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Results for “Optic Atrophy”

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At least 1,063 records · Page 59Linked to original sources
PubMed1986

Simultaneous recording of pattern electroretinography and visual evoked potentials in multiple sclerosis. A method to separate demyelination from axonal damage to the optic nerve.

Pattern electroretinograms (P-ERGs) and visual evoked potentials (VEPs) were recorded in 35 patients with multiple sclerosis and in 35 age-matched normal subjects. Four patterns of abnormalities were noted in the group with multiple sclerosis. The most frequent abnormality consisted of the following: normal P-ERG, delayed P100, and prolonged interpeak interval between the b-wave of the P-ERG and P100 (retinocortical time). This pattern indicates demyelination of the optic nerve. A second pattern consisted of absent P-ERG and absent VEP. This pattern was associated with optic atrophy and/or central scotoma, indicating severe optic nerve axonopathy with retrograde degeneration of ganglion cells. A third pattern consisted of normal P-ERG and absent VEP, suggesting a total block of transmission at the optic nerve. A fourth pattern consisted of present but low-amplitude P-ERG, delayed VEP, and prolonged retinocortical time, indicating a demyelinating process with partial axonal involvement. The concomitant use of P-ERG and VEP results in a better classification of the type and severity of dysfunction affecting the optic nerve. The prognostic value of the four patterns for recovery of visual function is discussed.

Electroretinography↗
PubMedDate not supplied

Posterior segment involvement in ocular Behçet's disease.

PURPOSE: Posterior segment involvement, which can lead to blindness with recurrent inflammatory attacks, has a very important prognostic value in ocular Behçet's disease (BD). This study evaluated the frequency and characteristics of posterior segment involvement and the causes of visual impairment in patients with ocular BD. METHODS: We retrospectively evaluated 257 eyes of the 131 patients who were followed from 1993 to 2001 in the Uveitis and BD division of SSK Ankara Eye Hospital, Turkey. RESULTS: The mean follow-up was 49.2 +/- 27.4 months. Thirty-one of the palents were female (23.6%) and 100 male (76.4%). The mean age was 25.1 +/- 7.9. Fundus changes were observed in 213 (82.9%) eyes and were bilateral in 87%, and vitritis was present in 239 (93%) eyes. The most frequent fundus changes were vascular sheathing in 61 eyes (23.7%), optic atrophy in 46 (17.9%), macular edema in 29 (11.3%), retinal hemorrhage in 23 (9%), macular scar in 21 (8.2%), optic disc paleness in 19 (7.4%), retinal edema in 17 (6.6%), branch retinal vein occlusion in 15 (5.8%). The most common fluorescein angiography findings were diffuse vascular leakage in 98 (38%), hyperfluorescence of the optic disc in 38 (14.8%) and hyperfluorescence of the macula in 29 eyes (11.3%). Visual acuity was <1/10 in 85 (33%) of the eyes. Optic atrophy was the main cause (54.1%) of permanent visual impairment. CONCLUSIONS: Posterior segment involvement is the most serious ocular complication of BD, leading to blindness with recurrent attacks. Following patients closely, performing fluorescein angiography in all patients diagnosed as BD even they have no clinical ocular involvement, and early treatment are very important in the prognosis of the disease.

Adolescent↗
PubMedDate not supplied

Wolfram syndrome: report of four new cases and a review of literature.

The Wolfram, or DIDMOAD, syndrome consists of diabetes insipidus, diabetes mellitus, optic atrophy, and deafness. Diabetes mellitus usually occurs as the first manifestation of this syndrome, followed by the development of optic atrophy, neurosensory hearing loss, and finally diabetes insipidus. We report on four cases with a review of the literature. The diabetes mellitus occurring in these patients is clinically indistinguishable from classic type I diabetes mellitus. Two of three patients continue to have measurable C-peptide secretion 8 yr after onset of diabetes. Two of three patients with Wolfram syndrome had the HLA-DR2 antigen. Combining our cases with those described in the literature, 7 of 11 patients have the HLA-DR2 antigen. The preponderance of the HLA-DR2 antigen in the Wolfram syndrome is different from classic type I diabetes. This is further evidence of the genetic heterogeneity of diabetes mellitus. Although the Wolfram syndrome is rare, it should be considered in diabetic patients with unexplained optic atrophy and hearing loss or with polyuria and polydipsia in the presence of adequate blood sugar control.

Adolescent↗
PubMed2006

The value of VEP in the diagnosis and post-operative monitoring of meningioma.

Meningiomas are the most frequently reported primary intracranial neoplasms. At first they usually cause unilateral visual loss but eventually result in a bilateral loss of vision. Depending upon the size and location of the tumour, the ocular signs and symptoms of meningiomas may include visual field abnormalities, optic atrophy, papilledema, diplopia and proptosis. This case report highlights the value of visual evoked potentials (VEP) in a patient with unexplained bilateral optic atrophy and a progressive loss of vision over 2 years. As a result of a delayed response in the VEP recorded from the right eye, a compressive lesion of the optic nerve was suspected. That prompted the referring ophthalmologist to request a MRI scan which led to the diagnosis of meningioma. Following the subtotal removal of the suprasellar meningioma, the remaining vision in the right eye improved and the latency of the VEP returned to the normal range. However, the VEP from the blind eye (left) did not show any measurable response either pre- or postoperatively. Experience with this patient suggests that early recognition of optic nerve compression is vital to an optimal outcome and the VEP technique, which is much more cost-effective than MRI, is clinically useful for detecting such compressive lesions.

Adult↗
PubMed1993

[Optic neuropathy in type-1 diabetes and acetylsalicylic acid-refractory thrombocyte activation].

A 26-year-old female with severe complications from type I diabetes mellitus of 17 years' duration (proliferative retinopathy, nephropathy with renal failure and nephrotic syndrome) developed rapid deterioration of vision in the right eye to 6/60 over a period of several weeks. There were no other neurological signs. Ophthalmological examination showed no worsening of the diabetic retinopathy, but the presence of bilateral optic atrophy, confirmed by visual evoked potentials. CT scan did not reveal any retrobulbar process, and MR scans of both the optic nerves and the visual pathways were unremarkable. The clinical features and the investigations pointed towards ischaemic optic atrophy. Detailed platelet studies showed intravascular platelet activation and an ADP-inducible increase in aggregation, although thromboxane formation was almost absent because of cyclooxygenase inhibition by acetylsalicylic acid. These findings suggest that the ischaemia was due to microcirculatory disturbances secondary to diabetic microangiopathy and platelet hyperreactivity.

Adult↗
PubMed1990

[Differential diagnosis of optic nerve atrophy].

Diseases of the visual pathway: early detection and therapy. Manual kinetic perimetry is faster in the detection of lesions of the visual pathway than automated static perimetry. When cases with a comparable reduction of visual acuity are considered, color desaturation is more pronounced in lesions of the visual pathway than in opacities of the optic media. Search for an afferent pupil defect allows the differential diagnosis between functional and unilateral organic visual impairment: in the case of bilateral visual impairment, a combination of VEP and pattern ERG is suitable for this differentiation. In chronic papilledema, secondary atrophy can best be detected in the arcuate retinal nerve fibers. Fistulation of the dural sheath of the optic nerve prevents visual loss in chronic papilledema. A similar operation could be useful in the progressive form of anterior ischemic optic neuropathy. Recently, the mitochondrial DNA mutation associated with Leber's hereditary optic neuropathy was identified. Prolactin secreting pituitary adenomas shrink with bromocriptine treatment.

Color Perception↗
PubMedDate not supplied

Ophthalmic findings in GAPO syndrome.

BACKGROUND: The main manifestations of GAPO syndrome are growth retardation (G), alopecia (A), pseudoanodontia (P), and optic atrophy (O). CASES: This syndrome has been described in 21 patients from 16 different families. Four cases are from Turkey and have been presented by Sayli and Gül. The purpose of our study is to document the cases from Turkey and discuss the ophthalmological and neuro-ophthalmolgical findings of these and other reported GAPO cases. OBSERVATIONS: All patients in the literature and our 4 cases have severe growth retardation with delayed bone age in infancy, characteristic facial appearance (high and bossed forehead, midface hypoplasia), alopecia or severe hypotrichosis, and pseudoanodontia. Optic atrophy was present in 1 of our cases and in 5 previous cases. Glaucoma was present in 5 cases, including 2 of ours. Buphthalmia and keratopathy secondary to glaucoma were also observed. White eyelashes, seen only in our cases, may be a sign of "early senility." CONCLUSIONS: Optic atrophy is not a constant finding in GAPO syndrome. Glaucoma may accompany the ocular findings. This syndrome has been attributed to either ectodermal dysplasia or the accumulation of extracellular connective tissue matrix, due to an enzyme deficiency involved in its metabolism. Current studies show that an elastin defect and secondary changes in collagen may be important in the pathogenesis of the disease.

Abnormalities, Multiple↗
PubMed1988

Onchocerciasis in Sierra Leone 3: Relationships between eye lesions and microfilarial prevalence and intensity.

The relationship between severe onchocercal eye lesions (iritis, sclerosing keratitis, optic atrophy and choroidoretinitis) and (i) the prevalence and intensity of microfilariae (mf) of Onchocerca volvulus in skin snips from the iliac crest and outer canthus, and (ii) the prevalence of mf in the cornea and anterior chamber of the eye, was studied in 1414 persons from forest and savanna villages and 312 attenders at eye clinics. Ecologically the savanna of Sierra Leone more closely resembles the forest than the dry Sudan-savanna areas of West Africa, and in persons aged 30 years or more the combined prevalence of anterior segment lesions (iritis and sclerosing keratitis) was higher in the forest villages (20.6%) than in the savanna (12.7%). The higher loads of mf found in the forest compared to savanna villages could explain these results. Prevalence rates for posterior segment lesions (optic atrophy and choroidoretinitis) were 28.1% and 22.6% in the forest and savanna respectively. Although in villages from both zones there was a close association between mf in the anterior chamber and optic atrophy, other associations between posterior segment lesions and mf were either not significant or weak. In contrast, there was a strong association between anterior segment lesions and mf in the eye and the concentration of mf at the outer canthus. This association was stronger for iritis than for sclerosing keratitis.

Adolescent↗
PubMed1997

Widespread choroidal insufficiency in primary open-angle glaucoma.

PURPOSE: The purpose of this study was to investigate choroidal perfusion in glaucoma, using histological and angiographic techniques. METHODS: We examined the choroidal vasculature in clinicopathological slides from 25 cases of primary open-angle glaucoma, five cases of optic atrophy, and 18 normal eyes. We measured choroidal thickness at fixed distances from the disk margin with light microscopy. Using a quantitative computer image analysis system, we established the depth of all vessels and the best fitting diameter and width-to-length ratio for each vessel in three pairs of eyes. Separate statistical analyses were done on the parapapillary area and the whole choroid. We compared standard fluorescein angiographic measures to peak choroidal filling time in a further 78 glaucoma and 84 normal eyes. RESULTS: Choroids were significantly (approximately 50 microns) thinner in glaucoma than in normal or optic atrophy irrespective of fundal position. Vessel frequency and mean diameter, relative to normal, showed greatest decrease near the choriocapillaris. Peak choroidal filling was the only fluorescein angiographic measure that was significantly delayed in glaucoma irrespective of age. CONCLUSIONS: Reduced choroidal thickness in primary open-angle glaucoma is primarily due to loss of the innermost choroidal vessels. Overall size decreases without significant flattening. These changes are not seen with optic atrophy alone and may be correlated with the delayed choroidal perfusion seen in fluorescein angiography.

Aged↗
PubMed2006

The role of the Met98Lys optineurin variant in inherited optic nerve diseases.

AIMS: To investigate the role of the common OPTN Met98Lys variant as a risk allele in open-angle glaucoma (OAG), autosomal dominant optic atrophy (ADOA) and Leber's hereditary optic neuropathy (LHON). METHODS: The presence of the Met98Lys variant was determined in a total of 498 (128 with normal-tension glaucoma (NTG)) patients with OAG, 29 patients who had myocilin-related OAG, 101 patients from ADOA pedigrees, 157 patients from LHON pedigrees and 218 examined OAG age-matched normal controls. RESULTS: 17 of 218 (7.8%) controls had the Met98Lys variant. 28 (5.6%) patients with OAG were Met98Lys positive. More Met98Lys carriers were found in the NTG group than in the high-tension glaucoma (HTG) group (p = 0.033). However, no significant difference was observed between the NTG and control cohorts (p = 0.609). Two MYOC mutation carriers were found to have the variant. The variant was found in 1 of 10 pedigrees with ADOA and in 8 of 35 pedigrees with LHON. CONCLUSION: Data from this study do not support a strong role for the OPTN Met98Lys variant in glaucoma, ADOA or LHON. However, a weak association was observed of the variant with NTG compared with that with HTG. Meta-analysis of all published data on the variant and glaucoma confirmed that the association, although weak, is highly statistically significant in the cohort with glaucoma versus controls.

Adolescent↗