• Archives of Plastic Surgery
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• 제32권3호
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• pp.389-392
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• 2005

A case of visual loss following cranio-maxillofacial trauma is reported. The patient had acute optic nerve injury associated with a fracture of the right zygomaticomaxillary and fronto-naso-ethmoido-orbital bone and epidural hematoma on the right temporal lobe of brain. Bony fragments compressing the optic nerve on lateral side was identified on computed tomography. Decompression of the optic nerve combined with evacuation of epidural hematoma has been performed via transfrontal craniotomy. The patient had complete recovery of visual acuity without any complications. The role of optic nerve decompression in the management of patients with traumatic optic neuropathy is discussed. Surgical indication is controversial and the procedure should be considered only within the context of the specific indication of the individual patient.

• Journal of Yeungnam Medical Science
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• 제16권1호
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• pp.114-118
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• 1999

LHON은 사립체 DNA의 점돌연변이에 의해서 유발되며 11778, 3460, 14484의 세 부위가 주된 사립체 DNA 점돌연변이의 위치로 알려져 있다. 이에 저자들은 점진적인 시력 저하를 호소하면서 사립체 DNA 분석 결과 11778 점돌연변이가 확인된 LHON환자 1례를 경험하였기에 문헌고찰과 함께 보고하는 바이다.

• 대한신경과학회지
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• 제36권4호
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• pp.366-368
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• 2018

• Maxillofacial Plastic and Reconstructive Surgery
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• 제31권1호
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• pp.81-85
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• 2009

As panfacial fractures are involved multiple fracture, there are possibility of many pre-operative & postoperative complications. It is necessary to do exact pre-operative evaluation, appropriate operation and care, for preventing and treating these complications, especially related to optic nerve injury. The complication occurs rarely after periorbital facial bone fracture, and indirect injuries may occur as a result of impact shearing force transmitted into the optic nerve axons or to the nutrient vessels of the optic nerve. Also indirect injuries may occur after the force of impact because of vasospasm and swelling of the optic nerve within the confines of the nonexpansile optic canal. It is necessary to active evaluation and treatments involving decompression of the orbit surgically and high dose steroid therapy in relation to panfacial fracture. But sometimes this treatments are limited due to severe swelling of the face and related multiple bone fractures in the body. This case showed the delayed neuropathy, at last visual loss, in spite of megadose methylprednisolone administration. The purpose of this article is to present indirect traumatic optic neuropathy that is one of many complications in panfacial bone fracture.

• 대한간학회지
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• 제24권4호
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• pp.417-423
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• 2018

Anemia appears frequently in patients with alcoholic liver disease (ALD) but has never been linked to bilateral nonarteritic anterior ischemic optic neuropathy (NAION). A 65-year-old woman with a medical history of alcoholic cirrhosis was admitted for bilateral NAION. On admission, she was found to have a low arterial pressure and severe normocytic anemia (48 g/L). The anemia was related to chronic bleeding due to antral gastritis along with other factors associated with ALD. The applied treatment consisted of urgent transfusion followed by high doses of proton-pump inhibitors, iron and vitamin supplementation, and support in lifestyle measures. Her hemoglobin levels remained stable after 2 years but the patient still suffered from visual loss. This case highlights the link between anemia and bilateral NAION in ALD patients. The optic nerve head is prone to infarction in this context due to the vascularization characteristics of ALD. Hemoglobin levels should be monitored in ALD patients to avoid the severe complication of NAION.

• 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.

• Annals of Clinical Neurophysiology
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• 제16권2호
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• pp.89-91
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• 2014

• Journal of Korean Neurosurgical Society
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• 제56권3호
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• pp.281-283
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• 2014

McCune-Albright syndrome (MAS) is characterized by a triad of poly/monostotic fibrous dysplasia, caf$\acute{e}$-au-lait macules and hyperfunctioning endocrinopathies including human growth hormone excess. Acromegaly as a manifestation of endocrine hyperfunction with MAS is uncommon. Surgical excision may be challenging due to the associated severe fibrous dysplasia of the skull base. Through the endoscopic procedures, we treated a case of MAS presenting with compressive optic neuropathy due to fibrous dysplasia and acromegaly caused by growth hormone secreting pituitary adenoma. We reviewed the literature on GH excess in MAS to highlight its surgical and medical challenges.

• 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.

• Journal of Korean Neurosurgical Society
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• 제29권10호
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• pp.1345-1351
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• 2000

Objective : Around the sellar area, there are many important structures. But, the optimal radiation dosage for minimal toxicity to surrounding neural tissue has not been firmly established. The purpose of this study is to evaluate the radiosurgical outcome of juxtasellar tumors and to investigate the relationship between radiation dosage and toxicity to neural tissue. Method : Between May 1992 and June 2000, we treated 65 juxtasellar tumors by using the Leksell Gamma Knife. Among them, 52 patients who could be followed more than 1 year were included in this study. The radiosurgical dosage to the optic pathway, cavernous sinus, Meckel's cave, hypothalamus, pituitary gland and stalk, and brain stem was analyzed and correlated with clinical outcome. The mean follow-up period was 33.5 months(range 12.2- 99.0 months). Result : The clinical response rate was 69.2%. The volume response rate was 61.0% and the radiologic control rate was 92.7%. There were 4 complications(7.7%) of 2 trigeminal neuropathy, 1 abducens nerve palsy, and 1 trigeminal and transient abducens nerve palsy. The optic apparatus appeared to tolerate doses greater than 10Gy. The risk of cranial nerve complications in cavernous sinus seemed to be related to doses of more than 16Gy. In 3 of 4 patients who received more than 16Gy to cavernous sinus, the abducens or trigeminal neuropathy occurred. Also, one patient who received more than 15Gy to the Meckel's cave, trigeminal neuropathy developed. The hypothalamus, pituitary gland and stalk, and brain stem were relatively tolerable to radiation. Conclusion : Gamma Knife radiosurgery seems to be an effective method to control the growth of juxtasellar tumors. To avoid injury to surrounding important neural tissue, careful dose planning and further study for radiation toxicity to neural tissue were needed.