가족성 해면혈관종에서 염색체 7q CCM1 염기서열의 가족간 연관성 분석

심기범(Ki-Bum Sim),이창섭(Chang Sub Lee),김승기(Seung-Ki Kim),왕규창(Kyu-Chang Wang),김영임(Young-Im Kim),조병규(Byung-Kyu Cho) 한국독성학회 2005 Toxicological Research Vol.21 No.2

Although the pathogenesis of cerebral cavernous malformation (CCM) is unknown, a familial predisposition has been recognized, with up to 55% of patients having an affected relatives. Genetic linkage studies have recently mapped a gene causing CCM to a segment of the long arm of chromosome 7 (7q). We report herein a genetic linkage analysis conducted on a Korean three generation family with CCM. It’s first report in Korean family. A Korean family in which one member had undergone surgery for intracerebral hematoma (ICH) and confirmed the CCM, was evaluated. They were examined clinically (n=18) and by magnetic resonance (MR) imaging (n=10). Polymorphic markers (D7S1813, D7S1789) spanning the CCM1 locus on 7q were genotyped by the polymerase chain reaction and analysis of linkage was performed in this family (n=17). Six had multiple lesions on brain MR image, one of them being symptomatic, and five were asymptomatic. Seven remaining members were asymptomatic and refused MR image study. One had died of ICH from presumed CCM. Analysis of the pedigree was consistent with an autosomal dominant pattern of inheritance. All affected patients were linked to CCM1. Linkage to CCM1 can account for inheritance of CCM in this family.They had some striking features with a low clinical penetrance and the presence of multiple lesions. These findings have implications for genetic testing of this disorder and represent an important step toward identification of the gene responsible for the pathogenesis of this disease.

정상 닭배자 (배양14-20일)의 후두와 발생과정에 대한 연구 : 자기공명영상 및 해부병리학적 소견

심기범(Ki-Bum Sim),이창섭(Chang Sub Lee),신태균(Taekyun Shin) 한국환경성돌연변이발암원학회 2005 한국환경성돌연변이·발암원학회지 Vol.25 No.1

The objective was to use MR imaging to provide a template of posterior fossa development during the late stages in the clrick embryos. The MR findings were then correlated with histological data. Fourteen normal formalin-fixed embryonic specimens with a gestational age of 14 to 20 days were examined with 1.5 TesJa unit MRI using a conventional clinical magnet and pulse sequences. The MR findings were correlated with the whole-mount histological specimens. Resolution of the morphological features of posterior fossa development in embryos greater than 14 days gestational age was possible. Development of cerebellum, brain stem, 4th ventricle and bony posterior fossa was documented. In the 14-day-old embryos, a premordial cerebellum was visualized in the enlarged bony posterior fossa, and it covered the the roof of the primitive fourth ventricle. The bony posterior fossa grows at the same rate along the supratentorial skull. The supratentorial skull and the rostral part of the brain grows at the same rate. The cerebellum begins to grow later than the rostral part of the brain. In the 19-to 20-day-old embryos, MRI revealed the rapid development of the cerebellar hemispheres, along with an increase in volume manifested by the more typical mushroom-shaped configuration observed in the newly hatched. At this stage, the cerebellum almost completely filled the posterior fossa and covered the entire fourth ventricle. The brain stem grew steadily, but the volume change was too subtle to evaluate. Features of cerebellar histogeneis were beyond the resolution of MRI. However, there were lots of artifacts in the features of the bony posterior fossa. An MR template of normal posterior fossa development would be useful to avoid confusion of normal development with abnormal development and to identify the expected developmental features when provided the estimated gestational age of a embryo.

척수내 종양과 감별을 요하는 비종양성 척수증 : 수술로 확진된 8례의 후향적 분석

김기정,정천기,심기범,김현집,Kim, Ki-Jeong,Chung, Chun-Kee,Sim, Ki-Bum,Kim, Hyun-Jib 대한신경외과학회 2000 Journal of Korean neurosurgical society Vol.29 No.7

Objective : It is difficult to differentiate intramedullary spinal cord tumors preoperatively from non-neoplastic pathologies in patients presenting as non-compressive myelopathies in magnetic resonance imaging(MRI). In this report, the authors reviewed nonneoplastic intramedullary spinal cord lesions preoperatively diagnosed as tumors and discussed their clinical and radiological characteristics and usefulness of surgical intervention. Methods : From January, 1985 to January, 1999, authors experienced eight non-neoplastic pathologies mimicking intramedullary spinal cord tumors and analysed their medical records, radiological findings and histopathological specimens retrospectively. Results : There were five males and three females and the duration of symptoms were from two to 20 months(mean, 9.8 months). The location of lesions were four cervical, one cervicothoracic and three thoracic. All patients manifested sensory abnormality, seven motor weakness, and six bladder symptom. All cases had swollen spinal cords and increased signal intensities in spin-echo sequences. Six cases showed contrast enhancement : four cases were focal and two diffuse. Under the impression of intramedullary tumors, the patients were operated upon. Final diagnoses on the base of clinical and pathologic finding were : three subacute necrotizing myelopathies, two multiple scleroses, two myelopathy of unknown etiology. One case showed no gross abnormality in surgical field in spite of adequate exposure of the lesion, so biopsy was not performed. In that case, postoperative MRI revealed spontaneous resolution of the lesion. Conclusion : MRI is invaluable diagnostic tool in screening myelopathies. However, its high sensitivity and lack of specificity make difficulty in preoperative differential diagnosis of non-compressive myelopathies. Although no surgical morbidity occurred in our series, we sometimes failed to confirm definite diagnosis even with biopsy. In such a circumstance, long-term follow up is needed.

전적출후 재발한 소아 두개인두종의 재발인자에 대한 분석

김승기,왕규창,정영섭,심기범,조병규,Kim, Seung-Ki,Wang, Kyu-Chang,Chung, Young Seob,Sim, Ki-Bum,Cho, Byung-Kyu 대한신경외과학회 2001 Journal of Korean neurosurgical society Vol.30 No.1

Objectives : A substantial number of craniopharyngiomas recur despite gross total excision. The purpose of our study was to investigate pattern of recurrence and to verify prognostic factors for recurrence after gross total excision of craniopharyngiomas in children. Methods : A series of 36 patients with craniopharyngiomas were reviewed. All patients had undergone gross total excision and none of them received radiotherapy after initial surgery. Fifteen were girls and twenty-one were boys, with a mean age of 7.3 years(range, one to 15 years). The mean follow-up period was 52 months(range, one to 149 months). Recurrence was noted in 14 patients within 83 months(mean 31.4 months). Results : The overall three-year recurrence free survival rate was 65%, and the five-year recurrence-free survival rate was 55%. Regular neuroimaging follow-up at six to 12-month intervals detected tumor recurrence of a smaller size before symptoms developed(p<0.05). At the first surgical procedure, the optic nerve/chiasm(n=23) was the most common adhesion site. The most frequent sites of recurrence were the optic nerve/chiasm(n=6) and the pitiutary fossa(n=6). Tumor location was the single significant clinical predictor of recurrence. The five-year recurrence-free survival rate was 39% for those who had an intrasellar tumor component and 81% for those who did not (p<0.05). Conclusion : Craniopharyngiomas with intrasellar components should be followed cautiously and regular followup of patients should be emphasized, even when the tumors are totally resected.

Extracellular signal regulated kinases in the spinal cord of rats with experimental autoimmune encephalomyelitis

안미정,허승담,지영흔,주홍구,이용덕,심기범,신태균,Ahn, Mee-jung,Heo, Seung-dam,Jee, Young-heun,Joo, Hong-gu,Lee, Yong-duk,Sim, Ki-Bum,Shin, Tae-kyun The Korean Society of Veterinary Science 2003 大韓獸醫學會誌 Vol.43 No.4

The phosphorylation of extracellular signal-regulated kinases (p-ERK) in the spinal cord of rats with acute monophasic experimental autoimmune encephalomyelitis (EAE) was studied using immunohistochemistry and treatment with inhibitor. P-ERK is constitutively expressed in glial cells in the normal spinal cord. In EAE, some inflammatory cells in the subarachnoid space were positive for p-ERK at the early stage, and its immunoreactivity declined when those cells infiltrated the parenchyma at the peak stage. In a blocking experiment using its inhibitor, the intravenous administration of PD98059 from day 7 to 13 post-immunization did not modulate EAE paralysis. Considering the results, we postulate that intravenous administration of PD98059 is not effective in ameliorating EAE paralysis, although many inflammatory cells express ERK in the subarachnoid space.

척수손상 장치의 충격량 정량화 모델

강철웅(Chul-Ung Kang),고석준(Seok-Jun Ko),심기범(Ki-Bum Sim) 한국정보기술학회 2012 한국정보기술학회논문지 Vol.10 No.11

In order to enhance the reproducibility, we developed an impact device to remedy the existing devices shortcoming of spinal cord injury model. The impactor is fixed for structure based on motion controller and electric cylinder. It is able to induce accurate impact location and maximize the reproducibility by using camera and laser sensor. Moreover, it has another advantage that can produce an experimental model indicating desired lesion. This is because it can directly set the variable related with amount of impact. Because it, in comparison to the previous impactor, functions contusion method and compression method with only one device, it is able to realize a diverse range of impact model. For this study, we first collected the force data of load-cell attached in impact area, and location data of cylinder stroke through experiment. Next, we quantify the amount of impact on the basic of the above data.

상악 분절골 절단술을 이용한 부분 무치악 환자의 보철적 수복

김영균(Young-Kyun, Kim),박현식(Hyun-Sik, Park),심기범(Ki-Bum Sim) 대한치과의사협회 2000 대한치과의사협회지 Vol.38 No.11

요추간판탈출 수술 실패에서의 재수술 경험

심기범,김영백,민병국,황성남,석종식,최덕영,임광세 대한신경외과학회 1988 Journal of Korean neurosurgical society Vol.17 No.1

Nine patents, who had once low back operation due to herniated lumbar disc, underwent repeated low back surgery at the same level because of persistent of recurred symptoms. All patients were proved to have no psychological or compensation problems. Seven of them had satisfactory results but two, one who had no definite symptom free interval and had surgically proven epidural fibrosis only and another who had no definite symptom free interval and had surgically proven epidural fibrosis only and another who underwent reoperation as early as 45 days after the first operation had persistent previous symtoms. After review of these cases we obtained several favorable factors which influence the postoperative results. When a patient had a definite symptom free interval longer than 2 years results could be anticipated.

Risk Factors of Ventriculitis Following Ventriculostomy

Sim, Ki-Bum,Chung, Chun Kee,Kim, Dong Gyu,Han, Dae Hee 대한신경외과학회 1994 Journal of Korean neurosurgical society Vol.23 No.5

본 저자들은 뇌실조루술 후 발생하는 뇌실염을 예방하고, 치료하는 지침을 마련하고자 본 연구를 시행하였다. 연구 대상은 1990년 3월부터 1991년 7월까지 서울대학교병원에서 경험하였던 뇌실염 환자 14명이었다. 그중 8명은 서울대학교병원에서 뇌실조루술을 시행한 100명의 환자 중에서 발생하였으며, 6명은 타병원에서 전원되었다. 뇌실염의 진단은 전적으로 뇌실내 뇌척수액의 배양 결과에 준하였다. 치료는 지속적인 뇌척수액의 배출, 뇌실조루술 도관의 간헐적 제거 및 재배치, 그리고 감도시험 결과에 따른 항생제의 투여를 시행하였다. 뇌실염의 원인균은 코아귤라제 음성 포도구균(9명), 황색포도상구균(2명), 녹색연쇄구균(1명), 엔테로박테르(1명), 그리고 장내구균(1명)이었다. 뇌실조루술후 뇌실염의 발생기간은 평균 7일(4일부터 15일)이었다. 본 연구결과에 의하면 뇌실조루술 시행후 4일째부터 뇌실염이 발생하였다. 뇌실염 환자중 10명은 평균적으로 뇌실염 치료 33일(9일부터 142일)만에 회복하였다. 4명은 사망하였다. 본 연구결과와 문헌고찰을 기초로 하여 본 저자들은 뇌실조루술 후 발생하는 뇌실염을 예방하고, 치료하기 위해서는 1) 뇌실조루술 도관을 엄격히 무균적으로 삽입하고, 피하터널을 만든 후 절대적으로 폐쇄된 상태로 유지하고, 2) 뇌실조루술을 필요로 한 원인질환을 가능한 빨리 치료하고, 3) 뇌실조루술 도관을 장기간 유지하여야 할 경우 3일 적어도 5일마다 제거후 재배치하고, 4) 뇌실조루술을 유지하고 있는 동안에는 주기적으로 세균 상용검사를 시행하고, 5) 치료를 위한 항생제는 반드시 감도시험 결과에 따라 선택하여 사용할 것을 권유한다.

제3뇌실에 발생한 낭포성 두개인두종 : 증례보고 Case Report

심기범,민병국,석종식,임광세 대한신경외과학회 1987 Journal of Korean neurosurgical society Vol.16 No.2

Craniopharyngiomas are usually considered to arise from the pituitary stalk and upper aspect of the pituitary gland. However, they very rarely develop from the floor of the third ventricle or the lamina terminalis, and are intrinsically confined to the third ventricle; about 40 cases have hitherto been reported in the literatures. We have recently experienced a case of intrinsic intraventricular craniopharyngioma with the density a little higher than cerebrospinal fluid on the brain CT.