• Journal of the Korean Child Neurology Society
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• v.26 no.4
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• pp.276-279
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• 2018

Klinefelter syndrome a genetic disorder with various clinical manifestations. Neurological symptoms, such as seizures, are rarely reported with Klinefelter syndrome, and it response well to anti-epileptic drugs. A 5-month-old boy visited the Inha university hospital due to jerking movements and hiccups. The patient had been diagnosed with Klinefelter syndrome at birth and had a medical history of admission to the neonatal intensive care unit due to opisthotonus and ocular deviation at 26 days of age. The patient's serum testosterone level was decreased and his anti-$M{\ddot{u}}llerian$ hormone level was increased. The brain image examination was normal and the electoencephalography and other blood test results showed no specific findings. However, after admission, the patient recurred generalized tonic-clonic-seizures recurred intermittently even after the administration of antiepileptic drugs. This paper reports a case of non-febrile seizures in a child with Klinefelter syndrome who presented with a refractory course.

• Yonsei Medical Journal
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• v.59 no.9
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• pp.1096-1106
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• 2018

Purpose: Alzheimer's disease (AD) is the sixth most common cause of death in the United States. MicroRNAs have been identified as vital players in neurodegenerative diseases, including AD. microRNA-128 (miR-128) has been shown to be dysregulated in AD. This study aimed to explore the roles and molecular mechanisms of miR-128 in AD progression. Materials and Methods: Expression patterns of miR-128 and peroxisome proliferator-activated receptor gamma ($PPAR-{\gamma}$) messenger RNA in clinical samples and cells were measured using RT-qPCR assay. $PPAR-{\gamma}$ protein levels were determined by Western blot assay. Cell viability was determined by MTT assay. Cell apoptotic rate was detected by flow cytometry via double-staining of Annexin V-FITC/PI. Caspase 3 and $NF-{\kappa}B$ activity was determined by a Caspase 3 Activity Assay Kit or $NF-{\kappa}B$ p65 Transcription Factor Assay Kit, respectively. Bioinformatics prediction and luciferase reporter assay were used to investigate interactions between miR-128 and $PPAR-{\gamma}$ 3'UTR. Results: MiR-128 expression was upregulated and $PPAR-{\gamma}$ expression was downregulated in plasma from AD patients and $amyloid-{\beta}$ $(A{\beta})-treated$ primary mouse cortical neurons (MCN) and Neuro2a (N2a) cells. Inhibition of miR-128 decreased $A{\beta}-mediated$ cytotoxicity through inactivation of $NF-{\kappa}B$ in MCN and N2a cells. Moreover, $PPAR-{\gamma}$ was a target of miR-128. $PPAR-{\gamma}$ upregulation attenuated $A{\beta}-mediated$ cytotoxicity by inactivating $NF-{\kappa}B$ in MCN and N2a cells. Furthermore, $PPAR-{\gamma}$ downregulation was able to abolish the effect of anti-miR-128 on cytotoxicity and $NF-{\kappa}B$ activity in MCN and N2a cells. Conclusion: MiR-128 inhibitor decreased $A{\beta}-mediated$ cytotoxicity by upregulating $PPAR-{\gamma}$ via inactivation of $NF-{\kappa}B$ in MCN and N2a cells, providing a new potential target in AD treatment.

• Journal of the Korean Chemical Society
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• v.68 no.3
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• pp.151-159
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• 2024

Indian spices are well known for their numerous health benefits, flavour, taste, and colour. Recent Advancements in chemical technology have led to better extraction and identification of bioactive molecules (phytochemicals) from spices. The therapeutic effects of spices against diabetes, cardiac problems, and various cancers has been well established. The present in silico study aims to investigate the binding affinity of 29 phytochemicals from 11 Indian spices with two prominent proteins, BCL3 and CXCL10 involved in invasiveness and bone metastasis of breast cancer. The three-dimensional structures of 29 phytochemicals were extracted from PubChem database. Protein Data Bank was used to retrieve the 3D structures of BCL3 and CXCL10 proteins. The drug-likeness and other properties of compounds were analysed by ADME and Lipinski rule of five (RO5). All computational simulations were carried out using Autodock 4.0 on Windows platform. The proteins were set to be rigid and compounds were kept free to rotate. In-silico study demonstrated a strong complex formation (positive binding constants and negative binding energy ΔG) between all phytochemicals and target proteins. However, piperine and sesamolin demonstrated high binding constants with BCL3 (50.681 × 10³ mol^-1, 137.76 × 10³ mol^-1) and CXCL10 (98.71 × 10³ mol^-1, 861.7 × 10³ mol^-1), respectively. The potential of these two phytochemicals as a drug candidate was highlighted by their binding energy of -6.5 kcal mol^-1, -7.1 kcal mol^-1 with BCL3 and -6.9 kcal mol^-1, -8.2 kcal mol^-1 with CXCL10, respectively coupled with their favourable drug likeliness and pharmacokinetics properties. These findings underscore the potential of piperine and sesamolin as drug candidates for inhibiting invasiveness and regulating breast cancer metastasis. However, further validation through in vitro and in vivo studies is necessary to confirm the in silico results and evaluate their clinical potential.

• Korean Journal of Radiology
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• v.25 no.3
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• pp.267-276
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• 2024

Objective: To evaluate the diagnostic performance of susceptibility map-weighted imaging (SMwI) taken in different acquisition planes for discriminating patients with neurodegenerative parkinsonism from those without. Materials and Methods: This retrospective, observational, single-institution study enrolled consecutive patients who visited movement disorder clinics and underwent brain MRI and ¹⁸F-FP-CIT PET between September 2021 and December 2021. SMwI images were acquired in both the oblique (perpendicular to the midbrain) and the anterior commissure-posterior commissure (AC-PC) planes. Hyperintensity in the substantia nigra was determined by two neuroradiologists. ¹⁸F-FP-CIT PET was used as the reference standard. Inter-rater agreement was assessed using Cohen;s kappa coefficient. The diagnostic performance of SMwI in the two planes was analyzed separately for the right and left substantia nigra. Multivariable logistic regression analysis with generalized estimating equations was applied to compare the diagnostic performance of the two planes. Results: In total, 194 patients were included, of whom 105 and 103 had positive results on ¹⁸F-FP-CIT PET in the left and right substantia nigra, respectively. Good inter-rater agreement in the oblique (κ = 0.772/0.658 for left/right) and AC-PC planes (0.730/0.741 for left/right) was confirmed. The pooled sensitivities for two readers were 86.4% (178/206, left) and 83.3% (175/210, right) in the oblique plane and 87.4% (180/206, left) and 87.6% (184/210, right) in the AC-PC plane. The pooled specificities for two readers were 83.5% (152/182, left) and 82.0% (146/178, right) in the oblique plane, and 83.5% (152/182, left) and 86.0% (153/178, right) in the AC-PC plane. There were no significant differences in the diagnostic performance between the two planes (P > 0.05). Conclusion: There are no significant difference in the diagnostic performance of SMwI performed in the oblique and AC-PC plane in discriminating patients with parkinsonism from those without. This finding affirms that each institution may choose the imaging plane for SMwI according to their clinical settings.

• Journal of Korean Neurosurgical Society
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• v.30 no.11
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• pp.1300-1307
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• 2001

Purposes : This study reports the possible causes of seizure recurrence in patients underwent previous epilepsy surgery, and surgical strategy for resection of the additional epileptogenic zone locating at the distant area to the site of first resection. Methods : A total of 10 patients with previous surgery due to intractable epilepsy were studied. Five of these underwent standard temporal lobectomy, four extratemporal resection, and one corticoamygdalectomy. Seizure outcome of these were class III-IV. Evaluation methods for reoperation included MRI, 3D-surface rendering of MRI, PET, prologned video-EEG recording with surface electrodes and subdural grid electrodes. Additional resection was done in the frontal lobe in two, in the temporal lobe in three, in the parietal lobe in two, and in the supplementary sensori-motor area in two. Tumor in the superior frontal gyrus in the left hemisphere was removed in one patient. Extent of resection was decided based on the results of ictal subdural grid EEGs and MRI findings. Awake anesthesia and electrocortical stimulation were performed in the two patients for defining the eloquent area. Results : Histopathologic findings revealed extratemporal cortical dysplasia in six, hippocampal sclerosis and cortical dysplasia of the temporal neocortex in one, neuronal gliosis in two, and meningioma in one. Previous pathology of the five patients with cortical dysplasia in the second operation was hippocampal sclerosis plus cortical dysplasia of the temporal neocortex. After reoperation, seizure outcomes were class I in six, class II in three, class III in one at the mean follow-up period of 17.5 months. Characteristically, patients in class II-III after reoperation showed histopathologic findings of hippocampal sclerosis plus temporal neocortical cortical dysplasia plus extratemporal cortical dysplasia. Conclusions : Seizure recurrence after epilepsy surgery was related with the presence of an additional epileptogenic zone distant to the site of first operation, and the majority of the histopathology of the surgical specimens was cortical dysplasia. In particular, hippocampal sclerosis plus temporal neocortical cortical dysplasia was highly related with seizure recurrence in patients with previous operation. In these patients, multimodal evaluation methods were necessary in defining the additional epileptogenic zone.

• Asian Pacific Journal of Cancer Prevention
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• v.15 no.19
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• pp.8383-8390
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• 2014

Background: Although the predictive value of the excision repair cross-complementing group 1 (ERCC1) C118T polymorphism in clinical outcomes of patients with colorectal cancer (CRC) receiving oxaliplatin-based chemotherapy has been evaluated in numerous published studies, the conclusions are conflicting. Therefore, we performed the present meta-analysis to determine the precise role of the ERCC1 C118T polymorphism in this clinical situation and help optimize individual chemotherapy. Materials and Methods: A multiple search strategy was used to identify eligible studies. Pooled odds ratios (ORs) and their 95% confidence intervals (CIs) were used to estimate objective response and oxaliplatin-induced toxicity, with hazard ratios (HRs) with 95%CIs for progression-free survival (PFS) and overall survival (OS). Results: A total of 22 studies including 2,846 CRC patients were eligible in the analysis. Overall, no significant correlation was found between the ERCC1 C118T polymorphism and objective response to oxaliplatin-based chemotherapy, in all patients or in the Asian and Caucasian subgroups. However, the pooled analysis showed that the PFS and OS were significantly shorter in patients who carried T/T or T/C genotypes of ERCC1 C118T as compared to the C/C genotype. On stratified analysis by ethnicity, the ERCC1 118T allele was associated with a favorable prognosis in Caucasians (PFS, HR=0.58, 95%CI: 0.24-1.44; OS, HR=0.38, 95%CI: 0.22-0.64) but an unfavorable prognosis in Asians (PFS, HR=2.49, 95%CI: 1.87-3.33; OS, HR=2.63, 95%CI: 1.87-3.69) based on a dominant model. In addition, we failed to find a statistically significant impact of ERCC1 C118T polymorphism on oxaliplatin-induced toxicity. Conclusions: The ERCC1 C118T polymorphism may have prognostic value in patients with CRC undergoing oxaliplatin-based chemotherapy.

• Sleep Medicine and Psychophysiology
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• v.7 no.2
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• pp.102-108
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• 2000

Objectives: Parkinson's disease (PD) patients may experience fluent mobility upon awakening from a night's sleep, which is called sleep benefit (SB). Although SB is a phenomenon closely associated with sleep, sleep features of PD are not well characterized. The objectives of this study were, first, to investigate if there are any clinical characteristic features between patients with SB and without SB (NSB), and second, to examine if SB patients are associated with any specific sleep variables compared with NSB patients. Methods: Thirty-three PD patients (14 men and 19 women) participated in this study. All subjects were interviewed to examine whether or not they had SB and overnight polysomnography was performed at the sleep center. Various clinical variables were collected through medical record review. Results: The 331 PD patients were divided into 16 SB group (48.5%) and 17 NSB group (51.5%). SB patients were younger (p<0.02), had higher sleep efficiency (p<0.05), and showed shortened sleep latency (p<0.02) as compared with NSB patients. However, no difference was found between SB and NSB with respect to gender, duration or stage of PD, antiparkinsonian medications prescribed, and predominant motor symptoms. SB did not clearly relate to a specific sleep stage and other sleep variables except sleep efficiency and sleep latency. Although primary snoring was more prevalent in SB patients (p<0.05), other sleep disorders were seen with equal frequency in SB and NSB groups. Conclusion: Our results suggest that good sleep efficiency, shortened sleep latency, and age may have an effect on morning motor function (i.e., SB) in Parkinson's disease.

• Annals of Clinical Neurophysiology
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• v.2 no.2
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• pp.70-80
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• 2000

Purpose : This study was performed to evaluate the antistress effects of two aromatic blends being composed of synergic essential oils and also to differentiate its effectiveness between two. Methods : The subject were 20(10 for men, 10 for women) for vital factors and another 20(10 for mem, 10 for women) for serum catecholamine. Vital factors(blood pressure, pulse), electroencephalograpy, psychological tests(SACL, STAI) and serum catecholamine were applied to the subjects. Results : 1. All two aromatic synergic blends revealed no significant differnce of vital factors after inhalation but stable conditions generally by lowering pulse and blood pressure after inhalation. 2. Both blends were significantly valuable in antianxiety and antistress effects statistically. There were no statistically difference between two blends. 3. There were no significant difference in all brain waves after inhalation of two blends but generally stable brain waves were seen in all areas. 4. There were antistress effects of both blends in accordance of decreased serum catecholamines after inhalation of both blends. There were no significantly difference between two blends statistically. Conclusion : Both two aromatic synergic blends reached effective antistress and antianxiety states after inhalation of each blends. There were no significant difference between two blends. Further studies about the effectiveness between the amount of aromatic essential oils and the duration of inhalation should be considered. Also clinical applications of these two aromatic synergic blending oils to develop the aromatic products would be affordable in the future.

• Clinical and Experimental Pediatrics
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• v.46 no.9
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• pp.893-897
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• 2003

Purpose : Benign childhood epilepsy with centrotemporal spikes(BECT) is characterized by an excellent prognosis. Therefore, the necessity for the use of antiepileptic drugs is controversial. The object of this study is to know about the long-term follow-up of BECT, comparing daily treatment with antiepileptic drug(AED) versus no medication. Methods : We retrospectively studied 56 cases of BECTs, examined at Pediatric Neurology Clinic, Korea University Medical Center as reference study time, August 2002. Thirty-eight patients didn't have mediation(group I), while eighteen patients received one or two AED(group II). We evaluated demographics, clinical manifestations at diagnosis, EEG findings and clinical courses in groups I & II. Results : There was no significant differences of sex, age, seizure frequency, seizure type, seizure onset time, family history and EEG findings between group I & II. The reasons to start AED were : parents' anxiety(three cases), frequent seizure(12 cases) and daytime seizure(three cases). There was no evolution of status epilepticus and no difficulty in school performance. Conclusion : AED treatment was of no advantage and long-term prognosis was good regardless of treatment strategy. So it is advisable not to choose AED as the first line of therapy, provided that the patient himself/herself or the family understands the benign nature of BECT.

• Clinical and Experimental Pediatrics
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• v.55 no.2
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• pp.63-67
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• 2012

A 4-year-old girl with Panayiotopoulos syndrome presented with a history of 4 prolonged autonomic seizures. The clinical features of her seizures included, in order of occurrence, blank staring, pallor, vomiting, hemi-clonic movement on the right side, and unresponsiveness. A brain magnetic resonance imaging (MRI) showed a slightly high $T_2$ signal in the left hippocampus. Interictal electoencephalogram revealed spikes in the occipital area of the left hemisphere. We analyzed the current-source distribution of the spikes to examine the relationship between the current source and the high $T_2$ signal. The current source of the occipital spikes was not only distributed in the occipital area of both cerebral hemispheres, but also extended to the posterior temporal area of the left hemisphere. These findings suggest that the left temporal lobe may be one of the hyperexcitable areas and form part of the epileptogenic area in this patient. We hypothesized that the high $T_2$ signal in the left hippocampus of our patient may not have been an incidental lesion, but instead may be related to the underlying electroclinical diagnosis of Panayiotopoulos syndrome, and particularly seizure. This notion is important because an abnormal $T_2$ signal in the hippocampus may represent an acute stage of hippocampal injury, although there is no previous report of hippocampal pathology in Panayiotopoulos syndrome. Therefore, long-term observation and serial follow-up MRIs may be needed to confirm the clinical significance of the $T_2$ signal change in the hippocampus of this patient.