• Journal of Korean Neurosurgical Society
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• 제59권5호
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• pp.533-536
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• 2016

Pilocytic astrocytomas (PAs) are World Heath Organization Grade I tumors and are most common in children. PA calcification is not a common finding and has been reported more frequently in the optic nerve, hypothalamic/thalamus and superficially located cerebral tumors. We present a cerebellar PA in a 3-year-old male patient with cystic components and massive calcification areas. The residual tumor grew rapidly after the first operation, and the patient was operated on again. A histopathological examination revealed polar spongioblastoma-like cells. Massive calcification is not a common feature in PAs and can lead to difficulties in radiological and pathological differential diagnoses.

• 대한임상독성학회지
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• 제16권1호
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• pp.57-59
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• 2018

Severe systemic responses including neurologic complications such as myasthenia gravis, myeloradiculopathy, optic neuropathy, parkinsonism, stroke and Guillain-$barr{\acute{e}}$ syndrome can occur after bee stings. This case describes a 78-year-old female who presented with symptoms of acute progressive bilateral symmetrical weakness in both lower legs after multiple bee stings. Nerve conduction study findings were consistent with acute sensorimotor axonal neuropathy and recovered by treatment with intravenous immunoglobulin. This case highlights that bee stings can result in acute onset Guillain-$barr{\acute{e}}$ syndrome, although the pathophysiologies of bee venoms need to be investigated accurately.

• 대한전기학회:학술대회논문집
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• 대한전기학회 1995년도 하계학술대회 논문집 B
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• pp.950-952
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• 1995

In this paper, a novel method which can recognize noised patterns by using a neural network is proposed. Patern recognition is processed by two networks and the processing mechanism is partially based on the results of neurophysiological studies. Synaptic activitoes of combining neurons between layers are implemented by spatial filters which approximate receptive fields of the modelled optic nerve cells.

• 대한두경부종양학회지
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• 제32권2호
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• pp.41-46
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• 2016

Sinonasal undifferentiated carcinoma (SNUC) is an extremely rare, highly aggressive malignancy of the nasal cavity and paranasal sinuses. Patients with SNUC usually being asymptomatic until the tumor has extensively progressed therefore frequently present with invasion of the orbit or cranial vault. Most case series of SNUC report very poor prognosis despite aggressive multimodality therapy. We recently experienced a 78-year-old male patient diagnosed as SNUC occurred from the left sphenoid sinus with invasion to the cavernous sinus and orbital content, which was treated intranasal endoscopic debulking surgery combined with curative radiation therapy successfully, and report this case with a review of literature.

• 대한의사협회지
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• 제61권12호
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• pp.740-748
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• 2018

Posttraumatic facial deformities (PTFDs) are very difficult to correct, and if they do occur, their impact can be devastating. It may sometimes be impossible for patients to return to normal life. The aim of surgical treatment is to restore the deformed bone structure and soft tissue to create symmetry between the affected side and the opposite side. In the process of managing PTFD, correcting enophthalmos is one of the most challenging aspects for surgeons because of difficulties in overcoming the scar tissue and danger of injuring to the optic nerve. In this article, surgical options for reconstruction of the medial wall, floor, lateral wall, and roof of the orbit are described. To optimize aesthetic improvement, additional cosmetic procedures such as facial contouring surgery, blepharoplasty and rhinoplasty can be used. Plastic surgeons should join emergency trauma teams to implement an overall treatment plan containing rational strategies to avoid or minimize PTFD.

• Journal of Genetic Medicine
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• 제18권2호
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• pp.83-93
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• 2021

Charcot-Marie-Tooth disease (CMT) is the most common hereditary motor and sensory peripheral neuropathy. CMT is usually classified into two categories based on pathology: demyelinating CMT type 1 (CMT1) and axonal CMT type 2 (CMT2) neuropathy. CMT1 can be distinguished by assessing the median motor nerve conduction velocity as greater than 38 m/s. The main clinical features of axonal CMT2 neuropathy are distal muscle weakness and loss of sensory and areflexia. In addition, they showed unusual clinical features, including delayed development, hearing loss, pyramidal signs, vocal cord paralysis, optic atrophy, and abnormal pupillary reactions. Recently, customized treatments for genetic diseases have been developed, and pregnancy diagnosis can enable the birth of a normal child when the causative gene mutation is found in CMT2. Therefore, accurate diagnosis based on genotype/phenotypic correlations is becoming more important. In this review, we describe the latest findings on the phenotypic characteristics of axonal CMT2 neuropathy. We hope that this review will be useful for clinicians in regard to the diagnosis and treatment of CMT.

• Clinical and Experimental Pediatrics
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• 제51권1호
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• pp.84-88
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• 2008

목 적 : 방사선조사는 망막모세포종의 국소적 치료에 효과적인 방법이나 장기 생존자에서 안면발육장애, 각막과 시신경 손상, 이차적인 암의 발생 등의 합병증을 유발하며 방사선조사로 인한 안면발육부전은 나이가 들수록 더욱 심각한 미용적 장애를 초래하고 환자의 삶의 질을 저하시킨다. 진행된 안구 내 망막모세포종과 안와 내 망막모세포종 환자에서 안구적출술과 항암화학치료 및 안와방사선조사를 받은 경우와 받지 않은 경우의 치료성적과 장기예후를 분석하고자 본 연구를 시도하였다. 방 법 : 1985년 10월부터 2006년 12월까지 영남대학교병원에서 망막모세포종으로 진단받은 35명의 환자를 대상으로 병록지를 후향적으로 분석하였다. 안구 내 병변은 Reese-Ellsworth(RE)의 분류에 따라, 안구 외로 진행된 경우는 Grabowski-Abramson (GA)의 분류에 따라 분류하여 RE group III, IV, V인 진행된 안구 내 종양과 GA stage II인 안와 내 종양 환자를 연구 대상으로 하였다. 결 과 : 연구에 포함된 환자는 18명이었고, 진단당시 나이의 중앙값은 27개월(범위 2-69개월), 추적기간의 중앙값은 73.5개월(범위 6-219개월)이었다. 안구 내에 국한된 경우는 6명이었고(RE group III; 2명, IV; 1명, V; 3명) 안와 내로 진행된 경우는 12명이었다(GA stage II). 대상 환아 모두에서 안구적출술을 시행한 후 항암화학치료를 시행하였는데 2001년 이전에는 vincristine, adriamycin, cyclophosphamide, cisplatin, VM-26을, 2001년 이후로는 vincristine, etoposide, carboplatin을 사용하였다. 9명의 환자에서는 국소적으로 방사선조사를 병행하였고, 총 방사선 조사량의 중앙값은 4,500 cGy(범위 3,500-5,000 cGy)이었다. 방사선조사를 병행하여 치료 받은 환아 9명 중 7명은 재발이나 전이 없이 장기생존하였으며, 장기 생존자 모두에서 심한 안면 골격의 비대칭적 발육부전이 나타났다. 방사선조사 없이 치료받은 9명 중에서는 재발이나 전이가 한명도 없이 모두 장기생존하였으며, 안구적출술 이후 인공안와삽입물을 삽입하였으며 안면 골격 발달은 모두 대칭적으로 정상 발육을 하였다. 결 론 : 시신경의 절제면을 침범하지 않은 안와내에 국한된 망막모세포종의 치료에 있어 방사선조사를 제외하고 안구적출술, 항암화학치료, 국소적 치료를 병행함으로써 높은 생존율을 유지하면서 안면발육부전과 같은 심각한 부작용을 줄여 삶의 질을 향상시킬 수 있을 것으로 생각된다. 그러나 시신경의 절제면을 침범한 경우에는 재발의 가능성이 있으므로 후기 후유증의 위험이 있더라도 안구적출술 후 방사선조사와 항암화학치료를 병행한 치료를 시행함으로써 생존률을 높일 수 있을 것으로 생각된다.

• 한국발생생물학회지:발생과생식
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• 제16권2호
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• pp.101-106
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• 2012

어류의 시각 구조와 시각 기능 연구에 있어서 행동적 요인이나 환경적 요인의 중요성은 시각생태학에서 매우 중요하게 다루어지고 있으며, 볼락, Sebastes inermis에 관하여는 아직까지 초기 눈의 성장 및 발달에 관한 발생생물학적 연구가 이루어진 바가 없음을 고려하여 이에 대한 연구를 수행하였다. 볼락의 전장, 두장, 두고, 눈의 직경과 수정체 직경은 산출 직후부터 산출 후 60일까지의 단계에서 양성의 상관관계(positive allometry)를 보여주었다. 전장과 관련된 두장과 두고에서의 증가와, 두고와 관련된 눈의 직경과 수정체 직경의 성장은 거의 동형(isometric)성장을 나타내었다. 눈은 산출단계에 완전히 형성되었다. 산출 단계의 눈은 시신경 섬유층, 신경절 세포층, 내망상층, 내과립층, 외망상층, 외과립층, 외경계막, 간체, 추체층 및 상피층을 가지며, 산출 후 60일의 망막 두께는 산출 직후의 망막 두께보다 더 크다. 본 실험기간 동안, 망막의 간체와 추체층의 비율 및 외과립층과 시신경의 비율은 상당히 증가한 반면, 망막의 외망상층과 내과립층, 신경절 세포층의 비율은 현저하게 감소하였다(P<0.05). 차후, 본 연구에 사용된 볼락 망막에 대한 빛 민감성과 공간 해상력에 관한 부수 연구가 필요하리라 사료된다.

• Journal of Korean Neurosurgical Society
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• 제54권6호
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• pp.477-483
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• 2013

Objective : Although surgical techniques for clipping paraclinoid aneurysms have evolved significantly in recent times, direct microsurgical clipping of large and giant paraclinoid aneurysms remains a formidable surgical challenge. We review here our surgical experiences in direct surgical clipping of large and giant paraclinoid aneurysms, especially in dealing with anterior clinoidectomy, distal dural ring resection, optic canal unroofing, clipping techniques, and surgical complications. Methods : Between September 2001 and February 2012, we directly obliterated ten large and giant paraclinoid aneurysms. In all cases, tailored orbito-zygomatic craniotomies with extradural and/or intradural clinoidectomy were performed. The efficacy of surgical clipping was evaluated with postoperative digital subtraction angiography and computed tomographic angiography. Results : Of the ten cases reported, five each were of ruptured and unruptured aneurysms. Five aneurysms occurred in the carotid cave, two in the superior hypophyseal artery, two in the intracavernous, and one in the posterior wall. The mean diameter of the aneurysms sac was 18.8 mm in the greatest dimension. All large and giant paraclinoid aneurysms were obliterated with direct neck clipping without bypass. With the exception of the one intracavenous aneurysm, all large and giant paraclinoid aneurysms were occluded completely. Conclusion : The key features of successful surgical clipping of large and giant paraclinoid aneurysms include enhancing exposure of proximal neck of aneurysms, establishing proximal control, and completely obliterating aneurysms with minimal manipulation of the optic nerve. Our results suggest that internal carotid artery reconstruction using multiple fenestrated clips without bypass may potentially achieve complete occlusion of large paraclinoid aneurysms.

• Clinical and Experimental Pediatrics
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• 제58권1호
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• pp.28-32
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• 2015

Purpose: Diencephalic syndrome is an uncommon cause of failure to thrive in early childhood that is associated with central nervous system neoplasms in the hypothalamic-optic chiasmatic region. It is characterized by complex signs and symptoms related to hypothalamic dysfunction; such nonspecific clinical features may delay diagnosis of the brain tumor. In this study, we analyzed a series of cases in order to define characteristic features of diencephalic syndrome. Methods: We performed a retrospective study of 8 patients with diencephalic syndrome (age, 5-38 months). All cases had presented to Seoul National University Children's Hospital between 1995 and 2013, with the chief complaint of poor weight gain. Results: Diencephalic syndrome with central nervous system (CNS) neoplasm was identified in 8 patients. The mean age at which symptoms were noted was $18{\pm}10.5$ months, and diagnosis after symptom onset was made at the mean age of $11{\pm}9.7$ months. The mean z score was $-3.15{\pm}1.14$ for weight, $-0.12{\pm}1.05$ for height, $1.01{\pm}1.58$ for head circumference, and $-1.76{\pm}1.97$ for weight-for-height. Clinical features included failure to thrive (n=8), hydrocephalus (n=5), recurrent vomiting (n=5), strabismus (n=2), developmental delay (n=2), hyperactivity (n=1), nystagmus (n=1), and diarrhea (n=1). On follow-up evaluation, 3 patients showed improvement and remained in stable remission, 2 patients were still receiving chemotherapy, and 3 patients were discharged for palliative care. Conclusion: Diencephalic syndrome is a rare cause of failure to thrive, and diagnosis is frequently delayed. Thus, it is important to consider the possibility of a CNS neoplasm as a cause of failure to thrive and to ensure early diagnosis.