The antiphospholipid antibody syndrome.

Antiphospholipid syndrome includes elevation of either the lupus anticoagulant titer or the anticardiolipin antibody titer on two occasions, separated by 6 weeks in a patient with an episode of thrombosis. The eye is involved frequently in patients with antiphospholipid syndrome. Patients with retinal vascular disease and none of the usual risk factors for retinal vascular disease should be considered at risk for expressing this syndrome.

A case of bilateral optic neuropathy in a patient on tacrolimus (FK506) therapy after liver transplantation.

PURPOSE: To report a case of bilateral optic neuropathy in a patient receiving tacrolimus (FK 506, Prograf; Fujisawa USA, Inc, Deerfield, Illinois) for immunosuppression after orthotropic liver transplantation. METHOD: Case report. In a 58-year-old man receiving tacrolimus after orthotropic liver transplantation, serial neuro-ophthalmologic examinations and laboratory studies were performed. RESULTS: The patient had episodic deterioration of vision in both eyes, with clinical features resembling ischemic optic neuropathies. Deterioration of vision occurred despite discontinuation of the tacrolimus. CONCLUSION: Tacrolimus and other immunosuppressive agents may be associated with optic nerve toxicity.

Skeinoid fibers in mesectodermal leiomyoma of the ciliary body.

Unlike smooth muscle elsewhere in the body, the smooth muscle of the iris and ciliary body is derived from neuroectoderm (mesectoderm). Leiomyomas that arise from the ciliary body, and therefore are of mesectodermal origin, may resemble spindle cell neurogenic tumors by light microscopy. They show positive immunostaining for smooth muscle actin but negative staining for neural markers. Ultrastructurally, the cells have the features of smooth muscle cells. The authors report a typical case of mesectodermal leiomyoma in a 47-year-old woman in which skeinoid fibers, considered to be an ultrastructural marker of neurogenic spindle cell tumors, were frequent together with other ultrastructural features often seen in neuroglial cell tumors. The findings indicate that mesectodermal leiomyoma is unique in its histogenesis as well as in its morphology.

Tropicamide eyedrops cannot be used for reliable diagnosis of Alzheimer's disease.

OBJECTIVE: To evaluate the mydriatic effect of tropicamide eyedrops as a diagnostic test for Alzheimer's disease. MATERIAL AND METHODS: In a double-blind, placebo-controlled study, we assessed pupillary responses in 22 normal control subjects, 23 patients with probable Alzheimer's disease, 4 patients with isolated memory difficulty, and 6 patients with non-Alzheimer's dementia. Three separate studies were performed, the second and third on a subset of the original group. With use of infrared binocular pupillography, after 5 minutes of dark adaptation, we averaged pupil size during a 1-minute interval for baseline determinations. We then instilled 0.01% tropicamide into one eye. In the first two studies, we averaged pupil size for a 1-minute period at 5-minute intervals for 30 minutes, followed by a pupil light reflex test. In the third study, we measured pupil size every 5 minutes for 45 minutes and omitted the light reflex test. RESULTS: No significant difference was noted in pupil dilatation between normal subjects and patients with Alzheimer's disease and between patients with non-Alzheimer's dementias and the Alzheimer's disease group in all three studies. Furthermore, on reperformance of the test in the same patients, more than 50% changed from a group above or below 13% pupil dilatation (a cutoff reported to distinguish Alzheimer's disease from normal control subjects) to the opposite group. CONCLUSION: Results of this study indicate that pupil measurement after instillation of tropicamide cannot be used as a reliable diagnostic test for Alzheimer's disease. Moreover, test-retest reliability with use of dilute tropicamide eyedrops is questionable.

Clinical features and treatment of seven patients with carcinoid tumor metastatic to the eye and orbit.

PURPOSE: To understand better the clinical features, treatment, and outcome of patients with carcinoid tumors metastatic to the eye and orbit. METHODS: We studied seven patients with carcinoid tumors metastatic to the eye and orbit who were initially examined at the Mayo Clinic between 1974 and 1992. Carcinoid tumors metastasized to the choroid of six eyes of four patients and unilaterally to the orbit in three additional patients. RESULTS: Decreased vision was the most frequent initial symptom with choroidal metastases. Choroidal metastases were treated with brachytherapy only, with brachytherapy and chemotherapy, with external beam irradiation and chemotherapy, or with chemotherapy only. The duration of follow-up of these four patients ranged from seven to eight years after the diagnosis of choroidal metastases. Two of the four patients died from tumor complications 7 1/2 and seven years after the diagnosis of choroidal metastases. Proptosis and limitation of ocular motility were the most common initial signs with orbital metastases. Orbital metastases were treated with external beam irradiation and chemotherapy, with chemotherapy only, or with external beam irradiation only. Duration of follow-up of these three patients ranged from one-half to two years after the diagnosis of orbital metastases. All three patients died from tumor complications during the follow-up period. CONCLUSIONS: Because of the relatively good prospects for long-term survival in patients with carcinoid tumors metastatic to the choroid, early detection and treatment of the metastases are important.

Intravitreal large-cell lymphoma.

Large-cell (non-Hodgkin's) lymphoma may occur in the eye as a cellular infiltrate in the vitreous, uveal tract (choroid), retina, or optic nerve. Lymphomatous involvement may be limited to the eye but also is frequently associated with lesions of the central nervous system. Ocular involvement may precede involvement of the central nervous system by months or, in some cases, years. Ocular large-cell lymphoma is bilateral in approximately 80% of cases but often is asymmetric. The mean age of patients with ocular large-cell lymphoma is 60 years, and women are affected almost twice as often as men. Intravitreal large-cell lymphoma may manifest as an infiltrate of large glassy-gray cells or clusters of cells, and it may mimic uveitis or other inflammatory and infectious conditions of the eye. The diagnosis is based on cytologic and immunocytochemical studies of a vitreous biopsy specimen obtained by aspiration or by vitrectomy through the pars plana. Advances in irradiation of the eyes and the central nervous system, supplemented with corticosteroids and intrathecally and intravenously administered chemotherapeutic agents, have resulted in improvement of the dismal prognosis for patients with large-cell lymphoma.

Simultanagnosia as the initial sign of degenerative dementia.

In a study of 10 patients with degenerative brain disease that manifested as simultanagnosia, our aims were (1) to elucidate their clinical, neuropsychologic, and radiologic findings to determine whether these patients might represent a group distinguishable from those with typical Alzheimer's disease and (2) to help clinicians recognize this entity. All patients were initially examined by ophthalmologists because of visual difficulties, and the simultanagnosia remained undiagnosed until nonophthalmologic complaints developed. Optic ataxia developed in six patients, and all patients had mildly impaired eye movements. All 10 patients could identify colors appropriately. Nine patients had language deficits (anomia, decreased auditory comprehension, alexia, and agraphia) but were fluent and had relative preservation of sentence repetition, and four performed in the normal range on a test of associative fluency. Two patients scored in the normal range on memory tests, all had preserved insight, and nine had no family history of degenerative dementia. The mean age at onset of the disorder was 60 years (range, 50 to 69). Neuroimaging disclosed prominent bilateral occipitoparietal atrophy in nine patients and generalized atrophy in one. With this unusual but consistent clinical, neuropsychologic, and anatomic profile, these patients are clinically distinguishable from those with typical Alzheimer's disease, but until a specific cause has been found, we cannot be certain that they constitute a specific biologic entity. Clinicians should consider this diagnosis in relatively young patients who have slowly progressive nonocular visual complaints.

Varicella-zoster virus retinitis in a patient with AIDS-related complex: case report and brief review of the acute retinal necrosis syndrome.

Retinitis reminiscent of the acute retinal necrosis syndrome was recognized in a patient with AIDS-related complex after he had experienced several episodes of a sacral, dermatomic zosteriform eruption. Varicella-zoster virus (VZV) was subsequently recovered from cell culture of retinal tissue. The literature on VZV retinitis, including that on acute retinal necrosis, is reviewed. Dissemination of VZV infection in AIDS is also reviewed. Features that differentiate the findings and course of VZV retinitis in patients with AIDS from those in otherwise healthy adults are noted and related to potentially different pathogenic mechanisms. This unusual and recently recognized complication of herpesvirus infection may be promoted by AIDS-related immunosuppression. Acute retinal necrosis and other more-recently described forms of VZV retinitis, which have primarily been subjects of the ophthalmologic literature, merit the attention of clinicians and investigators of infectious diseases.

When do optic disc edema and peripheral neuropathy constitute poetry?

A patient with chronic, bilateral optic disc edema developed multiple systemic manifestations of POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes). A serum immunoelectrophoresis showed an abnormal serum IgG lambda protein, and urine immunoelectrophoresis revealed a monoclonal lambda protein plus an IgG lambda fragment. Bone survey and MRI scan revealed a sclerotic lesion of the first lumbar vertebra, and lymph node biopsy showed changes consistent with Castleman's disease. Thus, his optic disc edema was the presenting feature of the POEMS syndrome and osteosclerotic myeloma.

Acquired retinal arteriovenous communications in occlusive disease of the carotid artery.

Retinal arteriovenous communications proximal to extensive areas of complete vascular closure developed in three patients with occlusive disease of the carotid arteries. In one case, the authors document the development of the arteriovenous communications through successive enlargement of small retinal vessels after progressive signs of retinal ischemia in the form of narrowed retinal arteries, venous dilation and beading, microaneurysms, retinal dot and blot hemorrhages, cotton-wool spots, and capillary nonperfusion. The development of arteriovenous communications in carotid occlusive disease has not been described previously.

Optic disk swelling with peripheral neuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS syndrome).

We reviewed charts of patients seen at Mayo Clinic Rochester and Mayo Clinic Jacksonville to determine the importance and frequency of optic disk swelling in patients with peripheral neuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS syndrome). A computer search identified 148 patients who were classified as having a monoclonal gammopathy and peripheral neuropathy. A review of the 148 charts disclosed that 11 patients had POEMS syndrome. Eight of these 11 patients had optic disk swelling identified by an ophthalmologist. In all eight patients, optic disk swelling was either the initial sign or was present the first time we saw the patient. Optic disk swelling may be an early sign in POEMS syndrome.

Conservative treatment of orbital roof blow-in fracture.

A two-year-old boy with orbital roof fracture had resolution of motility restriction without traditional surgical exploration. Orbital roof fractures may occur via a blow-in mechanism rather than by direct transmission of an outside force. As is the case with those caused by floor fractures, motility disturbances from roof fractures merit clinical observation before surgery is performed, since some motility defects will resolve spontaneously.

Traumatic extrusion of posterior chamber lenses: clinical and experimental correlations.

Five cases of traumatic expulsion of posterior chamber intraocular lens implants are presented. The syndrome is characterized by expulsion of the lens, iris damage, and intraocular hemorrhage. In experiments with donor human eyes we were able to reproduce the syndrome with 0.68 joules of energy, which is well below that encountered in common environmental situations.

Effect of ocular compression on intraocular pressure.

A prospective double-blind controlled study was undertaken to determine the effects of different compression times applied to soften the eye on normal subjects. Application of the Honan intraocular pressure reducer (HIPR) produced a significantly (p less than 0.02) greater decrease in intraocular pressure when applied for 40 minutes (14.3 mmHg SD = 1.7) when compared to a five-minute application (8.8 mmHg SD = 1.7). No untoward effects were noted in these patients. Physiologic effects of external ocular compression are discussed.