• 제목/요약/키워드: Focal epilepsy

검색결과 40건 처리시간 0.032초

결신발작 이후 확인된 일시적인 자기공명영상 이상 1예 (Transient Abnormalities on Magnetic Resonance Imaging after Absence Seizures)

  • 유혜원;윤리라;김혜영;곽민정;박경희;배미혜;이윤진;남상욱;김영미
    • 대한소아신경학회지
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    • 제26권4호
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    • pp.280-283
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    • 2018
  • 뇌전증 환자에서 자기공명영상은 기질적인 병변을 확인하기 위해 권장된다. 그러나 청소년기 결신 발작을 포함한 특발성 전신 발작에서 이상소견은 대개 자기공명영상과 같은 뇌 영상 기법을 사용하여 확인할 수 없다. 발작기 동안 자기공명영상 이상은 뇌전증 지속증에 의한 이차성으로 가장 흔히 보고되었으며, 부분발작과 전신 강직간대 발작환자에서 거의 나타나지 않는다. 결신 발작 동안 자기공명영상의 이상은 매우 드물다. 5세 소녀가 sodium valproate로 계속 치료를 했음에도 불구하고 3일간 발생한 결신 발작으로 내원하였다. 뇌파 검사에서 과다호흡 이후 3 Hz 극서파를 보였다. T2 강조 뇌 자기공명영상에서 좌측 외측구 주변의 비정상 피질 비후가 확인되었고, 피질 형성 이상이나 종양이 의심되었다. 환자는 lamotrigine으로 치료를 시작하였고 이후 발작은 없었다. 2개월 후 추적관찰로 뇌 자기공명영상을 시행하였고 이전의 비정상 부분은 확인되지 않았다. 저자들은 유년기 결신 발작 시 확인된 외측구 주변의 가역적인 뇌 자기공명영상 이상에 대해 보고하는 바이며 이것은 결신 발작 후 일과성 자기공명영상 이상 소견의 첫 번째 보고로 의미가 있다. 결신 발작과 부분 발작에서 자기공명영상 이상은 일시적인 이상 일 수 있으며 다른 질환과 감별 시 고려해야 할 것이다.

Cognitive and other neuropsychological profiles in children with newly diagnosed benign rolandic epilepsy

  • Kwon, Soonhak;Seo, Hye-Eun;Hwang, Su Kyeong
    • Clinical and Experimental Pediatrics
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    • 제55권10호
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    • pp.383-387
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    • 2012
  • Purpose: Although benign rolandic epilepsy (BRE) is a benign condition, it may be associated with a spectrum of behavioral, psychiatric, and cognitive disorders. This study aimed to assess the cognitive and other neuropsychological profiles of children with BRE. Methods: In total, 23 children with BRE were consecutively recruited. All children underwent sleep electroencephalography (EEG) and were assessed on a battery of comprehensive neuropsychological tests including the Korean versions of the Wechsler intelligence scale for children III, frontal executive neuropsychological test, rey complex figure test, Wisconsin card sorting test, attention deficit diagnostic scale, and child behavior checklist scale. Results: The study subjects included 13 boys and 10 girls aged $9.0{\pm}1.6$ years. Our subjects showed an average monthly seizure frequency of $0.9{\pm}0.7$, and a majority of them had focal seizures (70%). The spike index (frequency/min) was $4.1{\pm}5.3$ (right) and $13.1{\pm}15.9$ (left). Of the 23 subjects, 9 showed frequent spikes (>10/min) on the EEG. The subjects had normal cognitive and frontal executive functions, memory, and other neuropsychological sub-domain scores, even though 8 children (35%) showed some evidence of learning difficulties, attention deficits, and aggressive behavior. Conclusion: Our data have limited predictive value; however, these data demonstrate that although BRE appears to be benign at the onset, children with BRE might develop cognitive, behavioral, and other psychiatric disorders during the active phase of epilepsy, and these problems may even outlast the BRE. Therefore, we recommend scrupulous follow-up for children with BRE.

상호상관법에 의한 간질 초점부 피질뇌파 전파의 가시화 (Visualization of propagating process in the seizure discharge by use of cross-correlation analysis)

  • 김진우
    • 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.

Glucose transport 1 deficiency presenting as infantile spasms with a mutation identified in exon 9 of SLC2A1

  • Lee, Hyun Hee;Hur, Yun Jung
    • Clinical and Experimental Pediatrics
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    • 제59권sup1호
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    • pp.29-31
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    • 2016
  • Glucose transport 1 (GLUT-1) deficiency is a rare syndrome caused by mutations in the glucose transporter 1 gene (SLC2A1) and is characterized by early-onset intractable epilepsy, delayed development, and movement disorder. De novo mutations and several hot spots in N34, G91, R126, R153, and R333 of exons 2, 3, 4, and 8 of SLC2A1 are associated with this condition. Seizures, one of the main clinical features of GLUT-1 deficiency, usually develop during infancy. Most patients experience brief and subtle myoclonic jerk and focal seizures that evolve into a mixture of different types of seizures, such as generalized tonic-clonic, absence, myoclonic, and complex partial seizures. Here, we describe the case of a patient with GLUT-1 deficiency who developed infantile spasms and showed delayed development at 6 months of age. She had intractable epilepsy despite receiving aggressive antiepileptic drug therapy, and underwent a metabolic workup. Cerebrospinal fluid (CSF) examination showed CSF-glucose-to-blood-glucose ratio of 0.38, with a normal lactate level. Bidirectional sequencing of SLC2A1 identified a missense mutation (c.1198C>T) at codon 400 (p.Arg400Cys) of exon 9.

열성 경련에 대한 최신 지견 (Recent Advance in Febrile Seizure)

  • 한윤정;장규태
    • 대한한방소아과학회지
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    • 제21권3호
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    • pp.189-203
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    • 2007
  • Objectives The prognosis, recurrence rate and treatment of febrile seizure were studied through the research of recent western medicine and Chinese oriental medicine paper. Methods Recent western medicine paper of internal and external and chinese oriental medicine paper from 1999 to 2007 were investigated. Results and Conclusions The prognosis of febrile seizure was good in most cases, but children with febrile seizure who developed epilepsy range between 2 and 10%. The risk factors developed epilepsy including complex febrile seizure, focal and prolonged seizure, pre-existing neurodevelopmental abnormalities and recurrent febrile seizure. Recurrence rate of febrile seizure ranges between 30 and 50% was high. The risk factors can be predicted by their age at first febrile seizure happened, family medical history of febrile seizure and epilepsy, complex febrile seizure, and neurodevelopmental abnormalities. However, the most important factor of those is the age when they have first febrile seizure. Diazepam or Lorazepam was administrated for a child with prolonged seizure but only Diazepam was used for reducing recurrence of febrile seizurein febrile illness. However, there were some side effects such as lethargy, ataxia, and irritability. The study of chinese oriental medicine demonstrates that the acupuncture and venesection were used for seizure attack and reduced of recurrences and second attack. To reduce recurrence of febrile seizure, herbal medicine was also used for febrile illness or after seizure attack within a certain period of time, so reduce the recurrence, frequency of seizure and febrile illness. The most of herbs in prescription were used for removing heat and toxic meterials(淸熱解毒), extinguishing wind and to stopping the convulsion(熄風止痙)

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Epileptogenic Properties of Balloon Cells in Cortical Tubers of Tuberous Sclerosis : Upregulation of Drug Resistance Proteins

  • Kang, Nam-Gu;Chang, Hong-Joen;Ok, Young-Cheol;Lee, Rae-Seop;Park, Seung-Kyu;Lim, Jun-Seob;Cho, Kyu-Yong;Kim, Hyung-Ihl;Kim, Jae-Hyoo;Oh, Hyun-Sik;Lee, Min-Cheol
    • 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.

경련 질환 환아의 정신병리와 신경학적 요인과의 관계에 대한 연구 (THE STUDY ON RELATIONSHIP BETWEEN PSYCHOPATHOLOGY AND NEUROLOGICAL FACTORS IN CHRONIC EPILEPTIC CHILDREN)

  • 김붕년;조수철;황용승
    • Journal of the Korean Academy of Child and Adolescent Psychiatry
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    • 제7권1호
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    • pp.92-109
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    • 1996
  • 본 연구에서는 정상 지능 범위의 만성 특발성 경련 질환으로 치료중인 40명의 환아군과, 나이, 성별, 학업 성적, 지능 지수를 일치시킨 30명의 대조군을 대상으로, 국내외를 통해 신뢰도 및 타당도가 확인된 평가 도구들인, 부모용 아동 행동 조사표(Child Behavior Checklist)의 한국판, DSM-III-R에 따른 파탄적 행동 장애 척도(Disruptive behavior disorders scale according to DBM-III-R)의 한국판, 예일 아동 평가표(Yale children's inventory)의 한국판, 소아 우울 척도(Children's Depression Inventory)의 한국촌, 소아용 상태 및 특성불안 척도(State-Trait Anxiety Inventory for children)의 한국판, 피어스-해리스 자기 개념 척도(Piers-Harris self-concept questionnaire)의 한국판. 가정 환경 척도(Family environment Scale)의 한국판, 간이 정신진단 검사(Symptom check-list-90)의 한국판, 경련질환에 대한 부모태도 척도(Parent's attitude to epilepsy questionnaire)의 한국판, 아동 의존척도(self-administered dependency questionnaire)의 한국판을 사용하였고, 이들 결과로 부터 얻은 자료의 일부인 환아의 정신병리 부분과 경련 질환의 신경과적인 변수들인 환아의 성별 및 나이, 경련 질환 발병시 나이와 유병기간, 경련질환의 임상적 유형, 뇌파의 유형 최근 6개월간의 경련 빈도, 항경련 약물 치료력과 그 반응등과의 관련성을 평가하였다. 위에서 얻은 자료들을 토대로 분석하여 다음과 같은 연구 결과를 얻었다. 환아의 우울과 관계된 위험 요인은, 조기 발병(early onset), 복합성 부분 간질(complex partial seizure), 뇌파상 편측성 측두엽 이상(lateralized temporal focal abnormality in EEG), 약물 병합 투여(drug polypharmacy), 고 빈도 경련(high seizure frequency)임을 알 수 있었고, 이들 위험요인들은 상호 연관되어 있었다. 불안에 영향을 미치는 요인은 높은 연령. 뇌파상 편측성 측두엽 이상, 고 빈도 경련이었고, 학습 및 언어적 문제와 관련이 있는 위험 요인은 환아의 어린나이, 조기 발병, 복합성 부분 간질, 고빈도 경련 등이었다. 주의력 결핍 과잉행동 장애와 반항 및 품행장애에 영향을 미치는 위험 요인은 남성, 어린 나이, 조기 발병, 뇌파상 편측성 측두엽 이상, 고 빈도 경련등이었다. 위의 결과를 종합하면, 우울, 불안, 품행장애 및 주의력 결핍 과잉 행동장애에 공통적으로 관계되는 위험요인은, 뇌파상 편측성 측두엽 이상, 고 빈도 경련이라는 겻을 알 수 있었다. 향후 연구를 통해서는 신경학적 요인이외에 가족 및 사회-인구학적 위험 요인을 찾는 연구가 지속되어야 하며, 이를 통해 종합적 위험 요인 평가 작업과 치료적 대안을 제시하는 후속 작업이 지속 되어야 할 것 이다.

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Improving Diagnostic Performance of MRI for Temporal Lobe Epilepsy With Deep Learning-Based Image Reconstruction in Patients With Suspected Focal Epilepsy

  • Pae Sun Suh;Ji Eun Park;Yun Hwa Roh;Seonok Kim;Mina Jung;Yong Seo Koo;Sang-Ahm Lee;Yangsean Choi;Ho Sung Kim
    • Korean Journal of Radiology
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    • 제25권4호
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    • pp.374-383
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    • 2024
  • Objective: To evaluate the diagnostic performance and image quality of 1.5-mm slice thickness MRI with deep learningbased image reconstruction (1.5-mm MRI + DLR) compared to routine 3-mm slice thickness MRI (routine MRI) and 1.5-mm slice thickness MRI without DLR (1.5-mm MRI without DLR) for evaluating temporal lobe epilepsy (TLE). Materials and Methods: This retrospective study included 117 MR image sets comprising 1.5-mm MRI + DLR, 1.5-mm MRI without DLR, and routine MRI from 117 consecutive patients (mean age, 41 years; 61 female; 34 patients with TLE and 83 without TLE). Two neuroradiologists evaluated the presence of hippocampal or temporal lobe lesions, volume loss, signal abnormalities, loss of internal structure of the hippocampus, and lesion conspicuity in the temporal lobe. Reference standards for TLE were independently constructed by neurologists using clinical and radiological findings. Subjective image quality, signal-to-noise ratio (SNR), and contrast-to-noise ratio (CNR) were analyzed. Performance in diagnosing TLE, lesion findings, and image quality were compared among the three protocols. Results: The pooled sensitivity of 1.5-mm MRI + DLR (91.2%) for diagnosing TLE was higher than that of routine MRI (72.1%, P < 0.001). In the subgroup analysis, 1.5-mm MRI + DLR showed higher sensitivity for hippocampal lesions than routine MRI (92.7% vs. 75.0%, P = 0.001), with improved depiction of hippocampal T2 high signal intensity change (P = 0.016) and loss of internal structure (P < 0.001). However, the pooled specificity of 1.5-mm MRI + DLR (76.5%) was lower than that of routine MRI (89.2%, P = 0.004). Compared with 1.5-mm MRI without DLR, 1.5-mm MRI + DLR resulted in significantly improved pooled accuracy (91.2% vs. 73.1%, P = 0.010), image quality, SNR, and CNR (all, P < 0.001). Conclusion: The use of 1.5-mm MRI + DLR enhanced the performance of MRI in diagnosing TLE, particularly in hippocampal evaluation, because of improved depiction of hippocampal abnormalities and enhanced image quality.

Redox-modulation of NMDA receptor activity by nitric oxide congeners

  • Kim, Won-Ki;Stuart A. Lipton
    • 한국응용약물학회:학술대회논문집
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    • 한국응용약물학회 1995년도 제3회 추계심포지움
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    • pp.125-132
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    • 1995
  • In neurons, nitric oxide(NO) is produced by neuronal nitric oxide synthase following stimulation of N-methyl-D-aspartate(NMDA) receptors and the subsequent influx of Ca$\^$2+/. NO, induced in this manner, reportedly plays critical roles in neuronal plasticity, including neurite outgrowth, synaptic transmission, and long-term potentiation(LTP) (1-7). However, excessive activation of NMDA receptors has also been shown to be associated with various neurological disorders, including focal ischemia, epilepsy, trauma, neuropathic pain and chronic neurodegenerative maladies, such as Parkinson's disease, Hungtington's disease and amyotrophic lateral sclerosis(8). The paradox that nitric oxide(NO) has both neuroprotective and neurodestructive effects may be explained, at least in part, by the finding that NO effects on neurons are dependent on the redox state. This claim may be supported by the recent finding that tissue concentrations of cysteine approach 700 ${\mu}$M in settings of cerebral ischemia (9), levels of thiol that is expected to influence both the redox state of the system and the NO group itself(10).

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Genetic Basis of Early-onset Developmental and Epileptic Encephalopathies

  • Hwang, Su-Kyeong
    • Journal of Interdisciplinary Genomics
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    • 제3권1호
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    • pp.13-20
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    • 2021
  • Developmental and epileptic encephalopathies are the most devastating early-onset epilepsies, characterized by early-onset seizures that are often intractable, electroencephalographic abnormalities, developmental delay or regression, and various comorbidities. A large number of underlying genetic variants of developmental and epileptic encephalopathies have been identified over the past few decades. However, the most thorough sequencing studies leave 60-65% of patients without a molecular diagnosis. This review explores the genetic basis of developmental and epileptic encephalopathies that start within the first year of life, including Ohtahara syndrome, early myoclonic encephalopathy, epilepsy of infancy with migrating focal seizures, infantile spasms, and Dravet syndrome. The purpose of this review is to give an overview and encourage the clinicians to start considering genetic testing as an important investigation along with electroencephalogram for better understanding and management of developmental and epileptic encephalopathies.