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.

An anatomical study on the mandibular medial surface by CBCT analysis for safer implant placement

INTRODUCTION: This study examined the anatomical morphology of the medial surface of the posterior mandible using 3-dimensional cone-beam computed tomography (CT) images to reduce the number of complications related to dental implant placement. MATERIALS AND METHODS: Fifty patients were enrolled in this study with an average age (+/-standard deviation) of 44.28 (+/-13.05). On the coronal views cone-beam CT of the first molars, the distance between the top of the canal and alveolar crest vertical distance (VD), the distance between the upper-most point of the canal and the point perpendicular to the lingual cortical margin of the mandible lingual distance (LD), the location of the starting point of VD for reducing from the vertical reference line (VD point), and the inclination of the mandibular medial surface (lingual inclination) were measured, and a statistical evaluation was performed using SPSS for Windows version 15.0. RESULTS: The mean VD0 was 16.91+/-2.47 mm and VDx decreased with increasing x value. The mean LD was 5.27+/-1.36 mm. The VD began to decrease at the mean location of 6.12+/-0.96 mm from the vertical reference line. The mean lingual inclination was 1.52+/-0.72degrees. CONCLUSION: These results will assist in the accurate placement of dental implants and the reduction of complications, particularly in the case of preoperative implant planning using only 2-dimensional imaging methods. (ex. panoramic radiography)

Surgical treatment of chronic epilepsy with intracranial neoplasm and vascular lesion

The surgical treatment of epilepsy patients can be divided into various resection methods such as simple excision, lesionectomy, and lesionectomy plus cortisectomy. Recently, radiosurgery is included in the armamentarium. Several authors suggest that the radiosurgery is an alternative treatment to open respective surgery for intractable epilepsy, especially for mesial temporal lobe epilepsy. The optimal indication and effi cacy of radiosurgery for epilepsy patients are still controversial. In our study, 153 patients with seizure/epilepsy and MRI visible lesion from 1993 to 2006, were included (157 operations). We reviewed the cases of the patients with epileptogenic lesions of neoplasm and vasculopathy who had been treated surgically to determine surgical outcome in relation with types of surgical treatment, pathology, and location of lesion.

THE SUPERFICIAL AND DEEP SPREADING PATHWAYS OF INFECTION OF THE MAXILLOFACIAL AND NECK AREA: REPORTS OF 2 CASES

High-Level Cervical Spinal Cord Stimulation Used to Treat Intractable Pain Arising from Transverse Myelitis Caused by Schistosomiasis

The efficacy of spinal cord stimulation (SCS) for treatment of various chronic painful conditions is well established. Very few reports have documented the use of SCS for treatment of chronic pain after spinal cord injury. We present a case showing a good outcome after such treatment, and suggest that high cervical stimulation may be efficacious. A 53-year-old male underwent SCS on the C1-3 level for treatment of intractable neuropathic pain below the T3 level, and in the upper extremities, arising from spinal cord injury resulting from transverse myelitis caused by schistosomiasis. High cervical SCS significantly improved the pain in the upper extremities and at the T3-T10 dermatome level. The patient continues to report excellent pain relief 9 months later. The present case suggests that high cervical stimulation may improve chronic pain in the upper extremities and the T3-T10 dermatome level arising from spinal cord injury.

MR Spectroscopy and Perfusion MR Imaging Findings of Intracranial Foreign Body Granuloma: a Case Report

We report a case of intracranial foreign body granuloma that showed features of a high grade tumor on magnetic resonance (MR) imaging. However, the relative cerebral blood volume was not increased in the enhancing mass on perfusion MRI and the choline/creatine ratio only slightly increased on MR spectroscopy. The results suggest that the lesion is benign in nature. Perfusion MRI and MR spectroscopy may be helpful to differentiate a foreign body granuloma from a neoplastic condition.

Spinal Cord Stimulation for Intractable Visceral Pain due to Chronic Pancreatitis

Pain caused by chronic pancreatitis is medically intractable and resistant to conventional interventional or surgical treatment. We report a case of spinal cord stimulation (SCS) for intractable pain due to chronic pancreatitis. The patient had a history of nonalcoholic chronic pancreatitis and multiple emergency room visits as well as repeated hospitalization including multiple nerve block and morphine injection for 3 years. We implanted surgical lead at T6-8 level on this patient after successful trial of percutaneous electrode. The patient experienced a decreased visual analog scale (VAS) scores for pain intensity and amount of opioid intake. The patient was followed for more than 14 months with good outcome and no further hospitalization. From our clinical case, spinal cord stimulation on intractable pain due to chronic pancreatitis revealed moderate pain control outcome. We suggest that SCS is an effective, noninvasive treatment option for abdominal visceral pain. Further studies and long term follow-up are needed to fully understand the effect of SCS on abdominal visceral pain.

Multimodal Management of Lennox-Gastaut Syndrome

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.

Intracranial Epithelioid Hemangioendothelioma

Intracranial epithelioid hemangioendothelioma is extremely rare. We report a case of intracranial epithelioid hemangioendothelioma which developed in a 55-year-old man who presented with dysarthria for two weeks. The brain computed tomography scan and magnetic resonance image showed masses which had fat component at the left frontal convexity and at left posterior parietal area. Excisional biopsy at the left frontal convexity confirmed epithelioid hemangioendothelioma which is immunopositive for CD31, supporting endothelial differentiation, and negative for CD68, SMA and HMB-45.

Vagus Nerve Stimulation in Intractable Childhood Epilepsy: a Korean Multicenter Experience

We evaluated the long-term outcome of vagus nerve stimulation (VNS) in 28 children with refractory epilepsy. Of these 28 children, 15 (53.6%) showed a >50% reduction in seizure frequency and 9 (32.1%) had a >75% reduction. When we compared seizure reduction rates according to seizure types (generalized vs. partial) and etiologies (symptomatic vs. cryptogenic), we found no significant differences. In addition, there was no correlation between the length of the stimulation period and treatment effect. The seizure reduction rate, however, tended to be inversely related to the seizure duration before VNS implantation and age at the time of VNS therapy. VNS also improved quality of life in this group of patients, including improved memory in 9 (32.1%), improved mood in 12 (42.9%), improved behavior in 11 (39.3%), improved altertness in 12 (42.9%), improved achievement in 6 (21.4%), and improved verbal skills in 8 (28.6%). Adverse events included hoarseness in 7 patients, dyspnea at sleep in 2 patients, and wound infection in 1 patient, but all were transient and successfully managed by careful follow-up and adjustment of parameters. These results indicate that VNS is a safe and effective alternative therapy for pediatric refractory epilepsy, without significant adverse events.

Intracranial Ictal EEG Patterns Predicting the Surgical Outcome and the Yield of Intracranial Electrodes in Patients with Temporal Lobectomy

BACKGROUND: Analysis of intracranial ictal patterns may help to predict surgical outcomes. We investigated intracranial EEG patterns to correlate with surgical outcomes and compared the yield of 'subdural electrodes alone (SE)' versus 'combined depth and subdural electrodes (CDSE)' for ictal lateralization in temporal lobe epilepsy (TLE). METHODS: We reviewed a total of 95 seizures recorded by bilateral temporal depth and subdural electrodes in 25 TLE patients who underwent surgery. We classified surgical outcomes as 'seizure-free' or 'not-seizure-free'. Each seizure was analyzed based on the presence or absence of peri-ictal discharges, ictal distribution, waveform patterns, onset frequency and involved number of electrodes, and interhemispheric propagation time (IHPT). The accuracy of lateralizing seizure foci by CDSE was compared to that by SE. RESULTS: 20 patients (80.0%) were 'seizure-free' and 5 (20.0%) were 'not-seizure-free'. The presence of peri-ictal discharges (p<0.001), distribution of depth only or depth and medial electrodes (p<0.001) and higher onset frequency (p=0.021) were associated with 'seizure-free' outcomes. Ictal onset pattern with fast spike trains was common in 'seizure-free', whereas pattern with rhythmic activity was common in 'not-seizure-free' (p=0.005). SE correctly lateralized in 18 of 20 patients, and incorrectly lateralized in the remaining 2 patients, but CDSE correctly lateralized in all 20 patients. CONCLUSIONS: Some intracranial ictal patterns were significantly correlated with good surgical outcomes. These findings suggest that the analysis of ictal EEG patterns help to predict surgical outcomes. CDSE is more accurate for the lateralization of seizure foci compared to SE.

Surgical Treatment of Intractable Frontal Lobe Epilepsy / 대한간질학회지

PURPOSE: Frontal lobe epilepsy is difficult to be managed with medical or surgical treatment. So the authors analyzed and reviewed the clinical results of the frontal lobe epilepsy patients who underwent surgical treatment. METHODS: From 1996 to 2004, Thirty-nine patients were treated surgically. The authors reviewed retrospectively the radiological, clinical and pathological data of the patients. The surgical outcome was classified using Engel's classification. RESULTS: The mean follow-up period is 70.5 (+/-29.7) months. The preoperative MR image was normal in 19 cases and abnormal in 20 cases. There were 11 encephalomalacias, 4 cortical dysplasias and 5 brain tumors. The postoperative pathological finding was normal in 5 cases and abnormal finding in 34. There were 20 cortical dysplasias, 4 encephalomalacias, 3 gliosises, 2 fibrous scars, 2 cavernous malformations, 1 dysembryoplastic neuroepithelial tumor, 1 paragonimiasis and 1 venous thrombosis. The postoperative surgical outcomes were as follows: Class I, 41%; Class II, 25.6%; Class III, 20.5%; Class IV, 12.8%. CONCLUSIONS: The surgical outcomes of frontal lobe epilepsy have improved, compared with previous series. The pathological abnormality was observed in large portion of the frontal lobe epilepsy patients.

The Anatomical Location and Course of the Facial Nerve in Vestibular Schwannomas: A Study of 163 Surgically Treated Cases

OBJECTIVE: The aim of this study was to identify the anatomical location and course of the facial nerve (FN) and their relationship to the tumor size in surgically treated vestibular schwannomas. METHODS: A retrospective study was conducted on 163 patients who had been treated by the microsurgical resection for a newly diagnosed vestibular schwannoma between 1995 and 2005 (mean age of 46.1 years; 108 females and 55 males). Surgery was carried out via retrosigmoid approach in all patients with the electromyographic monitoring for the FN function. The anatomical location and course of the FN along the tumor surface were verified in each patient during the microsurgery, and were classified into 4 groups : 1) the FN displaced along the ventral and superior surface of the tumor (VS); 2) the ventral and central (VC); 3) the ventral and inferior (VI); and 4) the dorsal (Do). RESULTS: The FN displacement was identified as the followings : VS in 91 patients (55.8%); VC in 57 (35.0%); VI in 14 (8.6%); and Do in 1 (0.6%). In the subgroup with tumors less than 2 cm in diameter (n=23), the FN was displaced along the ventral and central surface of the tumor in the majority (65.2%), whereas, in the patients with tumors larger than 2cm (n=140), it was displaced along the ventral and superior surface most frequently (59.3%). CONCLUSION: The FN can be displaced variably in vestibular schwannomas, and most frequently along the ventral and superior surface of the tumor, especially in large ones.

A case of intrahepatic cholangiocarcinoma with orbital metastasis / 대한내과학회지

Orbital lesions may be the presenting sign of a systemic disease, such as a metastatic cancer. A metastatic cancer of the orbit, which has been reported to account for 2~3% of all orbital tumor, is a malignant neoplasm that had spread by hematogenous routes to the orbit from a distant primary site. Although metastatic orbital tumors are mostly from breast, lung, prostate, rarely, they can be originated from gastrointestinal tract such as large intestine, ileum and pancreas. However, there has been no report of metastatic orbital tumor from biliary system, until now. Herein, we report a unique case of metastatic orbital tumor from the intrahepatic cholangiocarcinoma, which has been successfully controlled with systemic chemotherapy and radiotherapy.

The Outcome of Corpus Callosotomy for Intractable Epilepsy: 10 Years Experience of Corpus Callosotomy

OBJECTIVE: The purpose of this study is to evaluate the effect of the corpus callosotomy and to elucidate possible prognostic factors. METHODS: The cases of 39 patients who underwent corpus callosotomy were reviewed retrospectively. Clinical outcomes were analyzed using Engel's classification, with consideration of various presurgical conditions and the extent of the callosal resection during follow-up more than one year. RESULTS: Satisfactory outcome (Engel's class I, II) was obtained in 20 patients (51%) of 39 patients. In 36 cases with drop attack seizures, the class I, II outcomes were 22 patients (61%). When the patients were grouped according to the extent of callosal resection, the class I, II outcomes were 50% of the patients with anterior 1/2 or 2/3, 50% of those with anterior 4/5 callosotomy, and 57% of those with total callosotomy, respectively. The mean follow-up period was 34 months (24 to 58 months). CONCLUSION: Although it is not statistically significant, the patients who had underwent total callosotomy show better outcomes than those with partial callosotomy. Corpus callosotomy is efficacious in controlling medically intractable epilepsy in appropriately selected patients.

Gamma Knife Radiosurgery in Patients with a Hypothalamic Hamartoma Associated with Intractable Gelastic Epilepsy: Report of Three Cases

Hypothalamic hamartoma(HH) is an unusual nonneoplastic developmental lesion associated with gelastic epilepsy and precocious puberty, mostly found in children. Although open surgery has been attempted when antiepileptic medication failed to control seizures, its deep location and surrounding vital structures often rendered surgery unsuccessful. We describe the outcome of gamma knife radiosurgery in three children with a HH associated with gelastic epilepsy and reviewed the literature for a possible therapeutic mechanism.

Posterior Cerebral Artery Aneurysm: Surgical Result of 11 Patients

OBJECTIVE: Eleven patients treated with posterior cerebral artery(PCA) aneurysm during 6.3-years period are retrospectively reviewed to determine treatment outcome. METHODS: Eleven patients with PCA aneurysm were treated from January 1998 to May 2004. Their medical records and radiologic studies were reviewed retrospectively. The records of these patients were analysed with particular reference to their demographic details, mode of presentation, and treatment outcome. RESULTS: Of the 11 patients, 8 patients presented with symptoms related aneurysmal bleeding. Three patients had unruptured PCA aneurysms. Open or endovascular surgery was performed in 9 patients; None of these patients exhibited a third nerve palsy, visual field deficit, or hemiparesis at the time of presentation. Postoperatively, 2 made a good recovery, 2 had a moderate disability because of cerebral infarction after surgery, and 5 had a severe disability because of cerebral infarction after surgery. Of 2 conservatively treated patients, 1 was doing well but the other died as a result of brain swelling. CONCLUSION: The treatment of the PCA aneurysms is difficult regardless of the aneurysmal size, site, and treatment modality. All reasonable treatment to reduce the risk of associated morbidity should be considered.

Outcome of Atypical Meningioma

OBJECTIVE: Limited data are available concerning the outcome of the patients with atypical meningioma due to lack of the studies with large series. The authors review atypical meningioma retrospectively and analyzed various parameters concerning its outcome. METHODS: Of the 866 meningioma patients treated between 1990 and 2003, pathologically proven 54 atypical meningiomas were reviewed. Various factors of the patients were analyzed, and surgical specimens were re-examined blindly by neuropathologist without any patient information. Extent of surgical resection was determined according to Simpson's classification by reviewing the chart and postoperative scan if possible. RESULTS: Twenty-three (42.6%) had local recurrences during the follow-up, of which 13 (32.5%) of 40 complete excisions and 10 (71.4%) of 14 incomplete excisions. The median time to recurrence was 47 months, and the overall 3-, 5-, and 10-year local control rates were 62.4%, 41.5%, and 31.1%, respectively. Five (9.3%) died during follow-up period. The mean survival time was 123 months, and the overall 3-, 5-, and 10-year survival rates were 94.2%, 87.2%, and 78.5%, respectively. The extent of surgical excision was the most significant prognostic factor not for survival but for local control (p=0.2179 and 0.0005, respectively). Extracranial metastasis was not seen in our cases. CONCLUSION: Complete surgical excision is the most important factor in improving local control. Careful long-term follow-up is mandatory because atypical meningioma shows a broad range of aggressiveness and natural history.

Prognostic Factors of Moyamoya Disease Evaluated by Activity of Daily Living / 대한뇌혈관외과학회지

BACKGROUNDS: Moyamoya disease is a progressive occlusive cerebrovascular disease which has characteristics of distal ICA stenosis and basal collateral vessels. Various methods of surgical treatment are recommended in the literatures but surgical strategies and outcome are not well established yet. AIMS AND METHODS: The aims of study is to evaluate surgical outcomes of moyamoya disease and to establish surgical indications. Total 155 patients diagnosed with moyamoya disease since 1990, were analyzed retrospectively. Female was more predominant by 1.5 : 1. There were two peaks of age of onset at the 1st decade (39.0%) and 4th (15.9%). Familial occurrence was 6.8% (n=17). The mean duration of follow-up was 41.1 months (12-156 months). Moyamoya syndrome was excluded in this study. Surgical outcomes were measured by grading activity of daily living (ADL) and prognostic factors were analyzed statistically with SAS. RESULT: The most common clinical presentations were transient ischemic attacks (69.1%), followed by cerebral infarction (26.0%) and hemorrhage (4.9%) in pediatric patients, but in adult hemorrhage (49.2%) was the most prevalent. Recurrence of symptoms developed in 8 patients (11.4%) among 70 of cerebral infarction with mean intervals of 30.8 months and 7 patients (21.9%) among 32 of hemorrhage with mean interval of 42.3 months. Forty five (29.0%) of 155 patients showed stenosis of posterior cerebral arteries on cerebral angiography at the age of diagnosis. The surgical treatment were performed at 183 hemispheres of 115 patients, direct bypass surgery was done in 10 hemispheres, indirect bypass surgery in 169 hemispheres (106 EDAMS, 14 EDAS, 40 frontal EDS or burr hole, 6 EDAMS and EDAS, 3 others), combined direct and indirect bypass surgery in 4 hemispheres. The improvement of ADL was not so different between 68 (59.1%) of 115 patients treated with surgery and 23 (57.5%) in 40 patients with conservative care. The initial and final grade of ADL of pediatric patients were better than those of adult (p0.1). Significant prognostic factors affecting outcomes of moyamoya disease age at onset, clinical features, and initial grade of ADL. CONCLUSION: Children with transient ischemic attack were the best, but adults with recurrent hemorrhage were the worst in outcomes. Surgical treatment for moyamoya disease should be carefully tailored according to age of onset and clinical features regardless of surgical methods. Further prospective study is indicated to determine optimal treatment guideline for moyamoya disease.

Vagus Nerve Stimulation In Intractable Pediatric Epilepsy Patients

PURPOSE: We intended to evaluate the efficacy, safety, and effects on quality of life of vagus nerve stimulation(VNS) in children with intractable epilepsy. METHODS: We retrospectively studied the medical records of 7 pediatric patients who underwent VNS implantation between December 2001 and April 2003. RESULTS: The ages at operation ranged from 6 years and 5 months to 11 years and 9 months. The duration of follow-ups ranged from 12 months to 44 months. All the patients had intractable seizures, in four of whom the epilepsy was symptomatic. Of the 4 patients, three had history of meningoencephalitis and one had pachygyria. 5 patients(71 %) had 50% or more reduction in seizure frequency and the onset of seizure reduction occurred within 3 months after VNS implantation. Seizure reduction of more than 50% was observed in head drop and atypical absence seizure(1/1, 100%, one with callosotomy), generalized tonic clonic seizure(3/4, 75%) and complex partial seizure(1/2, 50%). In 4 patients there were improvements in quality of life such as alertness, mood, verbal communication and motor function. Side effects were transient hoarseness in four patients and wound infection in one patient, but these were not too severe to lerate. CONCLUSION: VNS is an effective and safe adjunctive therapy in intractable pediatric epilepsy as wall as in improving quality of life.