A 37-year-old primigravida in her second trimester presented with bilateral painless progressive visual loss. Her vision was hand motion in both eyes. Both pupils were dilated with sluggish reaction to light. Both fundus appeared myopic with bilateral optic atrophy. Magnetic resonance imaging (MRI) of the brain revealed a suprasellar mass with optic chiasm compression and bilateral optic nerve atrophy. As the mass has compromised her vision, a semiemergency craniotomy and excision of tumour was performed. Histopathological examination confirmed the diagnosis of low grade meningothelial meningioma. Both mother and foetus were well after the surgery. However, post-operatively her vision remained poor due to optic nerve atrophy.
Optic Atrophy ; Pregnancy

The authors have experienced with three cases of Leber's hereditary optic atrophy one family which is a relatively rare condition characterized by acute or subacute failrure of central vision presenting as a retrobulbar neuritis or optic atrophy typically inypung males in late teens or in the early twenties, though the age range is very wide. The literature relating to Leber's hereditary optic atrophy was briefly reviewed.
Adolescent ; Humans ; Male ; Optic Atrophy ; Optic Atrophy, Hereditary, Leber* ; Optic Neuritis

The Leber's hereditary optic neuropathy, which affects mainly males in the late teens or in the early twenties, is a rare inherited disorder characterized by bilateral rapid loss of central vision. Leber's disease undergoes like optic neuritis in acute stage, but in late stage it results in optic atrophy with severe impairment of visual acuity and absolute central scotoma. Recently the authors have experienced two cases of Leber's optic neuropathy in a family. We observed a patient whose visual acuity of right eye was 0.8 at first examination, but reduced to 0.04 by 2 months after onset in spite of medical treatment, So we described the characteristic clinical findings of Leber's disease with brief review of the literatures.
Adolescent ; Humans ; Male ; Optic Atrophy ; Optic Atrophy, Hereditary, Leber* ; Optic Neuritis ; Scotoma ; Siblings* ; Visual Acuity

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

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

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

The pattern reversal visual evoked potential(PRVEP) and flash electroretinogram(flash ERG) were performed in 22 patients with optic atrophy. Patients with ophthalmologic problems other than optic atrophy or with systemic disorders were excluded from the analysis The results are as follows: 1. In the 41 eyes of patients with optic atrophy, 39 of them showed abnormal PRVEP, in which all the eyes had no consistent waveform except in one patient 2 eyes with delayed P1 latency. 2. 13 eyes were abnormal in both PRVEP and flash ERG but no eye was abnomnal in flash ERG only 3, Regarding the flash ERG examination, 13 eyes were abnomlal. Of these, there was a period of 1 to 2 years for 1 eye's disease, a period of 2 to 5 years for another eye' disease and after 5 years 11 eyes were diseased. Therefore, it showed that the longer the duration of disease lasted, the more flash ERG abnormalities developed. 4. The abnormalities of PRVEP haxe no significant relationship with the duration of the disease.
Evoked Potentials, Visual* ; Humans ; Optic Atrophy*

Juvenile retinoschisis is a vitreoretinal dystrophy with X-linked recessive mode of transmission that shows microcystic degeneration of the macula associated with splitting of the sensory retina, predominantly within the nerve fiber layer. We experienced X-linked juvenile retinoschisis, in four borthers within a family in which were the onset of visual disturbance between second decades and third decades, and all showed maculopathy, RPE atrophy, vitreous veils extended to vitreous, partial optic atrophy, specific electroretinographic findings.
Atrophy ; Humans ; Nerve Fibers ; Optic Atrophy ; Retina ; Retinoschisis* ; Siblings*