• 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.

• 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.

• Annals of Clinical Neurophysiology
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• 제1권2호
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• pp.147-153
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• 1999

Hippocampal slice models can be a powerful tool to study the mechanism of partial epilepsy. Despite the loss of connection with the rest of the brain, in vitro hippocampal slice preparations allow detailed physiological and pharmacological studies, which would be impossible, in vivo. There are several methods to induce electrographic seizures on hippocampal slice models. Those are electrical pulse train stimulation, 0 $Mg^{2+}$ artificial cerebrational fluid and high concentration of extracelluar $K^+$ on bath. Among them, the electrically triggered seizure may mimic the physiological communication between neuronal populations without any deterioration of normal physiologic and chemical status of the hippocampal slice models. Presumably, such communication from hyperexcitable areas to other neuronal populations is involved in the development of epilepsy. Electrographic seizures in hippocampal slice models occur in the network of neurons that are involved in epileptic seizures in the hippocampus in vivo. Because these models have many advantages and are very valuable to research of epileptogenesis on partial epilepsy, I would like to introduce the electrophysiological methods to induce electrographic seizure or epilepsy on hippocampal slice models briefly in this paper.

• Journal of Korean Neurosurgical Society
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• 제56권3호
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• pp.248-253
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• 2014

Objective : Cortical dysplasia (CD) is one of the common causes of epilepsy surgery. However, surgical outcome still remains poor, especially with frontal lobe epilepsy (FLE), despite the advancement of neuroimaging techniques and expansion of surgical indications. The aim of this study was to focus on surgical strategies in terms of extent of resection to improve surgical outcome in the cases of FLE with CD. Methods : A total of 11 patients of FLE were selected among 67 patients who were proven pathologically as CD, out of a total of 726 epilepsy surgery series since 1992. This study categorized surgical groups into three according to the extent of resection : 1) focal corticectomy, 2) regional corticectomy, and 3) partial functional lobectomy, based on the preoperative evaluation, in particular, ictal scalp EEG onset and/or intracranial recordings, and the lesions in high-resolution MRI. Surgical outcome was assessed following Engel's classification system. Results : Focal corticectomy was performed in 5 patients and regional corticectomy in another set of 5 patients. Only 1 patient underwent partial functional lobectomy. Types I and II CD were detected with the same frequency (45.45% each) and postoperative outcome was fully satisfactory (91%). Conclusion : The strategy of epilepsy surgery is to focus on the different characteristics of each individual, considering the extent of real resection, which is based on the focal ictal onset consistent with neuroimaging, especially in the practical point of view of neurosurgery.

• 대한임상검사과학회지
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• 제38권2호
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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.

• Annals of Clinical Neurophysiology
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• 제10권1호
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• pp.52-57
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• 2008

Even though the origin and nature of nocturnal paroxysmal dystonia (NPD) remains unclear, it has been considered as a manifestation of the nocturnal frontal lobe epilepsy. We report a 17-year-old man with abnormal stereotyped movement during sleep. Video-EEG monitoring, ictal SPECT and night polysomnography did not show any evidence of epilepsy. However, the partial response to large dose of carbamazepine and the scoring according to the frontal lobe epilepsy and parasomnias (FLEP) scale suggest his events could be classified as epilepsy. Therefore we think the FLEP scale might be a useful tool for differential diagnosis in a patient presenting NPD.

• Journal of Korean Neurosurgical Society
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• 제29권2호
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• pp.222-230
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• 2000

Purpose : Resection of the epileptogenic zone in the parietal and occipital lobes may be relevant although only few studies have been reported. Methods : Eight patients with parietal epilepsy and nine patients with occipital epilepsy were included for this study. Preoperatively, all had video-EEG monitoring with extracranial electrodes, MRI, 3D-surface rendering of MRI using Allegro(ISG Technologies Inc., Toronto, Canada), and PET scans. Sixteen patients underwent invasive recording with subdural grid. Eight had parietal resection including the sensory cortex in two. Seven had partial occipital resection. Two underwent total unilateral occipital lobectomy. The extent of the resection was made based mainly on the data of invasive EEG recordings, MRI, and 3D-surface rendering of MRI, not on the intraoperative electrocorticographic findings as usually done. During resection, electrocortical stimulation was performed on the motor cortex and speech area. Results : Out of eight patients with parietal epilepsy, three had sensory aura, two had gustatory aura, and two had visual aura. Six of nine patients with occipital epilepsy had visual auras. All had complex partial seizures with lateralizing signs in 15 patients. Four had quadrantopsia. One had mild right hemiparesis. Abnormality in MRI was noticed in six out of eight parietal epilepsy and in eight out of nine occipital epilepsy. 3D-surface rendering of MRI visualized volumetric abnormality with geometric spatial relationships adjacent to the normal brain, in all of parietal and occipital epilepsy. Surface EEG recording was not reliable in localizing the epileptogenic zone in any patient. The subdural grid electrodes can be implanted on the core of the structural abnormality in 3D-reconstructed brain. Ictal onset zone was localized accurately by subdural grid EEGs in 16 patients. Motor cortex in nine and sensory speech area in two were identified by electrocortical stimulation. Histopathologic findings revealed cortical dysplasia in 10 patients ; tuberous sclerosis was combined in two, hamartoma and ganglioglioma in one each, and subpial gliosis in six. Eleven patients were seizure free at follow-up of 6 months to 37 months(mean 19.7 months) after surgery. Seizures recurred in two and were unchanged in one. Six produced transient sensory loss and one developed hemiparesis and tactile agnosia. One revealed transient apraxia. Two patients with preoperative quadrantopsia developed homonymous hemianopsia. Conclusion : This study suggests that surgical treatment was relevant in parietal and occipital epilepsies with good surgical outcome, without significant neurologic sequelae. Neuroimaging studies including conventional MRI, 3Dsurface rendering of MRI were necessary in identifying the epileptogenic zone. In particular, 3D-surface rendering of MRI was very helpful in presuming the epileptogenic zone in patients with unidentifiable lesion in the conventional MRI, in planning surgical approach to lesions, and also in making a decision of the extent of the epileptogenic zone in patients with identifiable lesion in conventional MRI. Invasive EEG recording with the subdural grid electrodes helped to confirm a core of the epileptogenic zone which was revealed in 3D-surface rendered brain.

• Clinical and Experimental Pediatrics
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• 제53권4호
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• pp.565-570
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• 2010

목 적: LTG의 국외에서의 임상 연구는 다양한 발작과 간질증후군에 단일 또는 보조요법으로서 넓은 범위의 작용을 한다고 보고되었다. 국내에서는 아직까지 소아연령에서의 LTG 초기 단일요법에 대한 연구보고가 거의 없다. 이에 저자들은 단일 병원의 전향적 자료 분석을 통한 소아 간질 환자에서의 단일 LTG 약물요법의 효용성과 안정성에 대해 연구하고자 하였다. 방 법: 2002년 9월부터 2009년 6월까지 간질약 복용 병력이 없이 간질로진단 받고 LTG을 복약하였던 소아 환자 148명을 대상으로 전향적 연구를 통해 효용성과 안정성을 연구하였다. 결 과: 대상 환아 중 초기 단일 요법으로 LTG을 복용 후 최소 6개월간 외래 추적 관찰했을 때 발작이 전혀 없었던 환아(완전 관해군)는 87명(78.4%, n=111)이었고 발작 감소를 보인 환아(부분 관해군)는 13명(11.7%), 발작이 이전과 같은 빈도이거나 오히려 악화된 경우(발작 지속군)는 11명(9.9%)이었고 부작용인 피부발진과 틱 장애로 LTG복약을 중지해야만 했던 환아가 8명이었다. 발작의 유형에 따른 치료 결과는 완전 관해군을 기준으로 할 때 부분 간질에서 전신간질보다 발작 완해률이 81.6% vs 44.8 %로 더 높았다(${\kappa}^2$=26.75, $P$<0.05). 발작 유형별로는 각각 CPS가 41명(75.9%, n=54), BRE가 30명(90.9%, n=33) absence seizure가 3명(30.0%, n=10) JME가 5명(35.7%, n=14), IGE가 5명(100.0%, n=5)으로 완전 관해 되었고 IGE와 BRE에서 완전 관해률이 각각 100.0%, 90.9%로 높았다. 투약 기간 중 부작용이 관찰된 환아는 모두 17명(14.3%, n=119)이었다. 피부 발진이 11명, 틱 장애가 3명, 간염이 1명, 결막염이 1명, 졸림이 1명에서 관찰되었고 피부 발진이 생긴 환아 11명 중 7명은 발진이 심하여, 1명은 틱 증상이 심하여 투약을 중지하였다. Stevens-Johonson 증후군 같은 심각한 부작용이 생겼던 환아는 없었다. 결 론: 본 연구 결과 LTG 초기 단일요법의 효용성은 국외 보고와 비교하여 우수하거나 큰 차이가 없었으며, 위중한 부작용 사례는 없었다. 소아 간질 환자, 특히 양성 로란딕 간질 환자와 특발성 전신발작 환자에서 LTG의 초기 단일 요법 치료에 매우 우수한 것으로 사료되어 문헌 고찰과 함께 보고하는 바이다.

• 동의생리병리학회지
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• 제21권1호
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• pp.328-332
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• 2007

Epilepsy is any of various neurological disorders characterized by sudden, recurring attacks of motor, sensory, or psychic malfunction with or without loss of consciousness or convulsive seizures. It could be divided into subcategories due to the international classification of epileptic seizure and the complex partial seizure, that is one of epileptic seizure subcategories, is characterized by elaborate and multiple sensory, motor, and/or psychic components accompanying the clouding of consciousness, prodrome, automatism, postictal confusion. This study reports a patient who was presumptive diagnosed as complex partial seizure by having the clouding of consciousness, prodrome, postictal confusion. We also diagnosed him as a ‘dianxian’ patient induced by sum deficiency of liver & kidney. This patient was treated by acupuncture to tonifying eum of liver & kidney and it achieved markedly improved symptoms.

• 대한한방소아과학회지
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• 제28권1호
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• pp.61-70
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• 2014

Objectives The purpose of this study is to report three cases of epileptic children who were treated by oriental medicine. Methods Three epileptic children were treated by Gwakhyangjeonggi-san and Kuibiondam-tang. Two children had took combination therapy; anti-epilepsy drugs and oriental medicines and the other one took only oriental medicines. We measured frequency and intensity of seizure, and observed general conditions. Results The oriental medicine treatment is not only reducing seizures, but also improving general condition like digestion, constipation and reducing frequency of common cold and symptom of rhinitis. Conclusions Epilepsy in childhood is induced by various factor like Fung (風), Dam (痰), Sik (食), Kyung (驚). We administrated oriental medicine and prevented seizures.