Biomedical subjects
Publications and source records attributed to R M Burde.
A recent case of childhood dermatomyositis with striking retinopathy is reported. The patient's vision was initially at the 20/80 to 20/100 level with associated macular edema and extensive cotton-wool infarction of the retina. Visual recovery to the level of 20/20 in both eyes was achieved following treatment with high-dose corticosteroids.
Color contrast perimetry was used to evaluate central visual field defects in a group of 28 patients with visual loss resulting from optic nerve or retinal diseases. Kinetic perimetry was performed using colored test objects of constant luminance, equated to a white surround of 10 ft lamberts. Colored test objects were varied in size and in extent of color saturation. Test object color saturation was varied from a white that matched the color and luminance of the adapting background toward either the blue or the red color maxima of a video tangent screen. All central visual field defects that were demonstrable by luminance contrast perimetry were also detected by color contrast testing, and no defects were found for color contrast detection that could not also be demonstrated by conventional luminance increment perimetry. Retinal diseases usually produced scotomas for both color and luminance contrast detection, while optic nerve disorders tended to produce global depressions of both color and luminance contrast sensitivity across the entire visual field in addition to scotomas. There was no systematic difference in visual field defects for either class of disease when comparing color contrast in the blue (tritan) versus the red (protan) axes of color space. The apparent tritan or protan/deutan axes of color confusion found by hue discrimination testing in acquired dyschromatopsias may be determined by the relative spatial distribution of defects in the central visual field rather than by selective impairment of neural mechanisms for color or luminance information processing.
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Results of threshold static perimetry in nine cases of toxic retinopathy produced by chloroquine phosphate and hydroxychloroquine sulfate are reported. Pericentral visual field defects in an advanced case correlated well with ophthalmoscopically visible defects in the pigment epithelium. In a less advanced case, perifoveal defects in the visual field of one eye were found to be demonstrable in the absence of any fundus abnormalities by either ophthalmoscopy or fluorescein angiography. In both cases, visual field defects were deepest on the superior or vertical meridian, just above the point of fixation. Since defects were found to be deepest along the superior vertical meridian, threshold static perimetry that includes this location should be the most sensitive method of detecting the early stages of visual field damage in chloroquine retinopathy.
Subacute necrotizing encephalomyelopathy (SNE), or Leigh's disease, is an unusual neurologic disorder that is associated with nystagmus, strabismus, and optic atrophy in the majority of cases, and is most often described in infants. The defect currently thought to account for SNE is the presence of an inhibitor factor in the thiamine pathway. We saw a patient whose eye findings initially were believed to represent spasmus nutans, but whose clinical course, computed tomographic findings, and autopsy findings were typical of SNE.
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The organisation and adherence of a clot induced experimentally in the retinal vein of a cat was studied in vitro. For this purpose a retinal vein was treated in vivo with argon laser photocoagulation in 31 eyes, and each eye was removed at periods of time varying between 3 hours and 7 days after treatment. The freshly enucleated eye was then placed in a perfusion chamber with the cornea under a contact lens, and the retinal vessels were perfused under direct microscopic visualisation. It appears that the hydrostatic pressure and flow needed to perfuse the obstructed vein has to be increased with elapsing time, and that 7 days after laser treatment the retinal vessel becomes permanently occluded.
Optic neuropathy developed in a patient with rheumatoid arthritis who had been receiving D-penicillamine for about 1 year. An associated finding included a 2+ positive antinuclear antibody test with a titer of 1:320. Optic disc swelling was resolved on high doses of intravenous steroids. The case resembles two previously reported cases of optic neuropathy which occurred in patients with Wilson's disease who were receiving penicillamine.
It is not unusual for various ocular diseases to be associated with inflammatory bowel disease. Ocular involvement includes the inflammatory entities of keratitis, conjunctivitis, episcleritis, orbital pseudotumor, iritis and neuroretinitis , as well as those states in which the etiology remains undefined, i.e., scleromalacia perforans, peripheral corneal ulceration and furrow, retinal artery occlusion, and optic neuropathy. We report five cases of optic neuropathy associated with ulcerative colitis to emphasize that this may be the sole ocular manifestation of inflammatory bowel disease.
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Rhabdomyosarcoma commonly involves the orbit resulting in unilateral proptosis, swelling, and restricted ocular motility. We saw two unusual cases of children with nasopharyngeal rhabdomyosarcoma which caused rapid bilateral visual loss due to simultaneous spread of the tumor to the orbital apices. Initial CT scans revealed the tumor, but were misinterpreted as "normal," causing a significant delay in diagnosis. We recommend high resolution CT scans of the nasopharynx, orbits, and suprasellar region in any child with rapid bilateral visual loss to rule out this common childhood tumor.
A 25-year-old white man presented with gradual right-sided visual loss over a 3-week period resulting in a visual acuity of "no light perception" in the affected eye for 5 days prior to presentation. Visual-evoked potential testing showed no response to light with the right eye and normal response with the left eye. Evaluation revealed an anterior communicating artery aneurysm compressing the right optic nerve. Eleven days following surgical decompression, 20/200 vision was noted in the right eye; 2 months later, vision had improved to 20/30. Visual-evoked response testing postoperatively showed nearly complete return of function in comparison with the normal left eye. We believe this to be the first report of visual recovery following a prolonged absence of any "light perception" in a patient with compressive optic neuropathy.
Paraneoplastic retinopathy appears to represent a nonmetastatic remote effect of carcinoma and is characterized by rapid visual deterioration accompanied by narrow arterioles seen on ophthalmoscopic examination and an extinguished electroretinogram. This syndrome has been described in postmenopausal women, and it is possible that it represents an autoimmune disorder. Corticosteroid therapy may arrest the visual deterioration.
The hypothesis that a direct neural pathway exists between the midbrain and the intrinsic musculature was investigated. Wheat germ agglutinin labeled with HRP and WG-HRP were injected intraocularly into the ciliary muscle of rabbits and cynomolgus monkeys, and their retrograde movement was monitored. Sparse, but definite labeling was found in the AM nuclei, the EW nuclei, and Perlia's nucleus of the monkey, confirming the existence of a nonsynapsing pathway between intraocular structures and the midbrain in the primate.
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Thirty normal subjects were tested with the Titmus stereotest varying the binocular Snellen's visual acuity from 20/20 to 20/200. From the 36 possible combinations, a nomogram describing the relationship of binocular variations of Snellen's visual acuity and stereoacuity was constructed. The nomogram provides a useful reference for comparing expected and achieved clinical results of the Titmus stereotest in normal subjects. The effects of various pathologic conditions of the eye on the relationship between Snellen's visual acuity and stereoacuity needs to be studied.
The charts of 84 patients admitted to the Neurosurgery Service of the Washington University Medical Center between January, 1960, and July, 1981, with aneurysms at or near the junction of the internal carotid and posterior communicating arteries (ICA-PoCA) were reviewed. Special emphasis was placed on the pupillary size and reactivity of 51 patients with oculomotor nerve involvement. Seven initially had normal pupils, representing 8% of the total group and 14% of those presenting with oculomotor palsies. In 4 of these patients pupillary involvement developed within 5 days, and in 1 pupillary involvement developed in 4 months. Pupillary sparing appears to be more common than previously appreciated in patients with ICA-PoCA aneurysms and oculomotor nerve involvement. Patients with acute somatic oculomotor paresis should be observed closely for at least one week for the development of pupillary involvement. Arteriography may be indicated more frequently than previously recommended.
Idiopathic intracranial hypertension (pseudotumor cerebri) produces loss of visual field and visual acuity. We conducted a retrospective study of 12 patients (all female, ranging in age from 6 to 44 years) using computerized visual field analysis. In seven of the 12 patients, the visual field loss appeared to be permanent, and follow-up was too short for the final outcome to be determined in two others. The visual field defects were those known to be associated with optic disk lesions. The most common were blind spot enlargement (all 12 cases), isopter constriction (nine cases), and loss on the nasal side of the visual field (seven cases), especially in the inferonasal quadrant. Four patients had diminished visual acuities. The reversibility of the visual field defects was correlated with the presence (nonreversible) or absence (reversible) of ophthalmoscopic signs of chronic papilledema. Because visual loss is reversible if treatment is begun before the onset of the optic disk changes associated with chronic papilledema, patients with idiopathic intracranial hypertension should be monitored carefully with frequent perimetric and visual acuity testing.