Ocular-central nervous system lymphoma mimicking posterior scleritis with exudative retinal detachment

Objective: We describe an unusual ocular presentation of ocular-central ner vous system lymphoma in a young patient. Design: Interventional case report and literature review. Methods: A previously well 24-year-old white woman presented with left eye pain and reduced vision. Episcleral injection, globe tenderness, an afferen t pupil defect, and exudative retinal detachment were present. Computed tom ographic scan of the head and orbits demonstrated scleral thickening, retin al detachment, and no other abnormality. A provisional diagnosis of posteri or scleritis with exudative retinal detachment was made. Investigation for underlying connective tissue diseases was negative. There was an initial pr ompt response to corticosteroid therapy. The patient's symptoms and signs t hen recurred, and a left third cranial nerve palsy developed. Systemic inve stigations including lumbar puncture ultimately led to the diagnosis of pri mary T-cell central nervous system (CNS) lymphoma. Serologic tests for huma n immunodeficiency virus were negative. Main Outcome Measures and Results: The patient underwent orbital and crania l irradiation and intrathecal and systemic chemotherapy, Despite an initial response to treatment, she returned with a recurrence of the lymphoma in t he anterior segment of the left eye. Her systemic disease progressed rapidl y, and she died shortly thereafter. Conclusions: This patient's young age and initial presentation mimicking po sterior scleritis with unilateral exudative retinal detachment, without evi dence of vitreous involvement, are highly unusual for ocular involvement in primary CNS lymphoma. A review of the literature highlights the atypical n ature of this presentation. Ophthalmology 2000; 107:1955-1959 (C) 2000 by t he American Academy of Ophthalmology.