The authors clinically analyzed 240 patients of optic nerve diseases who visited the Department of Ophthalmology of Kyung Hee University hospital during four and a half years from July 1985 to February 1990. The most common optic nerve disease was optic nerve atrophy(59.2%), followed by optic neuritis, anterior ischemic optic neuropathy, papilledema, compressive optic neuropathy, retrobulbar optic neuritis, toxic neuropathy, and anomaly of optic nerve. According to sex, there was no significant difference between female and male. In comparison with age groups, the largest group was in the age group of 21-30 years. In cases of optic nerve atrophy, the most common cause was trauma(29.1%), most common age group was 21-30 years. In cases of optic neuritis, the most common cause was idiopathic(61.7%), most common age group was 31-40 years. Visual improvement was seen in 85.3%. In cases of anterior ischemic optic neuropathy, the most common cause was idiopathic(35.0%), most common age group was 41-50 years. Visual improvement was seen in 15%. In cases of papilledema, the most common cause was brain tumor (88.2%), most common age group was 41-50 years.
Atrophy ; Brain Neoplasms ; Female ; Humans ; Male ; Ophthalmology ; Optic Nerve Diseases* ; Optic Nerve* ; Optic Neuritis ; Optic Neuropathy, Ischemic ; Papilledema

Leber's hereditary optic neuropathy is caused by a single nucleotide change in the mitochondrial deoxynucleic acid(mtDNA). We identified a single guanine to adenine transition mutation in the mitochondrial DNA at nucleotide position 11778(Wallace mutation)in a 13 year old boy. To our knowldge, this is the first report confirming mtDNA mutation in Korea. This would be very helpful for the correct diagnosis of optic neuritis, optic neuropathy and optic atrophy of unknown etiology as well as for genetic counselling in the future.
Adenine ; Adolescent ; Diagnosis ; DNA, Mitochondrial ; Guanine ; Humans ; Korea ; Male ; Optic Atrophy ; Optic Atrophy, Hereditary, Leber* ; Optic Nerve Diseases ; Optic Neuritis

No abstract available.
DNA, Mitochondrial ; Optic Atrophy, Hereditary, Leber* ; Optic Nerve Diseases*

We performed full field pattern reversal VEP using UTAS-E 2000, in 87 eyes of the 70 patients with amblyopia(14 eyes) and optic nerve diseases; optic neuritis(21 eyes), optic nerve atrophy(23 eyes), toxic optic neuropathy(15 eyes) and optic nerve injury(14 eyes) from December 1993 to July 1994. This study was carried out to evaluate the relationship of the visual acuity with P1 amplitude, P1 latency, and to compare the latency of P1, and P1-N2 amplitude to each disease group and the normal groups. There was no correlation between the visual acuity and P1 latency, but significant correlation between the visual acuity and P1 amplitude(p<0.01). In the P1 implicit time, optic neuritis, optic nerve atrophy and toxic optic neuropathy patients presented marked delay and amblyopia patients presented moderate delay, but there was no other significant difference in each disease group. Over 50% of each disease group except amblyopia presented P1 destruction. Therefore, the authers concluded that P1 amplitude might not be good parameter in diagnosis of the optic nerve disease because of its variability to the visual acuity, but P1 latency and P1 destruction could be good parameter.
Amblyopia* ; Atrophy ; Diagnosis ; Humans ; Optic Nerve Diseases* ; Optic Nerve Injuries ; Optic Nerve* ; Optic Neuritis ; Visual Acuity

In order to investigate causative mechanisms of optic neuropathy, retrospective clinical studies including ophthalmologic examination, imaging study, and molecular biologic analyses were performed on 322 patients with optic neuropathy. The causes include hereditary optic neuropathy(71 patients, 22.1%), optic neuritis(66 patients, 20.5%), traumatic optic neuropathy(40 patients, 12.5%), ischemic optic neuropathy(35 patients, 10.9%), compressive optic neuropathy(31 patients, 9.6%), toxic optic neuropathy(23 patients, 7.1%), etc. In 29 patients of bilateral optic atrophy and 18 patients of unilateral optic atrophy, the causative mechanism was not clear. In conclusion, hereditary optic neuropathy was the most common causative mechanism of optic neuropathy in this study. The importance of meticulous history taking and molecular biologic test should be stressed in differential diagnosis of optic neuropathy.
Diagnosis, Differential ; Humans ; Optic Atrophy ; Optic Nerve Diseases* ; Optic Nerve Injuries ; Optic Neuritis ; Retrospective Studies

In order to investigate causative mechanisms of optic neuropathy, retrospective clinical studies including ophthalmologic examination, imaging study, and molecular biologic analyses were performed on 322 patients with optic neuropathy. The causes include hereditary optic neuropathy(71 patients, 22.1%), optic neuritis(66 patients, 20.5%), traumatic optic neuropathy(40 patients, 12.5%), ischemic optic neuropathy(35 patients, 10.9%), compressive optic neuropathy(31 patients, 9.6%), toxic optic neuropathy(23 patients, 7.1%), etc. In 29 patients of bilateral optic atrophy and 18 patients of unilateral optic atrophy, the causative mechanism was not clear. In conclusion, hereditary optic neuropathy was the most common causative mechanism of optic neuropathy in this study. The importance of meticulous history taking and molecular biologic test should be stressed in differential diagnosis of optic neuropathy.
Diagnosis, Differential ; Humans ; Optic Atrophy ; Optic Nerve Diseases* ; Optic Nerve Injuries ; Optic Neuritis ; Retrospective Studies

PURPOSE: We report an unusual case of Leber hereditary optic neuropathy presenting with optic disc hyperfluorescence. CASE SUMMARY: A 17-year-old male with sequential painless visual loss 3 weeks apart affecting first the left and then the right eye presented to our neuro-ophthalmology clinic. His best-corrected visual acuity was counting fingers in the right eye and 0.32 in the left eye. Fundus examination showed mild optic disc edema and hyperemia in both eyes, which were worse in the right eye. Fluorescein angiography revealed dye leakage from the right optic disc in the late phase. The results of magnetic resonance imaging of the brain and spinal cord were normal, and lumbar puncture study was unremarkable. Mitochondrial DNA sequencing revealed a pathognomonic 11778 mutation for Leber hereditary optic neuropathy. His vision deteriorated to 0.03 in both eyes 6 months later, but slowly started to improve 11 months after onset. At 2 years, his corrected visual acuity was 0.2 in both eyes. CONCLUSIONS: To our knowledge, this is the first report of optic disc hyperfluorescence in Leber hereditary optic neuropathy. This finding suggests that this mitochondrial optic neuropathy can masquerade as optic neuritis.
Adolescent ; Brain ; DNA, Mitochondrial ; Edema ; Fingers ; Fluorescein Angiography ; Humans ; Hyperemia ; Magnetic Resonance Imaging ; Male ; Optic Atrophy ; Optic Atrophy, Hereditary, Leber ; Optic Nerve Diseases ; Optic Neuritis ; Spinal Cord ; Spinal Puncture ; Visual Acuity

OBJECTIVE: Compared with other neural structures, optic apparatus are particularly sensitive to radiation. If tumors are adjacent to or in contact with optic apparatus, a number of limitations need to be addressed for planning radiosurgery. To avoid radiation induced optic neuropathy, we treated these lesions with fractionated stereotactic radiosurgery (FSRS). This study is undertaken to assess the efficacy of FSRS for sella and parasella tumors adjacent to or in contact with optic apparatus. METHODS: We treated 19 sellar region tumors located adjacent to or in contact with optic apparatus with fractionated stereotactic radiosurgery using the Novalis system. Seventeen patients who could be followed were included in this study. They consisted of 8 pituitary adenomas, 4 optic gliomas, 3 meningiomas, 2 craniopharyngiomas. The mean tumor volume was 16.1cc(range 1-61.1). When planning FSRS, the prescribed fractionation dose to optic apparatus below 200cGy. Follow up examinations consisted of neurological, neuroradiological, and neuroopthalmological evaluations. RESULTS: Follow-up ranged from 2 to 34 months(mean 15 months). Serial magnetic resonance imaging revealed no increase in volume of tumor in all 17 patients. No patients had radiation induced optic neuropathy. CONCLUSION: Fractionated stereotactic radiosurgery is proper innovative treatment modality for sella or parasella tumors adjacent to or in contact with optic apparatus to avoid radiation induced optic neuropathy. We need further follow-up and clinical experiences.
Craniopharyngioma ; Follow-Up Studies ; Humans ; Magnetic Resonance Imaging ; Meningioma ; Optic Nerve Diseases ; Optic Nerve Glioma ; Pituitary Neoplasms ; Radiosurgery ; Tumor Burden

Leber's hereditary optic neuropathy(LHON) is an optic nerve disease that causes blindness and is associated with maternally inherited mitochondrial DNA(mt DNA) mutations. The most common mitochondrial DNA mutation among LHON patients is a point mutation at the nucleotide 11778 in the subunit 4 of complex I. In one 45-year old male LHON patient with bilateral optic neuropathy, we investigated the presence of a point mutation of mitochondrial DNA and identified a single guanine to adenine transition mutation in the mitochondrial DNA at nucleotide point 11778.
Adenine ; Blindness ; DNA, Mitochondrial* ; Guanine ; Humans ; Male ; Middle Aged ; Optic Atrophy, Hereditary, Leber ; Optic Nerve Diseases ; Point Mutation*

PURPOSE: To assess the correlation between retinal nerve fiber layer (RNFL) thickness measured by optical coherence tomography (OCT, Cirrus HD-OCT®) and visual acuity in optic neuritis, ischemic optic neuropathy and traumatic optic neuropathy. METHODS: Thirty-eight patients were recruited. RNFL thickness and visual acuity in optic neuritis, ischemic optic neuropathy and traumatic optic neuropathy were measured at least 6 months after the event. The correlation between log MAR best-corrected visual acuity (BCVA) and retinal nerve fiber thickness in each quadrant was analyzed. RESULTS: log MAR BCVA and RNFL thickness of each quadrant in optic neuropathy exhibited a statistically significant correlation. In optic neuritis, RNFL thickness of the superior quadrant was significantly thicker than in ischemic optic neuropathy and traumatic optic neuropathy (p = 0.009, 0.003). In addition, RNFL thickness of the inferior quadrant in optic neuritis was significantly thicker than in traumatic optic neuropathy (p = 0.012). CONCLUSIONS: There was a statistically significant correlation between log MAR BCVA and RNFL thickness by OCT in patients with optic neuropathies. The RNFL thickness may predict visual acuity after an optic neuropathy attack and help to differentiate malingering patients with impaired vision loss.
Humans ; Malingering ; Nerve Fibers* ; Optic Nerve Diseases* ; Optic Nerve Injuries ; Optic Neuritis ; Optic Neuropathy, Ischemic ; Retinaldehyde* ; Tomography, Optical Coherence ; Visual Acuity*