• 제목/요약/키워드: Juvenile myoclonic epilepsy

검색결과 6건 처리시간 0.018초

복잡한 사고에 의해 유발되는 간질발작 2예 (Two Patients with Epilepsy Induced by Complex Thinking)

  • 김재문;이경목;손은희;정기영
    • 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.

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Development and Validation of MRI-Based Radiomics Models for Diagnosing Juvenile Myoclonic Epilepsy

  • Kyung Min Kim;Heewon Hwang;Beomseok Sohn;Kisung Park;Kyunghwa Han;Sung Soo Ahn;Wonwoo Lee;Min Kyung Chu;Kyoung Heo;Seung-Koo Lee
    • Korean Journal of Radiology
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    • 제23권12호
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    • pp.1281-1289
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    • 2022
  • Objective: Radiomic modeling using multiple regions of interest in MRI of the brain to diagnose juvenile myoclonic epilepsy (JME) has not yet been investigated. This study aimed to develop and validate radiomics prediction models to distinguish patients with JME from healthy controls (HCs), and to evaluate the feasibility of a radiomics approach using MRI for diagnosing JME. Materials and Methods: A total of 97 JME patients (25.6 ± 8.5 years; female, 45.5%) and 32 HCs (28.9 ± 11.4 years; female, 50.0%) were randomly split (7:3 ratio) into a training (n = 90) and a test set (n = 39) group. Radiomic features were extracted from 22 regions of interest in the brain using the T1-weighted MRI based on clinical evidence. Predictive models were trained using seven modeling methods, including a light gradient boosting machine, support vector classifier, random forest, logistic regression, extreme gradient boosting, gradient boosting machine, and decision tree, with radiomics features in the training set. The performance of the models was validated and compared to the test set. The model with the highest area under the receiver operating curve (AUROC) was chosen, and important features in the model were identified. Results: The seven tested radiomics models, including light gradient boosting machine, support vector classifier, random forest, logistic regression, extreme gradient boosting, gradient boosting machine, and decision tree, showed AUROC values of 0.817, 0.807, 0.783, 0.779, 0.767, 0.762, and 0.672, respectively. The light gradient boosting machine with the highest AUROC, albeit without statistically significant differences from the other models in pairwise comparisons, had accuracy, precision, recall, and F1 scores of 0.795, 0.818, 0.931, and 0.871, respectively. Radiomic features, including the putamen and ventral diencephalon, were ranked as the most important for suggesting JME. Conclusion: Radiomic models using MRI were able to differentiate JME from HCs.

간질 치료에서 뇌파의 임상적 유용성에 관한 논란: 긍정적 관점에서 (Controversies in Usefulness of EEG for Clinical Decision in Epilepsy: Pros.)

  • 손영민;김영인
    • Annals of Clinical Neurophysiology
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    • 제9권2호
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    • pp.63-68
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    • 2007
  • The EEG plays an important diagnostic role in epilepsy and provides supporting evidence of a seizure disorder as well as assisting with classification of seizures and epilepsy syndromes. There are a variety of electroclinical syndromes that are really defined by the EEG such as Lennox-Gastaut syndrome, benign rolandic epilepsy, childhood absence epilepsy, juvenile myoclonic epilepsy and also for localization purposes, it is vitally important especially for temporal lobe epilepsy. The sensitivity of first routine EEG in diagnosis of epilepsy has been known about 20-50%, but this proportion rises to 80-90% if sleep EEG and repetitive recording should be added. Convincing evidences suggest that the EEG may also provide useful prognostic information regarding seizure recurrence after a single unprovoked attack and following antiepileptic drug (AED) withdrawal. Moreover, patterns in the EEG make it possible to disclose an ictal feature of nonconvulsive status epilepticus, separate epileptic from other non-epileptic episodes and clarify the clues predictive of the cause of the encephalopathy (i.e., triphasic waves in metabolic encephalopathy). Therefore, regardless of its low sensitivity and other pitfalls, EEG should be considered not only in the situation of new onset episode such as a newly developed, unprovoked seizure or a condition manifesting decreased mentality from obscure origin, but also as a barometer of the long-term outcome following AED withdrawal.

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간질 환아에서 Lamotrigine 초기 단독 요법의 효능 및 안정성에 대한 연구 (Clinical efficacy and safety of lamotrigine monotherapy in newly diagnosed pediatric patients with epilepsy)

  • 한지혜;오정은;김선준
    • 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.