• Journal of Korean Neurosurgical Society
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• 제58권1호
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• pp.22-29
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• 2015

Objective : Perinatal hypoxic-ischemic encephalopathy (HIE) and prolonged febrile seizures (pFS) are common neurologic problems that occur during childhood. However, there is insufficient evidence from experimental studies to conclude that pFS directly induces hippocampal injury. We studied cognitive function and histological changes in a rat model and investigated which among pFS, HIE, or a dual pathologic effect is most detrimental to the health of children. Methods : A rat model of HIE at postnatal day (PD) 7 and a pFS model at PD10 were used. Behavioral and cognitive functions were investigated by means of weekly open field tests from postnatal week (PW) 3 to PW7, and by daily testing with the Morris water maze test at PW8. Pathological changes in the hippocampus were observed in the control, pFS, HIE, and HIE+pFS groups at PW9. Results : The HIE priming group showed a seizure-prone state. The Morris water maze test revealed a decline in cognitive function in the HIE and HIE+pFS groups compared with the pFS and control groups. Additionally, the HIE and HIE+pFS groups showed significant hippocampal neuronal damage, astrogliosis, and volume loss, after maturation. The pFS alone induced minimal hippocampal neuronal damage without astrogliosis or volume loss. Conclusion : Our findings suggest that pFS alone causes no considerable memory or behavioral impairment, or cellular change. In contrast, HIE results in lasting memory impairment and neuronal damage, gliosis, and tissue loss. These findings may contribute to the understanding of the developing brain concerning conditions caused by HIE or pFS.

• 정보보호학회논문지
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• 제26권1호
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• pp.135-152
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• 2016

모바일 포렌식은 스마트폰의 대중화와 다양한 모바일 기기의 증가로 인해 그 중요성 및 필요성이 급격히 증가하고 있다. 하지만 그 방안 및 절차는 아직 모바일 포렌식의 특성에 충분히 맞게 적용되고 있지 않다. 이에 따라 본 논문에서는 현재 모바일 포렌식이 직면한 문제점을 파악하기 위해 법 제도 기술적 관점에서의 분석을 수행하였으며 이를 통해 모바일 기기에 대해서는 현재 디지털 포렌식 수사과정에서 큰 이슈가 되고 있는 선별압수에 있어서 제약사항이 있음을 확인하였다. 또한 모바일 포렌식에서 디지털 증거 수집 방안에 대한 분석 및 실사용 도구의 무결성 연구를 진행함으로써 현재 기술의 적합성 검증 및 추후 발생될 문제점에 대해 분석하였으며 결과적으로 모바일 포렌식에서 수집된 데이터가 증거능력을 확보할 수 있는 방안을 위해 전반적인 고려사항을 제시하였다.

• 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.

• BMB Reports
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• 제39권4호
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• pp.400-405
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• 2006

A 16-month old boy was referred to our hospital for evaluation of recurrent generalized tonic clonic seizures. Metabolic evaluation revealed significant hyperammonemia ($1,112\;{\mu}g/dl$). Amino acid/acylcarnitine screening using tandem mass spectrometry showed markedly increased plasma levels of citrulline ($1,350\;{\mu}M/l$) with undetectable levels of arginine and arginosuccinic acid. Urinary excretion of citrulline was markedly increased ($38,617\;{\mu}M/g$ creatinine). Brain MRI findings showed diffuse high-signal intensity lesions, that involved gray and white matter in both frontal lobes and insula with edematous changes; these findings were consistent with the acute stage of citrullinemia (CTLN). Mutation analysis of the argininosuccinate synthetase (ASS) gene, in this patient, showed a Gly324Ser mutation in exon 13, and a 67-bp duplication mutation in exon 15 (c.1128-6_1188dup67). The patient was confirmed as having late-onset CTLN1 and treated with anticonvulsants, lactulose enema, protein restricted diet and arginine. Here we describe a case of late-onset CTLN1 in a patient by biochemical analyses and ASS gene mutation confirmation. This is the first report of a Korean patient with late-onset CTLN1 confirmed by ASS gene mutation identification.

• 한국발생생물학회지:발생과생식
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• 제13권2호
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• pp.63-77
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• 2009

신경리포푸신증(NCLs)은 신경퇴행 축적 질환으로 뇌에 자기형광물질을 포함한 다양한 물질의 축적이 야기되어 발생하며, 노던에필렙시를 제외한 모든 신경포푸신증은 리소즘 축적 질환으로 분류된다. 이러한 신경리포푸신증은 전세계적으로 12,500명 중 1명에게 발생되는 높은 발병 빈도를 나타내며, 그 발병 시기에 따라 영아형, 영유아형, 유년형, 그리고 성인형과 같이 분류된다. 신경리포푸신증이 유발하는 의학적 증상로는 시각 손실, 발작, 간질, 진행성 정신지체등을 야기하여 소아성 치매라는 이야기를 들으며, 증상이 심할 경우 환자가 사망에 이르게 된다. 신경퇴행성 리포푸신증의 원인은 유전자의 돌연변이 때문이라고 알려져 있으며, 일부의 연구를 통해 태아의 발생과정 상 문제를 통해 질병이 야기되는 경우도 관찰이 되고 있으나, 아직 그 분자 발생학적 기전이 명확하게 규명되어 있지 않은 현실이다. 현재 전 세계적으로 많은 연구가 수행되고 있어 그 결과가 주목되는 바이다.

• The Korean Journal of Physiology and Pharmacology
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• 제13권4호
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• pp.265-271
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• 2009

Nitric oxide (NO) has both neuroprotective and neurotoxic effects depending on its concentration and the experimental model. We tested the effects of NG-nitro-L-arginine methyl ester (L-NAME), a nonselective nitric oxide synthase (NOS) inhibitor, and aminoguanidine, a selective inducible NOS (iNOS) inhibitor, on kainic acid (KA)-induced seizures and hippocampal CA3 neuronal death. L-NAME (50 mg/kg, i.p.) and/or aminoguanidine (200 mg/kg, i.p.) were administered 1 h prior to the intracerebroventricular (i.c.v.) injection of KA. Pretreatment with L-NAME significantly increased KA-induced CA3 neuronal death, iNOS expression, and activation of microglia. However, pretreatment with aminoguanidine significantly suppressed both the KA-induced and L-NAME-aggravated hippocampal CA3 neuronal death with concomitant decreases in iNOS expression and microglial activation. The protective effect of aminoguanidine was maintained for up to 2 weeks. Furthermore, iNOS knockout mice ($iNOS^{-1-}$) were resistant to KA-induced neuronal death. The present study demonstrates that aminoguanidine attenuates KA-induced neuronal death, whereas L-NAME aggravates neuronal death, in the CA3 region of the hippocampus, suggesting that NOS isoforms play different roles in KA-induced excitotoxicity.

• Clinical and Experimental Pediatrics
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• 제58권2호
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• pp.69-72
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• 2015

Insulinoma, which arises from insulin-producing pancreatic beta cells, is a rare tumor in children. Only 5%-10% of insulinomas are malignant and undergo metastasis. We report a case of an 11-year-old girl who experienced hypoglycemia-related seizures induced by an insulinoma; after resection of the primary tumor, she developed hepatic focal nodular hyperplasia (FNH). Laboratory test results indicated marked hypoglycemia with hyperinsulinemia. Abdominal ultrasonography (US) and computed tomography results were normal; however, magnetic resonance imaging (MRI) showed a solid mass in the pancreatic tail. Therefore, laparoscopic distal pancreatectomy was performed. Two months after the surgery, an abdominal MRI revealed multiple nodular lesions in the liver. An US-guided liver biopsy was then performed, and histological examination revealed FNH without necrosis or mitotic activity. The patient has been free of hypoglycemia for 2 years, and recent MRI studies showed a decrease in the size of FNH lesions, without any evidence of metastasis. Even though no metastatic lesions are noted on imaging, close observation and follow-up imaging studies are required in a child with insulinoma that has malignant potential on histopathologic findings.

• The Korean Journal of Pain
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• 제31권4호
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• pp.296-304
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• 2018

Epiduroscopy is defined as a percutaneous, minimally invasive endoscopic investigation of the epidural space. Periduroscopy is currently used mainly as a diagnostic tool to directly visualize epidural adhesions in patients with failed back surgery syndrome (FBSS), and as a therapeutic action in patients with low back pain by accurately administering drugs, releasing inflammation, washing the epidural space, and mechanically releasing the scars displayed. Considering epiduroscopy a minimally invasive technique should not lead to underestimating its potential complications. The purpose of this review is to summarize and explain the mechanisms of the side effects strictly related to the technique itself, leaving aside complications considered typical for any kind of extradural procedure (e.g. adverse reactions due to the administration of drugs or bleeding) and not fitting the usual concept of epiduroscopy for which the data on its real usefulness are still lacking. The most frequent complications and side effects of epiduroscopy can be summarized as non-persistent post-procedural low back and/or leg discomfort/pain, transient neurological symptoms (headache, hearing impairment, paresthesia), dural puncture with or without post dural puncture headache (PDPH), post-procedural visual impairment with retinal hemorrhage, encephalopathy resulting in rhabdomyolysis due to a dural tear, intradural cyst, as well as neurogenic bladder and seizures. We also report for first time, to our knowledge, a case of symptomatic pneumocephalus after epiduroscopy, and try to explain the reason for this event and the precautions to avoid this complication.

• Journal of Korean Neurosurgical Society
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• 제58권5호
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• pp.462-466
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• 2015

Objective : Post-craniotomy seizure (PCS) is reported only rarely. However, our department noted a 433% increase in PCS for a year beginning September 2010, especially after cerebrovascular surgery. Our goal was to identify the cause of our unusual outbreak of PCS. Methods : For almost one year after September 2010, cases of PCS increased significantly in our department. We analyzed 973 patients who had received a major craniotomy between January 2009 and November 2011. We included seizures that occurred only in the first 24 postoperative hours, which we defined as early PCS. After verifying the presence of PCS, we analyzed multiple seizure-provoking factors and their relation to the duration and character of seizure activity. Results : Overall PCS incidence was 7.2% (70/973). Cefazolin (2 g/L saline) was the antibiotic drug used for intraoperative irrigation in 88.4% of the operations, and no PCS occurred without intraoperative cefazolin irrigation. When analyzed by operation type, clipping surgery for unruptured aneurysms was the most frequently associated with PCS (80%). Using logistic regression, only 2 g cefazolin intraoperative irrigation (p=0.024) and unruptured aneurysm clipping surgery (p<0.001) were associated with early PCS. The seizure rate of unruptured aneurysm clipping surgery using 2 g cefazolin intraoperative irrigation was 32.9%. Conclusion : Intraoperative cefazolin irrigation must be avoided in patients undergoing craniotomy, especially for clipping of unruptured aneurysms, because of the increased risk of early PCS.

• Clinical and Experimental Pediatrics
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• 제45권4호
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• pp.540-544
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• 2002

저자들은 임상적으로 Rett 증후군이 의심된 2세 환아에서 MECP2 유전자의 직접 염기 서열 분석을 통해 P152R 돌연변이를 확인하였다. 이에 대한 문헌 고찰과 함께 보고하는 바이다.