• Journal of Korean Neurosurgical Society
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• v.29 no.7
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• pp.861-867
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• 2000

Purpose : Neuronal migration disorder(NMD) is a major underlying pathology of patients with intractable epilepsy. The role of NMD on seizure susceptibility or epileptogenecity, however, has not been documented. Methods : External irradiation of total amount of 250 cGY was performed to the fetal rats on days 16(E16) and 17(E17) of gestation. After delivery, the rats of 230-260g were decapitated for the histopathologic study. Epileptog-enecity of the NMD was studied by observing electroclinical events after intraperitoneal kainic acid(KA) injection in the control rats and NMD rats. Results : Histopathologic findings revealed focal and/or diffuse cortical dysplasia consisting of dyslamination of the cerebral cortex and appearance of the cytomegalic neurons, neuronal heterotopia in the periventricular white matter, dispersion of the pyramidal layer and the dentate gyrus of the hippocampus, and agenesis of the corpus callosum. Abnormal expression of neurofilaments protein(NF-M/H) was characteristically observed in the dysplastic neurons of the neocortex and hippocampus. Early ictal onset and prolonged ictal activity on EEG and clinical seizures were observed from the NMD rats unlike with the control rats. Conclusions : Exteranl irradiation on the fetal rats produced NMD. And the rats with NMD were highly susceptible to kainic acid provoked seizures. This animal model would be useful to study the pathophysiology of clinically relevant NMDs.

• Journal of Korean Medical Science
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• v.33 no.44
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• pp.279.1-279.16
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• 2018

Magnetic resonance-guided focused ultrasound (MRgFUS) is a new emerging neurosurgical procedure applied in a wide range of clinical fields. It can generate high-intensity energy at the focal zone in deep body areas without requiring incision of soft tissues. Although the effectiveness of the focused ultrasound technique had not been recognized because of the skull being a main barrier in the transmission of acoustic energy, the development of hemispheric distribution of ultrasound transducer phased arrays has solved this issue and enabled the performance of true transcranial procedures. Advanced imaging technologies such as magnetic resonance thermometry could enhance the safety of MRgFUS. The current clinical applications of MRgFUS in neurosurgery involve stereotactic ablative treatments for patients with essential tremor, Parkinson's disease, obsessive-compulsive disorder, major depressive disorder, or neuropathic pain. Other potential treatment candidates being examined in ongoing clinical trials include brain tumors, Alzheimer's disease, and epilepsy, based on MRgFUS abilities of thermal ablation and opening the blood-brain barrier. With the development of ultrasound technology to overcome the limitations, MRgFUS is gradually expanding the therapeutic field for intractable neurological disorders and serving as a trail for a promising future in noninvasive and safe neurosurgical care.

• Clinical and Experimental Pediatrics
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• v.53 no.1
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• pp.106-110
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• 2010

Hippocampal sclerosis (HS) is one of the most common features of intractable temporal lobe epilepsy. Generally it can be identified through brain magnetic resonance imaging (MRI) with high degree of sensitivity and specificity. Typical brain MRI findings of HS are hippocampal atrophy with hyperintense signal confined to the lesion. On the other hand cortical dysplasia exhibits blurring of the gray-white matter junction and abnormal white matter signal intensity. We present a case where preoperative brain MRI strongly suggested the presence of diffuse cortical dysplasia in the left temporal lobe but postoperative pathology revealed the temporal lesion to be unremarkable except for hippocampal sclerosis.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.17 no.1
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• pp.18-23
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• 2017

${\beta}$-ureidopropionase deficiency (${\beta}$-UPD; OMIM # 613161) is a rare autosomal recessive inborn error of pyrimidine metabolism caused by mutations in the UPB1 gene and approximately 30 cases have been reported in the world. The clinical features of patients with ${\beta}$-UPD have been reported to vary from asymptomatic to severe developmental delays. However, the long-term clinical courses of patients with ${\beta}$-UPD have not yet been reported. A Korean girl was diagnosed with ${\beta}$-UPD at the age of 8 years and 10 months by targeted next-generation sequencing which was subsequently confirmed by Sanger sequencing. She had many clinical features such as poor oral feeding, failure to thrive, global developmental delay, microcephaly, frequent infection, and intractable epilepsy. She died suddenly of an unknown cause at the age of 11 years and 5 months. Here we report the long-term (i.e. lifelong) clinical aspects of a Korean patient with ${\beta}$-UPD.

• Toxicological Research
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• v.17
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• pp.11-16
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• 2001

The development of the central nervous system is a continuous process during the embryonic and fetal periods. For a better understanding of congenital anomalies of central nervous system, three major events of normal development, i.e., neurulation (3 to 4 weeks), brain vesicle formation (4 to 7 weeks) and mantle formation (over 8 weeks) should be kept in mind. The first category of anomalies is neural tube defect. Neural tube defects encompass all the anomalies arise in completion of neurulation. The second category of central nervous system anomalies is disorders of brain vesicle formation. This is anomaly that applies for "the face predicts the brain". Holoprosencephaly covers a spectrum of anomalies of intracranial and midfacial development which result from incomplete development and septation of midline structures within the forebrain or prosencephalon. The last category of central nervous system malformation is disorders involving the process of mantle formation. In the human, neurons are generated in two bursts, the first from 8 to 10 weeks and next from 12 to 14 weeks. By 16 weeks, most of the neurons have been generated and have started their migration into the cortex. Mechanism of migration disorders are multifactorial. Abnormal migration into the cortex, abnormal neurons, faulty neural growth within the cortex, unstable pial-glial border, degeneration of neurons, neural death by exogenous factors are some of the proposed mechanism. Agyria-pachygyria are characterized by a four-layerd cortex. Polymicrogyria is gyri that are too numerous and too small, and is morphologically heterogeneous. Cortical dysplasia is characterized by the presence Q[ abnormal neurons and glia arranged abnormally in focal areas of the cerebral cortex. Neuroglial malformative lesions associated with medically intractable epilepsy are hamartia or hamartoma, focal cortical dysplasia and microdysgenesis.ysgenesis.

• Journal of Korean Neurosurgical Society
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• v.42 no.1
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• pp.16-19
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• 2007

Objective : This report describes the clinical study of the surgical method of lateral third infraclavicular implantation of vagal nerve stimulation (VNS) generator through the axillary wrinkle incision. Methods : In a retrospective study, the data for 20 patients with medically intractable epilepsy treated by this approach were examined. The mean age was 31.4 years (range : 14-50), and the mean follow-up period was 12.15 months (range : 4-21 months). The male to female ratio was 2.3 : 1. The subcutaneous pocket for the generator was located in the lateral third infraclavicular area through the axillary wrinkle. Our method was a modification of the standard VNS generator implantation in the mid-infraclavicular pocket through anterior axillary incision. Results : There were the excellent or good cosmetic satisfaction in 95% of the cases and fair in 5%. The generator was located outside the lung field in 15%, periphery of the lung field in 45%, and crossed over the lung field in 40%. Discomfort from shoulder motion occurred transiently in 35% of cases. Other complications were minimal. Conclusion : These results demonstrate that the lateral third infraclavicular apporach will offers cosmetic benefits and reduction of obscuration of the lung field without serious complications. Thus, this technique provides an attractive alternative among the surgical techniques for the vagal nerve stimulation.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.13 no.1
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• pp.28-32
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• 2017

Lennox-Gastaut syndrome (LGS) is a type of childhood epilepsy, and this syndrome can compromise dental treatment. LGS represents variable etiologies, multiple types of intractable seizures, and cognitive impairment in most patients. A 7-year-4-month old girl with LGS was referred to our hospital for dental treatment under general anesthesia. Clinically, the patient presented with multiple dental caries. The patient had been prescribed a ketogenic diet for the management of repetitive seizures. In addition, she experienced severe types of hypoxia during medical examination using chloral hydrate. Therefore, dental treatment was performed under general anesthesia. LGS patients have a high risk of seizure during dental treatment and can induce a lot of complications. Dental treatment under general anesthesia for LGS patients can be carried out careful preoperative evaluation and in cooperation with medical care.

• Clinical and Experimental Pediatrics
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• v.48 no.11
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• pp.1225-1231
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• 2005

Purpose : This study compares the first epileptic seizures between preterm and term-born children with periventricular leukomalacia and epilepsy. Methods : From 108 cases having lesions of high signal intensity around the ventricles in T2 weighted imaging of a brain magnetic resonance study, we selected 37 cases that showed epileptic seizures two times or more and divided them into the group of preterm-born(27 cases) and term-born children(10 cases). A retrospective study was made by comparing the two groups with regard to age, type of the first epileptic seizures, EEG findings and responsiveness to anticonvulsants. Results : The age of the first epileptic seizure was $22.2{\pm}18.3$ months in the preterm-born group and $26.9{\pm}21.1$ months in the term-born group(P=0.505). As for the first epileptic seizure, 11 out of the 27 cases in the preterm-born group had infantile spasms. Out of the 10 cases in the term-born group, 7 had complex partial seizures. In the preterm group, hypsarrhythmias were found in 11 cases, focal epileptiform discharges in 6 cases. In term-born group, focal epileptiform discharges were found in 5 cases but no epileptiform discharge was found in 3 cases. Intractable epilepsies were diagnosed in 6 cases and all of them belonged to the preterm-born group. Conclusion : More severe epilepsies such as infantile spasm and intractable epilepsies seem to be more common in preterm-born epileptic children with PVL as well as more severely abnormal EEG finding compared to term-born epileptic children.

• Clinical and Experimental Pediatrics
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• v.52 no.4
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• pp.458-463
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• 2009

Purpose : Schizencephaly is a uncommon congenital brain anomaly characterized by congenital clefts spanning the cerebral hemispheres from pial surface to lateral ventricles and lined by gray matter. In this study, we investigated the clinical manifestation and radiologic findings of pediatric schizencephaly. Methods : The data of 13 patients who were diagnosed with schizencephaly in Severance Childrens Hospital and Yongdong Severance Hospital from January 2005 to December 2007 were analyzed retrospectively. Results : The mean age at diagnosis was $9.08{\pm}2.67$ months old and ranged from 1 to 30 months. The ratio of male to female patients was 3.33:1. Five (38.5%) patients had bilateral clefts, while 8 (61.5%) had unilateral clefts. Five (38.5%) patients had closed lip clefts, and 4 (30.8%) had opened lip clefts. Four (30.8%) patients had multiple clefts. Associated anomalies showed in all cases. The clinical features consisted of mild unilateral weakness in 7 (53.8%) cases and a hemiparesis was present in 3 (23.1%) patients. A tetraparesis was in 3 (23.1%) patients. There was no difference in motor deficit between unilateral and bilateral clefts. Delayed development was observed in all cases. Epilepsy was present in 7 (53.8%) patients, 5 patients with unilateral clefts and 2 patients with bilateral clefts. Three (42.8%) patients showed intractable seizures. Conclusion : Schizencephaly showed variable clinical manifestations and radiologic findings in association with the types and locations of the clefts. It is necessary to diagnose schizencephaly early and to detect the development of epilepsy. Intensive and large studies of the correlation of clinical outcomes and radiologic findings should be continued for more effective treatment.

• Annals of Clinical Neurophysiology
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• v.1 no.2
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• pp.112-117
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• 1999

Background : Posterior tibial nerve somatosensory evoked potentials (PTSEP) have cortical potentials on primary sensory area of foot around 40 msec. The direct cortical recordings of the cortical potentials shows high voltage positive wave on medial hemisphere, especially on paracentral lobule (PCL). However, it is so difficult to record the potential directly on PCL that the cortical potential of PTSEP is not well understood. We investigated the cortical potential of PTSEP on subdural electrodes. Methods : We recorded cortical potentials to posterior tibial nerve stimulation on subdural electrodes which were on medial hemisphere near PCL in 15 intractable neocortical epilepsy patients. The numbers of subdural electrodes were 8 in 10 subjects ($1{\times}8array$) and 16 in 5 subjects ($2{\times}8arrays$). Seven subjects had three-dimensional imaging fusion (3D-fusion) of MRI and the electrodes using Analyze program. We investigated the amplitude, latency, polarity, and phase of the waves regarding location. Results : The waves had maximal amplitude on PCL in 4 subjects, precuneus in 1, cingulate gyrus nearest to PCL in 2 among 7 subjects with 3D-fusion. Also the electrodes were located on posterior area of PCL (2 out of 2 subjects with more than two electrodes put on PCL in 3D-fusion) and superior area of it (5 out of 5 subjects with $2{\times}8arrays $). All the high (more than 20 uV) amplitude around 40msec had positive polarity in 7 subjects. The phase reversals were detected between the electrodes with the highest amplitude and the just posterior (2 subjects) or anterior (6 subjects) located electrodes. The just posterior located electrodes had sharper phase reversal than the anterior one. Conclusion : PTSEP might have maximal amplitude of cortical potentials on the more superior and posterior area of PCL. The highest amplitude potential has positivity. The wave with maximal amplitude could have phase reversal of cortical potentials with surrounding electrodes, especially shaper with posterior part than with anterior one.