title: Prevalence of Antiretinal Antibodies in Acute Zonal Occult Outer Retinopathy: A Comprehensive Review of 25 Cases.
abstract: OBJECTIVE
  To perform a comprehensive review and to investigate the presence and role of autoimmune antibodies in 25 cases of acute zonal occult outer retinopathy (AZOOR) identified using the classification originally proposed by J. Donald Gass.
  
  
  METHODS
  Observational case series.
  
  
  METHODS
  Setting: Institutional.
  
  
  METHODS
  Twenty-five patients were identified by characteristic symptoms (abrupt onset of photopsias, followed by large scotomata at or connected to the blind spot), ocular findings (paucity of pigmentary changes with no sign of vitreous inflammation and abnormal electroretinogram in at least 1 eye), and a negative family history for retinitis pigmentosa.
  
  
  METHODS
  Patients underwent a full comprehensive ophthalmologic examination, fundus retinography, Goldmann kinetic visual field (GVF), and full-field electroretinogram (ffERG). Blood samples were also obtained to verify for the presence of antiretinal antibodies by Western blot analysis. MainOutcome Measures: Clinical presentation, best-corrected visual acuity (BCVA), fundus abnormalities, visual field defects, ffERG changes, and presence of antiretinal antibodies.
  
  
  RESULTS
  Sixteen patients (64%) presented with photopsias, 56% (14/25) with night blindness, and 56% (14/25) with loss of peripheral vision. Sixty-four percent (16/25) of cases were bilateral. All patients demonstrated retinal vascular attenuation, optic nerve head pallor, and mottling of retinal pigment epithelium. The most common visual field changes included enlargement and expansion of the blind spot extending into large pericentral or other types of scotomata (64%). Both scotopic and photopic ffERG values were abnormal and affected to a similar degree in our patients. Nine patients (36%) had a greater than 20% asymmetry in ERG values between the 2 eyes. All patients had antiretinal antibodies on Western blot with an average of 6.6 bands.
  
  
  CONCLUSIONS
  Evidence suggests that AZOOR is a unique form of autoimmune retinopathy and retinal manifestation suggests possible antiretinal antibody leakage from the disc margin with spread of immune products under the retina, resulting in large scotomata that connect to the optic nerve head.
authors.affiliation: Department of Ophthalmology and Visual Sciences, University of Michigan Medical School, Ann Arbor, Michigan.
authors.forename: Cynthia X
authors.initials: CX
authors.lastname: Qian
authors.affiliation: Department of Ophthalmology and Visual Sciences, University of Wisconsin Medical School, Madison, Wisconsin.
authors.forename: Angeline
authors.initials: A
authors.lastname: Wang
authors.affiliation: Department of Ophthalmology and Visual Sciences, University of Michigan Medical School, Ann Arbor, Michigan.
authors.forename: David L
authors.initials: DL
authors.lastname: DeMill
authors.affiliation: Department of Ophthalmology and Visual Sciences, University of Michigan Medical School, Ann Arbor, Michigan.
authors.forename: Thiran
authors.initials: T
authors.lastname: Jayasundera
authors.affiliation: Department of Ophthalmology and Visual Sciences, University of Michigan Medical School, Ann Arbor, Michigan.
authors.forename: Kari
authors.initials: K
authors.lastname: Branham
authors.affiliation: Department of Ophthalmology and Visual Sciences, University of Michigan Medical School, Ann Arbor, Michigan.
authors.forename: Maria Fernanda
authors.initials: MF
authors.lastname: Abalem
authors.affiliation: Department of Ophthalmology and Visual Sciences, University of Michigan Medical School, Ann Arbor, Michigan.
authors.forename: Naheed
authors.initials: N
authors.lastname: Khan
authors.affiliation: Department of Ophthalmology and Visual Sciences, University of Michigan Medical School, Ann Arbor, Michigan. Electronic address: jrheck@med.umich.edu.
authors.forename: John R
authors.initials: JR
authors.lastname: Heckenlively
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001323:Autoantibodies / Q000097:blood*; D015551:Autoimmunity; D015153:Blotting, Western; D004596:Electroretinography; D005260:Female; D005451:Fluorescein Angiography; D005654:Fundus Oculi; D006801:Humans; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D012189:Retrospective Studies; D012607:Scotoma / Q000097:blood / Q000175:diagnosis / Q000276:immunology*; D041623:Tomography, Optical Coherence; D000080363:White Dot Syndromes; D055815:Young Adult
chemical_list: D001323:Autoantibodies