Clinical and electrophysiologic characterization of paraneoplastic and autoimmune retinopathies associated with antienolase antibodies.
OBJECTIVE
Paraneoplastic and autoimmune retinopathies are immunologically mediated retinal degenerations that are associated with antibodies directed against any of several retinal proteins, including alpha-enolase. We report the clinical and electrophysiological features of antienolase retinopathy in contrast to the features of antirecoverin retinopathy.


METHODS
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 alpha-enolase.


RESULTS
Of 87 patients with unexplained retinal visual symptoms associated with abnormal ERGs, 37 (43%) demonstrated autoantibodies to retinal antigens, including 12 against alpha-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.
Weleber
Richard G
RG
Casey Eye Institute, Department of Ophthalmology, Oregon Health & Science University, 3375 SW Terwilliger Boulevard, Portland, OR 97239, USA. weleberr@ohsu.edu
Watzke
Robert C
RC
Shults
William T
WT
Trzupek
Karmen M
KM
Heckenlively
John R
JR
Egan
Robert A
RA
Adamus
Grazyna
G
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001323:Autoantibodies / Q000097:blood*; D001324:Autoantigens / Q000276:immunology; D001327:Autoimmune Diseases / Q000175:diagnosis* / Q000276:immunology / Q000503:physiopathology; D014408:Biomarkers, Tumor; D015153:Blotting, Western; D002135:Calcium-Binding Proteins / Q000276:immunology; D018288:Carcinoma, Small Cell / Q000276:immunology; D004268:DNA-Binding Proteins / Q000276:immunology*; D004596:Electroretinography; D005136:Eye Proteins / Q000276:immunology*; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008074:Lipoproteins / Q000276:immunology; D008175:Lung Neoplasms / Q000276:immunology; D008297:Male; D008875:Middle Aged; D010257:Paraneoplastic Syndromes / Q000175:diagnosis* / Q000276:immunology / Q000503:physiopathology; D010751:Phosphopyruvate Hydratase / Q000276:immunology*; D011471:Prostatic Neoplasms / Q000276:immunology; D051603:Recoverin; D012160:Retina / Q000276:immunology / Q000473:pathology; D017949:Retinal Cone Photoreceptor Cells / Q000503:physiopathology; D012162:Retinal Degeneration / Q000175:diagnosis* / Q000276:immunology / Q000503:physiopathology; D025521:Tumor Suppressor Proteins / Q000276:immunology*; D014794:Visual Fields
D001323:Autoantibodies; D001324:Autoantigens; D014408:Biomarkers, Tumor; D002135:Calcium-Binding Proteins; D004268:DNA-Binding Proteins; D005136:Eye Proteins; D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D008074:Lipoproteins; C496731:RCVRN protein, human; D025521:Tumor Suppressor Proteins; D051603:Recoverin; C095157:ENO1 protein, human; D010751:Phosphopyruvate Hydratase