Clinical and electrophysiologic characterization of paraneoplastic and autoimmune retinopathies associated with antienolase antibodies

Richard G. Weleber, Robert C. Watzke, William T. Shults, Karmen M. Trzupek, John R. Heckenlively, Robert A. Egan, Grazyna Adamus

Research output: Contribution to journalArticle

133 Scopus citations

Abstract

• PURPOSE: Paraneoplastic and autoimmune retinopathies are immunologically mediated retinal degenerations that are associated with antibodies directed against any of several retinal proteins, including α-enolase. We report the clinical and electrophysiological features of antienolase retinopathy in contrast to the features of antirecoverin retinopathy. • DESIGN: Retrospective, observational case series. • METHODS: Patients were referred for evaluation of unexplained acquired visual symptoms, including photopsias, and loss of visual acuity or field considered of possible retinal origin. Full-field and multifocal electroretinograms (ERGs) were performed. Sera from patients were examined for antiretinal antibodies by Western blot analysis using proteins extracted from human retinas and by immunohistochemistry; antienolase was confirmed by incubating patient sera with purified α-enolase. • RESULTS: Of 87 patients with unexplained retinal visual symptoms associated with abnormal ERGs, 37 (43%) demonstrated autoantibodies to retinal antigens, including 12 against α-enolase, of whom 4 had cancer. Initial visual loss was typically central and often asymmetric. The ERGs demonstrated mostly normal rod responses but central cone abnormalities (evident on multifocal ERG) and, for many, global cone abnormalities. Seven patients developed optic disk pallor. Corticosteroid and immunosuppressive therapy, when attempted, was clinically ineffective. • CONCLUSIONS: Antienolase retinopathy is a protean autoimmune retinopathy that characteristically presents with cone dysfunction. The visual impairment and course vary from relative stability for years to slow progression with loss of central vision. With time, optic disk pallor can evolve, presumably from attrition of ganglion cells.

Original languageEnglish (US)
Pages (from-to)780-794
Number of pages15
JournalAmerican journal of ophthalmology
Volume139
Issue number5
DOIs
StatePublished - Apr 2005

ASJC Scopus subject areas

  • Ophthalmology

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