• Clinical and Experimental Pediatrics
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• v.63 no.8
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• pp.291-300
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• 2020

Advances in autoimmune encephalitis studies in the past 10 years have led to the identification of new syndromes and biomarkers that have transformed the diagnostic approach to the disorder. The disorder or syndrome has been linked to a wide variety of pathologic processes associated with the neuron-specific autoantibodies targeting intracellular and plasma membrane antigens. However, current criteria for autoimmune encephalitis are quite dependent on antibody testing and responses to immunotherapy, which might delay the diagnosis. This form of encephalitis can involve the multifaceted presentation of seizures and unexpected behavioral changes. The spectrum of neuropsychiatric symptoms in children is less definitive than that in adults, and the incorporation of clinical, immunological, electrophysiological, and neuroradiological results is critical to the diagnostic approach. In this review, we document the clinical and immunologic characteristics of autoimmune encephalitis known to date, with the goal of helping clinicians in differential diagnosis and to provide prompt and effective treatment.

• Biomedical Science Letters
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• v.18 no.4
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• pp.428-434
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• 2012

Epilepsy is a chronic neurological disease showing a symptom of repeated seizures without any other physical disorders. Among the diagnostic examination for epilepsy, the electroencephalogram (EEG) has been known as an important test. This study aimed to investigate the EEG with photic stimulation in the pediatric epilepsy patients. They underwent digital sleep and waking EEGs or waking EEGs with photic stimulation. Epilepsy type, seizure history, and season of occurring seizure were analyzed. Epilepsy patients showed more response during the period of photic-on and eye close at the frequency of 10~20 Hz during the EEG activation procedure. Photoparoxysmal response (PPR) was shown in 206 patients out of total 1,551 epilepsy patients. PPR was appeared more frequently during summer and winter seasons, and especially in the patients who had a history of seizure. During the PPR, EEG pattern showed spike (77.18%), theta (9.71%), and spike + theta (13.11%). On the other hand, beta and theta waves were not significantly changed by photic stimulation. However, alpha wave was decreased and delta wave was increased by photic stimulation (P<0.05). These changes may be due to temporarily altered electrophysiological function of the epileptic patient's brain by the photic stimulation. There was no difference in the EEG pattern between the left and right side in the brain. In conclusion, condition of photic-on with closed eyes and frequency of 10~20 Hz during the procedure of EEG activation could be appropriate for obtaining a definite photoparoxysmal response in the electroencephalogram of the pediatric epilepsy patients.

• The Journal of Pediatrics of Korean Medicine
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• v.20 no.1
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• pp.195-206
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• 2006

Objective : The purpose of this study is to investigate the current trends of children patients with convulsion. Children convulsion patients who visited the Pediatric Department of the Oriental Medicine hospital were investigated and analyzed. Method : 90 children complaining convulsion who visited the Department of Pediatrics of Kyung Hee University Oriental Medicine Hospital, between March, 2005 and October 2005 were statistically analyzed. Results : The percentage of boys outnumbered the girls. The percentage of children under the age of 5 18 66.7%. The percentage of children with family history is 43%. The majority of children had visited the hospital twice (n=21). As for intercurrent diseases, there were URI, C.P., ADHD, CHD, sequela of encephalitis and etc. As for the result of EEG examinations, 25 children were normal and 18 were abnormal. As for the appearance of seizures, Tonic-Clonic seizure was the most common seizure among the children (n=13). As for the type of convulsion, simple febrile convulsion was the most common (n=35). As for the curative effect. the percentage of children who showed improvement to treatment was 41.2%. Jangdambosintang, sunbangpaedoktang, gamichodeungeunm, gamichungeumchowitang and etc were the most frequently prescribed medicines.

• Molecules and Cells
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• v.41 no.10
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• pp.881-888
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• 2018

During the cortical development, cells in the brain acquire somatic mutations that can be implicated in various neurodevelopmental disorders. There is increasing evidence that brain somatic mutations lead to sporadic form of epileptic disorders with previously unknown etiology. In particular, malformation of cortical developments (MCD), ganglioglioma (GG) associated with intractable epilepsy and non-lesional focal epilepsy (NLFE) are known to be attributable to brain somatic mutations in mTOR pathway genes and others. In order to identify such somatic mutations presenting as low-level in epileptic brain tissues, the mutated cells should be enriched and sequenced with high-depth coverage. Nevertheless, there are a lot of technical limitations to accurately detect low-level of somatic mutations. Also, it is important to validate whether identified somatic mutations are truly causative for epileptic seizures or not. Furthermore, it will be necessary to understand the molecular mechanism of how brain somatic mutations disturb neuronal circuitry since epilepsy is a typical example of neural network disorder. In this review, we overview current genetic techniques and experimental tools in neuroscience that can address the existence and significance of brain somatic mutations in epileptic disorders as well as their effect on neuronal circuitry.

• Journal of Veterinary Clinics
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• v.33 no.1
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• pp.70-73
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• 2016

Necrotizing meningoencephalitis (NME) is a unique idiopathic nonsuppurative inflammatory disease of central nervous system in small-sized breed dogs. A 9-year-old intact male Shih-Tzu dog with anorexia, vomiting, salivation and intermittent seizures was submitted to the Jeju National University for diagnosis. Grossly, there were no obvious lesions in the brain, except dilatation of most blood vessels in meninges. Histopathologically, brain revealed severe multifocal nonsuppurative inflammation in perivascular area of meninges and cerebral cortex. Some areas of cerebral parenchyma were replaced with lots of macrophages contained periodic acid-Schiff positive materials. Many new-formed blood vessels were observed around the necrotic regions using Gomori reticulum stain. Immunohistochemistry and reverse transcription-polymerase chain reaction were negative for toxoplasmosis and canine distemper virus. Based on the gross, histopathologic features and antigen detection methods, this case was diagnosed as NME. Here we reported the NME in relatively uncommon breed, Shih-Tzu dog, than other small breed dogs.

• Biomolecules & Therapeutics
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• v.5 no.4
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• pp.402-411
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• 1997

ln order to investigate pharmacological properties of New Woohwangchungsimwon Liquid (NCL) and Woohwangchungsimwon Liquid (CL), effects of NCL and CL on cerebral ischemia and central nervous system were compared. Cerebral ischemia insult was performed using unilateral carotid artery occlusion in Mongolian gerbils. The histological observations showed a preventive effect of NCL and CL treatments with ischemia-induced brain damage. The ATP in brain tissue was decreased in vehicle-treated ischemic gerbils. This decrease was prevented by CL treatment. In contrast to what was seen with ATP, the lactate and lipid peroxide were both elevated in vehicle-treated ischemic gerbils. This elevation was inhibited by NCL and CL treatments. While NCL and CL had no effects on the hexobarbital-induced sleeping time, they prevented the seizures induced by electric shock and pentetrazol. NCL and CL showed sedative effect in rotarod and spontaneous activity test. Respiration rate and depth were increased at the high dose of NCL and CL. Furthermore, NCL and CL showed anti-stress effect. Our findings suggest that the pharmacological profile of NCL on cerebral ischemia and central nervous system are similar to that of CL.

• Neonatal Medicine
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• v.17 no.1
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• pp.132-135
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• 2010

Although the majority of abnormal bleeding during the neonatal period results from acquired coagulation disorders, inherited coagulation disorders can also manifest at this time. Hemophilia is the most common of inherited coagulation disorder. Although 40-70% of cases with hemophilia are diagnosed in the neonatal period, few cases have been reported in premature infants. We report a case of a premature infant born at 31 weeks of gestation, diagnosed with hemophilia A by blood coagulation test, coagulation factor assay and study of the F8 gene. The baby was treated with recombinant factor VIII (Recombinate$^{(R)}$, USA) because of repeated seizures and intramuscular hematoma.

• Korean Journal of Clinical Laboratory Science
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• v.39 no.1
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• pp.56-62
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• 2007

Lennox-Gastaut syndrome(LGS) is a severe age-specific epilepsy syndrome that causes medication-resistant seizures in childhood. Vagal nerve stimulation (VNS) has been proposed as a possible way to improve the treatment of refractory epilepsy. We treated 9 patients with Lennox-Gastaut syndrome between the ages of 2 and 11 years (mean 5.8 years), by using the vagus nerve stimulation. The mean follow-up duration was 35 months. The mean reduction of seizure frequency compared with baseline before VNS was 52% after 6 months (range, 0% to 89%; P<0.011), and 58% after 1 year (range 0% to 89%; P<0.012). Seven patients showed improvements of quality of life (QOL) such as alertness, mood, and language skills. The most common side effects were transient hoarseness (6 patients) and drooling (1 patient). Our results suggest that the vagus nerve stimulation could be an effective and safe adjunct therapy for the treatment of Lennox-Gastaut syndrome.

• Proceedings of the Korean Society for Emotion and Sensibility Conference
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• 2008.10a
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• pp.147-150
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• 2008

Vitamin C ascorbic acid (AA) and dehydroascorbic acid (DHA) as an antioxidant have been shown to have protective effects in experimental neurological disorder models such as stroke, ischemia, and epileptic seizures. The present study was conducted to examine the protective effect of AA and DHA on Kainic acid (KA) neurotoxicity using organotypic hippocampal slice cultures (OHSC). After 12h KA treatment, significant delayed neuronal death was detected in CA3 region, but not in CA1. Intermediate dose of AA and DHA pretreatment significantly prevented cell death and inhibit ROS level, mitochondrial dysfunction and capase-3 activation in CA3 region. In the case of low or high dose, however, AA or DHA pretreatment were not effective. These data suggest that both AA and DHA pretreatment have neuroprotective effects on KA-induced neuronal injury depending on the concentration, by means of inhibition of ROS generation, mitochondrial dysfunction, and caspase-dependent apoptotic pathway.

• Journal of Korean Neurosurgical Society
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• v.46 no.3
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• pp.252-256
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• 2009

Meningioangiomatosis (MA) is a rare congenital tumor that occurs mostly in 5-15 year old children. There have been only 5 cases previously reported that described the cystic nature within these tumors. We present a case of a MA accompanied by a separate macrocyst. A normally developed 2 year-old female patient presented with partial and generalized seizures. The brain computerized tomogram and magnetic resonance imaging revealed the presence of a calcified mass accompanied by a cyst in the right parietal area, surrounded by low density and high attenuation edema and hemorrhage. Upon right parietal craniotomy, a $1.6cm{\times}1.2cm{\times}0.5cm$ sized plate-like, gray-white, slightly hard mass was seen and it was completely excised. Approximately 1 cm from the mass in the anterior lateral direction, a cyst was found and subsequent biopsy of the cyst wall revealed no tumor tissue, and therefore the cyst was not removed. Pathologic report demonstrated the meningioangiomatosis. Follow up examination 2 years later showed no recurrence of the tumor, and there was no evidence of neurological deficits. Authors suggest that cysts that arise in the surrounding tissues of tumors may not be tumor cysts, and do not require surgical removal.