Acute Zonal Occult Outer Retinopathy (AZOOR) and Acute Idiopathic Blind Spot Enlargement Syndrome (AIBSE):

34-year-old female awoke with painless loss of vision OS 3 weeks prior to presentation

Andrew Doan, MD, PhD, Andrew G. Lee, MD, and H. Culver Boldt, MD

February 21, 2005

Chief Complaint: 34-year-old female awoke with painless loss of vision OS 3 weeks prior to presentation.

History of Present Illness: She saw her local ophthalmologist immediately after the onset of vision loss. Her ophthalmologist noted 1+ vitreous cells, VF defect OS, and a head MRI was reported normal.  A diagnosis of retrobulbar optic neuritis was made. Her vision continued to decline. One week prior to our evaluation, she complained of photopsias x 1 week and a diffuse, frontal headache x 3 days.

PMH/FH/POH: Previously healthy without prior ocular surgeries or trauma. She is 5'7" and weighs 250 lbs.

EXAM
Figure 1: HVF
OS OD
HVF OS HVF OD
Enlarge blind spot with superior and inferior visual field (VF) loss. The VF loss does not respect the vertical midline; thus, this lesion is unlikely to be cortical. Full VF OD.

Figure 2: GVF
OS OD

GVF OS

GVF OD

Extensive superior & inferior VF loss around where the blind spot should be located. The VF loss does not respect the vertical midline. Constriction of I2e (red), I4e (blue), I3e (grey), and V4e (purple) isopters. Full VF.

Figure 3: DFE
OD OS

Fundus OD

Fundus OS

Normal optic nerve and macula. Incidental melanocytoma and peripapillary choroidal nevus. No optic nerve edema. Macula normal.
Figure 4: Multifocal Electroretinogram (MERG)
OD OS

MERG OD

MERG OS

OD Waveform

Waveform OD

OS Waveform

Waveform OS

Normal MERG OD. Marked depression of retinal function OS.

Discussion

Our patient presented with: a normal optic nerve (except for the incidental finding of melanocytoma OS), normal dilated fundus exam, mild vitreous cells, decreased vision OS associated with RAPD, photopsias, headache, VF loss, and depression of retinal function OS illustrated by the MERG. Considering a normal MRI, age of the patient, and heavy stature of the patient, we felt that the patient may have presented as an atypical pseudotumor cerebri. Without disc swelling, the diagnosis of pseudotumor cerebri is not typical. The patient had a spinal tap which demonstrated a normal opening pressure.

The other symptoms and clinical presentation in this patient is more typical for acute idiopathic blind spot enlargement (AIBSE) syndrome or acute zonal occult outer retinopathy (AZOOR). This led us to perform a MERG which demonstrated depression of retinal function OS and confirmed the diagnosis of AIBSE/AZOOR. Some will call this disease AIBSE but others will call it AZOOR when there are large areas of retina involved. Several clinicians believe that AIBSE and AZOOR fall in the spectrum of the white dot syndromes, which are idiopathic chorioretinal inflammatory diseases. Some have suggested that AIBSE and AZOOR are multiple evanescent white dot syndrome (MEWDS) without the dots. AIBSE and AZOOR are less aggressive than the other white dot syndromes, and many patients with AIBSE/AZOOR will recover vision.

Diagnosis: Acute Zonal Occult Outer Retinopathy (AZOOR) and Acute Idiopathic Blind Spot Enlargement Syndrome (AIBSE)

EPIDEMIOLOGY

  • young women (30s)
  • incidence unknown
  • etiology: unknown

SIGNS

  • Normal Exam (33%)
  • RPE changes (26%)
  • Optic disc edema (20%)
  • Grayish peripapillary retina (15%)
  • White/gray dots-MEWDS (15%)
  • Chorioretinal scars (7%)

SYMPTOMS

  • Photopsias (74%)
  • RAPD (41%)
  • Decreased vision (22%)
  • Recurrent (20%)
  • Bilateral presentation ( 15%)
  • Headache (9%)
  • Eye Pain (6%)

TREATMENT

  • Follow the patient:
    • 86% will improve
    • 36% with residual photopsias
    • 65% with residual large blind spot

Differential Diagnoses: White Dot Syndromes

References

  1. Brown J Jr, Folk JC. Current controversies in the white dot syndromes. Multifocal choroiditis, punctate inner choroidopathy, and the diffuse subretinal fibrosis syndrome. Ocul Immunol Inflamm. 1998 Jun;6(2):125-7.
  2. Callanan D, Gass JD. Multifocal choroiditis and choroidal neovascularization associated with the multiple evanescent white dot and acute idiopathic blind spot enlargement syndrome. Ophthalmology. 1992 Nov;99(11):1678-85.
  3. Gass JD Acute zonal occult outer retinopathy. Donders Lecture: The Netherlands Ophthalmological Society, Maastricht, Holland, June 19, 1992. J Clin Neuroophthalmol. 1993 Jun;13(2):79-97.
  4. Jacobson SG, Morales DS, Sun XK, Feuer WJ, Cideciyan AV, Gass JD, Milam AH. Pattern of retinal dysfunction in acute zonal occult outer retinopathy. Ophthalmology. 1995 Aug;102(8):1187-98.
  5. Jampol LM, Wiredu A. MEWDS, MFC, PIC, AMN, AIBSE, and AZOOR: one disease or many? Retina. 1995;15(5):373-8.
  6. Oh KT, Christmas NJ, Folk JC. Birdshot retinochoroiditis: long term follow-up of a chronically progressive disease. Am J Ophthalmol. 2002 May;133(5):622-9.
  7. Tsai L, Jampol LM, Pollock SC, Olk J. Chronic recurrent multiple evanescent white dot syndrome. Retina. 1994;14(2):160-3.
  8. Volpe NJ, Rizzo JF 3rd, Lessell S. Acute idiopathic blind spot enlargement syndrome: a review of 27 new cases. Arch Ophthalmol. 2001 Jan;119(1):59-63.

Suggested citation format: Doan A, Lee AG, Boldt HC: Acute Zonal Occult Outer Retinopathy (AZOOR) and Acute Idiopathic Blind Spot Enlargement Syndrome (AIBSE): 34-year-old female awoke with painless loss of vision OS 3 weeks prior to presentation. February 21, 2005; Available from: http://www.EyeRounds.org/cases/case18.htm.