Bernadette Snyers

Retinal and choroidal vaso-occlusive disease in systemic lupus erythematosus associated with antiphospholipid antibodies.

Three patients with systemic lupus erythematosus (SLE) associated with anti-phospholipid antibodies are reported. All three had severe vaso-occlusive disease: the first had an unilateral vaso-occlusive retinopathy, the second an unilateral central retinal venous obstruction, and the third a bilateral ischemic choroidopathy. The association of these occlusive ocular vascular diseases with the presence of the lupus anticoagulant and other associated factors in SLE is discussed.

Fundus changes in membranoproliferative glomerulonephritis type II

A total of 23 patients aged between 11 and 64 years who had biopsy-proven membranoproliferative glomerulonephritis type 11 (dense deposit disease) were studied using fluorescein angiography of the retina. With the exception of two adolescents, all patients exhibited small subretinal nodules that were similar to basal laminar drusen. Subjects with a long history of renal disease displayed more numerous and larger nodules as well as atrophic changes. Four subjects presented with subretinal neovascular membranes.

Sequential observation of fundus changes in patients with long standing membranoproliferative glomerulonephritis type II (MPGN type II)

Specific fundus changes have been reported in patients with membranoproliferative glomerulonephritis type II (MPGN type II). We studied the clinical course of this retinopathy in four patients who all had a long follow-up with several fundus examinations. Sequential observation was indicative of a slow progression of the retinopathy. Most eyes maintained in the chronic stages a nearly normal visual acuity, and a full visual field despite the existence of marked drusen and atrophic changes. The prognosis however must be somewhat guarded, since choroidal neovascularization developed in three eyes and caused bilateral severe visual loss in one patient

Bilateral Granulomatous Panuveitis as Initial Presentation of Diffuse Systemic T Cell Lymphoma

A high-grade diffuse T cell lymphoma, initially simulating bilateral panuveitis, was diagnosed by analysis of a vitreous biopsy specimen and a breast tumor in a 57-year-old woman. It responded favorably to aggressive chemotherapy before it relapsed in leukemic transformation. This case emphasizes the misleading initial symptoms of primary intraocular lymphoma and the role of immunophenotyping in the diagnosis and classification of lymphoproliferative ocular disorders. The presentation and management of uveal lymphoid neoplasia are discussed.

Susac syndrome in four male patients.

Purpose: To report the clinical and imaging features in four male patients presenting with Susac syndrome, a microangiopathy affecting the brain, the retina, and the cochlea. Methods: Retrospective review of clinical data, fluorescein angiograms, and magnetic resonance imaging findings in these four cases. Results: All four patients were young men (range, 20–35 years). The axiomatic triad of ocular, cochlear, and neurologic involvement was present in three patients. Neurologic symptoms were absent in the fourth one. Fluorescein angiography showed arteriolar wall hyperfluorescence in all four patients. Magnetic resonance images showed in three patients multifocal hyperintense lesions in the white matter and the corpus callosum with typical involvement of the central fibers. Therapeutic modalities and clinical course are described. Three patients had a follow-up of 3, 5, and 13 years with complete remission of the disease within 1 year in all three cases. One patient had severe neuropsychological sequelae. Conclusion: Susac syndrome seems to be less unusual in men than previously reported. Though presenting as a self-limited monophasic course disease in most cases, it may result in severe neuropsychological sequelae. Early diagnosis of the syndrome is enabled by the combination of the ophthalmologic, audiometric, and brain magnetic resonance features.