Search

We compared the prevalence of atrophic-appearing macular lesions between black and white patients with isolated or various genetic types of retinitis pigmentosa to determine if an appreciable difference existed between these two groups. The study included 720 patients of whom 138 (19.2%) were black patients from 115 families and 582 (80.8%) were white patients from 478 families. A logistic regression analysis combining isolated and all genetic types but randomly selecting one patient per family showed a statistically significant difference in the prevalence of atrophic-appearing macular lesions between black and white patients for the right eye (P = .0012) and left eye (P = .002). When considering either all patients or one patient per family, the estimated odds ratios were approximately 2.0 for blacks relative to whites. Our findings indicate that black patients with retinitis pigmentosa are approximately twice as likely as white patients to develop an atrophic-appearing macular lesion. This observation has implications for the prognosis of central visual function in such patients.

We report three patients with chronic headaches and optic neuropathy due to widespread meningeal thickening shown on enhanced MRI; all had biopsy-proven intracranial pachymeningitis (fibrosclerosis of the meninges). Two patients had bilateral optic neuropathy, elevated CSF protein, and polyclonal serum hypergammaglobulinemia. They developed temporal lobe cortical necrosis or sagittal sinus thrombosis, presumably due to compromised dural venous drainage from extensive meningeal fibrosis. The other patient had multiple cranial nerve palsies and unilateral optic neuropathy with normal CSF. Corticosteroid therapy improved visual function in all three patients, although all had persisting visual deficits. Gadolinium-enhanced MRI was essential in identifying meningeal inflammation and locating suitable biopsy sites.

After transsphenoidal resection of a pituitary (or other) tumor, the remaining intrasellar cavity, and sphenoid sinus are usually packed with exogenous fat or muscle to prevent cerebrospinal leak and prolapse of the optic chiasm into an empty sella. We treated two patients in whom chiasmal compression occurred postoperatively because of packing of fat. In one patient, the expected visual improvement in the postoperative period was suboptimal. The subsequent removal of fat resulted in total visual recovery. In the other patient, chiasmal compression persisted from intrasellar fat and residual tumor. Iatrogenic compression of the optic nerves or chiasm should be considered in all patients in whom visual recovery is incomplete.

Iris crystals in three patients with idiopathic iridocyclitis, Fuchs' heterochromic iridocyclitis, and iridocyclitis associated with systemic pseudolymphoma are described. Two patients had iris crystals in one eye, and the other patient had bilateral involvement. The number of iris crystals in each eye ranged from one to multiple crystals. Iris crystals were minute and refractile and glistened with light; they have previously been reported to be Russell bodies and to be associated with hypergammaglobulinaemia. However, two of the patients, who underwent serum protein electrophoresis, did not demonstrate hypergammaglobulinaemia.

We measured the serum levels of sialic acid in 12 patients with idiopathic acute iridocyclitis and 18 normal controls. Sialic acid levels were significantly elevated in the patients with idiopathic acute iridocyclitis. Since animal studies have shown that intraocular inflammation alone cannot elevate serum level of sialic acid, the result of our study suggest that idiopathic acute iridocyclitis may be a multiorgan disease with systemic effects.

Background: Two cases of sympathetic ophthalmia occurring after noncontact neodymium:YAG (Nd:YAG) cyclotherapy have previously been reported. In each case, the patient had undergone filtering surgery in the exciting eye. Although in each case Nd:YAG cyclotherapy was the last surgery performed, the inciting event of sympathetic ophthalmia was unclear., Methods: The authors studied three additional patients who developed sympathetic ophthalmia after Nd:YAG cyclotherapy for glaucoma., Results: Two patients developed sympathetic ophthalmia 4 months after noncontact Nd:YAG cyclotherapy, and 1 patient developed sympathetic ophthalmia 18 months after contact Nd:YAG cyclotherapy. All patients had previous cataract extractions but no filtering surgery in the exciting eye. Clinical features included chronic iridocyclitis, choroidal folds, Dalen-Fuchs nodules, and optic disc edema. Combining these cases with the two previously reported cases, the incidence of sympathetic ophthalmia at our institution thus far is 5.8% (4 of 69) and 0.67% (1 of 150) after noncontact and contact Nd:YAG cyclotherapy, respectively., Conclusions: The incidence of sympathetic ophthalmia after Nd:YAG cyclotherapy is high compared with other ocular procedures. The clinician should vigilantly monitor patients after Nd:YAG cyclotherapy and report additional cases that may have occurred at other institutions.

Hodgkin's disease is a rare cause of infiltrative optic neuropathy, which typically evolves late in the disease course. We managed an unusual case of isolated optic neuropathy in a 21-year-old man occurring during clinical remission from Hodgkin's disease. Radiotherapy and treatment with high-dose systemic corticosteroids resulted in dramatic improvement in vision. Even without other evidence of recurrent disease, acute-onset optic neuropathy in a patient with a history of a lymphoproliferative disorder should raise the question of a reemergence of the malignancy.

Purpose: The effect of mydriasis on Goldmann visual field area in patients with retinitis pigmentosa has not been suitably defined. The aim of this study is to determine whether visual field area in these patients varies with pharmacologic mydriasis., Methods: Fifteen adult patients with retinitis pigmentosa were studied prospectively. Goldmann visual fields with II4e and V4e isopters were obtained in both eyes before and after full pharmacologic mydriasis of the right eye. The isopter areas were quantified and analyzed to determine the effect of mydriasis on visual field area., Results: The difference in the right eye isopter areas was compared with the difference in the left eye isopter areas using paired t tests, where the differences were computed from areas obtained before and after dilation of the right eye. Mydriasis had no significant effect on the visual field in terms of isopter area difference (II4e, P = 0.87; V4e, P = 0.45) and percent change in isopter area (II4e, P = 0.81; V4e, P = 0.24)., Conclusion: Pharmacologic mydriasis had no appreciable effect on the Goldmann visual field area in a selected group of patients with retinitis pigmentosa. These findings suggest that visual fields of such patients obtained in the dilated or undilated state can be meaningfully compared.

Dural sinus hypertension from cerebral venous outflow impairment is a cause of pseudotumor cerebri. The authors documented six such patients: two with unilateral neck dissection, one with surgical ligation of the dominant sigmoid sinus, two with thrombosed central intravenous catheterization, and one with dural sinus thrombosis. The site of cerebral venous outflow obstruction was variable and identified in three patients with computed tomography, conventional magnetic resonance imaging, magnetic resonance angiography, and/or angiography. Magnetic resonance angiography used in two patients characterized the venous flow pattern and identified the site of obstruction, confirming magnetic resonance angiography as an effective noninvasive blood flow technique in diagnosing and following these patients. Three patients were treated successfully with medical therapy and one patient with optic nerve fenestration. The two patients with thrombosed central venous catheters had serious systemic illnesses and suffered permanent visual loss.

A 33-year-old woman who was a chronic alcohol abuser and had bilateral visual loss was found to have megaloblastic anemia with thrombocytopenia. Both fundi showed retinal venular dilatation and tortuosity, superficial and deep intraretinal hemorrhages, white-centered retinal hemorrhages, and optic disc edema. Her megaloblastic anemia was caused by vitamin B12 and folate deficiencies, resulting from an inadequate diet and alcohol abuse. The retinal changes were believed to be the result of the severe anemia and thrombocytopenia. Both the megaloblastic anemia and retinal changes resolved promptly after she received vitamin B12 and folate supplements. This case shows that megaloblastic anemia should be suspected as a cause of bilateral retinal hemorrhages.

A corneal perforation developed in the right eye of a 46-year-old man after removal of a corneal foreign body. Two attempts to seal the perforation with cyanoacrylate glue failed. The patient subsequently underwent lamellar corneal autograft, which successfully closed the perforation. To the best of our knowledge, this is the first report of repairing a corneal perforation with lamellar corneal autograft.

Humphrey visual fields (30-2 program) were performed on 24 otherwise healthy patients before and after cataract extraction to examine the effect of cataract on automated visual fields. All patients met reliability index criteria and recovered visual acuity of 20/25 or better. The effect of learning associated with repeated testing was controlled with visual fields of the fellow eye. Although a greater absolute threshold recovery occurred in the central region of the visual field after cataract extraction, the percent of threshold recovery did not vary across the visual field except for the most peripheral testing points, which demonstrated less recovery. Thus, cataracts depress an automated visual field fairly uniformly. Clinical grading of cataracts by a single experienced clinician was generally a poor predictor of visual field loss. Only the presence of posterior subcapsular plaque in the visual axis and the preoperative visual acuity correlated significantly with postoperative central threshold recovery. Pattern standard deviation remained unchanged after cataract removal, confirming it as a useful way of estimating visual loss from cataracts.

We studied a four-generation family with early-onset autosomal dominant retinitis pigmentosa, severe hyperopia, and axial eye lengths of less than 20 mm. The affected members had decreased vision, night blindness, typical peripheral retinal pigmentary changes, and electroretinographic abnormalities characteristic of retinitis pigmentosa. This pedigree suggests there is another variant of retinitis pigmentosa associated with hyperopia besides Leber's congenital amaurosis and preserved para-arteriole retinal pigment epithelium.

Since 1979, we have treated 11 patients who had macular retinoblastomas. Two patients eventually recovered 20/20 visual acuity despite the presence of subretinal fluid in the fovea at the time of diagnosis. The diagnosis was made at 11 and 14 months of age, and follow-up periods were ten and seven years, respectively. One case was sporadic and the other was hereditary. Both patients were treated with external radiation; one patient was also treated with chemotherapy. The lesions regressed markedly after treatment. These cases demonstrate that visual prognosis in macular retinoblastomas is not uniformly poor even when a foveal detachment is present. Visual acuity may be good in some cases, which supports the merits of medical treatment rather than enucleation in selected patients.

The relative afferent pupillary defect (RAPD) was measured with neutral density filters in ten patients, each of whom had a dense cataract in one eye only. In each instance, the cataract reduced visual acuity to counting fingers or worse, whereas visual acuity in the other eye was 20/25 or better. All patients with mature or nuclear cataracts had a measureable RAPD in the other eye (mean, 0.44 log unit). In each instance, after extraction of the cataract, the RAPD disappeared. Thus, a dense cataract causes an RAPD in the contralateral eye by increasing the pupillomotor effectiveness of the stimulus light and that this is the reason why even a brunescent cataract does not cause an RAPD in the same eye. In addition, two clinical cases are reported. One case records the development of a cataract in an eye with a preexisting RAPD resulting from optic neuritis; as the cataract became more opaque, the afferent defect became smaller and finally was reversed. The other case shows that a traumatic cataract can produce an RAPD in the contralateral eye. Thus, when an afferent pupillary defect is seen in an eye with a cataract, a visual pathway defect in that eye should definitely be suspected.

We photographed the pupils of 128 normal subjects in dim light, morning and afternoon, for five consecutive days. Fifty-two of the subjects (41%) had an anisocoria of 0.4 mm or more at one time or another during these five days, but only four (3%) had unequal pupils of 0.4 mm or more in all ten photography sessions. At any given examination, a fairly constant 19% (24 of 128) of the subjects showed this amount of anisocoria. These numbers shifted dramatically when anisocoria was defined as a pupillary inequality of greater than, or less than, 0.4 mm. The prevalence of anisocoria did not vary with the time of day, from day to day, or from week to week, nor was it influenced by the sex, age, or iris color of the subject.

We compared the direct light response of both eyes in 90 patients who had anterior visual pathway disease using two different testing methods. We measured Kestenbaum's number in millimeters of pupillary diameter. Kestenbaum's number (K) is the difference in the pupil size attained in each eye under direct illumination while the other eye is occluded. We then measured the relative afferent pupillary defect (RAPD) in log units using neutral density filters. The two tests gave similar results (K = 0.88 x RAPD). Kestenbaum's number is the less precise measure, but it can be quickly and cheaply estimated even in dark brown eyes. The filter test requires a set of filters and at least one well-innervated iris sphincter. Kestenbaum's number can be measured without filters, but the iris sphincter and dilator muscles in both eyes must be normally innervated.

In a search for inhibitors of epinephrine biosynthesis as potential therapeutic agents, a series of 13 ring-chlorinated 1,2,3,4-tetrahydroisoquinolines was prepared. These compounds were tested initially for their ability to inhibit rabbit adrenal phenylethanolamine N-methyltransferase (PNMT) in vitro. Enzyme-inhibitor dissociation constants, determined for the six most potent members of the series, indicated the following order of decreasing potency: 7,8-Cl2 greater than 6,7,8-Cl3 greater than 7-Cl approximately 5,6,7,8-Cl4 greater than 5,7,8-Cl3. These compounds were subsequently examined for PNMT-inhibiting activity in intact rats and mice. 7,8-Dichloro-1,2,3,4-tetrahydroisoquinoline (13, SK&F 64139) was the most potent member of the series both in vitro and in vivo and is currently undergoing clinical investigation.