• Title/Summary/Keyword: 난치성 간질

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Brain F-18 FDG PET for localization of epileptogenic zones in frontal lobe epilepsy: visual assessment and statistical parametric mapping analysis (전두엽 간질에서 F-18-FDG PET의 간질병소 국소화 성능: 육안 판독과 SPM에 의한 분석)

  • Kim, Yu-Kyeong;Lee, Dong-Soo;Lee, Sang-Kun;Chung, Chun-Kee;Yeo, Jeong-Seok;Chung, June-Key;Lee, Myung-Chul
    • The Korean Journal of Nuclear Medicine
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    • v.35 no.3
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    • pp.131-141
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    • 2001
  • Purpose: We evaluated the sensitivity of the F-18 FDG PET by visual assessment and statistical parametric mapping (SPM) analysis for the localization of the epileptogenic zones in frontal lobe epilepsy. Materials and Methods: Twenty-four patients with frontal lobe epilepsy were examined. All patients exhibited improvements after surgical resection (Engel class I or II). Upon pathological examination, 18 patients revealed cortical dysplasia, 4 patients revealed tumor, and 2 patients revealed cortical scar. The hypometabolic lesions were found in F-18 FDG PET by visual assessment and SPM analysis. On SPM analysis, cutoff threshold was changed. Results: MRI showed structural lesions in 12 patients and normal results in the remaining 12. F-18 FDG PET correctly localized epileptogenic zones in 13 patients (54%) by visual assessment. Sensitivity of F-18 FDG PET in MR-negative patients (50%) was similar to that in MR-positive patients (67%). On SPM analysis, sensitivity decreased according to the decrease of p value. Using uncorrected p value of 0.05 as threshold, sensitivity of SPM analysis was 53%, which was not statistically different from that of visual assessment. Conclusion: F-18 FDG PET was sensitive in finding epileptogenic zones by revealing hypometabolic areas even in MR-negative patients with frontal lobe epilepsy as well as in MR-positive patients. SPM analysis showed comparable sensitivity to visual assessment and could be used as an aid in the diagnosis of epileptogenic zones in frontal lobe epilepsy.

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Two Cases of Renal Stone Associated with Ketogenic Diet (케톤 식이요법 후에 발생한 신장 결석 2례)

  • Chung, Ju-Young;Koo, Ja-Wook;Kang, Hoon-Churl;Kim, Sang-Woo;Kim, Heung-Dong
    • Clinical and Experimental Pediatrics
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    • v.48 no.2
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    • pp.204-207
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    • 2005
  • Ketogenic diet is a high-fat, low-carbohydrate, low-protein diet used in the treatment of epilepsy since 1920's. Recently, it's use for intractable epilepsy in childhood has increased. Complications of ketogenic diet are known to include dehydration, vomiting, diarrhea, renal stones, metabolic derangement, hypercholesterolemia and refusal to eat. We experienced two cases of renal stones in children with intractable epilepsy during ketogenic diet.

DENTAL TREATMENT IN A PATIENT WITH LENNOX-GASTAUT SYNDROME UNDER GENERAL ANESTHESIA (레녹스-가스토 증후군 (Lennox-Gastaut syndrome) 환자의 전신마취 하 치과치료)

  • Baik, Minkyung;Shin, Teo-Jeon;Hyun, Hong-Keun;Kim, Young-Jae;Kim, Jung-Wook;Jang, Ki-Taeg;Lee, Sang-Hoon;Kim, Chong-Chul
    • The Journal of Korea Assosiation for Disability and Oral Health
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    • v.9 no.1
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    • pp.51-55
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    • 2013
  • Lennox-Gastaut syndrome (LGS) is a severe form of childhood epilepsy that is defined by generalized multiple type seizures, slowness of intellectual growth, and a specific EEG disturbance. Children affected might previously have infantile spasms or underlying brain disorder but etiology can be idiopathic. LGS seizures are often treatment resistant and the long term prognosis is poor. A 14-year-3-month old, 20.5 kg girl with LGS was scheduled for dental treatment under general anesthesia. The patient presented with multiple caries and heavy calculus. General anesthesia was induced and maintained with inhalation agent, Sevoflurane. And caries treatment, scaling, subgingival curretage were performed. LGS patients usually have poor oral hygiene due to mental retardation and have high risk of seizure during dental treatment. In dental treatment under general anesthesia for LGS, it must be considered of seizure control during peri-operative period. Also periodic recall check, instruction of oral hygiene must be performed.

Vagus Nerve Stimulation Treatment for Children with Refractory Epilepsy (Lennox-Gastaut Syndrome) (난치성 간질 환자에서 미주신경자극술 치료결과 분석(레녹스-가스토 증후군 환자))

  • Kim, Dae-Sik;Kim, Cheon-Sik
    • Korean Journal of Clinical Laboratory Science
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    • v.39 no.1
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    • pp.56-62
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    • 2007
  • Lennox-Gastaut syndrome(LGS) is a severe age-specific epilepsy syndrome that causes medication-resistant seizures in childhood. Vagal nerve stimulation (VNS) has been proposed as a possible way to improve the treatment of refractory epilepsy. We treated 9 patients with Lennox-Gastaut syndrome between the ages of 2 and 11 years (mean 5.8 years), by using the vagus nerve stimulation. The mean follow-up duration was 35 months. The mean reduction of seizure frequency compared with baseline before VNS was 52% after 6 months (range, 0% to 89%; P<0.011), and 58% after 1 year (range 0% to 89%; P<0.012). Seven patients showed improvements of quality of life (QOL) such as alertness, mood, and language skills. The most common side effects were transient hoarseness (6 patients) and drooling (1 patient). Our results suggest that the vagus nerve stimulation could be an effective and safe adjunct therapy for the treatment of Lennox-Gastaut syndrome.

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Surgical Results of Functional Hemispherectomy and Peri-insular Hemispherotomy (난치성 간질에 대한 기능적 대뇌반구 절제술 및 Peri-insular 대뇌반구 절제술의 효과)

  • Lee, Dong Kul;Lee, Wan Su;Lee, Jung Kyo;Kim, Chung Ho;Ko, Tae Seong;Lee, Sang Am
    • Journal of Korean Neurosurgical Society
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    • v.29 no.9
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    • pp.1195-1203
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    • 2000
  • Objective : To confirm the efficacy of functional hemispherectomy and peri-insular hemispherotomy on treatment of intractable epilepsy. Materials & Methods : From April 1997 to February 1999, we performed 1 functional hemispherectomy and 6 peri-insular hemispherotomy in 7 consecutive patients. These procedures result in completely disconnected hemisphere while maintaining the disconnected portion of the hemisphere intact within the surgical cavity. The indications were hemimegalencephaly in 2 cases, infarction with encephalomalacia in 2, Sturge-Weber syndrome in 1, hemiconvulsion hemiplegia epilepsy syndrome in 1, cortical dysplasia with leptomeningeal cyst in 1. Mean follow-up is 15.8 months(range 8-28 months). Results : Among 7 patients, 1 patient died immediately after peri-insular hemispherotomy. Five patients became seizure free with reduced doses of medications. One patient developed rare disabling seizure with medication. In 6 patients, there were improvements in the function of the hemiparetic limbs in the postoperative phase. A 3-year-old boy with infarction and encephalomalacia died few hours after surgery due to postoperative hypothermia. Two patients required shunt after surgery. Two patients developed postoperative brain swelling but were successfully managed with conservative care. Conclusion : In conclusion, functional hemispherectomy and peri-insular hemispherotomy may provide substantial seizure control in selected cases of young hemiplegic patients with intractable epilepsy.

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Effects of Vagus Nerve Stimulation on Adults and Children with Refractory Epilepsy (소아와 성인의 난치성 간질 환자에서 미주신경 자극술의 효과)

  • Kim, Cheon-Sik;Ro, Yeong-Joo;Choi, Sang-Yong;Kim, Dae-Sik
    • Korean Journal of Clinical Laboratory Science
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    • v.38 no.2
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    • pp.141-146
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    • 2006
  • Vagal nerve stimulation (VNS) has been proposed as a possible way to improve the control of refractory epilepsy. We report the effects following VNS treatment in patients with refractory epilepsy. Seventeen patients with a mean age of 12.8 years, ranging from 5 to 29 years, underwent the implantation of vagal nerve stimulation (Cyberonics, Houston, TX). We reviewed the clinical findings before and after VNS in seizure frequency, number of antiepileptic drugs (AED), and quality of life (QOL). All of the patients had intractable seizures, eleven of the patients had additional medical complications, three had hippocampus atrophy, one had encephalomalacia, five had encephalitis, one had pachygyria, and one had schizencephaly. Thirteen patients had symptomatic partial epilepsies, three patients had Lennox-Gastaut syndrome and one had cryptogenic partial epilepsy. The mean follow up duration was 35 months. The mean reduction of seizure frequency compared with baseline before VNS was 26.1% after 3 months (p<0.005), 41.9% after 6 months (p<0.001), 46.9% after 1 year (p<0.001), and 53% at the latest follow-up (p<0.001). Twelve patients showed an improvement of QOL such as mood, language, alertness, expression, and motor function. The most common side effects were transient hoarseness or voice change or cough, which was detected in six patients (35%) and wound infection in one patient (5%). This study has shown a good anti-seizure effect of VNS, decrease in seizure frequency and improvements in QOL. We concluded that VNS is a beneficial therapy in refractory epilepsy with a non-resectable epileptic focus. Further studies should be focused on the prediction of unresponsiveness and the adjustment of VNS parameters for maximum efficacy in patients with various medical histories.

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Clinical manifestations and neuroimaging findings of schizencephaly in children (소아 뇌갈림증의 신경영상학적 소견 및 임상 양상)

  • Lee, Jae Rang;Kim, Seung;Lee, Young Mock;Lee, Joon Soo;Kim, Heung Dong
    • Clinical and Experimental Pediatrics
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    • v.52 no.4
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    • pp.458-463
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    • 2009
  • Purpose : Schizencephaly is a uncommon congenital brain anomaly characterized by congenital clefts spanning the cerebral hemispheres from pial surface to lateral ventricles and lined by gray matter. In this study, we investigated the clinical manifestation and radiologic findings of pediatric schizencephaly. Methods : The data of 13 patients who were diagnosed with schizencephaly in Severance Childrens Hospital and Yongdong Severance Hospital from January 2005 to December 2007 were analyzed retrospectively. Results : The mean age at diagnosis was $9.08{\pm}2.67$ months old and ranged from 1 to 30 months. The ratio of male to female patients was 3.33:1. Five (38.5%) patients had bilateral clefts, while 8 (61.5%) had unilateral clefts. Five (38.5%) patients had closed lip clefts, and 4 (30.8%) had opened lip clefts. Four (30.8%) patients had multiple clefts. Associated anomalies showed in all cases. The clinical features consisted of mild unilateral weakness in 7 (53.8%) cases and a hemiparesis was present in 3 (23.1%) patients. A tetraparesis was in 3 (23.1%) patients. There was no difference in motor deficit between unilateral and bilateral clefts. Delayed development was observed in all cases. Epilepsy was present in 7 (53.8%) patients, 5 patients with unilateral clefts and 2 patients with bilateral clefts. Three (42.8%) patients showed intractable seizures. Conclusion : Schizencephaly showed variable clinical manifestations and radiologic findings in association with the types and locations of the clefts. It is necessary to diagnose schizencephaly early and to detect the development of epilepsy. Intensive and large studies of the correlation of clinical outcomes and radiologic findings should be continued for more effective treatment.

Video-Assisted Thoracic Surgery Under Epidural Anesthesia -in High-Risk Group (경막외마취하에 비디오 흉강경수술 - 고위험군에서)

  • Lee, Song-Am;Kim, Kwang-Taik;Kim, Il-Hyeon;Park, Sung-Min;Baek, Man-Jong;Sun, Kyung;Kim, Hyoung-Mook;Lee, In-Sung
    • Journal of Chest Surgery
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    • v.32 no.8
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    • pp.732-738
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    • 1999
  • Background: Video-assisted thoracoscopic surgery has become a standard therapy for several diseases such as pneumothorax, hyperhidrosis, mediastinal mass, and so on. These methods usually required single-lung ventilation with double-lumen endobronchial tube to collapse the lung under general anesthesia. However, risks of general anesthesia itself and single-lung ventilation must be considered in high-risk patients. Material and method: Between December 1997 and July 1998, eight high-risk patients (6: empyema, 1: intractable pleural effusion, 1: idiopathic pulmonary fibrosis) with underlying pulmonary disease and poor general condition were treated by video-assisted thoracoscopic surgerys under epidural anesthesia and spontaneous breathing. Result: Video-assisted thoracoscopic surgerys were successfully per formed in 7 patients. Conversion to general anesthesia was required in 1 patient because of decrease in spontaneous breathing. But, conversion to open decortication was not required. In two patients with chronic empyema, one patient required thoracoplasty as a second procedure and one patient required re-video-assisted thoracoscopic procedure due to a recurrence. The mean operative time was 31.8$\pm$15.2 minutes. No significant postoperative respiratory com plication was encountered. Conclusion: Video-assisted thoracoscopic surgerys can be per formed safely under epidural anesthesia for the treatment of empyema and diagnosis of pulmonary abnormalities in high-risk patients.

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Mesenchymal Stem Cell-derived Exosomes: Applications in Cell-free Therapy (중간엽줄기세포유래 엑소좀: 비세포치료제로서의 활용)

  • Heo, June Seok;Kim, Jinkwan
    • Korean Journal of Clinical Laboratory Science
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    • v.50 no.4
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    • pp.391-398
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    • 2018
  • Mesenchymal stem cells (MSCs) are an attractive resource for refractory patients because of their anti-inflammatory/immunomodulatory capability and multi-lineage differentiation potential. The transplantation of MSCs has led to positive results in preclinical and clinical application to various diseases, including autoimmune disease, cardiovascular disease, cancer, liver cirrhosis, and ischemic stroke. On the other hand, studies have shown that paracrine factors, not direct cell replacement for damaged cells or tissue, are the main contributors in MSC-based therapy. More recently, evidence has indicated that MSC-derived exosomes play crucial roles in regulating the paracrine factors that can mediate tissue regeneration via transferring nucleic acids, proteins, and lipids to the local microenvironment and cell-to-cell communication. The use of these exosomes is likely to be beneficial for the therapeutic application of MSCs because their use can avoid harmful effects, such as tumor formation involved in cell transplantation. Therefore, therapeutic applications using MSC-derived exosomes might be safe and efficient strategies for regenerative medicine and tissue engineering. This review summarizes the recent advances and provides a comprehensive understanding of the role of MSC-derived exosomes as a therapeutic agent.

A case of hippocampal sclerosis diagnosed as cortical dysplasia due to preoperative brain MRI finding (수술 전 뇌 자기공명 영상에서 겉질 형성이상증 소견 보였으나 수술 후 병리학적으로 확인된 해마경화증 1례)

  • Lee, Jun Seok;Kim, Kyo Ryung;Kim, Jeong Tae;Choi, Min Jung;Lee, Young Mock;Kim, Heung Dong;Lee, Joon Soo;Kim, Dong Seok;Kim, Tae Seong
    • Clinical and Experimental Pediatrics
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    • v.53 no.1
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    • pp.106-110
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    • 2010
  • Hippocampal sclerosis (HS) is one of the most common features of intractable temporal lobe epilepsy. Generally it can be identified through brain magnetic resonance imaging (MRI) with high degree of sensitivity and specificity. Typical brain MRI findings of HS are hippocampal atrophy with hyperintense signal confined to the lesion. On the other hand cortical dysplasia exhibits blurring of the gray-white matter junction and abnormal white matter signal intensity. We present a case where preoperative brain MRI strongly suggested the presence of diffuse cortical dysplasia in the left temporal lobe but postoperative pathology revealed the temporal lesion to be unremarkable except for hippocampal sclerosis.