Acute multifocal placoid pigment epitheliopathy and central nervous systeminvolvement - Nine new cases and a review of the literature

Objective: The authors describe nine new cases of acute multifocal placoid pigment epitheliopathy (AMPPE) with associated central nervous system (CNS) involvement and permanent visual sequelae. The study includes a review of the literature and discussion of evaluation, management, and treatment opti ons. Design: Retrospective, noncomparative case series. Participants: Nine patients were identified with AMPPE and CNS involvement in addition to 22 patients reviewed in the literature. Main Outcome Measures: A review of nine patients with AMPPE and CNS involve ment was performed. Charts were reviewed for age, gender, preceding viral p rodromes, visual acuity, ophthalmologic examination findings, CNS findings, and treatment. Results: Thirty-one patients (nine new patients) were diagnosed with AMPPE and various degrees of CNS involvement. Ages ranged from 8 to 54 years, wit h an average of 27 years. Twenty-one males (68%) and 10 females (32%) were identified. Eleven patients (35%) had antecedent viral illnesses. Visual ac uity was variable and ranged from 20/20 to count fingers, The spectrum of C NS findings ranged from headaches to sagittal sinus thrombosis. Conclusions: Acute multifocal placoid pigment epitheliopathy can be associa ted with CNS abnormalities and permanent visual deficits. Neuroimaging, lum bar puncture, and cerebral angiography analysis provide useful diagnostic t ools when CNS involvement is suspected. Intravenous corticosteroids and col laboration with neurovascular colleagues should be considered in these situ ations. in cases complicated by CNS arteritis, immunosuppressive agents can be a beneficial adjunct to corticosteroids. Ophthalmology 2001;108:861-868 (C) 2001 by the American Academy of Ophthalmology.