1/8. optic disk pallor and retinal atrophy in sotos syndrome (cerebral gigantism).PURPOSE: To report a case of sotos syndrome (cerebral gigantism) with megalophthalmos, optic disk pallor, and retinal atrophy. methods: Case report. RESULTS: A 22-year-old man was diagnosed with sotos syndrome with optic atrophy. His corneal diameters were 13 mm horizontally, and his optic disks were pale. His retinal vessels were attenuate in diameter, and small white spots were found on the retina. The results of a Goldmann visual field test were normal. His visually evoked potential to flash stimuli showed extended latent times and normal amplitudes. Keratometry was 40.5 diopters (8.33 mm) in both eyes. The axial length was 25.9 mm in the right eye and 25.4 mm in the left eye. CONCLUSION: sotos syndrome may be associated with optic disk pallor and retinal atrophy.- - - - - - - - - - ranking = 1keywords = pallor (Clic here for more details about this article) |
2/8. Development of atrophy of the retinal pigment epithelium around disciform scars.BACKGROUND/AIMS: Eyes with burnt out disciform scars secondary to age related macular degeneration (AMD) are regarded as visually stable. The aim of this study is to report the subsequent development of atrophy of the retinal pigment epithelium (RPE) around the scars and discuss the possible basis. methods: 20 eyes from 18 patients were observed to develop atrophy around choroidal neovascularisation (CNV). A method of measuring expansion of the atrophy over time is described using the Topcon Imagenet 2000 system. An additional 10 clinicopathological examples were reviewed. RESULTS: Clinically CNV became surrounded initially by a ring of pallor that progressed to an expanding band of atrophy of the RPE. It developed most rapidly in the first 3 years after CNV became quiescent but then continued to expand slowly to more than three times the size of the scar. Histopathological specimens showed large choroidal vessels entering the scars directly and a reduced number of small choroidal vessels beneath and around the scar CONCLUSIONS: Disciform scars may become surrounded by an expanding band of atrophy of the RPE, postulated to result from remodelling of the choroidal circulation. The ongoing enlargement of the resulting scotoma may need to be considered when planning management and assessing treatment outcomes.- - - - - - - - - - ranking = 0.16666666666667keywords = pallor (Clic here for more details about this article) |
3/8. The AB-variant of GM2-gangliosidosis. Clinical, biochemical, and pathological studies of two patients.Clinical, neuropathological, and biochemical studies are reported in two children with the AB-variant of GM2-gangliosidosis. One patient had become symptomatic by 1--1.5 years, initially showing cerebellar signs, and then progressive psychomotor retardation, with hypotonia, spasticity, dementia, and macular cherry red spots, until death at the age of 4.5 years. The second patient showed an earlier onset of retardation and a more rapidly progressive course. At postmortem, the brains were of normal or near normal weights and displayed grossly only mild cerebral cortical and cerebellar atrophy, and mild pallor or attenuation of the white matter. Neuronal storage was widespread throughout the CNS, and both neurons and glia contained a variety of abnormal, membranous inclusions. Visceral organs were not involved. Ganglioside sialic acid was increased several fold in gray matter, with GM2 the predominant ganglioside species. N-acetyl-beta-glucosaminidase activities in serum, leukocytes, fibroblasts, and postmortem gray matter, assayed with an artificial, fluorogenic substrate, were normal, as were activities of other lysosomal hydrolases.- - - - - - - - - - ranking = 0.16666666666667keywords = pallor (Clic here for more details about this article) |
4/8. Ocular manifestations of congenital muscular dystrophy (Fukuyama type).Ocular manifestations in two cases of congenital muscular dystrophy of Fukuyama type were reported. This disease is characterized by early onset of hypotonia, generalized muscle weakness and atrophy, mental retardation, and elevated serum creatine-phosphokinase activity. The symptoms include entropion of lower lids, pathological myopia with astigmatism, optic nerve pallor, and irregular grayish subretinal mottling. Case 1 showed additional features of posterior staphyloma, dragged papillomacular vessels, peripheral grayish-white discoloration of the retina, and rete mirabile as well as abnormal vascular anastomosis.- - - - - - - - - - ranking = 0.16666666666667keywords = pallor (Clic here for more details about this article) |
5/8. An autopsy case of atypical senile dementia with atrophy of the temporal lobes--a clinical and histopathological report.A man aged 70, descendant of an apparently healthy family, showed disorientation, delusional ideas and rages at 66. Later there was slowly advancing deterioration with muteness, disorientation and dysphagia. He died of cardiac failure. There was the diffuse atrophy of the cerebrum, which was remarkably accentuated on both temporal lobes (poles, T2 and T3), where the loss of nerve cells and proliferation of astrocytes were found in the cortex and pallor and conspicuous fibrillary gliosis were noted in the white matter. These findings fundamentally suggest Pick's disease. On the one hand, numerous senile plaques and Alzheimer's neurofibrillary changes, suggestive of Alzheimer's disease (senile dementia), were observed throughout the cerebral cortex. On the other hand, a few inflated cells were also seen in the cingulate, superior frontal gyri and temporal lobes. basilar artery was moderately atherosclerotic and cerebrovascular disorders were distributed throughout the cerebral cortex and basal ganglia, especially in the field of supply of middle cerebral artery. This case is similar to rare cases reported by berlin (1949), Neumann (1949) and Oyanagi et al. (1975). The nosological situation as a disease entity remains to be determined.- - - - - - - - - - ranking = 0.16666666666667keywords = pallor (Clic here for more details about this article) |
6/8. Neuro-Behcet disease with demyelination and gliosis of the frontal white matter.A rare case of neuro-Behcet disease with diffuse demyelination and gliosis of the frontal white matter is reported clinico-pathologically. The disease began with genital ulcer and recurrent oral aphthosis when the patient was 42 years of age. There was erythema, moderate fever, CSF (cerebrospinal fluid)-pleocytosis and elevated CSF-globulin. He was diagnosed as having neuro-Behcet disease and treated with prednisolone. He gradually became euphoric, disinhibited, indifferent and demented. His cranial CT showed diffuse low density areas in the bilateral frontal white matter. He became bedridden, akinetic mute and died from respiratory dysfunction 3 1/2 years after onset. The following neuropathological findings were observed: 1) Moderate demyelination and gliosis was present mainly in the frontal and parietal white matter. 2) There were many micro-spongious necrotic foci in the gray and white matters of the cerebrum, basal ganglia, thalamus, midbrain and pons, some of which were accompanied by gliosis. 3) From 1/2 to 1/3 of all micro-necrotic foci in the frontal white matter were old and accompanied by gliosis. The white matter containing numerous micro-necrotic foci had myelin pallor and gliosis. 4) There was neither micro-necrosis nor gliosis in the occipital lobe. The pathogenetic correlation of white matter lesions with primary and secondary circulatory disturbances is discussed.- - - - - - - - - - ranking = 0.16666666666667keywords = pallor (Clic here for more details about this article) |
7/8. Optic disc pallor: a false localizing sign.A 20-year-old pregnant woman was referred with bilateral mild visual acuity loss and optic disc pallor. Because of profound color vision deficits out of proportion to her acuity loss, an abnormality of the cone photoreceptors was suspected. An electroretinogram confirmed the diagnosis of a cone dystrophy and precluded further costly and invasive testing. Cone dystrophy should be considered in the differential diagnosis of any patient with bilateral, nonrefractive visual loss, especially if color vision is disproportionately affected, even with a normal retinal appearance and no significant family history.- - - - - - - - - - ranking = 0.83333333333333keywords = pallor (Clic here for more details about this article) |
8/8. Bilateral optic disk pallor after unilateral internal carotid artery occlusion.Bilateral pallor of the optic disks was observed in a 52-year-old man after dissection of an internal carotid artery. Diffuse pallor of the ipsilateral optic disk reflected infarction of the ipsilateral optic nerve and "bow-tie" atrophy of the contralateral optic disk reflected infarction of the ipsilateral optic tract. The findings were due to an occlusion of the internal carotid artery proximal to the origin of the ophthalmic artery, resulting also in insufficiency in the area of supply of the anterior choroidal artery.- - - - - - - - - - ranking = 1keywords = pallor (Clic here for more details about this article) |