Multimodal magnetic resonance imaging for assessment of motor function after hemispherectomy in children with refractory epilepsy / 中华实用儿科临床杂志

Hemispherectomy (HS) is an effective surgical therapy to treat refractory epilepsy caused by diffuse hemispheric disorders.After HS surgery, the seizures are often well controlled in patients, and the bilateral limb motor function improves even recovers in some children.This result reveals the strong neuronal plasticity of the brain.The brain, especially of young children, can achieve functional reorganization and cortical remodeling after extensive damage.Multimodal magnetic resonance imaging techniques can jointly analyze the structure and function of the brain and associated neuroimaging features to reveal the neurophysiological mechanisms underlying improved motor function.In this paper, the progress of multimodal magnetic resonance imaging research on motor function alterations in children after hemispherectomy was reviewed.

Hemispherectomy in treatment of seizures: current perspectives of the indications, techniques and complications: literature review

Introducción: A hemisferectomía es un procedimiento valioso en el tratamiento de trastornos convulsivos causados por desordenes hemisféricos unilaterales. El hemisferectomía anatómica se ha utilizado para este fin desde 1938, sin embargo, se abandonó este procedimiento después de informes de complicaciones postoperatorias causadas por hemosiderosis superficial, ependimitis e hidrocefalia obstructiva. Así que, se ha mostrado en la literatura modificaciones en las indicaciones y técnicas de hemisferectomía anatómica cuya finalidad es la de reducir la incidencia de esta complicación sin dejar de lograr control de las convulsiones. Sobre la base de la literatura, la hemisferectomía mejora la calidad de vida de los pacientes que tiene la indicación para realizar este procedimiento, ya que permite reducir la frecuencia de las convulsiones, si tónica o átona, tónico-clónicas Objetivo: El objetivo de esta revisión de la literatura es discutir los detalles técnicos, modalidades, riesgos, complicaciones, resultados y de pronóstico de hemisferectomía basado en la revisión crítica de la literatura. Casuística y Métodos: Se realizó la consulta bibliográfica, utilizando la base de datos MEDLINE, LILACS, SciELO, que utiliza el lenguaje como criterios de selección, la elección de los artículos recientes preferiblemente en portugués, español o inglés. Conclusión: Según las referencias, hemisferectomía es un procedimiento con buen resultado para las personas con convulsiones derivadas cuando está indicado para casos seleccionados y la tasa de éxito no es proporcional a la extensión de la resección del tejido neuronal. A mayor resección puede o no reducir la frecuencia de las crisis, sin embargo, la incidencia de la morbilidad puede ser mayor.

Background: The hemispherectomy is a valuable procedure in the management of seizure disorders caused by unilateral hemispheric disease. The anatomical hemispherectomy has been used for this purpose since 1938, however, it was abandoned after reports of postoperative complications caused by superficial hemosiderosis, ependymitis and obstructive hydrocephalus. So that, it has been showed modifications in the techniques of hemispherectomy whose the purpose is reduce the incidence of this complications while still achieving seizure control. Based on literature, the hemispherectomy improves the quality of life of patients that has the indication to perform this procedure because it allows reducing the frequency of seizures, whether tonic or atonic, tonic-clonic. Aim: The aim of this literature review is discuss the indications, technical details, modalities, risks, complications, results as well de prognosis of callosotomy based on critical literature review and the authors experience. Casuistry and Methods: It was performed bibliographical consultation, using the databases MEDLINE, LILACS, SciELO, utilizing language as selection criteria, choosing preferably recent articles in Portuguese, Spanish or English. Conclusion: According to references, the functional hemispherectomy has a good outocome for those with seizures arisin when indicated to selected cases and the success rate is not proportional to the extent of neuronal tissue resection. So that, a greater resection cannot necessarily reduce the seizure frequency, however the morbidity may also be larger.

Absence of hemispatial neglect following right hemispherectomy in a 7-year-old girl with neonatal stroke

Neonatal stroke leads to cognitive deficits that may include hemispatial neglect. Hemispatial neglect is a syndrome after stroke that patients fail to be aware of stimuli on the side of space and body opposite a brain lesion. We report here a 7-year-old girl who suffered neonatal right brain stroke and underwent right hemispherectomy due to refractory epilepsy. Post-surgical observation of the child’s behavior and tests did not show any signs of hemispatial neglect. We concluded the spatial attention function of the child with neonatal stroke might be transferred to the contralateral side during early childhood.

Hemispherotomy for hemisphericepilepsy: outcome and early follow up for complications / 基础医学与临床

Objective To investigate the results and complications of hemispherotomy for drug resistant hemispheric epilepsy.Methods The authors reviewed 5 patients who were diagnosed as drug resistant hemispheric epilepsy and operated in the neurosurgery department of Peking Union Medical College Hospital from 2011 to 2013.All the 5 patients were underwent hemispherotomy after carefully multidisciplinary pre-operation evaluation.Results All patients tolerated the procedure well and the postoperative course was smooth.All the 5 patients didn`t have seizure in the period of following up of 46 to 69 months.Conclusions According to literatures and the authors` experience, hemispherotomy is as safe and efficient for hemispheric epilepsy as hemispherectomy.It is most important advance for hemispherectomy.The procedure of hemispherotomy is complex but not very difficult, illustrating a good prospect of application and extension.

Factors Related with Neurological Function in Children after Hemispherectomy for Intractable Epilepsy / 中国康复理论与实践

Objective To explore the factors related with neurological function in children after hemispherectomy for intractable epilep-sy. Methods Thirty-three children suffered hemispherectomy from May, 2014 to June, 2015 were analyzed. Their preoperative data were col-lected. A structured questionnaire was used to evaluate the neurological function. The relationship between preoperative parameters and post-operative functional outcomes was analyzed. Results Bilateral lesions in MRI (P<0.001) and age (P<0.001) were related with functional out-come. Conclusion The prognosis of hemispherectomy for children with intractable epilepsy is related to the contralateral lesion and age.

Cirugía de epilepsia en el Hospital Nacional Guillermo Almenara: primeros siete casos / Epilepsy surgery in Guillermo Almenara Hospital: First seven cases

La epilepsia tiene una incidencia de 1 % en la población, de los cuales un tercio no responde al tratamiento farmacológico, que conlleva a discapacidad y morbilidad secundaria. Los pacientes con epilepsia refractaria requieren un estudio multidisciplinario para el origen de la epilepsia y realizar la resección quirúrgica. En cinco pacientes del Hospital Almenara hicimos la evaluación clínica, neuropsicológica, estudio estructural con resonancia magnética, PET y SPECT y videoelectroencefalografía de cuero cabelludo e intracraneal, con lo que realizamos la cirugía resectiva correspondiente. En 71 % de los pacientes se logró la supresión completa de las crisis epilépticas y en 29 % restantes, la disminución de la frecuencia en 75 %. La morbilidad adicional en los pacientes operados fue leve y manejable. El manejo multidisciplinario y tratamiento quirúrgico de la epilepsia refractaria en el hospital Almenara es altamente eficaz y se requiere la implementación de una unidad de Cirugía de Epilepsia.

Epilepsy has 1 % prevalence, until one third are refractory to pharmacological treatment, so it produces disability, morbidity and mortality. These patients requires multidisciplinary diagnostic approach to localize the origin and ideally resect it. In Almenara Hospital, seven patients has been studied clinically, neuropsychologically, brain imaging: structure magnetic resonance and functional with positron emission tomography and single photon emission computed tomography and video electroencephalography non invasive initially and electrocorticography. After the study we decided and performed respective surgery. In 71 % of patients we have got complete suppression of seizures and in the remain 29 %, more than 75 % in seizure frequency. The side effects were mild and possible to treat. The multidisciplinary approach and surgical treatment of refractory epilepsy in the Almenara Hospital is highly efficient and it is needed to install an Specialized Unit.

Clinical study of functional hemispherectomy for pediatric intractable epilepsy with hemisphere lesions / 实用医学杂志

Objective To study the results of functional hemispherectomy for pediatric intractable epilepsy with hemisphere lesions , and to explore the indications of functional hemispherectomy , as well as the prognosis. Methods Twenty-four children with intractable epilepsy caused by hemisphere lesions were included , who underwent functional hemispherectomy. All patients were followed up for 3 ~ 48 months. Results All 24 cases obtained improvement of abnormal behavior , and no significant exacerbation of neurological deficits was observed. During the follow-up, 18 cases were in EngelⅠ(75%), 5 in Engel Ⅱ(21%), and 1 in Engel Ⅰ(4%) respectively. No long-term complication was observed. Conclusion Comprehensive presurgical evaluation is a prerequisite for the surgical treatment of pediatric intractable epilepsy caused by hemisphere lesions. Functional hemispherectomy can effectively control seizures and obviously improve the prognosis.

Long-Term Outcomes of Hemispheric Disconnection in Pediatric Patients with Intractable Epilepsy

BACKGROUND AND PURPOSE: Hemispherectomy reportedly produces remarkable results in terms of seizure outcome and quality of life for medically intractable hemispheric epilepsy in children. We reviewed the neuroradiologic findings, pathologic findings, epilepsy characteristics, and clinical long-term outcomes in pediatric patients following a hemispheric disconnection. METHODS: We retrospectively studied 12 children (8 males) who underwent a hemispherectomy at Asan Medical Center between 1997 and 2005. Clinical, EEG, neuroradiological, and surgical data were collected. Long-term outcomes for seizure, motor functions, and cognitive functions were evaluated at a mean follow-up of 12.7 years (range, 7.6-16.2 years) after surgery. RESULTS: The mean age at epilepsy onset was 3.0 years (range, 0-7.6 years). The following epilepsy syndromes were identified in our cohort: focal symptomatic epilepsy (n=8), West syndrome (n=3), and Rasmussen's syndrome (n=1). Postoperative histopathology of our study patients revealed malformation of cortical development (n=7), encephalomalacia as a sequela of infarction or trauma (n=3), Sturge-Weber syndrome (n=1), and Rasmussen's encephalitis (n=1). The mean age at surgery was 6.5 years (range, 0.8-12.3 years). Anatomical or functional hemispherectomy was performed in 8 patients, and hemispherotomy was performed in 4 patients. Eight of our 12 children (66.7%) were seizure-free, but 3 patients with perioperative complications showed persistent seizure. Although all patients had preoperative hemiparesis and developmental delay, none had additional motor or cognitive deficits after surgery, and most achieved independent walking and improvement in daily activities. CONCLUSIONS: The long-term clinical outcomes of hemispherectomy in children with intractable hemispheric epilepsy are good when careful patient selection and skilled surgical approaches are applied.

The usefulness of neuronavigation in functional hemispherectomy / Aplicabilidade da neuronavegação em hemisferectomia funcional

Catastrophic epileptic encephalopathies in children comprise devastating conditions that features cerebral dysfunction in association with refractory epileptic seizures. The diagnosis is based on the clinical findings, on magnetic resonance imaging (MRI) of the brain and on electroencephalographic findings. For these conditions, surgery remains essential for attaining seizure control. We report two cases of 5-year-old girls. The first one had a diagnosis of Rasmussen’s syndrome. The second one had a large porencephalic cyst secondary to perinatal cerebral ischemia. Despite trials of anticonvulsants, both patients deteriorated, and a functional hemispherectomy guided by neuronavigation was indicated and performed, with low morbidity and excellent seizure control. The neuronavigation proved to be a valuable guidance tool in performing the functional hemispherectomy, making the disconnections more accurate, and thus decreasing the surgical time and blood loss.

Aplicabilidade da neuronavegação em hemisferectomia funcional As encefalopatias epilépticas catastróficas da infância compreendem condições graves que associam disfunção cerebral e crises epilépticas refratárias. Seu diagnóstico é firmado com base nos dados clínicos e nos achados de ressonância magnética e eletrencefalográficos. Para algumas destas condições o tratamento cirúrgico continua sendo essencial para o controle das crises. Relatamos dois casos de pacientes de 5 anos. A primeira teve diagnóstico de síndrome de Rasmussen. A segunda tinha antecedentes de encefalopatia hipóxico-isquêmica perinatal. Ambas apresentaram epilepsia parcial refratária em associação com rápida deterioração neurológica, e foram submetidas à hemisferectomia funcional com auxílio da neuronavegação, com baixa morbidade e excelente controle das crises. A neuronavegação se mostrou como uma valiosa ferramenta na realização da hemisferectomia funcional, possibilitando desconexões mais precisas, menor tempo de cirurgia e menor perda sanguínea.

Cerebral Paragonimiasis and Bo Sung Sim's Hemispherectomy in Korea in 1950s-1960s / 의사학

This paper deals with cerebral paragonimiasis and cerebral hemispherectomy conducted as a treatment of cerebral paragonimiasis by Bo Sung Sim in Korea in 1950s-1960s. He demonstrated that cerebral hemispherectomy could be used for unilateral diffuse cerebral paragonimiasis. Sim learned cerebral hemispherectomy from Dr. L. A. French. at the University of Minnesota from 1955 to 1957 in America. The authors argues that Bo Sung Sim's introduction of cerebral hemispherectomy to Korea was not a simple application of an advanced medical technology, but a complicated and active process in that Sim used the technique to intervene intractable complications from cerebral paragonimiasis such as generalized convulsions, spastic hemiplegia and mental deterioration. Bo Sung Sim, one of the neurosurgeons of the first generation in Korea, was trained in neurology, neuropathology, neuroradiology and animal experiments as well as in neurosurgery at the University of Minnesota. After returning to Korea, Sim faced parasitic diseases, one of the most serious public health problems at that time, which were far different from what he learned in America. As a neurosurgeon, Sim tackled with parasitic diseases of the central nervous system with various diagnostics and therapeutics. In 1950s, more than one million populations suffered from pulmonary paragonimiasis acquired by eating raw crabs or by feeding juice of crushed crayfish for the treatment of measles in Korea. About 26.6 percent of people with paragonimiasis had cerebral paragonimiasis. Before bithionol therapy was introduced in 1962, neurosurgery was the only available treatment to control increased intracranial pressures, intractable epilepsy, paralysis and mental deterioration. Between 1958 to 1962, Bo Sung Sim operated on 24 patients of cerebral paragonimiasis. In two of them, he performed cerebral hemispherectomy to control intractable convulsions when he found diffuse cerebral paragonimiasis and cerebral atrophy at the operating table. The two patients were recovered dramatically after the operation. The first patient became a part of medical campus for 20 years after hemispherectomy, doing chores at the hospital and helping Bo Sung Sim for his teaching neuroanatomy. The presence of the hemispherectomized patient in the classroom impressed the students deeply. Furthermore, the hemispherectomized patient stimulated Sim and his school to perform research upon the neuroanatomy and neurophysiology of the brain with hemispherectomized animals.

Epilepsy Surgery II

Epilepsy surgery is classified into two types: curative epilepsy surgery and palliative surgery. The most frequently performed curative epilepsy surgery is an anterior temporal lobectomy with amygdalohippocampectomy (ATL with AH). ATL with AH includes the resection of epileptic hippocampus/amygdala and anterior temporal lobe (3~4cm from temporal pole) and is performed for treating drug refractory mesial temporal lobe epilepsy. A literature reports that more resection of epileptic hippocampus had a better surgical outcome. However, a surgery should be planned to prevent or minimize a postsurgical memory decline especially in resection of a dominant temporal lobe. Cortisectomy is a resection of localized epileptic focus in patients with neocortical epilepsy such as frontal, parietal, occipital, and lateral temporal lobe epilepsies. Most of neocortical epilepsy patients need an intracranial electrode implantation for determination of resection margin and a brain stimulation on intracranial electrodes for functional mapping. For a successful cortisectomy, an epilepsy surgery team should have a good amount of knowledge and experiences in intracranial EEG monitoring for intractable epilepsy patients. It is very important to place the intracranial electrodes at a brain region where epileptic focus is located because a wrong placement of intracranial electrodes results in failure of surgery. The surgical principles of functional hemispherectomy (FH) aim at disconnecting the hemisphere while leaving as much of the ipsilateral brain as possible intracranially; it has been characterized as anatomically subtotal but physiologically complete hemispherectomy. The original technique consists of a large central tissue removal, complete callosotomy, frontal and parieto-occipital disconnection, temporal lobectomy and insular corticectomy. The candidates of FH are drug refractory partial epilepsy patients who have unilateral epileptic focus and severe brain damage in ipsilateral hemisphere with loss of finger movements of contralateral hand. Corpus callosotomy is a surgical technique severing the corpus callosum so that communication between the cerebral hemispheres is interrupted. In contrast with lobectomy, corpus callosotomy does not involve removing any brain tissue. Instead, it usually involves cutting the front two-thirds of this bundle (anterior callosotomy). Sometimes the other one-third is cut later (complete callosotomy). Corpus callosotomy is most effective for atonic seizures ("drop attacks"), less effective for tonic-clonic seizures and tonic seizures. Additionally, multiple subpial transection and neurostimulation techniques are described.

Surgical Treatment of Epilepsy: Extratemporal Operative Methods

The extratemporal operative approaches for intractable epilepsy are reviewed. Intracranial recordings are often necessary for extratemporal epilepsy to define the lateratization as well as the localization of epileptogenic regions and tailor the resection. The operative procedures include cortical resections, corpus callosotomy, and functional hemispherectomy. The author presents an overview of indications for surgery, oprative methods, and risks in the medically intractable epileptic patient with extratemporal focus.