• Journal of Korean Neurosurgical Society
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• v.59 no.5
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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.

• Journal of The Korean Society of Clinical Toxicology
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• v.16 no.1
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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.

• Proceedings of the KIEE Conference
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• 1995.07b
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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.

• Korean Journal of Head & Neck Oncology
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• v.32 no.2
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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.

• Journal of the Korean Medical Association
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• v.61 no.12
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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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• v.18 no.2
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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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• v.51 no.1
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• pp.84-88
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• 2008

Purpose : Radiotherapy is effective in local treatment for retinoblastoma. However, asymmetric facial hypoplasia after radiation is a serious late effect. This study was performed to investigate the effects of enucleation and chemotherapy with or without radiotherapy in advanced intraocular and intraorbital retinoblastoma. Methods : Between 1985 October and 2006 December, the records of thirty five patients who were diagnosed as retinoblastoma at Yeungnam University Hospital were reviewed. Advanced intraocular and intraorbital retinoblastoma patients classified as Reese-Ellsworth group III, IV, and V and Grabowski- Abramson class II were selected for the study. Results : Eighteen patients were enrolled in this study. All patients were enucleated and had received chemotherapy. Nine patients received radiotherapy and nine patients didn't receive radiotherapy. Tumor cells were found on resection margin of optic nerve in five of nine patients who received radiotherapy, but none of nine who didn't receive radiotherapy. Chemotherapy included vincristine, adriamycin, cyclophosphamide, VM-26, cisplatin before 2001, and vincristine, etoposide, and carboplatin after 2001. There were no recurrences or metastases in nine patients who didn't receive radiotherapy. But two of nine patients who received radiotherapy had metastases to brain. However, all survivors who received radiotherapy had significant facial asymmetry. Conclusion : In advanced intraocular and intraorbital retinoblastoma without tumor cell on resection margin of optic nerve, enucleation and chemotherapy without local radiotherapy appears to be safe for long-term survival. However, in those with tumor cells on resection margin of optic nerve, enucleation and chemotherapy with local radiotherapy seems to be necessary to improve survival.

• Development and Reproduction
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• v.16 no.2
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• pp.101-106
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• 2012

Importance of behavior factors or environmental factors in visual organization and visual function of fish is treated with great care in visual ecology, and there is no study about initial ocular growth and development on the dark banded rockfish, Sebastes inermis. Thus, this study was performed. The total length, head length, head depth, eye diameter and lens diameter of the dark banded rockfish showed positive allometric relationship between parturition stage and 60 days post-parturition (dpp). The increase in total length relative to head length and head depth, head length growth relative to eye diameter and lens diameter, and head depth growth relative to eye and lens diameter were nearly isometric. The eyes were formed completely at parturtion stage. At this age, the eye has an optic nerve fiber layer, a ganglion cell layer, an inner plexiform layer, an inner nuclear layer, an outer plexiform layer, an outer nuclear layer, an outer limiting membrane, a rod and cone layer and an epithelial layer. Thickness of retina at 60 dpp was higher than that of at parturition stage. During this experiment, the proportion of the rod and cone layer, outer nuclear layer, and optic nerve fiber layer of retina were significantly increased, while the proportion of the outer plexiform layer, inner nuclear layer and ganglion cell layer of retina were significantly decreased (P<0.05). The essential demands that must be met by the retina in this species pertain to light sensitivity and spatial resolution.

• Journal of Korean Neurosurgical Society
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• v.54 no.6
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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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• v.58 no.1
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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.