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The histologic basis of optic disk pallor in experimental optic atrophy.

We studied the clinical and microscopic appearances of the optic nerve head in squirrel monkeys with optic nerve degeneration produced by optic nerve transection at the orbital apex. The ophthalmoscopic development of optic disk pallor coincided with the loss of nerve fiber bundles and the rearrangement of the remaining disk astrocytes into dense parallel layers across the nerve head. No astrocytic mitoses were observed and the estimated volume of astrocytes increased only slightly from normal. Among the astrocytes in atrophic disks, many capillaries had patent lumens and ultrastructurally normal endothelial cells. Pallor of the optic disk seems to result from a decrease in the transmission of light into the cytoarchitecture of the atrophic nerve head, not from the absence of capillaries or from extensive astrocytic proliferation.

Animals↗

Ictal pallor is associated with left temporal seizure onset zone in children.

PURPOSE: To describe the frequency, localizing and lateralizing value of ictal pallor (IP) in children with focal epilepsy. METHODS: A retrospective review of medical charts and 514 archived seizures from 100 children < or =12 years old was performed. All patients had a history of therapy-resistant partial epilepsy and a seizure-free postoperative outcome. The presence and attributes of IP were analyzed. RESULTS: No IP was detected by reviewing the archived seizures. According to medical charts, IP was reported in 11/100 children (six girls) aged 14 months to 12 (mean 5.5+/-4.1) years. Ten of them had temporal lobe epilepsy (p=0.046) - including nine temporo-medial and one temporo-lateral cases - while only one child had an extratemporal (parietal lobe) seizure onset zone. All but one children had left-sided operation (p=0.01). Presence of IP had a positive predictive value of 91% for both lateralizing (left) and localizing (temporal lobe) the seizure onset zone. History of IP was neither age-, epilepsy onset-, nor gender-related. CONCLUSIONS: Ictal pallor is a frequently reported autonomic symptom during childhood focal seizures but difficult to assess purely by video-monitoring. It has a high predictive value to localize the seizure onset zone to the left temporal region already in very young patients.

Child↗

Increase of pallor and fluorescein-filling defects of the optic disc in the follow-up of ocular hypertensives measured by computerized image analysis.

In 24 ocular hypertensive patients, two optic disc fluorescein angiograms were obtained a mean of 3.9 years apart. Using computerized image analysis, we measured the area of pallor and the fluorescein-filling defect area within the disc and the fluorescein circulation parameters representing the filling rates of optic disc, choroid, and retinal vessels. The results indicated that increase in pallor and fluorescein-filling defect areas, as well as a slowed filling rate of the retinal veins, correlated with glaucomatous progression.

Aged↗

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.

Aortic Dissection↗

Dyspnea on exertion, fatigue, and pallor in a 50-year-old active duty soldier.

OBJECTIVE: To discuss a comprehensive diagnostic approach to an active duty patient presenting with dyspnea on exertion, fatigue, and pallor. A 50-year-old active duty E-6 white male in Al Udeid, Qatar, presented with progressive dyspnea on exertion, fatigue, and new pallor. This case illustrates the course of events from Al Udeid to the Walter Reed Army Medical Center, where the final diagnosis was made. Along the way, questions with discussions explore the various diagnostic and management aspects of his case and highlight military relevant issues that include efficient diagnostic algorithms in the field and transfusion of scarce blood products.

Algorithms↗

The mechanism of disc pallor in experimental optic atrophy. A fluorescein angiographic study.

Ascending optic atrophy was produced in 13 eyes of owl monkeys (Aotestrivirgatus) by retinal photocoagulation. Color fundus photography and fluorescein angiography were used to study and document the evolution of nerve head abnormalities. The optic nerve heads were also studied histopathologically. Except in certain instances of early transient (relative) filling defects, normal disc fluorescent patterns were preserved, despite clinically apparent optic nerve head pallor. Sectorial defects did not persist into the later phases of the angiogram. These findings may suggest a reduced blood flow, but neither angiographic nor histopathologic studies detected a reduced vascularity in the atrophic optic nerve. Pallor of the optic nerve head seems to result from alterations in the tissue reflectance and translucency following axonal loss and glial reorganization rather than from a decreased microvascular bed.

Animals↗

Development of cupping and pallor in posterior ischemic optic neuropathy.

A case of arteriosclerotic posterior ischemic optic neuropathy without optic disc edema is described and documented photographically. The development of optic disc cupping and pallor with ischemia in this patient supports the vascular basis for development of similar cupping and pallor in open angle glaucoma and low tension glaucoma.

Aged↗

Colour measurements of pallor mortis.

Little interest has yet been focused on the development of postmortem paleness (pallor mortis). Using an opto-electronical colour measurement device, we examined pallor mortis in 126 bodies and compared these findings to the average skin colour of 72 living Caucasian volunteers. It was shown that (a) hairy skin influences the results and any hair must be removed by shaving before colour determination, (b) among the living, there is a skin colour difference between the sexes which disappears after death, (c) postmortem paleness is caused by lack of capillary circulation after death and (d) paleness develops so rapidly after death that it has no or little use in determining time of death.

Adult↗

Quantitative study of optic nerve head capillaries in experimental optic disk pallor.

We obtained quantitative measurements of capillary numbers, areas, and diameters in atrophic (pale) and normal primate optic nerve heads. The number of capillaries per square millimeter in pale optic disks was not significantly different from that in normal optic disks. Because the loss of all nerve fibers leads to a 50% decrease in nerve head substance, capillaries must atrophy to maintain a constant relationship between capillary number and tissue volume. The mean size of individual capillaries in atrophic nerve heads was smaller than normal, leading to a decrease of more than 27% in the percentage of tissue volume occupied by capillaries. When this decrease in capillary volume was mimicked in the normal optic disk by reducing the hematocrit value, optic disk pallor did not result. Hence, the development of optic disk pallor appears to be the result of thinning of the neural tissue of the rim of the optic disk and the consequent change in tissue composition and optical transparency, rather than of a loss of optic disk capillaries.

Animals↗

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.

Abnormalities, Multiple↗

Pathological study of the diffuse myelin pallor in the anterolateral columns of the spinal cord in amyotrophic lateral sclerosis.

An immunohistochemical study using a monoclonal antibody against macrophage (Ki-M1p) was performed to examine which fiber tracts are affected in the spinal cords and brainstems of ALS patients. In 21 out of 30 ALS patients, various degrees of macrophage infiltration were observed diffusely in the anterolateral columns beyond the corticospinal tracts. On the other hand, a few macrophages were scattered in 20 non-ALS patients in the anterolateral columns outside the corticospinal tracts. In ALS brainstems, the macrophages were mainly localized in the corticospinal tracts. The result suggests that the diffuse myelin pallor in the anterolateral columns beyond the corticospinal tracts may be derived from intrinsic spinal cord lesions. Quantitative investigation using a monoclonal antibody against phosphorylated neurofilaments (SMI-31) revealed that the decrease in the numbers of small fibers would induce the diffuse myelin pallor in anterolateral columns of ALS patients. From these findings, we propose that the propriospinal bundles are candidates for the degenerating fibers in the anterolateral columns of ALS.

Adult↗

Comparison of computer-assisted versus manual optic nerve head pallor measurement.

Computerized methods of examining the optic nerve head offer the possibility of providing consistent, reliable, and accurate measurements of the nerve head. The reliability and accuracy of such instruments must be examined to confirm this. This research was undertaken to gain an insight into the accuracy of such a system, which takes its measurements by tracking the luminance boundaries of the pallor area and the optic nerve head after an observer marks the starting points. Measurements made using the computer-tracked boundary were compared with those made using a boundary tracked by an image-processing system of greater sophistication, the human visual system. The comparison was made with images from one eye from each of 30 subjects (10 normal, 10 ocular hypertensive, and 10 glaucomatous). The measurements were made by two observers. Each observer made each set of measurements twice, and the results were analyzed with a five-factor analysis of variance. The measurements made using the computerized method did not differ significantly from those made using the manual boundary tracking for the two observers, and they were highly correlated (r = 0.99 for the area pallor-disc ratio). Significant differences (P < 0.05) were found between the two observers in both the manual and computerized boundary tracking. The computerized method, however, did not find differences between the two sets of measurements made by each observer; the manual method did show such differences. The computerized method appeared to trace the luminance boundaries successfully in the image, and it might reduce errors related to the observer marking the starting points on different occasions.(ABSTRACT TRUNCATED AT 250 WORDS)

Analysis of Variance↗

Measurement of optic nerve head pallor with a video-ophthalmograph and with computerized boundary analysis.

Optic disc pallor was recorded in 53 eyes of 53 patients by means of global analysis with a video-ophthalmograph (Rodenstock Disk Analyzer) and computerized boundary analysis. The coefficients of correlation between the values obtained with the Rodenstock Disk Analyzer and those obtained with boundary analysis were r = 0.24 (p = 0.08) at the 0.29 cutoff level, r = 0.39 (p = 0.004) at the 0.39 level and r = 0.46 (p = 0.0005) at the 0.50 level. Although the correlation was significant, the correlation coefficients were not high. The Rodenstock Disk Analyzer uses global analysis, which does not take into account the spatial relationship of the pallor on the disc, whereas boundary analysis is sensitive to spatial relationship, which may account for the poor correlation.

Adolescent↗

Optic disk cupping and pallor measurements of patients with a disk hemorrhage.

We evaluated whether the rate of occurrence of glaucomatous disk damage in eyes with an optic disk hemorrhage differed from the rate of occurrence in control eyes. We compared 24 patients with a disk hemorrhage to 24 control patients, who had been matched for diagnosis, disk appearance, and visual fields. Cup volume and cup-to-disk area ratio of the optic disk were measured using the stereophotogrammetric technique, and area of pallor was measured using computerized image analysis. We found that diabetes increased the probability that a patient would have a disk hemorrhage, and that a hemorrhage was associated with progression of glaucomatous disk changes in half of the cases during the mean 3.1-year follow-up period. However, the mean rate of glaucomatous disk damage in patients with a hemorrhage was not different from matched control patients. A disk hemorrhage is a sign of later disk damage, but it does not alter the rate of glaucomatous disk progression.

Eye Hemorrhage↗

Pallor of the optic disc in children.

This report discusses the subject of pallor of the optic disc from the viewpoint of a paediatric neurologist. The paper is divided into two sections, one on optic atrophy in childhood and the second on optic nerve hypoplasia. Optic atrophy in children is a topic which is very poorly covered in standard textbooks either of neurology or ophthalmology. This review attempts to develop a working approach to the management of children who present with optic nerve atrophy as their primary neurological finding.

Child↗

Visual function in patients with optic nerve pallor (optic atrophy).

This cross-sectional study assessed the relationship between the degree of optic nerve pallor (optic atrophy) and visual function. Using a set of "gold standard" stereoscopic slides, the severity of optic atrophy for 270 eyes, each having sustained a bout of optic neuropathy, was graded. Good visual acuity was found in 55/86 (64.0%) mild, 54/119 (45.4%) moderate, and 21/65 (32.3%) marked optic atrophy eyes. Good visual field was found in 6/28 (21.4%) mild, 4/43 (9.3%) moderate, and 2/28 (7.1%) marked optic atrophy eyes. Good color vision was found in 31/46 (67.4%) mild, 12/62 (19.4%) moderate, and 7/31 (22.6%) marked optic atrophy eyes. A significant rank correlation was observed between optic atrophy and visual acuity (P < 0.001; rs = 0.356), visual field (P < 0.001; rs = -0.398), and color vision (P < 0.001; rs = -0.492). As the graded severity of optic atrophy increases, the proportion of eyes with good visual function decreases. Visual field, rather than visual acuity or color vision, appears to be a better indicator of the severity of visual loss, when optic atrophy is present.

Cross-Sectional Studies↗

The pathology of the posterior root ganglia in AIDS and its relationship to the pallor of the gracile tract.

The spinal cord and the thoracic and lumbar posterior root ganglia (PRGs) of 14 HIV-positive men and 7 age- and sex-matched controls were studied by routine histology, morphometric analysis of the number of nodules of Nageotte (nN) and the diameters of sensory ganglion cells, immunohistochemistry and in situ hybridization. In 7 patients (2 of whom had evidence of cytomegalovirus ganglionitis) there were increased numbers of nN and diffuse, mild infiltration with CD45R+ T lymphocytes; no B lymphocytes were observed. Macrophages were increased in number in all cases. Whenever more than one ganglion was examined from the same patient, the appearances were similar in all. There was no alteration in the distribution of ganglion cell diameters. Changes in the spinal cord included vacuolar myelopathy (5 cases), HIV myelitis (1 case), microglial nodules (3 cases) and pallor of the gracile tracts (GTP) in 7 cases, in 6 of whom it co-existed with increased numbers of nN. Seven cases had no abnormalities, except the increase in number of macrophages in PRGs. In spite of a correlation between sensory nerve cell loss and GTP our findings suggest that other mechanisms, such as 'dying back' may contribute to the pathogenesis of GTP. Moreover, sensory disturbances were found most commonly in association with nerve cell loss; however, loss of sensory ganglion cells was not necessarily associated with evidence of sensory impairment.

Acquired Immunodeficiency Syndrome↗