Subclinical optic neuropathy in multiple sclerosis. How early VER components reflect axon loss and conduction defects in optic pathways.
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The pathological effect of multiple sclerosis in the visual pathways consists of axonal demyelination and axonal loss. These two consequences of the disease, even in its subclinical stages, are reflected in changes in the initial component of the visual evoked response (VER) affecting its latency, configuration, or both. These abnormal early components of the VER were recorded in 25 patients with multiple sclerosis, only 10 of whom had any indication of visual involvement that could be documented historically or by conventional ophthalmic investigation. (+info)
A family with apparently sex-linked optic atrophy.
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A family is described in which a probable new form of sex-linked optic atrophy was found in eight individuals. Some additional neurological abnormalities were noted. Results of studies of the Xg blood group excluded close linkage between the optic atrophy and Xg genes. As a probable coincidence, Huntington's chorea was found in a side branch of the family. (+info)
Menkes' kinky hair disease: a light and electron microscopic study of the eye.
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Light and electron microscopic studies of the ocular tissue of a case of Menkes' kinky hair disease are described. The copper deficiency responsible for this systemic and neurologic disease appears to cause a progressive degeneration of retinal ganglion cells, loss of nerve fibers, and optic atrophy. The pigment epithelium is also abnormal with only small and irregular melanin granules present among electron-dense inclusion bodies. Abnormal elastica is present in Bruch's membrane. (+info)
Quantitative analysis of axonal loss in band atrophy of the optic nerve using scanning laser polarimetry.
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AIMS: To measure axonal loss in patients with band atrophy from optic chiasm compression using scanning laser polarimetry (GDx, Laser Diagnostic Technologies, Inc, San Diego, CA, USA) and to evaluate the ability of this instrument to identify this pattern of retinal nerve fibre layer (RNFL) loss. METHODS: 19 eyes from 17 consecutive patients with band atrophy of the optic nerve and permanent temporal hemianopia due to chiasmal compression, and 19 eyes from an age and sex matched control group of 17 healthy individuals were prospectively studied. All patients were submitted to an ophthalmic examination including Goldmann perimetry and evaluation of the RNFL using scanning laser polarimetry. Mean RNFL thickness around the optic disc were compared between the two groups. The diagnostic performance of the deviation from normal analysis provided by the GDx software was also assessed. RESULTS: The peripapillary RNFL thickness (mean (SD)) of eyes with band atrophy was 47.9 (7.63) micro m, 37.1 (8.48) micro m, 57.0 (9.31) micro m, and 37.2 (8.86) micro m in the superior, temporal, inferior, and nasal regions, respectively. The total average was 43.7 (12.0) micro m. In the control group, the corresponding values were 71.1 (12.2) micro m, 40.4 (10.9) micro m, 85.4 (14.0) micro m, and 49.8 (10.1) micro m. The total average measured 67.9 (11.2) micro m. The measurements from eyes with optic atrophy were significantly different from those in the control group in all regions but the temporal. The deviation from normal analysis provided by the GDx software failed to identify the majority of abnormalities in the temporal and nasal regions of patients with band atrophy. CONCLUSIONS: Scanning laser polarimetry was able to identify axonal loss in the superior, inferior, and nasal regions, but failed to detect it in the temporal region of the optic disc, despite the fact that this area was clearly altered in eyes with band atrophy. This examination also showed poor sensitivity to detect axonal loss in the nasal region when GDx software analysis was used. The results of this study emphasise that RNFL evaluation using scanning laser polarimetry should be interpreted with caution in the study of eye diseases that lead to axonal loss predominantly in the nasal and temporal areas of the optic disc. (+info)
Influence of cilioretinal arteries on neuroretinal rim and parapapillary atrophy in glaucoma.
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PURPOSE: The pattern of neuroretinal rim loss and increase in the area of parapapillary atrophy in glaucoma depend on the localization of the central retinal vessel trunk in the lamina cribrosa. The purpose of the present study was to determine whether, in a similar way, the pattern of rim loss and progression of parapapillary atrophy are influenced by the presence and position of cilioretinal arteries. METHODS: Color stereo optic disc photographs (15 degrees) for morphometric evaluation of the optic nerve head were used to compare the appearance of the optic disc in 41 patients exhibiting unilateral or bilateral cilioretinal arteries in the temporal horizontal disc region with the optic disc morphology of 127 patients without cilioretinal arteries. The areas of the neuroretinal rim and alpha and beta zones of parapapillary atrophy were measured in the total disc and in four disc sectors. RESULTS: Eyes with and eyes without cilioretinal arteries did not differ significantly in the areas of neuroretinal rim and alpha and beta zones of parapapillary atrophy, when measured in the whole optic disc and in the four disc sectors separately; in ratios of the temporal horizontal area to total area of rim and parapapillary atrophy; and in the ratio of temporal horizontal rim area-to-nasal rim area, neither in an interindividual comparison nor in an intraindividual intereye comparison. CONCLUSIONS: In contrast to the position of the central retinal vessel trunk, presence and position of cilioretinal arteries do not markedly influence the pattern of neuroretinal rim loss and progression of parapapillary atrophy in glaucoma. (+info)
Scleroderma, stroke, optic neuropathy: a rare association.
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A known case of scleroderma presented with right hemiparesis, focal seizures, optic atrophy and gangrene of digits. There was no evidence of peripheral nerve or muscle involvement. MRI showed multifocal infarcts in both cerebral hemispheres. MR angiography revealed poor flow in bilateral carotid arteries with collateralization from posterior circulation. She improved with phenytoin, nifedipine, antibiotics and immunosuppressants. The rarity of central nervous system affliction in scleroderma and large vessel vasculitis is discussed along with review of literature. (+info)
Incidence of blindness in southern Germany due to glaucoma and degenerative conditions.
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PURPOSE: To estimate population-based incidence rates of registered blindness separately, to determine its main causes. METHODS: The files of all newly registered blindness-allowance recipients in Wurttemberg-Hohenzollern, Germany (population: approximately 5 million), between 1994 and 1998 were reviewed. From ophthalmological reports on file the fulfillment of the German criteria for blindness (visual acuity of 1/50 or less or equivalent reduction of visual function) was ascertained, and the causes of blindness were obtained. Incidence rates of blindness due to macular degeneration, glaucoma, cataract, optic atrophy, and diabetic retinopathy were estimated. RESULTS: There were 3531 newly registered blindness-allowance recipients (67.1% female; mean age, 72.8 +/- 21.0 years). Standardized incidence rates in the general population (per 100,000 person-years; 95% confidence interval): All causes 12.27 (11.87-12.68), macular degeneration 5.29 (5.02-5.55), cataract 3.32 (3.11-3.52), optic atrophy 2.86 (2.66-3.05), glaucoma 2.43 (2.25-2.61), diabetic retinopathy 2.13 (1.96-2.30), other or unknown causes 5.17 (4.91-5.43). In many cases, blindness was attributable to more than one cause. Assuming that incidence rates are the same in other parts of the country, 9,939 (9,608-10,270) new cases of blindness were estimated to occur in Germany per year. CONCLUSIONS: The most common single cause of blindness was macular degeneration. Incidence rates of blindness due to such treatable conditions as glaucoma were also high. This finding suggests that the taking of measures for secondary prevention (e.g., early detection and optimal treatment of patients with glaucoma and diabetic retinopathy) should be intensified. (+info)
Morphology of posterior segment lesions of the eye in patients with onchocerciasis.
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Posterior segment ocular lesions were examined in 244 selected patients with onchocerciasis from the Sudan-savanna and rain-forest areas of the United Cameroon Republic. Fluorescein fundus angiography was performed on 210 cases. As a result of this study the following conclusions were drawn: 1. The heterogeneity of single lesions, and the occurrence of different types of lesion in a single eye or two eyes, prevented subdivision of the disease into distinct forms and suggested that inflammation alone was responsible for fundus lesions in onchocerciasis. 2. Optic nerve disease, alone or in the presence of choroido-retinal changes, was responsible for a large proportion of the blindness due to posterior segment lesions in onchocerciasis (87.6 per cent in this series). (+info)