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.

Seizure Outcome after Anterior versus Complete Corpus Callosotomy in Children: A Systematic Review with Meta-Analysis / Controle das crises epilépticas após calosotomia anterior versus completa em crianças: uma revisão sistemática com metanálise

Introduction Refractory epilepsy is a debilitating and challenging condition to manage. Corpus callosotomy (CC) seems to be an effective treatment option for patients with seizures not amenable to focal resection. The aim of the present study is to compare seizure outcome of pediatric patients following anterior CC, compared with complete CC. Method The authors performed a systematic review and meta-analysis of the English literature involving comparative studies. Results The present investigation includes four retrospective case-controlled studies and authors perform a pooled analysis of the surgical results. Seizure outcome presented favorable results in patients who underwent complete CC (Odds Ratio, M-H, Fixed, 95% CI: 3.02 [1.43, 6.387], p-value: 0.005). Clinical and neurological complications occurred independently when a complete or anterior CC was performed. Conclusion Complete CC seems to be the most effective treatment option to control intractable seizure in children not amenable to focal resection.

Introdução Epilepsia refratária é uma condição debilitante e desaadora para lidar. Calosotomia parece ser uma opção de tratamento ecaz para pacientes com convulsões não passíveis de ressecção focal. O objetivo do presente estudo é comparar o resultado de convulsões em pacientes pediátricos de acordo com calosotomia anterior e completa. Métodos Uma revisão sistemática e metanálise da literatura médica em inglês envolvendo estudos comparativos. Resultados Quatro casos retrospectivos foram incluídos na presente investigação e uma análise dos resultados cirúrgicos foi realizada. Convulsões decorrentes tiveram resultados favoráveis em pacientes submetidos a calosotomia complete (odds ratio, M-H, xo, 95% IC: 3,02 [1,43; 6,387], valor de p: 0,005). Complicações clínicas e neurológicas ocorreram independentemente de se calosotomia complete ou anterior. Conclusão Calosotomia completa parece ser a opção de tratamento mais ecaz para controlar convulsões não rastreáveis e não passíveis de ressecção focal em crianças.

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.

Pathologic features of ulegyria in refractory epilepsy by modified anatomic hemispherectomy: a clinicopathologic study of 39 cases / 中华病理学杂志

<p><b>OBJECTIVE</b>To investigate the clinicopathologic features of the brain tissue diagnosed as ulegyria from modified anatomic hemispherectomy for refractory epilepsy.</p><p><b>METHODS</b>The clinical and pathologic findings were reviewed in 39 patients who underwent modified anatomic hemispherectomy and diagnosed as ulegyria in the Epilepsy Center of Tsinghua University Yuquan Hospital from 2007 to 2011.</p><p><b>RESULTS</b>All patients including 30 males and 9 females had medically intractable seizures, and the mean age of seizure onset and disease duration were 4.0 years and 7.3 years respectively. Significant history included febrile seizure in 14 patients (35.9%), cerebral hemorrhage in 8 patients (20.5%), fetal distress and surgical trauma each in 6 patients (15.4%), vascular malformation and cerebral hemorrhage in 1 patient (2.6%), and unclear history in 4 patients (10.2%). Histologically, all cases were characterized by cortical destruction, with neuronal loss and gliosis. All cases were accompanied by varying degree of cortical dysplasia, which were diagnosed as focal cortical dysplasia IIId. Hippocampus sclerosis was identified in 2 cases. Seizure outcome after surgery revealed 37 patients (94.9%) had an Engel grade I, two patients (5.1%) had an Engel grade II.</p><p><b>CONCLUSIONS</b>Febrile seizure, cerebral hemorrhage, fetal distress and surgical trauma in childhood can lead to refractory epilepsy. Histopathological change in the brain is ulegyria accompanied by focal cortical dysplasia IIId. Modified anatomic hemispherectomy is an effective therapy to treat those patients with extensive changes of one hemisphere.</p>

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.

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.

Upper limbs motor maps in cortex and plasticity after the anatomical hemispherectomy / 中华外科杂志

<p><b>OBJECTIVE</b>To locate motor functional area of patients who undergone modified anatomical hemispherectomy in order to analysis the plasticity of upper limbs motor.</p><p><b>METHODS</b>The patients who undergone modified anatomical hemispherectomy were performed BOLD sequences, to locate functional cortical areas in their residual brain.</p><p><b>RESULTS</b>6 patients have performed examination of BOLD sequences by 3.0-T MRI.5 of them obtained contralateral upper limb motor areas in their residual brain, and 3 of them obtained ipsilateral and contralateral upper limb motor area map in cortex. The ipsilateral upper limb motor areas in the M1, SMA and posterior parietal cortex.</p><p><b>CONCLUSIONS</b>The patients who undergone modified anatomical hemispherectomy is an excellent model to investigate mechanism of plasticity in the developing brain. Functional magnetic resonance (fMRI) provided fine spatial detail of brain responses, would describe the motor functional area of cortical maps. These patients exist ipsilateral motor areas in their residual mono hemisphere. The study indicated there maybe have somewhat extent of correlation between the surgical procedure and the outcome of neuroplasticity.</p>

Long-term P300 in hemispherectomized patients / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>In the years around 1990, in Beijing Tiantan Hospital Affiliated to Capital Medical University many children with infantile hemiplegia and intractable epilepsy were treated with further modified anatomical hemispherectomy. We report the follow up of the first six cases. To make good use of these precious clinical data and make clear their neuropsychological state, we performed neuropsychological and neurophysiological measurements in these patients, who were at a median of 17.8 years after hemispherectomy.</p><p><b>METHODS</b>Oddball task was given to the patients and to a normal control group to collect the peak latency (PL) and peak amplitude (PA) of event-related potentials (ERPs)-P300. The P300 data of the two groups were analyzed and the P300 patterns of the six patients are presented. The baseline characteristics and long-term follow-up of the six hemispherectomized patients, especially the long-term seizure control and cognitive function after surgery, are described.</p><p><b>RESULTS</b>Five patients had no seizures and one was almost seizure-free during the years after surgery. Clear P300 was obtained from every electrode in the patients. Differences of P300 between patients and normal control group had no statistical significance. And the maximum PA was at the site of electrode Pz or Cz which was consistent with that of the control group and with previous findings.</p><p><b>CONCLUSIONS</b>Further modified anatomical hemispherectomy has preferable long-term antiepileptic effects. The P300 results of the patients mean that the basic cognitive function of the patients has no difference from the control group. This reflects the plasticity of the hemisphere to some extent and increases the affirmation of the long-term curative effects of further modified anatomical hemispherectomy from both neuropsychological and neurophysiological aspects.</p>

Motor evoked potential of both upper extremities long term after anatomical hemispherectomy / 中华外科杂志

<p><b>OBJECTIVE</b>To explore the function and motor pathway of remained cerebral hemisphere by studying motor evoked potential of both upper extremities on patients long term after anatomical hemispherectomy.</p><p><b>METHODS</b>Five patients after anatomical hemispherectomy, who were marked 5 dispersive sites on head to perform transcranial magnetic stimulation. Recording motor evoked potential of target muscles (brachioradialis muscle and abductor pollicis brevis) of both upper extremities respectively when muscle resting and contracting.</p><p><b>RESULTS</b>Only affected abductor pollicis brevis of case 2 and only affected brachioradialis muscle of case 4 and 5 recorded motor evoked potential when muscle resting. Motor evoked potential of some cases can be recorded simultaneously in homonymous muscles of both sides when muscle resting or contracting.</p><p><b>CONCLUSIONS</b>There exists motor cortex that controls movement of ipsilateral limbs and also ipsilateral motor pathway of corticospinal connection at patients after anatomical hemispherectomy. It also means that the motor function of affected limbs has potency to recover well after hemispherectomy. The mirror movement after hemispherectomy is possible relate to overlap of both limbs' motor cortex.</p>

Epilepsy surgery in children

In the majority of patients with intractable epilepsy, seizures can be well controlled with appropriate medication. However, current estimates indicate that some of patients with epilepsy are refractory to all forms of medical therapy. The surgical treatment of intractable epilepsy in children has evolved with advances in technical innovations. These medically intractable patients are candidates for surgical treatment in an attempt to achieve better seizure control. The definitive successful outcome of epilepsy surgery is a seizure-free state without significant neurological impairments. In this article, we will outline the essential elements of presurgical evaluation and describe a variety of therapeutic surgical options, and the related indications, techniques, results and complications of each procedure

Hemisferectomías y hemi-hemisferectomías: nuestra experiencia acerca de 49 casos / Hemispherectomy and hemi-hemispherectomy: Review of 49 cases

Objective. To review the results of hemispherectomy in the treatment of refractive epilepsy. Methodology. Analytic retrospective cohort study including every patient presenting refractive epilepsy to pharmacologic therapy, operated with hemispherectomy techniques from 1988 to 2008 (n=49). Of 507 patients, operated for refractive epilepsy in the last 20 years, in 49 cases we used any kind of hemispherectomy techniques (9,7%). The male/female relationship was 1.13-1, with 53% males and 47% females. The mean age was 8±5 years old, minimum 4 months and maximum 19 years old. The epilepsy time evolution was 3±2 years. The age of initial presentation was 3±1 years old. The more frequent pathologies were: Rasmussen encephalopathy (30,6%) and secuelar lesions (34,7%). Results. The results were evaluated with the Engel score. Using this classification, our patients were distributed in this manner: 40 patients (81,6%) were in class I of Engel; 4 patients (8,2%) in class II of Engel and 5 patients (10,2%) en Engel III and IV. 88% (43 cases) without complications, 4% (2 cases) present hematomas and the same occur with hydrocephalus and postoperative meningitis. One patient died a few days posterior to surgery because of hematologic complications. Conclusion. The hemispherectomy for the management of refractive epilepsy is a safe procedure with high positive results and small morbimortality in selective pathologies.

Hemispherectomy

Hemispherectomy became a more widely accepted surgical treatment for intractable epilepsy, secondary to severe unilateral hemisphere damage. Basic concept of the hemispherectomy changed from hemispheric resection in anatomical hemispherectomy to a less tissue removal with disconnection of the rest of the hemisphere in the functional hemispherectomy, up to minimal tissue resection with maximal disconnection in the hemispherotomy. This change away from extended resections to predominant disconnection methods lead to reduction of the complications and at the same time maintained the favourable seizure freedom rate. In our current review, we review the hemispherectomy history and techniques in literature with details of our modified surgical technique of hemispherotomy. We also outline the indications, appropriate patient selection, and present our results in a large series of 74 patients who underwent hemispherotomy in our clinic between 1995 and 2006. In our clinical series, the 2-year follow-up shows that 72% of our patients [54 out of 74] had class I and II outcome according to Engel's classification system. No mortality occurred in the current series and postoperative complication was significantly lowered. Hemispherotomy represents an efficacious, technically simple and safe surgical treatment for the management of patients with medically intractable seizures

Role of surgery in pediatric epilepsy.

Twenty five percent of patients with intractable epilepsy have surgically remediable epilepsy syndromes. This article reviews the treatment paradigm for pediatric epilepsy and also the indications, methods, and surgical options for the subgroup of patients with surgically remediable epileptic disorders based on our experience in the management of these children. The article also discusses the rationale for offering surgery and the timing of surgery in these patients. The study of surgically remediable epilepsy can best be divided into focal, sub hemispheric, hemispheric and multifocal epileptic syndromes. These syndromes have both acquired and congenital etiologies and can be treated by resective or disconnective surgery. The surgical management of these conditions (with the exception of multifocal epilepsy) provides Engel's Class 1 outcome(complete seizure freedom) in approximately 80% of children. The consequences of seizure freedom leads to a marked improvement in the quality of life of these children.The benefits to society, of allowing a child to grow to adulthood with normal cognition to earn a livelihood and contribute actively to society, cannot be understated.

Multimodal Management of Lennox-Gastaut Syndrome / 대한소아신경학회지

PURPOSE: Lennox-Gastaut syndrome(LGS) is a childhood epileptic encephalopathy characterized by an electroclinical triad of generalized slow spike and wave activities in the EEG, multiple types of epileptic seizures, and slow mental development. We have attempted to evaluate the efficacy of diverse treatment modalities including epilepsy surgery, ketogenic diet and vagus nerve stimulation as well as new antiepileptic drugs(AEDs) in patients with LGS. METHODS: We retrospectively reviewed the medical records of 79 patients who were managed as Lennox-Gastaut syndrome at Asan Medical Center from April 1993 to March 2006. Their clinical characteristics, EEG, brain MRI findings, and applied treatment modalities were analyzed. Pre- and post-treatment seizure counts were obtained from monthly diary annotations by their families or caregivers. RESULTS: Among these 79 patients, 51 were males. The mean age of their first seizure was 1.9 years and the mean age of their first clinic visit was 4.6 years. Seizure freedom longer than one year was noted in 23 of the 79 patients(29.1%; AED in only 10 of 78 patients, three of 22 patients on a ketogenic diet, seven of 28 patients callosotomy, one patient hemispherectomy, and 2 patients cortical lesionectomy). Measurable improvement (>50%) was noted in 67 of the 79 patients(84.8%) by multimodal treatments. CONCLUSION: Lennox-Gastaut syndrome is recognized as intractable epilepsy. However, patient-designed multi-modality treatment can usually be very helpful for most of these patients.

Seizure Control in Patients with Extratemporal Lobe Epilepsy

OBJECTIVE: This study was designed to analyze seizure outcome and to investigate the prognostic factors for predicting seizure outcome according to the preoperative evaluations, surgical procedures, topectomy sites and histopathological findings in patients with extratemporal lobe epilepsy (ETLE). METHODS: This study comprised 63 patients with ETLE who underwent surgery. Preoperative evaluations included semiologic analysis, chronic video-EEG monitoring, and neuroimaging studies. Surgical procedures consisted of topectomy in 51 patients, corpus callosotomy in 9, functional hemispherectomy in 2, and vagus nerve stimulation (VNS) in 1. Histopathological findings were reviewed. Postoperative seizure outcomes were assessed by Engel's classification at the average follow up period of 66.8 months. Chi-square test was used for statistics. RESULTS: Total postoperative seizure outcomes were class I in 51 (80%) patients, class II in 6 (10%), class III in 6 (10%). Patients with structural abnormalities on neuroimaging study showed class I in 49 (88%) patients (p0.05). CONCLUSION: A good seizure outcome was obtained in patients with ETLE. The factors for favorable seizure outcome are related to the presence of structural abnormalities on neuroimaging study, and focal and regional ictal EEG onset.

Clinical characteristics and treatment of Rasmussen syndrome in 16 children / 中华儿科杂志

<p><b>OBJECTIVE</b>Rasmussen syndrome (RS) is a chronic inflammatory disease of unknown origin, usually affecting one brain hemisphere. The present study aimed to analyze the electroclinical characteristics and treatment of RS.</p><p><b>METHODS</b>The medical records of 16 children with RS were retrospectively reviewed.</p><p><b>RESULTS</b>Of the 16 children, 8 were males and 8 were females. The age of onset was from 1 year and 11 months to 11 years and 6 months. The first symptom was seizure in all patients. The main seizure type was partial motor seizures. In all the patients, seizures gradually became frequent and in the form of epilepsia partialis continua (EPC). Thirteen cases developed hemiparesis. Fixed hemiparesis occurred from 2 months to 3 years after the onset of seizures. The cognitive deterioration was present in 14. The EEG background activity was abnormal in all the cases, asymmetric slow wave disturbances were bilateral but with unilateral predominance in 11, unilateral delta or theta wave in 8. The presence of interictal epileptiform discharges were found in all cases, unilateral in 11 and bilateral in 5. Seizures were recorded in all patients, no electroclinical correlation was found in 5. Serial magnetic resonance imaging (MRI) showed progressive unihemispheric or focal cortical atrophy in all cases. Six cases transiently showed focal cortical swelling or T2/FLAIR hyperintense signal on early scans. Antiepileptic drugs were not effective in any of the patients. Three of 10 patients receiving immunoglobulin, and 4 of 8 receiving corticosteroids, had some reduction of seizure frequency for a short period. Six patients accepted functional hemispherectomy, in 4 of them seizure no longer occurred and cognitive function was improved. The results of multiple subpial transection in 2 cases and focal resection in one patient were disappointing.</p><p><b>CONCLUSION</b>The clinical features of RS were refractory partial epilepsy, progressive hemiplegia and cognitive deterioration. The EEG background was asymmetric with slow wave activity, interictal epileptiform discharges were unilateral or bilateral, no electroclinical correlation occurred. Serial MRI showed progressive unihemispheric focal cortical atrophy. Antiepileptic drugs were not effective for RS. In some patients, immunoglobulin or corticosteroids could reduce seizure frequency in the short term. Functional hemispherectomy could lead to seizure control and prevent further development of neurological impairment and cognitive deterioration.</p>

Early Onset Hemiparesis with Epilepsy: Pattern of Surgical Outcome according to Ictal Onset Zone / 대한간질학회지

PURPOSE: To reveal the pattern of surgical outcome according to the location of ictal onset zone in the patients who had a history of early onset (less than two years old) hemiparesis and delayed epilepsy. Many children with acute infantile hemiplegia (AIH) develop delayed epilepsy. This can lead to HHE (Hemiplegia, hemiconvulsion, and epilepsy) syndrome. Epilepsy patients associated with AIH or HHE syndrome generally have been thought to be medically intractable and difficult to treat surgically. METHODS: Patients with a history of early onset hemiparesis with epilepsy who had undergone surgical treatment from November 1995 to May 2002 at Seoul National University Comprehensive Epilepsy Center were recruited. Diagnostic criteria include convulsions, followed by a flaccid hemiplegia, and later epilepsy with partial seizures. Multidisciplinary presurgical evaluations were performed which include a complete neurological examination, brain MRI, long-term video-EEG monitoring, FDG-PET, intracarotid amobarbital test, and ictal and interictal SPECT if possible. Patients with epileptogenic zone outside the hippocampus underwent implantation of intracranial electrodes. The surgical side was localized by the clinical, neuroimaging, and electrophysiological results includeing results of invasive studies. Anterior temporal lobectomy (ATL), cortical resection, functional hemispherectomy, and callosotomy were performed according to the results of presurgical evaluation. RESULTS: Twenty-five patients were included. Mean age was 29.8 ranging from 19 to 60 years old. Fifteen were male and 10 were female. All had a previous history of febrile convulsions. The onset age of febrile convulsion and hemiplegia was one to 48 months (mean=18.0+/-13.2) and the onset age of epileptic seizures were 0.5 to 40 years (mean=9.9+/-8.2). Seventeen of them were right-handed, eight were left-handed and one was bilateral-handed. Follow-up periods after surgery were one to eleven years (mean=5.6+/-2.2). Twelve patients were diagnosed as medial TLE and underwent ATL. The other thirteen patients were diagnosed as neocortical or multifocal epilepsy. Eleven of medial TLE patients were seizure free after ATL and only one had aura. However, only four patients with neocortical epilepsy were seizure free and nine were not. Surgical outcome was significantly different between ATL only and other surgical procedures (p=0.004). CONCLUSION: In patients of early onset hemiparesis with epilepsy, various ictal onset zones can be possible. The medial TLE patients diagnosed by comprehensive presurgical evaluation, in spite of hemiatrophy on brain MRI, showed a good surgical outcome. Surgical treatment should be considered for the selected patients.

Functional hemispherectomy for the treatment of intractable seizures

Hemispherectomy is the most successful operation for the control of seizures in a very select group of patients with infantile hemiplegia and intractable epilepsy. The efficacy of anatomical hemispherectomy has been offset to some degree by the fact that up to one third of those operated upon will subsequently develop obstructive hydrocephalus, and the so-called superficial subpial cerebral hemosiderosis, with potentially fatal complications. This review article details the operative technique of a modified "functional" hemispherectomy. In essence, the technique consists of removing the central [Rolandic] cortex and the temporal lobe of the affected hemisphere and leaving the remainder of the hemisphere intact, but neurogenically disconnected from the remaining brain

rare, late complication in a hemispherectomised patient: a brucella abscess in the contralateral hemicranium

Pathology in the contralateral hemicranium following hemispherectomy has been reported only once; a meningioma was found at autopsy, compressing the remaining hemisphere of a patient who had been submitted to a hemispherectomy for a malignant glioma [14] Intracranial brucella abscess have been reported only 10 times in the literature. We are reporting a case of cerebral brucella abscess in the remaining hemisphere following a hemispherectomy for infantile uncontrollable seizures and behaviour problems. The literature is reviewed and a new approach to the medial aspect of the remaining hemisphere is described