• Clinical and Experimental Pediatrics
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• 제55권5호
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• pp.171-176
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• 2012

Purpose: The aim of this study was to investigate the natural history of epilepsy and response to anti-epileptic drug treatment in patients with Angelman syndrome (AS) in Korea. Methods: We retrospectively reviewed the clinical records of 14 patients diagnosed with epilepsy out of a total of 17 patients with a genetic diagnosis of AS. These patients were seen at the Department of Pediatric Neurology at Severance Children's Hospital from March 2005 to March 2011. Results: Fourteen (9 males and 5 females) subjects (82.3%) were diagnosed with epilepsy in AS. The most common seizure types were generalized tonic-clonic (n=9, 27%) and myoclonic (n=9, 27%), followed by atonic (n=8, 24%), atypical absence (n=4, 12%) and complex partial seizure (n=3, 9%). The most commonly prescribed antiepileptic drug (AED) was valproic acid (VPA, n=12, 86%), followed by lamotrigine (LTG, n=9, 64%), and topiramate (n=8, 57%). According to questionnaires that determined whether each AED was efficacious or not, VPA had the highest response rate and LTG was associated with the highest rate of seizure exacerbation. Complete control of seizures was achieved in 6 patients. Partial control was achieved in 7 patients, while one patient was not controlled. Conclusion: Epilepsy is observed in the great majority of AS patients. It may have early onset and is often refractory to treatment. There are few reports about epilepsy in AS in Korea. This study will be helpful in understanding epilepsy in AS in Korea.

• 정신신체의학
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• 제4권1호
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• pp.64-70
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• 1996

The prevalence rate of psychiatric symptoms of the refractory epileptic patients was evaluated according to the location of the epileptic focus. The subjects were 91 patients admitted to Epilepsy Monitoring Unit of Seoul National University Hospital. The psychiatric symptoms were assessed by Korean version of Symptom Checklist-90-R(SCL-90-R). The locus of epileptic focus was assessed by clinical features, 2-hour interictal EEG, long-term video-EEG monitoring, brain MRI, interictal and ictal brain SPECT, and interictal brain PET The subjects were divided into three groups according to the epileptic focus, non-temporal(N=29), left temporal (N=26), and right temporal(N=32). There were no statistical differences in demographic and seizure-related variables among groups. The number of patients with $T-score {\geq} 65$ at any subscale of the SCL-90-R were compared by $X^2-test$ among groups. The mean T-scores of each subscale of the SCL-90-R were compared by oneway-ANOVA among groups. The prevalence rate of psychiatric symptoms of the refractory epileptic patients was 38.5%. There was no statistical difference in the prevalence rate of psychiatric symptoms among groups. However, the patients with non-temporal or right temporal epileptic foci showed statistically significant higher mean T-scores of interpersonal sensitivity, depression, hostility, and phobic subscales than the patients with left temporal epileptic foci. These results suggest that the epileptic focus plays an important role in the production of interictal psychiatric symptoms of the refractory epileptics.

• Journal of Korean Neurosurgical Society
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• 제62권3호
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• pp.302-312
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• 2019

Stereoelectroencephalography (SEEG) is an invasive technique used during the surgical management of medically refractory epilepsy. The utility of SEEG rests in its ability to survey the three-dimensional organization of the epileptogenic zone as well as nearby eloquent cortices. Once concentrated to specialized centers in Europe and Canada, the SEEG methodology has gained worldwide popularity due to its favorable morbidity profile, superior coverage of deep structures, and ability to perform multi-lobar explorations without the need for craniotomy. This rapid shift in practice represents both a challenge and an opportunity for pediatric neurosurgeons familiar with the subdural grid approach. The purpose of this review is to discuss the indications, technique, and safety of long-term SEEG monitoring in children. In addition to reviewing the conceptual and technical points of the diagnostic evaluation, attention will also be given to SEEG-based interventions (e.g., radiofrequency thermo-coagulation).

• Annals of Clinical Neurophysiology
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• 제2권1호
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• pp.27-30
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• 2000

Reflex epilepsies are distinct but not clearly understood clinical entity. Various cerebral activities induced by simple stimulation including visual, auditory, somatosensory stimulation, as well as diverse functional tasks such as reading, calculation, complex thinking are believed to be seizure-inducing factors. We experienced two patients whose seizures were readily precipitated by complex, strenuous thinking. Both patients was teen-aged boy at the onset of seizure(13, and 15 years of age each) with normal physical and mental growth. Although first seizure was precipitated by watching TV and playing puzzles in each patient, initial diagnosis was idiopathic generalized epilepsy, possibly juvenile myoclonic epilepsy( JME). For the first few years, seizures were infrequent but mostly precipitated by the tasks needs concentration such as playing computer games, decision-making, mathematics, reading, or during the examination. EEG revealed various thinking process including reading hard books, drawing complex figure, complex calculation induced epileptic discharges even if it usually needs certain period of concentration. Phenytoin, valproic acid, clonazepam, vigabatrin, and lamotrigine sometimes abated their seizures but none of these made them seizure-free. Complex reflex epilepsy induced by thinking was proposed to be a separate type of epilepsy or a variant of JME. Age, sex, stereotypic seizure-inducing factors, clinical course, and refractory epilepsies in these patients highly suggested this type of epilepsy as a variant of JME but its refractoriness and unique provocation still needs more speculation.

• 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.

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

• Clinical and Experimental Pediatrics
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• 제56권7호
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• pp.275-281
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• 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
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• 제60권3호
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• pp.294-300
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• 2017

Hypothalamic hamartoma (HH) is a benign indolent lesion despite the presentation of refractory epilepsy. Behavioral disturbances and endocrine problems are additional critical symptoms that arise along with HHs. Due to its nature of generating epileptiform discharge and spreading to cortical region, various management strategies have been proposed and combined. Surgical approaches with open craniotomy or endoscopy, stereotactic approaches with radiosurgery and gamma knife surgery or radiofrequency thermos-coagulation, and laser ablation have been introduced. Topographical dimension and the surgeon's preference are key factors for treatment modalities. Endoscopic disconnection has been one of the most favorable options performed in treating HHs. Here we discuss presurgical evaluation, patient selection, surgical procedures, and complications.

• 한국정보통신학회논문지
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• 제10권8호
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• pp.1471-1477
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• 2006

뇌파 기록은 난치성 간질 환자인 성인을 대상으로 하였다. 경막하 전극으로 부터 기록된 피질뇌파를 위상차해석에 의해서 간질 초점의 동정 및 발작파 전파의 가시화를 행하였다. 발작파의 세밀한 시간변화를 조사하기 위해서 자기 회귀모델, 웨이브렛해석을 이용하여 발작파 성분을 구하고, 상호상관법에 의해 각 전극 간의 위상차를 해석했다. 그 결과, 발작파의 초점은 적어도 2종류가 존재하였고, 각각의 전파방법도 달랐다. 이로 부터 발작파의 출현 기구는 동시에 적어도 2종류 존재하는 것을 확인할 수 있었다. 또한, 발작파 출현의 변화를 경시적으로 해석할 수 있기 때문에 발작파 전파의 가시화에 유효하다고 생각되어 진다.

• Journal of Korean Neurosurgical Society
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• 제41권6호
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• pp.397-402
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• 2007

Objective : Balloon cells and dysplastic neurons are histopathological hallmarks of the cortical tubers of tuberous sclerosis complex [TSC] and focal cortical dysplasia [FCD] of the Taylor type. They are believed to be the epileptogenic substrate and cause therapeutic drug resistant epilepsy in man. P-glycoprotein [P-gp] is the product of multidrug resistance gene [MDR1], and it maintains intracellular drug concentration at a relatively low level. The authors investigated expression of P-gp in balloon cells and dysplastic neurons of cortical tubers in patients with TSC. Methods : An immunohistochemical study using the primary antibody for P-gp, as an indicative of drug resistance, was performed in the cortical tuber tissues in two patients of surgical resection for epilepsy and six autopsy cases. Results : Balloon cells of each lesion showed different intensity and number in P-gp immunopositivity. P-gp immunopositivity in balloon cells were 28.2%, and dysplastic neurons were 22.7%. These immunoreactivities were more prominent in balloon cells distributed in the subpial region than deeper region of the cortical tubers. Capillary endothelial cells within the cortical tubers also showed P-gp immunopositivity. Conclusion : In this study, the drug resistance protein P-glycoprotein in balloon cells and dysplastic neurons might explain medically refractory epilepsy in TSC.