for surgical management of hypothalamic hamartomas in children.

Purpose: Hypothalamic hamartomas (HH) are rare benign lesions frequently associated with gelastic seizures early in life. Epilepsy can progress to multiple seizure types with cognitive impairment and behavioural disturbance, leading in some cases to epileptic encephalopathy.

Methods: We reviewed a retrospective series of 112 children treated in a single center, between 1998 and 2017.

Dissecting the genetic basis of focal cortical dysplasia: a large cohort study.

Genetic malformations of cortical development (MCDs), such as mild MCDs (mMCD), focal cortical dysplasia (FCD), and hemimegalencephaly (HME), are major causes of severe pediatric refractory epilepsies subjected to neurosurgery. FCD2 are characterized by neuropathological hallmarks that include enlarged dysmorphic neurons (DNs) and balloon cells (BCs). Here, we provide a comprehensive assessment of the contribution of germline and somatic variants in a large cohort of surgical MCD cases.

ILAE survey of neuropsychology practice in pediatric epilepsy surgery evaluation.

To determine the extent to which specific neuropsychological measures are in common use around the world for the assessment of children who are candidates for epilepsy surgery. As part of the work of the International League Against Epilepsy Pediatric Surgical Task Force, a survey was developed and distributed online. The survey consisted of questions related to demographics, training experience, general practice, and specific measures used and at what frequency.

Language plasticity after hemispherotomy of the dominant hemisphere in 3 patients: Implication of non-linguistic networks.

The neural networks involved in language recovery following hemispherotomy of the dominant hemisphere after language acquisition in children remain poorly known. Twelve hemispherotomized children (mean age at surgery: 11.3years) with comparable post-operative neuropsychological patterns underwent multi-task language functional MRI.

Anti-tumor necrosis factor alpha therapy (adalimumab) in Rasmussen's encephalitis: An open pilot study.

Objective: Rasmussen's encephalitis (RE) is a severe chronic inflammatory brain disease affecting one cerebral hemisphere and leading to drug-resistant epilepsy, progressive neurologic deficit, and unilateral brain atrophy. Hemispherotomy remains the gold standard treatment but causes permanent functional impairment. No standardized medical treatment protocol currently exists for patients prior to indication of hemispherotomy, although some immunotherapies have shown partial efficacy with functional preservation but poor antiseizure effect.