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.
Child ; Chungcheongnam-do ; Cohort Studies ; Electroencephalography ; Encephalitis ; Encephalomalacia ; Epilepsy* ; Follow-Up Studies ; Hemispherectomy ; Humans ; Infant ; Infant, Newborn ; Infarction ; Paresis ; Patient Selection ; Quality of Life ; Retrospective Studies ; Seizures ; Spasms, Infantile ; Sturge-Weber Syndrome ; Walking

OBJECTIVETo investigate the clinicopathologic features of the brain tissue diagnosed as ulegyria from modified anatomic hemispherectomy for refractory epilepsy.

METHODSThe 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.

RESULTSAll 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.

CONCLUSIONSFebrile 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.

Adolescent ; Adult ; CD3 Complex ; metabolism ; Cerebral Cortex ; abnormalities ; pathology ; surgery ; Child ; Child, Preschool ; Epilepsy ; metabolism ; pathology ; surgery ; Female ; Follow-Up Studies ; Hemispherectomy ; methods ; Humans ; Infant ; Magnetic Resonance Imaging ; Male ; Malformations of Cortical Development ; metabolism ; pathology ; surgery ; Retrospective Studies ; Young Adult

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.
Animals ; Brain/parasitology ; Hemispherectomy/*history ; History, 20th Century ; Humans ; Paragonimiasis/*history/surgery ; Parasitic Diseases/history/therapy ; Trematoda

OBJECTIVETo locate motor functional area of patients who undergone modified anatomical hemispherectomy in order to analysis the plasticity of upper limbs motor.

METHODSThe patients who undergone modified anatomical hemispherectomy were performed BOLD sequences, to locate functional cortical areas in their residual brain.

RESULTS6 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.

CONCLUSIONSThe 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.

Cerebral Cortex ; physiopathology ; Epilepsy ; physiopathology ; surgery ; Female ; Hemispherectomy ; Humans ; Magnetic Resonance Imaging ; methods ; Male ; Neuronal Plasticity ; physiology ; Postoperative Period ; Upper Extremity ; innervation ; physiopathology

OBJECTIVETo 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.

METHODSFive 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.

RESULTSOnly 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.

CONCLUSIONSThere 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.

Adult ; Evoked Potentials, Motor ; physiology ; Female ; Follow-Up Studies ; Hemispherectomy ; Humans ; Male ; Motor Cortex ; physiopathology ; Postoperative Period ; Transcranial Magnetic Stimulation ; Upper Extremity ; physiopathology

BACKGROUNDIn 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.

METHODSOddball 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.

RESULTSFive 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.

CONCLUSIONSFurther 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.

Adult ; Disease-Free Survival ; Event-Related Potentials, P300 ; Female ; Follow-Up Studies ; Hemispherectomy ; Humans ; Male ; Seizures ; surgery ; Treatment Outcome

OBJECTIVERasmussen 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.

METHODSThe medical records of 16 children with RS were retrospectively reviewed.

RESULTSOf 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.

CONCLUSIONThe 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.

Anticonvulsants ; therapeutic use ; Child ; Child, Preschool ; Cognition ; drug effects ; Electroencephalography ; Encephalitis ; drug therapy ; physiopathology ; prevention & control ; Epilepsia Partialis Continua ; drug therapy ; prevention & control ; Epilepsy ; etiology ; prevention & control ; Female ; Hemispherectomy ; methods ; Humans ; Infant ; Magnetic Resonance Imaging ; Male ; Treatment Outcome

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 (p<0.05). Patients with focal and regional ictal EEG onset revealed class I in 47 (90%) patients (p<0.05). Semiologic findings, surgical procedures, topectomy sites and histopathological findings did not show statistical correlation with seizure outcome (p>0.05). 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.
Classification ; Electroencephalography ; Epilepsy* ; Follow-Up Studies ; Hemispherectomy ; Humans ; Neuroimaging ; Psychosurgery ; Seizures* ; Vagus Nerve Stimulation

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.
Ambulatory Care ; Brain ; Caregivers ; Child ; Chungcheongnam-do ; Combined Modality Therapy ; Electroencephalography ; Epilepsy ; Freedom ; Hemispherectomy ; Humans ; Ketogenic Diet ; Magnetic Resonance Imaging ; Male ; Medical Records ; Retrospective Studies ; Seizures ; Vagus Nerve Stimulation

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.
Age of Onset ; Amobarbital ; Anterior Temporal Lobectomy ; Brain ; Child ; Electrodes ; Epilepsy* ; Female ; Follow-Up Studies ; Hemiplegia ; Hemispherectomy ; Hippocampus ; Humans ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Neuroimaging ; Neurologic Examination ; Paresis* ; Seizures ; Seizures, Febrile ; Seoul ; Tomography, Emission-Computed, Single-Photon