• Annals of Clinical Neurophysiology
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• v.20 no.2
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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.

• Proceedings of the KOSOMBE Conference
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• v.1997 no.11
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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.

• Korean Journal of Veterinary Research
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• v.59 no.2
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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.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.22 no.2
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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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• v.20 no.4
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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.

• Journal of The Korean Ophthalmological Society
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• v.59 no.11
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• pp.1097-1102
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• 2018

Purpose: To report a case of toxic optic neuropathy caused by chlorfenapyr ingestion accompanied by central nervous system involvement. Case summary: A 44-year-old female visited our clinic complaining of reduced visual acuity in both eyes for 7 days. She had ingested a mouthful of chlorfenapyr for a suicide attempt 2 weeks prior to the visit. Gastric lavage was performed immediately after ingestion at the other hospital. Her best-corrected visual acuity was finger count 30 cm in the right eye and hand motion in the left eye. Both pupils were dilated by 5.0 mm and the response to light was sluggish in both eyes. A relative afferent pupillary defect was detected in her left eye. Funduscopy revealed optic disc swelling in both eyes. Magnetic resonance imaging of the brain showed a symmetric hyper-intense signal in the white matter tract including the internal capsule, corpus callosum, middle cerebellar peduncle, and brainstem. The patient was diagnosed with toxic optic neuropathy induced by chlorfenapyr ingestion, and underwent high-dose intravenous corticosteroid pulse therapy. Three days later, the best-corrected visual acuity was no light perception in both eyes. Three months later, optic atrophy was observed in both eyes. Optical coherence tomography revealed a reduction in the thicknesses of the retinal nerve fiber layer and ganglion cell and inner plexiform layer in the macular area. Conclusions: Ingestion of even a small amount of chlorfenapyr can cause severe optic nerve damage through the latent period, despite prompt lavage and high-dose steroid treatment.

• Korean Journal of Veterinary Pathology
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• v.7 no.1
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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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• v.57 no.1
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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.

• Journal of The Korean Ophthalmological Society
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• v.59 no.11
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• pp.1091-1096
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• 2018

Purpose: We report a case of bilateral nonarteritic anterior ischemic optic neuropathy (NAION) following acute angle-closure crisis (AACC). Case summary: A 76-year-old female visited our clinic because of a 1-day history of ocular pain and vision loss in both eyes. The visual acuity was 0.02 in both eyes and her intraocular pressure (IOP) was 52 mmHg in the right eye (RE) and 50 mmHg in the left eye (LE). She had corneal edema and a shallow anterior chamber in both eyes, with 4 mm fixed dilated pupils. After decreasing the IOP with intravenous mannitol, laser iridotomy was performed. However, 2 days later, visual acuity was further reduced to finger counting at 10 cm RE and at 50 cm LE, and her optic disc was swollen. Bilateral NAION following AACC was diagnosed. One month later, visual acuity slightly improved to 0.02 RE and 0.04 LE, and the optic disc edema resolved. A small cup-disc ratio, optic disc pallor, and atrophy were observed. Humphrey visual fields demonstrated superior and inferior altitudial visual field defects in the LE, and almost total scotoma in the RE. Conclusions: AACC can be a predisposing factor for NAION, so the relative afferent pupillary defect, papilledema, and presentation of other risk factors are important clues to a diagnosis of NAION.

• 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.