• Annals of Clinical Neurophysiology
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• 제20권2호
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• pp.85-88
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• 2018

Septo-optic dysplasia is a congenital anomaly with diverse phenotypes from normal to mixtures of visual abnormality, endocrine dysfunction, psychomotor retardations and epileptic seizures. It is characterized by optic atrophy, pituitary dysfunction and midline structure abnormalities in corpus callosum or septum pellucidum. Diagnosis of septo-optic dysplasia plus is made when cortical malformations accompanied. Here we report a middle-aged woman with septo-optic dysplasia plus having unilateral optic atrophy, agenesis of septum pellucidum and cortical malformations.

• 대한의용생체공학회:학술대회논문집
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• 대한의용생체공학회 1997년도 추계학술대회
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• pp.146-149
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• 1997

Computerized densitometry was developed or the quantitative measurement of diffuse retinal nerve fiber layer (RNFL) atrophy and intra- and inter-operator reliability and clinical validity of this system were evaluated. Vertical diameter, center of the optic disc, and peripapillary circles which had radii of 1.5 and 2.5 times that of the optic disc were user-interactively determined in digitized RNFL photograph and density profile along each circle was measured and normalized. The areas under the normalized density profiles of the superior and the inferior segments in both circle were used or the study of RNFL. To determine the variability and correspondence in the measurements of density variations, 21 RNFL photographs of glaucoma patients which showed varying degrees of atrophy underwent computerized densitometry by two operators on two separate occasions. Coefficient of variation in the densitometric measurements was $1.2{\sim}5.4%$. Intra- and inter-operator reliabilities were excellent. The correlations between the densitometric values and mean deviations of Humphrey C30-2 visual field showed statistical significance. Computerized densitometry of RNFL photographs was useful in the objective and quantitative assessment of diffuse RNFL atrophy.

• 대한수의학회지
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• 제59권2호
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• pp.113-117
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• 2019

A 6-year-old intact male Maltese dog presented with a history of blindness and ataxia. Neuro-ophthalmic examination revealed dilated pupils with absent pupillary light reflexes and menace response in both eyes. Mild peripapillary edema was noted in the fundus of the right eye. After magnetic resonance imaging, the dog was provisionally diagnosed with meningoencephalitis of unknown etiology. Follow-up funduscopy was performed to monitor the condition of the optic discs for three years. Despite of the treatment with prednisolone, the optic nerve progressed to atrophy and the dog couldn't restore vision.

• 대한유전성대사질환학회지
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• 제22권2호
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• pp.37-45
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• 2022

Inherited metabolic disorders (IMD) are a large group of rare disorders affecting normal biochemical pathways. The ophthalmic involvement can be very varied affecting any part of the eye, including abnormalities of cornea, lens dislocation and cataracts, retina and the optic nerve, and extraocular muscles. Eye disorders can be initial symptoms of some IMD and can be clue for diagnosis of IMD. However, eye disorders can evolve later in the natural history of an already diagnosed metabolic disorder. Awareness of IMDs is important to facilitate early diagnosis and in some cases instigate early treatment if a patient presents with eye involvement suggestive of a metabolic disorder. Ophthalmological interventions are also an important component of the multisystem holistic approach to treating patients with metabolic disorders.

• Journal of Cerebrovascular and Endovascular Neurosurgery
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• 제20권4호
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• pp.241-247
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• 2018

Treatment of paraclinoid aneurysms weather by surgery, or endovascular embolization has a risk of visual loss due to optic neuropathy, or diplopia due to cranial nerve palsies. Visual complications occur immediately after the clipping, whereas they can occur variable time after endovascular coiling. Recently, endovascular coiling for paraclinoid aneurysm is regarded as a safe and feasible treatment. But it still has risks of acute thromboembolic complication, or cranial nerve palsies. A 45-year-old woman was referred from local hospital to our hospital due to ruptured large ICA dorsal wall aneurysm. A total of 12 coils (195 cm) were used for obliteration of aneurysm. Postoperative diffusion weighted image showed no abnormal signal intensity lesion and magnetic resonance angiography demonstrated no sign of vasospasm, or vessel narrowing. But, she complained visual problem 23 days after coil embolization. Ophthalmologist confirmed the left optic disc atrophy on fundoscopy. Although steroid was started, but monocular blindness did not recover completely. The endovascular embolization of paraclinoid aneurysm, especially projecting superiorly with large irregular shape, has the risk of progressive visual loss because of the proximity to optic nerve.

• 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.

• 한국수의병리학회지
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• 제7권1호
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• pp.17-21
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• 2003

From February to September in 1996, a lot of blind cattle were reported in Dangjin Area of Chungnam Province. Blindness were observed in the 143 calves of the 1,496 calves from 79 farms. Blind cattle were found only in the farms where green pasture was not fed. After administration of vitamin A as the type of feed additives and parenteral injection, the blindness cases were not occurred in those farms. Both pupils were totally dilated with the absence of pupillary light reflex in all blind cattle. In the ophthalmoscopic examination, the fundus revealed multi focal linear white mottling which was more severe in nontapetal fundus than tapetal fundus. Serum vitamin A concentration was as low as 4.1 ,$\mu\textrm{g}$/dl in the calves of the affected farms. Narrowing of optic foramen, retinal degeneration and optic disc atrophy were shown in pathological findings.

• Journal of Korean Neurosurgical Society
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• 제57권1호
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• pp.23-31
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• 2015

Objective : The aims of this study were to investigate the quantitative relationship between pituitary macroadenoma size and degree of visual impairment, and assess visual improvement after surgical resection of the tumor. Methods : The medical records of patients with pituitary adenoma, who had undergone trans-sphenoidal adenectomy between January 2009 and January 2011, were reviewed. Patients underwent an ocular examination and brain MRI before and after surgery. The visual impairment score (VIS) was derived by combining the scores of best-corrected visual acuity and visual field. The relationship between VIS and tumor size/tumor type/position of the optic chiasm was assessed. Results : Seventy-eight patients were included (41 male, 37 female). Thirty-two (41%) patients experienced blurred vision or visual field defect as an initial symptom. Receiver operating characteristic curve analysis showed that tumors <2.2 cm tended to cause minimal or no visual impairment. Statistical analysis showed that 1) poor preoperative vision is related to tumor size, displacement of the optic chiasm in the sagittal view on MRI and optic atrophy, and 2) poorer visual prognosis is associated with greater preoperative VIS. In multivariate analysis the only factor significantly related to VIS improvement was increasing pituitary adenoma size, which predicted decreased improvement. Conclusion : Results from this study show that pituitary adenomas larger than 2 cm cause defects in vision while adenomas 2 cm or smaller do not cause significant visual impairment. Patients with a large macroadenoma or giant adenoma should undergo surgical resection as soon as possible to prevent permanent visual loss.

• 대한안과학회지
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• 제59권11호
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• pp.1091-1096
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• 2018

목적: 양안 급성 폐쇄각녹내장 후 발생한 양안 비동맥염 앞허혈시신경병증 1예를 보고하고자 한다. 증례요약: 76세 여자 환자가 하루 전 시작된 양안 통증 및 시력저하로 방문하였다. 양안 시력 0.02, 안압은 우안 52 mmHg, 좌안 50 mmHg, 양안 각막부종과 얕은 전방이 관찰되었으며, 동공은 4 mm로 고정되어 있었다. 만니톨 주사로 안압하강 시킨 후 양안 레이저홍채절개술을 시행하였다. 그러나 2일 후 시력은 우안 안전수지 10 cm, 좌안 안전수지 50 cm로 더 감소되었고 양안 시신경 유두부종이 관찰되었다. 양안 급성 폐쇄각녹내장 후 발생한 비동맥염 앞허혈시신경병증으로 진단하고 경과관찰하였다. 한 달 뒤, 시력은 우안 0.02, 좌안 0.04로 약간 호전되었고, 양안 시신경 유두부종은 사라졌다. 매우 작은 유두함몰비와 시신경 창백과 위축이 관찰되었으며, 표준자동시야검사상 우안은 완전시야결손, 좌안에 상하 수평시야결손이 관찰되었다. 결론: 급성 폐쇄각녹내장이 비동맥염 앞허혈시신경병증의 선행 위험인자가 될 수 있으며, 구심동공운동장애와 유두부종, 다른 위험인자의 동반이 있는 경우 비동맥염 앞허혈시신경병증의 동반 가능성을 고려해야 한다.

• 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.