1/9. Bilateral optic nerve atrophy in myotonic dystrophy.PURPOSE: To document a case of bilateral optic atrophy in a patient with myotonic dystrophy. myotonic dystrophy is an autosomal dominant disorder, genetically resulting from an expansion of an unstable CTG repeat in the 3'-untranslated region of a protein kinase gene (DMPK) on chromosome 19q13.3. methods: Case report, clinical examination, fundus photographs, visual fields, visual evoked potentials, electroretinograms, and genetic studies of a 56-year-old woman clinically diagnosed with myotonic dystrophy. RESULTS: The patient experienced decreased vision consisting of light perception with the right eye and 20/25 with the left. Fundus examination showed bilateral pallor of the optic disks. intraocular pressure was normal. Visual field testing, visual evoked potentials, and electroretinogram were abnormal. A pathologic CTG expansion in the myotonic dystrophy gene was found. CONCLUSIONS: In a patient with myotonic dystrophy, confirmed with genetic molecular diagnosis, bilateral optic atrophy was present. optic atrophy should be considered a possible complication of myotonic dystrophy.- - - - - - - - - - ranking = 1keywords = perception (Clic here for more details about this article) |
2/9. arachnoid cyst of the optic nerve: a case report.BACKGROUND: To report a case of an arachnoid cyst of the optic nerve with homonymous optic atrophy. history AND SIGNS: A 17-year-old female patient presented with severe visual loss, limited to light perception, of the left eye. She underwent complete ophthalmologic examination, color fundus photography and electrophysiological study. magnetic resonance imaging (MRI) revealed the presence of a fusiform structure involving the left optic nerve from the orbital apex to the posterior surface of the globe. The clinical and radiographic features are consistent with an arachnoid cyst of the optic nerve. THERAPY AND OUTCOME: The patient refused to be treated. Two years later, the VA remained unchanged. CONCLUSIONS: The arachnoid cyst of the optic nerve is a rare entity. Its differential diagnosis from the primary optic nerve glioma is difficult and important to be made in order to be treated properly.- - - - - - - - - - ranking = 1keywords = perception (Clic here for more details about this article) |
3/9. optic atrophy and macular degeneration as initial presentations of subacute sclerosing panencephalitis.PURPOSE: To report a case of subacute sclerosing panencephalitis in which the initial clinical presentations were optic atrophy and macular degeneration. DESIGN: Observational case report. methods: A 9-year-old boy with visual loss attributable to optic atrophy and macular degeneration had a diagnostic examination. RESULTS: The visual acuity levels of both eyes were light perception at presentation. Fundus examination revealed bilateral optic atrophy and macular degenerative changes including retinal pigment epithelial atrophy, macular scarring, and epiretinal membrane formation. The antimeasles IgG titers were high both in serum and cerebrospinal fluid samples. Neurologic symptoms of myoclonus, ataxia, motor, and mental disabilities occurred 4 months after the onset of first visual disturbances and 2 weeks after the patient applied to our clinic. CONCLUSION: Children with optic atrophy and macular degenerative lesions should be examined for sclerosing panencephalitis, even if the classical neurologic symptoms of this disease are not present.- - - - - - - - - - ranking = 1keywords = perception (Clic here for more details about this article) |
4/9. Bilateral visual loss in craniodiaphysial dysplasia.PURPOSE: To report a rare case of craniodiaphysial dysplasia (CDD) that resulted in a profound loss of vision in both eyes. DESIGN: Observational case report. methods: A 2-year-old girl presented with midfacial anomaly. Marked thickening and sclerosis in the calvaria and facial bones were detected on the plain x-rays, which were compatible with CDD. Two years later, she visited our clinic because of visual loss in both eyes. RESULTS: The visual acuity was light perception in both eyes. The optic disk swelling with temporal pallor was observed in her both eyes. Orbital computed tomography scan revealed near-total obliteration of the optic canal in both eyes. CONCLUSIONS: CDD is a severe bone disorder characterized by massive generalized hyperostosis and sclerosis, especially involving the facial bones. Bony encroachment on the cranial foramina causes optic nerve compression, and this may lead to progressive visual impairment and ultimately to blindness.- - - - - - - - - - ranking = 1keywords = perception (Clic here for more details about this article) |
5/9. Sarcomatoid carcinoma and orbital apex syndrome.PURPOSE: To report a case of sarcomatoid carcinoma and orbital apex syndrome in a previously healthy adult. methods: A previously healthy 45-year-old man presented with exophthalmos of the left eye and a mass visible through his left nostril. A biopsy was performed and immunohistochemistry was used to confirm the diagnosis of the tumor. The patient was treated with chemotherapy and radiotherapy. RESULTS: After treatment, we observed a substantial reduction in the size of the mass, but side effects of treatment developed. The visual acuity of the left eye was no light perception. Eight months later, the patient presented with bone and liver metastases, and he died 4 months later. CONCLUSIONS: Sarcomatoid carcinoma is an aggressive tumor that can produce compressive symptoms with very poor visual and survival prognoses. A cranio-orbital computed tomography scan should be performed when patients present with symptoms of conjunctivitis and orbital apex syndrome.- - - - - - - - - - ranking = 1keywords = perception (Clic here for more details about this article) |
6/9. adult-onset cyclic esotropia and optic atrophy.A 37-year-old man developed periodic esotropia with 4-day cycles. He was unique because of his age, negative family history for strabismus, bilateral optic atrophy, and absence of light perception in the deviating eye. adult-onset cyclic esotropia in a blind eye is reported. This afferent defect may lead to secondary changes in the central nervous system, and fusional potential is not necessary for cyclic esotropia to occur.- - - - - - - - - - ranking = 1keywords = perception (Clic here for more details about this article) |
7/9. Human pattern-evoked retinal responses are altered by optic atrophy.Electrical signals in response to both diffuse flashes of light and phase-alternating spatial patterns were recorded from the eyes and from the occipital scalp of a subject with a traumatic unilateral (right) optic nerve section. Clinical examination disclosed a grossly normal right eye with no light perception, a Marcus Gunn pupil, and an atrophic optic disc. The left eye was normal. The electroretinogram responses on the lesioned side (OD) were normal, but there was no pattern-evoked retinal response (PERR) and no recordable visual-evoked response present. The conclusion is that the optic nerve and ganglion cells appear to be selectively responsible for the PERR potential.- - - - - - - - - - ranking = 1keywords = perception (Clic here for more details about this article) |
8/9. Posterior ischemic optic neuropathy.3 patients (5 eyes) with posterior ischemic optic neuropathy (PION) are presented. PION is a distinct clinical entity, caused by ischemia of the posterior part of the optic nerve. These eyes presented with a visual acuity varying from normal to no light perception, optic nerve-related visual field defects, and no fundus abnormality (on ophthalmoscopy or fluorescein fundus angiography) at the onset of the disease and for about a month thereafter, but after about 5-6 weeks the optic disc developed atrophic changes. The pathogenesis, differential diagnosis and possible management of PION are discussed.- - - - - - - - - - ranking = 1keywords = perception (Clic here for more details about this article) |
9/9. Atrophy of myelinated retinal nerve fibers after acute optic neuropathy.A 45-year-old man with extensive myelinated retinal nerve fibers had an attack of anterior ischemic optic neuropathy. Serial fundus photographs documented the loss of myelin. visual acuity in the affected eye decreased to 6/90 (20/300) and color perception was markedly reduced. The visual fields in the affected eye showed dense superior and inferior arcuate scotomas and central depression. These findings were unchanged nine months later, but the areas of myelinated nerve fibers had become smaller.- - - - - - - - - - ranking = 1keywords = perception (Clic here for more details about this article) |