• 한국수정란이식학회지
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• 제29권3호
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• pp.249-255
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• 2014

Programmed cell death or apoptosis is associated with changes in $K^+$ concentration in many cell types. Recent studies have demonstrated that two-pore domain $K^+$ ($K_{2P}$) channels are involved in mouse embryonic development and apoptotic volume decrease of mammalian cells. In cerebellar granule neurons that normally undergo apoptosis during the early developmental stage, TASK-1 and TASK-3, members of $K_{2P}$ channels, were found to be critical for cell death. This study was performed to identify the role of $K^+$ channels in the $H_2O_2$-induced or cryo-induced cell death of mouse and bovine embryos. Mouse and bovine two-cell stage embryos (2-cells) exposed to $H_2O_2$ for 4 h suffered from apoptosis. The 2-cells showed positive TUNEL staining. Treatment with high concentration of KCl (25mM) inhibited $H_2O_2$-induced apoptosis of 2-cells by 19%. Cryo-induced death in bovine blastocysts showed positive TUNEL staining only in the cells near the plasma membrane. Cryoprotectant supplemented with 25 mM KCl reduced apoptosis slightly compared to cryoprotectant supplemented with 5 mM KCl. However, the combination of antioxidants (${\beta}$-mercaptoethanol) with 25 mM KCl significantly decreased the rate of $H_2O_2$-induced and cryo-induced apoptosis compared to treatments with only antioxidants or 25 mM KCl. These results show that blockage of $K^+$ channel efflux for a short-time reduces $H_2O_2$- and cryo-induced apoptosis in mouse and bovine embryos. Our findings suggest that apoptosis in mouse and bovine embryos might be controlled by modulation of $K^+$ channels which are highly expressed in a given cell type.

• Journal of Genetic Medicine
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• 제7권2호
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• pp.145-150
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• 2010

선단이골증(acrodysostosis)은 매우 드문 골격계 이형성 질환의 하나로, 말단골 이형성을 보이는 짧은 손가락과 발가락, 코뼈의 저형성 및 정신지체를 그 특징으로 한다. 본 증례에서 저자들은 전형적인 임상 양상을 보이면서 신경 증상 및 징후와 경추관 협착을 동반한 선단이골증 16세 남아를 경험하여 보고 하고자 한다. 환아는 양쪽 상지의 간헐적인 통증과 위약감을 호소하였으며 양팔을 높이 들어올리는 것이 불가능함을 주소로 내원하였다. 신체 검진상 저신장을 보였으며 넓으면서도 낮은 콧등, 작고 위로 들려 올라간 코끝, 양쪽 눈의 안쪽 눈구석 주름 및 경한 양안격리증 등 특징적 안면 소견을 보였다. 신경심리검사상 중등도의 정신지체가 확인되었으며 청력검사상 양측의 신경성 난청 소견이 동반되어 있었다. 방사선학적 검사에서는, 원뿔 모양의 골단을 보이는 넓으면서도 짧은 중수골 및 지골, 양측 전와부의 마델룽 변형(Madelung deformity), 과형성된 첫 번째 중족골과 두꺼워진 두개골이 확인되었다. 뇌 및 척추 자기공명영상검사에서는 경추관 협착, 경추-연수 접합부의 압박을 동반한 편평두개저 및 소뇌 편도의 하방 이동 소견을 보였다. 선단이골증에서 동반되는 경추관 협착증은 시간이 지남에 따라 진행하는 경향을 보이므로, 6개월 후 추적 영상검사에서 진행되는 소견을 보이거나 증상이나 징후가 악화될 경우에는 수술적 갑압술을 실시할 계획이다. 본 증례는 선단이골증의 국내 보고로는 두 번째이나, 경추관 협착증의 증상 및 징후를 동반한 선단이골증의 첫 번째 보고이다. 선단이골증에서의 척추관 협착증은 시간이 지남에 따라 진행하는 증상이며 심각한 합병증이 초래될 수 있기 때문에, 특징적 임상 양상을 보이는 환자를 조기에 진단할 수 있다면 이러한 합병증에 대한 적절한 치료로서 치명적인 신경학적 후유증의 발생을 피할 수 있다. 따라서, 말단골의 이형성을 보이는 환자의 중요한 감별 질환 중 하나로 선단이골증은 반드시 고려되어야 할 것이다.

• Journal of Korean Neurosurgical Society
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• 제30권2호
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• pp.207-210
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• 2001

Objective : The prognosis of spontaneous intracerebral hemorrhage often depends on initial neurologic condition, size and location of hemorrhage and associated intraventricular hemorrhage. However, age of patient, coagulation state and other associated vascular diseases may also play a role when present. In recent years, the geriatric population has been increasing. The age distribution of the patients with intracerebral hemorrhages also has been increased, accordingly. However, such patients, especially when associated with poor initial conditions often tend to be managed rather conservatively. The authors analyzed retrospectively on forty-five patients with spontaneous intracerebral hemorrhage over the seventies with poor initial condition to find out whether there exists a difference of outcome between surgery and non-surgery group. Material and Method : A total of 45 patients over seventies with spontaneous intracerebral hemorrhage with Glasgow Coma Scale(GCS) 4-8 treated over last six years were included. The validity of surgical management for these patients as well as clinical variables which might have been operated on the outcome of these patients were evaluated. The Glasgow Outcome Scale(GOS) after three months was used for comparison of outcome. Results : In surgical group(19 cases), mean age was 74.5 years old, mean hematoma volume 67.2ml and mean GCS score 5.7 points. In nonsurgical group(26 cases), mean age was 79.3 years old, mean hematoma volume 32.1ml, and mean GCS score 6.8 points. Mortality rate in surgical group was 47.4%(9 patients), including 2 cases of post-operative rebleeding, while that in nonsurgical group was 46.2%. However, when patients with initial GCS 4-6 points and over 30ml in hematoma volume were regrouped, mortality rate in surgical group was 46.2%, whereas mortality rate in nonsurgical group was 66.7%. Conclusion : It is concluded that the mortality rate is much low in surgery group with initial GCS less than 6 points and hematoma volume over 30cc. There was no significant difference of outcome in patients with basal ganglia and thalamic hemorrhage. However, surgical treatment lowered the mortality and morbidity rate in patients with subcortical and cerebellar hemorrhage.

• Experimental and Molecular Medicine
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• 제50권11호
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• pp.8.1-8.14
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• 2018

We previously demonstrated that the direct transplantation of human umbilical cord blood-derived mesenchymal stem cells (hUCB-MSCs) into the dentate gyrus ameliorated the neurological symptoms of Niemann-Pick type C1 (NPC1)-mutant mice. However, the clinical presentation of NPC1-mutant mice was not fully understood with a molecular mechanism. Here, we found 14,15-epoxyeicosatrienoic acid (14,15-EET), a cytochrome P450 (CYP) metabolite, from hUCB-MSCs and the cerebella of NPC1-mutant mice and investigated the functional consequence of this metabolite. Our screening of the CYP2J family indicated a dysregulation in the CYP system in a cerebellar-specific manner. Moreover, in Purkinje cells, CYP2J6 showed an elevated expression level compared to that of astrocytes, granule cells, and microglia. In this regard, we found that one CYP metabolite, 14,15-EET, acts as a key mediator in ameliorating cholesterol accumulation. In confirming this hypothesis, 14,15-EET treatment reduced the accumulation of cholesterol in human NPC1 patient-derived fibroblasts in vitro by suppressing cholesterol synthesis and ameliorating the impaired autophagic flux. We show that the reduced activity within the CYP system in the cerebellum could cause the neurological symptoms of NPC1 patients, as 14,15-EET treatment significantly rescued cholesterol accumulation and impaired autophagy. We also provide evidence that the intranasal administration of hUCB-MSCs is a highly promising alternative to traumatic surgical transplantation for NPC1 patients.

• 대한안과학회지
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• 제59권11호
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• pp.1097-1102
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• 2018

목적: 클로르페나피르 음독 후 중추신경계 손상을 동반한 독성 시신경병증 1예를 보고하고자 한다. 증례요약: 44세 여자가 7일 전부터의 양안 시력저하를 주소로 내원하였다. 환자는 내원 2주 전 자살 목적으로 클로르페나피르 한 모금을 음독했고, 직후 근처 병원에서 위세척을 시행하였다. 초기 최대교정시력은 우안 안전수지 30 cm, 좌안 안전수동이었다. 양안 동공은 5.0 mm로 커져 있었고, 빛에 대한 반응은 느렸으며 좌안에는 상대구심동공운동장애가 관찰되었다. 안저검사에서 양안 시신경유두부종이 관찰되었고, 뇌자기공명영상에서 양안 시신경과 속섬유막, 뇌량, 중소뇌각, 뇌간 등 백질 신경로를 따라 양쪽에 대칭적인 고강도신호가 관찰되었다. 클로르페나피르 중독으로 인한 독성 시신경병증으로 진단 후, 고용량 스테로이드치료를 3일간 시행하였으나 양안 최대교정시력은 광각무로 악화되었다. 3개월 후, 안저검사에서 양안 시신경위축이 관찰되었고, 빛간섭단층촬영에서 망막신경섬유층 및 신경절세포-내망상세포층 두께가 감소하였다. 결론: 매우 적은 양이라도 클로르페나피르에 노출되면 적절한 치료에도 불구하고 잠복기를 거쳐 심각한 시신경손상이 발생할 수 있으므로 주의해야 하겠다.

• 대한한방내과학회지
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• 제40권5호
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• pp.851-864
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• 2019

Multiple system atrophy is a neurodegenerative disease that causes diverse bodily dysfunctions (cerebellar, pyramidal, automatic, and urological, in any combination), as well as Parkinsonism. Patients with multiple system atrophy commonly display antecollis, a condition where the patient's head tilts forward more than 45 degrees. Despite its common occurrence in these patients, no current standardized therapies are effective for treating antecollis. In this study, Korean medicinal treatments, including Chuna manual therapy, pharmaco-acupuncture, bee venom acupuncture, acupuncture, herbal medicine, and moxibustion therapy, were administered to the patient over a 27-day period. After the treatment, assessments of the head position on the EPIS-PD scale (Part I) and at a standing position from the side (Part II) both revealed improvements. As the head flexion angle decreased, the patient's head posture improved, as determined by a decrease in angle from 80 degrees to 30 degrees in the upright, standing position. As a result, patients who previously were unable to walk without the support of walking frames could now roam freely and independently, with significant increases in both walking speed and distance. In essence, this study suggests that Korean medicine is an effective treatment for patients with multiple system atrophy who suffer from antecollis and gait disorders.

• 한국방사선학회논문지
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• 제16권6호
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• pp.761-769
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• 2022

고유전 재료를 사용한 패드는 임상 MRI 연구에서 RF 펄스의 필드 감도와 균질성을 국부적으로 개선하기 위해 다양한 응용 분야에서 사용되었다. 본 연구에서는 실제 임상 영상의 적용과 관련된 실질적인 문제를 고려하여 패드를 개선하는 것을 목표로 하였다. 감쇠된 B1 필드 강도를 증가시키기 위한 고유전율 패드를 제작하고 7T MRI에서 테스트했다. Sim4Life 시뮬레이션 및 실험 결과는 B1 근거리장에서 더 강하고 상대적으로 균일함을 보여준다. 민감도가 낮은 영역으로 알려진 전체 소뇌의 영상 품질을 향상시키기 위해 패드의 기계적 변화를 줄이는 가이드를 만들었다. 또한 착용감을 향상시키기 위해 패드를 상하로 나누어 디자인하였다. 안면 패드는 비강 내 비갑개와 같은 부위에서 전반적인 신호 증가 효과를 보였다. 전두엽, 눈 등의 영역에서 신호 증가가 예상됐지만 효과가 미미하거나 영상 프로토콜에서 효과를 보기 어려웠다. 결론적으로, 본 논문은 그 효과를 유지하면서 개선된 비강 신호를 갖는 소뇌 최적화 패드를 보여주었다.

• Journal of Korean Neurosurgical Society
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• 제66권6호
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• pp.672-680
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• 2023

Objective : We evaluated the diagnosis, treatment, and long-term results of patients with dural arteriovenous fistula (dAVF), which is a very rare cause of posterior fossa hemorrhage. Methods : This study included 15 patients who underwent endovascular, surgical, combined, or Gamma Knife treatments between 2012 and 2020. Demographics and clinical features, angiographic features, treatment modalities, and outcomes were analyzed. Results : The mean age of the patients was 40±17 years (range, 17-68), and 68% were men (11/15). Seven of the patients (46.6%) were in the age group of 50 years and older. While the mean Glasgow coma scale was 11.5±3.9 (range, 4-15), 46.3% presented with headache and 53.7% had stupor/coma. Four patients (26.6%) had only cerebellar hematoma and headache. All dAVFs had cortical venous drainage. In 11 patients (73.3%), the fistula was located in the tentorium and was the most common localization. Three patients (20%) had transverse and sigmoid sinus localizations, while one patient (6.7%) had dAVF located in the foramen magnum. Eighteen sessions were performed on the patients during endovascular treatment. Sixteen sessions (88.8%) were performed with the transarterial (TA) route, one session (5.5%) with the transvenous (TV) route, and one session (5.5%) with the TA+TV route. Surgery was performed in two patients (14.2%). One patient (7.1%) passed away. While there were nine patients (64.2%) with a Rankin score between 0 and 2, the total closure rate was 69.2% in the first year of control angiograms. Conclusion : In the differential diagnosis of posterior fossa hemorrhages, the differential diagnosis of dAVFs, which is a very rare entity, should be considered, even in the middle and elderly age groups, in patients presenting with good clinical status and pure hematoma. The treatment of such patients can be done safely and effectively in a multidisciplinary manner with a good understanding of pathological vascular anatomy and appropriate endovascular treatment approaches.

• 한국식품영양과학회지
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• 제33권2호
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• pp.262-270
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• 2004

에탄올이 지속적으로 뇌 신경세포에 미치는 영향을 조사하기 위하여 흰쥐의 신경세포로부터 유래 된 B103 neuroblastoma cell을 사용하여 세포독성이 나타나지 않는 에탄을 농도(0, 50, 100, 200 mM)에서의 1, 2, 8, 18, 24시간 경과에 따라 유도되는 PKC $\alpha$, ${\gamma}$, $\varepsilon$, ζ isozyme들의 양을 세포질 분획과 세포막 분획으로 나누어 Western blot으로 각각 분석하였다. 100 mM의 에탄올 농도에서 분석된 PKC isozyme들 중 PKC-$\varepsilon$는 18시간대의 세포질에서 그리고 PKC-$\varepsilon$은 8∼18시간대의 세포막분획에서 각각 현저한 유도현상을 보였다 PKC-$\alpha$는 200 mM의 에탄을 첨가 후 18시간과 24시간에 세포질과 세포막 분획에서 모두 대조군의 150%까지 현저한 증가를 나타낸 반면 PKC-ζ는 100, 200 mM 에탄올농도에서 배양(18, 24시간 동안)한 세포의 세포막분획에서만 유도되었다. 그리고 50, 100, 200 mM의 에탄올 농도에서 24시간동안 배양한 세포질 분획에서 PKC-${\gamma}$는 농도 의존적으로 감소하여 200 mM의 에탄올 농도에서는 대조군의 47%까지 현저한 감소를 나타내었으며, 세포내에 세포독성을 나타내지 않는 농도 특히 100∼200mM농도범위의 에탄올을 첨가하여 24시간 동안 지속적으로 배양할 때 PKC-${\gamma}$ 및 $\varepsilon$이 관련된 신호전달체계가 억제됨을 보였다. 이는 에탄올이 PKC isozyme들의 상호간 조절을 통해 신호전달계 또는 신경전달 물질들의 변화에 영향을 줄 수 있음을 시사하며 에탄올의 중추신경계에 미치는 지속적 영향으로 나타나는 행동장애 및 뇌 기능의 손상 또는 보호과정 에 PKC-isozyme들이 관여할 수 있음을 시사한다.

• 대한핵의학회지
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• 제30권3호
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• pp.299-314
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• 1996

The purpose of the present study was to validate the use of tissue radioactivity ratios instead of regional metabolic rates for the assessment of regional metabolic changes in Alzheimer's disease(AD) with [$^{18}F$]FDG PET and to examine the correlation of ratio indices with the severity of cognitive impairment in AD. Thirty-seven AD Patients(age $68{\pm}9 yrs$, $mean{\pm}s.d.$; 36 probable and 1 definite AD), 28 patients with dementia of non-Alzheimer type(age $66{\pm}7 yrs$), and 17 healthy controls(age $66{\pm}4 yrs$) underwent [$^{18}F$]FDG PET imaging. Two simplified radioactivity ratio indices were calculated from 37-66 min image: region-to-cerebellar radioactivity ratio(RCR) and a composite radioactivity ratio(a ratio of radioactivity in the most typically affected regions over the least typically affected regions: CRR). Local cerebral metabolic rate for glucose(LCMRglu) was also measured using a three-compartment, five-parameter tracer kinetic model. The ratio indices were significantly lower in AD patients than in controls(RCR in temporoparietal cortex, $0.949{\pm}0.136$ vs. $1.238{\pm}0.129$, p=0.0004; RCR in frontal cortex, $1.027{\pm}0.128$ vs. $1.361{\pm}0.151$, p<0.0001; CRR, $0.886{\pm}0.096$ vs. $1.032{\pm}0.042$. p=0.0024). On the RCR analysis, 86% of AD patients showed a pattern of bilateral temporoparietal hypometabolism with or without frontal involvement; hypometabolism was unilateral in 11% of the patients. When bilateral temporoparietal hypometabolism was considered to be suggestive of AD, the sensitivity and specificity of the RCR analysis for the differential diagnosis of AD were 86% and 73%, respectively. The RCR was correlated significantly with the macroparameter K [$K_1k_3/(k_2+k_3)$] (r=0.775, p<0.0001) and LCMRglu(r=0.633, p=0.0002) measured using the kinetic model. In patients with AD, both average RCR of cortical association areas and CRR were correlated with Mini-Mental Status Examination(r=0.565, p=0.0145; r=0.642, p=0.0031, respectively), Clinical Dementia Rating(r=-0.576, p=0.0124; r=-0.591, p=0.0077), and total score of Mattis Dementia Rating Scale (r=0.574, p=0.0648; r=0.737, p=0.0096). There were also significant correlations between memory and language impairments and corresponding regional RCRs. The results suggest that the [$^{18}F$]FDG PET ratio indices, RCR and CRR, reflect global and regional metabolic rates and correlate with the severity of cognitive impairment in AD. The simplified ratio analysis may be clinically useful for the differential diagnosis and serial monitoring of the disease.