• Journal of Korean Neurosurgical Society
• /
• 제62권3호
• /
• pp.361-365
• /
• 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.

• 대한예방한의학회지
• /
• 제14권2호
• /
• pp.135-148
• /
• 2010

The major purpose of this study is to evaluate the classification of epileptic seizure types and epilepsy described in the literatures of both Western and East Asian medicine, especially based on the two criteria- a theoretical and a practical aspect of the classification systems. Currently, the 1981 classification of epileptic seizure types, and the 1989 classification of epilepsy syndromes and epilepsies which were proposed and approved by the International League Against Epilepsy(ILAE) have been generally accepted worldwide, although a variety of modifications have been consistently suggested. A large proportion of epilepsy cases cannot be easily classified as either 'focal' or 'generalized' or as either 'symptomatic' or 'idiopathic', so they fail to be precisely fallen into any of the ILAE categories. Terms and concepts used in the East Asian medicine are also inadequate to identify epileptic seizure types and epilepsy syndromes as discrete diagnostic entities because of ambiguities in definition and use. Therefore, this article suggests an alternative approach not only more helpful in understanding mechanism of epilepsy but also more easily applicable and effective in clinical value.

• Journal of Korean Neurosurgical Society
• /
• 제62권3호
• /
• pp.288-295
• /
• 2019

In 2011, the International League against Epilepsy (ILAE) proposed a first international consensus of the classification of focal cortical dysplasia (FCD). This FCD classification had been widely used in worldwide. In this review paper, the authors would like to give helpful comments for better understanding of the current FCD classification. Especially, the basic concepts of FCD type I, such as "radial", "tangential" and "microcolumn" will be discussed with figures. In addition, the limitations, genetic progress and prospect of FCD will be suggested.

• Journal of Korean Neurosurgical Society
• /
• 제62권3호
• /
• pp.353-360
• /
• 2019

Epilepsy surgery that eliminates the epileptogenic focus or disconnects the epileptic network has the potential to significantly improve seizure control in patients with medically intractable epilepsy. Magnetic resonance-guided laser interstitial thermal therapy (MRgLITT) has been an established option for epilepsy surgery since the US Food and Drug Administration cleared the use of MRgLITT in neurosurgery in 2007. MRgLITT is an ablative stereotactic procedure utilizing heat that is converted from laser energy, and the temperature of the tissue is monitored in real-time by MR thermography. Real-time quantitative thermal monitoring enables titration of laser energy for cellular injury, and it also estimates the extent of tissue damage. MRgLITT is applicable for lesion ablation in cases that the epileptogenic foci are localized and/or deep-seated such as in the mesial temporal lobe epilepsy and hypothalamic hamartoma. Seizure-free outcomes after MRgLITT are comparable to those of open surgery in well-selected patients such as those with mesial temporal sclerosis. Particularly in patients with hypothalamic hamartoma. In addition, MRgLITT can also be applied to ablate multiple discrete lesions of focal cortical dysplasia and tuberous sclerosis complex without the need for multiple craniotomies, as well as disconnection surgery such as corpus callosotomy. Careful planning of the target, the optimal trajectory of the laser probe, and the appropriate parameters for energy delivery are paramount to improve the seizure outcome and to reduce the complication caused by the thermal damage to the surrounding critical structures.

• Molecules and Cells
• /
• 제41권10호
• /
• pp.881-888
• /
• 2018

During the cortical development, cells in the brain acquire somatic mutations that can be implicated in various neurodevelopmental disorders. There is increasing evidence that brain somatic mutations lead to sporadic form of epileptic disorders with previously unknown etiology. In particular, malformation of cortical developments (MCD), ganglioglioma (GG) associated with intractable epilepsy and non-lesional focal epilepsy (NLFE) are known to be attributable to brain somatic mutations in mTOR pathway genes and others. In order to identify such somatic mutations presenting as low-level in epileptic brain tissues, the mutated cells should be enriched and sequenced with high-depth coverage. Nevertheless, there are a lot of technical limitations to accurately detect low-level of somatic mutations. Also, it is important to validate whether identified somatic mutations are truly causative for epileptic seizures or not. Furthermore, it will be necessary to understand the molecular mechanism of how brain somatic mutations disturb neuronal circuitry since epilepsy is a typical example of neural network disorder. In this review, we overview current genetic techniques and experimental tools in neuroscience that can address the existence and significance of brain somatic mutations in epileptic disorders as well as their effect on neuronal circuitry.

• Clinical and Experimental Pediatrics
• /
• 제49권5호
• /
• pp.529-532
• /
• 2006

목 적 : 뇌성마비는 뇌의 여러 가지 원인에 의해 뇌에 발생한 비진행성 손상으로, 이러한 뇌성마비를 가진 환아의 간질 발생 빈도는 15-90%로 다양하게 나타난다. 이렇게 뇌성마비와 간질이 중복 되서 나타나는 경우에 심각한 중복 장애를 남길 수 있다. 따라서 뇌성마비아 중 간질의 발생아와 비발생아의 특징을 비교하여 장기적인 진료 및 예후의 지표를 삼고자 하였다. 방 법 : 1995년부터 2004년까지 9년간 경희의료원 소아과 외래에서 뇌성마비로 진단받고 추적관찰이 가능하였던 93명을 대상으로 조사하였으며, 이들의 뇌성마비를 분류하고, 간질의 형태와 발병 당시의 나이, 뇌영상소견과 뇌파 소견을 비교하였다. 결 과 : 뇌성마비에서의 간질의 유병률은 93명 중 43명으로 46.2%이었으며, 발작의 초 발 연령은 평균 21.5개월로 1세 미만에서 발생한 경우가 21례(48.8%)로 가장 많았고, 1세 미만에서는 영아 연축과 근간대성 발작의 빈도가 의미 있게 높았으며, 전체적으로는 강직성-간대성 발작이 44.2%로 가장 많이 나타났다. 뇌영상소견에서 가장 많이 동반된 이상은 뇌 피질 위축으로 간질 발생군과 비간질 발생군에서 각각 22례(23.6%)와 12례(12.1%)였으며, 뇌파 소견에서는 간질 발생아의 전례에서 이상 소견을 보였다. 결 론 : 뇌성마비아에서 간질이 동반된 경우 어린 나이에 발생하는 경우가 많았으며, 간질의 형태로는 전신성 간대성-강직성 발작이 가장 많았다. 또한 뇌 영상검사와 뇌파검사에서 뇌성마비아 중 간질 발생아와 비간질 발생아에서 의미 있는 빈도 차이를 보여 조기 진단에 도움을 줄 것으로 생각된다.

• Clinical and Experimental Pediatrics
• /
• 제56권7호
• /
• pp.275-281
• /
• 2013

Temporal lobe epilepsy (TLE) is the most common type of medically intractable epilepsy in adults and children, and mesial temporal sclerosis is the most common underlying cause of TLE. Unlike in the case of adults, TLE in infants and young children often has etiologies other than mesial temporal sclerosis, such as tumors, cortical dysplasia, trauma, and vascular malformations. Differences in seizure semiology have also been reported. Motor manifestations are prominent in infants and young children, but they become less obvious with increasing age. Further, automatisms tend to become increasingly complex with age. However, in childhood and especially in adolescence, the clinical manifestations are similar to those of the adult population. Selective amygdalohippocampectomy can lead to excellent postoperative seizure outcome in adults, but favorable results have been seen in children as well. Anterior temporal lobectomy may prove to be a more successful surgery than amygdalohippocampectomy in children with intractable TLE. The presence of a focal brain lesion on magnetic resonance imaging is one of the most reliable independent predictors of a good postoperative seizure outcome. Seizure-free status is the most important predictor of improved psychosocial outcome with advanced quality of life and a lower proportion of disability among adults and children. Since the brain is more plastic during infancy and early childhood, recovery is promoted. In contrast, long epilepsy duration is an important risk factor for surgically refractory seizures. Therefore, patients with medically intractable TLE should undergo surgery as early as possible.

• Journal of Korean Neurosurgical Society
• /
• 제41권5호
• /
• pp.283-290
• /
• 2007

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.

• Journal of Korean Neurosurgical Society
• /
• 제67권3호
• /
• pp.299-307
• /
• 2024

Cerebral cavernous malformation (CCM) is a vascular anomaly commonly found in children and young adults. Common clinical presentations of pediatric patients with CCMs include headache, focal neurological deficits, and seizures. Approximately 40% of pediatric patients are asymptomatic. Understanding the natural history of CCM is crucial and hemorrhagic rates are higher in patients with an initial hemorrhagic presentation, whereas it is low in asymptomatic patients. There is a phenomenon known as temporal clustering in which a higher frequency of symptomatic hemorrhages occurs within a few years following the initial hemorrhagic event. Surgical resection remains the mainstay of treatment for pediatric CCMs. Excision of a hemosiderin-laden rim is controversial regarding its impact on epilepsy outcomes. Stereotactic radiosurgery is an alternative treatment, especially for deep-seated CCMs, but its true efficacy needs to be verified in a clinical trial.

• 대한핵의학회지
• /
• 제34권3호
• /
• pp.169-182
• /
• 2000

목적: Ictal SPECT의 간질 병소를 찾는 민감도와 정확도는 각 연구마다 다르다. 발작 중에 발생할 수 있는 여러 가지 형태의 뇌혈류변화를 알아보고 이와 관련된 임상 요인들을 찾아보기 위하여 본 연구를 수행하였다. 대상 및 방법: 간질 수술을 받을 61명의 부분적 간질 환자들에서 interictal SPECT와 ictal SPECT를 시행한 후 두 영상을 차감하여 subtraction SPECT (sub-SPECT)영상을 구하였다. Sub-SPECT에서 보인 발작 중 뇌혈류변화의 형태는 1) 국소 뇌혈류증가(focal hyperperfusion), 2) 뇌혈류증가 플러스(hyperperfusion-plus), 3) 국소 뇌혈류감소(focal hypoperfusion), 그리고, 4) 혈류증가-감소 복합형(combined hyperperfusion-hypoperfusion)의 4가지로 나누었다. 각 형태 별로 간질초점 (epielptic focus)과의 일치율 (concordance rate)을 산출하였다. 결과: Conventional ictal SPECT 분석법의 간질촛점 진단율은 68.9%이었고, sub-SPECT의 진단율은 뇌혈류증가 만을 분석하였을때는 85.2%이었고, 뇌혈류 증가 및 감소를 모두 평가하였을 때는 91.3%이었다. 발작 중 국소 뇌혈류 감소는 측두엽외 간질에 비하여, 측두엽 간질에서 더 드물게 관찰되었다. 내측 측두엽에서만 발생한 뇌혈류증가는 내측 측두엽간질에서만 관찰되었고, 외측 측두엽에서만 발생한 뇌혈류증가는 신피질 측두엽간질에서만 관찰되었다. 해마 경화를 동반한 측두엽간질은 다른 병리소견에 비하여 발작 중 뇌혈류감소 현상을 매우 적게 보였다. 결론: Ictal SPECT를 분석할 때는 뇌혈류증가 뿐만 아니라 뇌혈류감소 현상도 유심히 보아야 하며, 발작 중 국소 뇌혈류감소의 기전으로 간질초점의 발작 중 초기 에너지고갈 (intra-ictal early exhaustion) 또는 간질초점 주변부로의 발작파 전파에 의한 steal 현상 등을 생각할 수 있지만 앞으로 규명하여야 할 과제이다.