• 제목/요약/키워드: Intractable epilepsy

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간질에서의 핵의학 영상 (Nuclear Imaging in Epilepsy)

  • 천경아
    • Nuclear Medicine and Molecular Imaging
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    • 제41권2호
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    • pp.97-101
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    • 2007
  • Correct localization of epileptogenic zone is important for the successful epilepsy surgery. Both ictal perfusion single photon emission computed tomography (SPECT) and interictal F-18 fluorodeoxyglucose positron emission tomography (FDG-PET) can provide useful information in the presurgical localization of intractable partial epilepsy. These imaging modalities have excellent diagnostic sensitivity in medial temporal lobe epilepsy and provide good presurgical information in neocortical epilepsy. Also provide functional information about cellular functions to better understand the neurobiology of epilepsy and to better define the ictal onset zone, symptomatogenic zone, propagation pathways, functional deficit zone and surround inhibition zones. Multimodality imaging and developments in analysis methods of ictal perfusion SPECT and new PET ligand other than FDG help to better define the localization.

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

  • 이동걸;이완수;이정교;김정호;고태성;이상암
    • Journal of Korean Neurosurgical Society
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    • 제29권9호
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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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Epilepsy Surgery in 2019 : A Time to Change

  • Phi, Ji Hoon;Cho, Byung-Kyu
    • Journal of Korean Neurosurgical Society
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    • 제62권3호
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    • pp.361-365
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    • 2019
  • Epilepsy has been known to humankind since antiquity. The surgical treatment of epilepsy began in the early days of neurosurgery and has developed greatly. Many surgical procedures have stood the test of time. However, clinicians treating epilepsy patients are now witnessing a huge tide of change. In 2017, the classification system for seizure and epilepsy types was revised nearly 36 years after the previous scheme was released. The actual difference between these systems may not be large, but there have been many conceptual changes, and clinicians must bid farewell to old terminology. Paradigms in drug discovery are changing, and novel anti-seizure drugs have been introduced for clinical use. In particular, drugs that target genetic changes harbor greater therapeutic potential than previous screening-based compounds. The concept of focal epilepsy has been challenged, and now epilepsy is regarded as a network disorder. With this novel concept, stereotactic electroencephalography (SEEG) is becoming increasingly popular for the evaluation of dysfunctioning neuronal networks. Minimally invasive ablative therapies using SEEG electrodes and neuromodulatory therapies such as deep brain stimulation and vagus nerve stimulation are widely applied to remedy dysfunctional epilepsy networks. The use of responsive neurostimulation is currently off-label in children with intractable epilepsy.

Brain somatic mutations in MTOR leading to focal cortical dysplasia

  • Lim, Jae Seok;Lee, Jeong Ho
    • BMB Reports
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    • 제49권2호
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    • pp.71-72
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    • 2016
  • Focal cortical dysplasia type II (FCDII) is a focal malformation of the developing cerebral cortex and the major cause of intractable epilepsy. However, since the molecular genetic etiology of FCD has remained enigmatic, the effective therapeutic target for this condition has remained poorly understood. Our recent study on FCD utilizing various deep sequencing platforms identified somatic mutations in MTOR (existing as low as 1% allelic frequency) only in the affected brain tissues. We observed that these mutations induced hyperactivation of the mTOR kinase. In addition, focal cortical expression of mutant MTOR using in utero electroporation in mice, recapitulated the neuropathological features of FCDII, such as migration defect, cytomegalic neuron and spontaneous seizures. Furthermore, seizures and dysmorphic neurons were rescued by the administration of mTOR inhibitor, rapamycin. This study provides the first evidence that brain somatic activating mutations in MTOR cause FCD, and suggests the potential drug target for intractable epilepsy in FCD patients.

난치성 뇌전증 치료를 위한 심부뇌자극술: 임상적 관점에서 (Deep Brain Stimulation for Controlling Refractory Epilepsy: a Clinical Perspective)

  • 김우준;손영민
    • Annals of Clinical Neurophysiology
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    • 제14권2호
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    • pp.59-63
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    • 2012
  • Epilepsy has continued to provide challenges to epileptologists, as a significant proportion of patients continue to suffer from seizures despite medical and surgical treatments. Deep brain stimulation (DBS) has emerged as a new therapeutic modality that has the potential to improve quality of life and occasionally be curative for patients with medically refractory epilepsy who are not surgical candidates. Several groups have used DBS in drug-resistant epilepsy cases for which resective surgery cannot be applied. The promising subcortical brain structures are anterior and centromedian nucleus of the thalamus, subthalamic nucleus, and other nuclei to treat epilepsy in light of previous clinical and experimental data. Recently two randomized trials of neurostimulation for controlling refractory epilepsy employed the strategies to stimulate electrodes placed on both anterior thalamic nuclei or near seizure foci in response to electroencephalographically detected epileptiform activity. However, the more large-scale, long-term clinical trials which elucidates optimal stimulation parameters, ideal selection criteria for epilepsy DBS should be performed before long. In order to continue to advance the frontier of this field, it is imperative to have a good grasp of the current body of knowledge.

난치성 소아 간질에서 levetiracetam의 효과 (Efficacy of levetiracetam in refractory childhood epilepsy)

  • 이건수;강준원
    • Clinical and Experimental Pediatrics
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    • 제53권4호
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    • pp.571-578
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    • 2010
  • 목 적: 간질 환자의 20-30%는 난치성 간질로 알려졌는데, 기존의 항경련제나 새로운 항경련제에 반응하지 않는 난치성 간질환자에게 LEV를 부가요법으로 투여 후 효과와 부작용을 알아보고자 본 연구를 시행하였다. 방 법: 1989년 3월부터 2009년 3월까지 충남대학교병원 소아청소년과를 방문하여 난치성 간질로 진단받은 환자 중 6개월 이상 추적 관찰한 86명을 대상으로, LEV 투여 전 6개월간의 평균 경련 횟수를 기준으로 LEV외에 다른 항경련제를 추가하기 전까지의 평균 경련 감소 정도를 백분율로 분석하였다. 결 과: 86명의 환자 중 남자는 47명, 여자는 39명이었고, 발작 소실은 86명 중 44명(51.1%), 50% 이상 발작 감소는 86명 중 62명(72.1%)이었고, 19.8%는 변화가 없었으며, 5.8%는 발작이 증가했다. 나이, 동반질환, 발작형 및 치료 전 6개월간 발작 횟수는 발작 소실이나 50% 이상 발작 감소율과 관계가 없었다. 통계적으로 의미 있는 발작 소실과 50% 이상 발작 감소를 보인 경우는, 질병기간, LEV 투여 전 치료기간, LEV의 용량과 치료 전 사용한 항경련제의 숫자였으며, LEV 치료 기간과 뇌파는 발작 소실에만 의의가 있었고, 원인은 50% 이상 감소율에만 의의가 있었다. 부작용은 86명 중 40명(46%)에서 나타났고, 부작용은 기면, 과잉 행동, 과민성, 공격성, 피로 등이었으며, 대부분 일시적이거나 경미하여 치료 도중 소실되었다. 결 론: 난치성 소아 간질의 치료에 LEV의 부가 요법은 부분 발작뿐만 아니라 전신발작에도 효과와 반응이 좋고, 심각한 부작용도 없었다.

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

  • Seo, Jeong-Suk;Lee, Jong-Ju;Lee, Jung-Kyo;Kang, Jung-Gu;Lee, Sang-Am;Ko, Tae-Sung
    • Journal of Korean Neurosurgical Society
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    • 제39권1호
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    • pp.16-19
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    • 2006
  • 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.

측두엽내 공간 점유 병소와 동반된 난치성 간질의 수술적 치료 성적 (Surgical Outcome of Intractable Seizure with Space-Occupying Lesion in Temporal Lobe)

  • 박준범;이완수;이정교;전상룡;김정훈;노성우;나영신;김창진;권양;임승철;권병덕;강중구;이상암;고태성
    • Journal of Korean Neurosurgical Society
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    • 제30권1호
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    • pp.26-32
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    • 2001
  • Objective : The increasing use of sensitive neuroimaging techniques has demonstrated that significant percentage of patients with intractable complex partial seizures have brain masses, especially in temporal lobe. The optimal surgical solution for these patients is still open to debate. The purpose of our investigation is to evaluate the surgical outcome of patient with lesion-related temporal lobe epilepsy with respect to the types of surgery and the location of lesion. Patients and Methods : From DEC. 1993 to Dec. 1997, 35 patients with intractable epilepsy and space occupying temporal lobe lesion identified in preoperative MRI were included in this study. The types of surgery were lesionectomy, anterior temporal lobectomy with or without hippocampectomy. The location of lesion was divided as anteromedial group and lateral cortical group. The postoperative seizure outcomes according to the type of surgery and location of the lesion were compared. Results : Twenty-six of 34 patients(76.5%) were seizure-free after surgery. The Engel's class was favorable after anterior temporal lobectomy with or without hippocampectomy(p=.044) Conclusion : It is favorable to perform anterior temporal lobectomy for the treatment of intractable epilepsy with space-occipying lesion in temporal lobe. The resection of the hippocampus can be individualized.

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대뇌 피질 발달 기형을 동반한 난치성 소아 간질에서 케톤생성 식이요법의 효과 (The efficacy of ketogenic diet in childhood intractable epilepsy with malformation of cortical development)

  • 이영목;강두철;정다은;강훈철;김흥동
    • Clinical and Experimental Pediatrics
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    • 제49권2호
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    • pp.187-191
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    • 2006
  • 목 적 : MCD는 항경련제에 반응하지 않는 난치성 간질의 중요한 원인으로 수술적 치료의 대상으로 고려되어지지만, 병변의 범위나 분포에 따라 제한적이고 치료효과에 있어서도 차이가 보고되고 있다. 케톤생성 식이요법은 최근까지 대부분의 연구들에서 뛰어난 간질 억제효과를 보고하고 있으나, MCD 병변을 보이는 난치성 간질을 대상으로 시행한 케톤생성 식이요법의 치료 효과에 대한 보고는 국내외적으로 지금까지 없는 상태로, 본 연구에서는 이 환자군들에 대해 간질 수술보다 덜 침습적인 치료법인 케톤생성 식이요법의 간질 억제효과를 알아보고자 하였다. 방 법 : 1998년 이후 난치성 간질로 케톤생성 식이요법을 시행하였던 소아 환아들 중 뇌 MRI상 MCD 소견을 보이는 30명을 대상으로 후향적 의무기록 고찰과 분석을 시행하였으며, 케톤 생성 식이요법 시행 후 경련의 감소효과를 분석하였다. 결 과 : 전체 대상 30명 환아들의 남녀비는 1 : 1.1이었고, 경련을 처음 시작한 연령은 평균 $2.0{\pm}2.9$세, 케톤생성 식이요법을 시작한 평균 연령은 $5.4{\pm}4.6$세, 케톤생성 식이요법을 시작할 때까지의 경련 지속기간은 평균 $3.5{\pm}3.3$년, 환아들의 추적 관찰기간은 평균 $29.0{\pm}21.0$개월이었다. MCD의 종류는 대뇌 피질 이형성증(cortical dysplasia)이 24명(80.0%)으로 가장 많았고, MCD의 분포는 일측 대뇌 반구에만 있는 경우가 23명(76.7%), 양측 대뇌 반구 모두에 병변이 있는 경우가 7명(23.3%)이었다. 케톤생성 식이요법에 의한 경련의 감소 정도는 전체 30명의 환아 중 9명(30.0%)에서 경련이 완전히 소실되었으며, 50% 이상 경련이 감소된 경우는 14명(46.7%)이었는데, 경련을 처음 시작한 연령이나 케톤생성 식이요법을 시작할 때까지의 경련 지속기간은 경련의 감소효과와 통계학적 유의성이 없었고, 케톤생성 식이요법을 시작한 나이가 어릴수록, 케톤생성 식이요법의 기간이 길수록 경련의 감소효과가 높은 경향을 나타내었으나 통계적 유의성은 없었으며, MCD의 분포에 따른 특별한 연관관계는 없었다. 결 론 : 케톤생성 식이요법이 MCD 소견을 동반하고 있어 수술적 치료가 고려되어지는 난치성 소아 간질 환아에서도 간질 수술의 여러 가지 제한적인 측면과 침습성을 고려해 볼 때 효과적인 치료 결과를 기대할 수 있을 것으로 판단되며, 아직까지 국내외적으로 MCD를 동반한 난치성 소아 간질 환아에 대한 케톤생성 식이요법의 효과에 대한 자료가 미미한 상태이므로 전향적인 대규모의 비교 연구 및 분석이 필요할 것으로 사료된다.

난치성 간질환자에서 간질관련요인이 발작간 정신증상에 미치는 영향 (The Effect of Seizure-Related Variables on Interictal Psychiatric Symptoms in the Intractable Epileptic Patients)

  • 김종훈;한우상;이상건;류인균;하규섭
    • 정신신체의학
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    • 제5권2호
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    • pp.205-213
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    • 1997
  • The aim of this study is to find the relationship between interictal psychiatric symptoms and seizure-related variables such as structural changes, regional interictal perfusion changes, the number of interictal epileptic discharges and the presence of accompanying generalized tonic clonic seizure(GTC). The subjects were 75 patients (47 males : mean age $28.3{\pm}7.7$) with intractable epilepsy, Interictal psychiatric symptoms were rated by Symptom Checklist-90-R(SCL-90-R). Each of 4 seizure variables was measured by MRI, $^{99m}TC-HMPAO$ SPECT and the prolonged EEG monitoring. The mean SCL-90-R subscale T-scores and the ratio of the patients with definite psychiatric symptoms(T-score > 65 at any one subscale of SCL-90-R) were compared among the groups with different seizure variables. Demographic variables and clinical variables were not statistically different among the groups with different seizure variables. The patients with right mesial temporal sclerosis had higher mean SCL-90-R scores for obsessive compulsive symptom, interpersonal sensitivity, depression, hostility and psychotic symptoms than the patients with left mesial temporal sclerosis, and they also showed higher ratio of definite psychiatric symptoms. The presence of interictal epileptic discharges was related with higher T-scores of paranoid subscale. The areas of hypoperfusion, the presence of the GTC, and other clinical seizure variables showed no significant influences on the mean SCL-90-R subscale T-scores. from these results, the authors speculated that interictal subictal epileptic dischrages may be related with psychiatric symptoms of the intractable epileptic patients, especially with right mesial temporal sclerosis.

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