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

Autor: William T. Shults, Karmen M Trzupek, Robert A. Egan, John R. Heckenlively, Richard G. Weleber, Robert C. Watzke, Grazyna Adamus
Rok vydání: 2004
Předmět:
Male
Pathology
Visual acuity
Lung Neoplasms
genetic structures
Paraneoplastic Syndromes
Eye disease
Autoimmune retinopathy
Autoantigens
chemistry.chemical_compound
Recoverin
Carcinoma
Small Cell
Aged
80 and over
medicine.diagnostic_test
Retinal Degeneration
Middle Aged
DNA-Binding Proteins
Retinal Cone Photoreceptor Cells
Immunohistochemistry
Female
medicine.symptom
Immunosuppressive Agents
Retinopathy
Adult
medicine.medical_specialty
Lipoproteins
Blotting
Western
Retina
Autoimmune Diseases
medicine
Biomarkers
Tumor
Electroretinography
Humans
Eye Proteins
Glucocorticoids
Aged
Autoantibodies
business.industry
Tumor Suppressor Proteins
Calcium-Binding Proteins
Autoantibody
Prostatic Neoplasms
Retinal
medicine.disease
eye diseases
Ophthalmology
chemistry
Phosphopyruvate Hydratase
sense organs
Visual Fields
business
Zdroj: American journal of ophthalmology. 139(5)
ISSN: 0002-9394
Popis: 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.
Databáze: OpenAIRE
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