Case ReportAcute Zonal Occult Outer Retinopathy with Atypical Findings

Dimitrios Karagiannis1 Georgios A Kontadakis1

Artemios S Kandarakis1 Nikolaos Markomichelakis2 Ilias Georgalas2

Efstratios A Parikakis1 and Stamatina A Kabanarou3

1 Ophthalmiatreio Eye Hospital of Athens Eleftheriou Venizelou 26 106 72 Athens Greece2 Department of Ophthalmology General Hospital of Athens Leoforos Mesogion 154 115 27 Athens Greece3 Ophthalmology Department Red Cross Hospital Erythrou Stavrou 1 115 26 Athens Greece

Correspondence should be addressed to Georgios A Kontadakis kontadasyahoocom

Received 22 January 2014 Revised 11 June 2014 Accepted 12 June 2014 Published 21 July 2014

Academic Editor Aaron S Dumont

Copyright copy 2014 Dimitrios Karagiannis et al This is an open access article distributed under the Creative Commons AttributionLicense which permits unrestricted use distribution and reproduction in any medium provided the original work is properlycited

Background To report a case of acute zonal occult outer retinopathy (AZOOR) with atypical electrophysiology findings CasePresentation A 23-year-old-female presented with visual acuity deterioration in her right eye accompanied by photopsia bilaterallyCorrected distance visual acuity at presentation was 2050 in the right eye and 2020 in the left eye Fundus examination wasunremarkable Visual field (VF) testing revealed a large scotoma Pattern and full-field electroretinograms (PERG and ERG)revealed macular involvement associated with generalized retinal dysfunction Electrooculogram (EOG) light rise and the Ardenratio were within normal limits bilaterally The patient was diagnosed with AZOOR due to clinical findings visual field defect andERG findings Conclusion This is a case of AZOOR with characteristic VF defects and clinical symptoms presenting with atypicalEOG findings

1 Introduction

Acute zonal occult outer retinopathy (AZOOR) is a rareretinal disorder first described in the literature byGass in 1992[1 2] Patients with AZOOR typically present with photopsiaand scotomas Fundoscopy in patients at initial presentationmay be normal and diagnosis is confirmedwith the abnormalfindings in electrophysiology [1 2]

According to Gass AZOOR belongs to ldquowhite dotrdquo syn-dromes which is a wide spectrumof idiopathic inflammatoryretinal disorders [1 2] Acute zonal occult outer retinopathyaffects mostly women with an average age at presentationof all patients described in the literature about 37 yearsTypical characteristic of the disease is the presentation withvision deterioration in an area of the visual field accompaniedby photopsia Most of the patients present with unilateraldisease but involvement of the contralateral eye ultimatelyappears in themajority of the cases according to the literature[1]

In electrophysiologymost of the patients described in theliterature have abnormal electroretinogram (ERG) in at leastone eye (the most severely affected in asymmetric disease) [12] The electrooculogram (EOG) was also tested in many ofthe cases described in the literature and so far all of them hadabnormal results [1 3]

The purpose of our study is to describe a case ofAZOORwith typical clinical findings and abnormal ERG thatpresented with normal EOG in contrast to the up till nowpublished reports

2 Case Presentation

A 23-year-old-female patient presented in the OutpatientsDepartment of the Ophthalmiatreio Eye Hospital of Athensdue to vision deterioration in her right eye and photop-sias bilaterally The patient underwent complete evaluationwith detailed ophthalmic and systemic history assessmentof corrected distance visual acuity (CDVA) and slit lamp

Hindawi Publishing CorporationCase Reports in MedicineVolume 2014 Article ID 290696 7 pageshttpdxdoiorg1011552014290696

2 Case Reports in Medicine

Single field analysis Eye rightNameID

DOB 01-01-1989

Central 30-2 threshold testFixation monitor offFixation target centralFixation losses 00False POS errors 1False NEG errors 66Test duration 0922

Fovea off

Date 27-09-2012Time 1442Age 23

Pupil diameterVisual acuityRX DS DC X

Stimulus III whiteBackground 315 ASBStrategy SITA standard

GHTOutside normal limitsPattern deviation not

shown for severelydepressed fields Referto total deviation

Pattern deviation notshown for severelydepressed fields Referto total deviation

Pattern deviation

VFI 24

MDPSD

Ophthalmiatrio AthinonGlaukoma Department26 Panepistimiou stTel 3623191-2

lt5lt2

lt1lt05

copy 2007 Carl Zeiss MeditecHFA II 750-9124-41422

14

12

12

1525

25

24

25

30 30

26 26

12

12

19

19 1913

17

13

13

14

14

10

10

10

lt0

lt0

lt0

lt0

lt0

lt0

lt0

lt0

lt0

lt0

lt0

lt0

lt0lt0

lt0lt0

lt0

lt0

lt0

lt0

lt0

lt0

lt0

lt0

lt0

lt0

8

8 8

7

7

1

9 3

9

6 6

4

4

4

3

5

5

5

0

0

0

0

0

0

0

0

minus14

minus18

minus13minus15

minus15

minus15

minus19

minus18

minus19

minus31 minus31

minus31

minus31

minus31

minus31minus31

minus31minus31

minus33

minus33minus33

minus33

minus33

minus33

minus33

minus33

minus33minus32

minus32

minus30

minus30

minus33minus34

minus31

minus36

minus37

minus36

minus32

minus31

minus31

minus21

minus21

minus22

minus22

minus23

minus23

minus27

minus27minus25

minus25

minus26

minus26

minus26minus28

minus24

minus24

minus24

minus23

minus20

minus20

minus28

minus27minus20minus20

minus22

minus29

minus29

minus29

minus8

minus8

minus8

minus9

minus6minus7

Total deviation

minus2447dB P lt 05989dB P lt 05

Figure 1 Visual field of the patientrsquos right eye demonstrating a generalized depression

Case Reports in Medicine 3

OD

(a)

OS

(b)

Figure 2 Optical coherence tomography of the macula without any abnormal findings

Right eye Left eye25 120583Vdiv

+

+

+

+

+

+

N35 N35

P50N95

N95

NormalP50

20msdiv

5 120583Vdiv

++++++++++++++++++

++++++++++++

+++++++++N35

P50NN95

(a)

+

+

+

+

+

+N b

a

N b

a

+

+

N

b

a

250120583Vdiv

20msdiv

(b)

+

++

++

N

ba

bb

aa

250120583Vdiv

20msdiv

+++++++++

++++++++++++

Na

(c)

+

+

+

+

+

+

N1N1 N1

P1

P1P1

100120583Vdiv

20msdiv

(d)

Figure 3 (a) Pattern electroretinogram (PERG) showing reduction of the P50 amplitude in the right eye (b) Bright flash dark adapted ERGs(DA 110) demonstrating a ldquonegativerdquo ERG in the right eye as b-wave is of a lower amplitude than a-wave (c) Photopic single flash ERGs(LA 30) responses were subnormal in the right eye (d) Light adapted 30Hz flicker ERG (LA 30Hz) demonstrating implicit time delay in theright eye

4 Case Reports in Medicine

Right

Peak

1998400div

Dark (15998400) Light (15998400)

Trough

(a)

Left

Peak

Trough

1998400div

Dark (15998400) Light (15998400)

Peak

Troughggg

(b)

Figure 4 EOG recordings demonstrating a normal light rise and Arden ratio bilaterally

inspection of anterior and posterior segments The patientsrsquoCDVA was 2050 in the right eye and 2020 in the lefteye Intraocular pressure was 14mmHg in the right eye and15mmHg in the left eye The patient had no history ofother ophthalmic or systemic diseases Anterior andposteriorsegments examination was unremarkableThe patient under-went visual field testing that revealed a large central scotomain the right eye and was normal in the left eye (Figure 1)

Fluorescein angiography and indocyanine green angiog-raphy were unremarkable bilaterally Optical coherencetomography (OCT) (Spectralis SD-OCT Heidelberg Engi-neering Inc Germany) also revealed no macular pathology(Figure 2)

Additionally the patient underwent PERG and ERGelectroretinograms according to the protocols recommendedby the International Society for Clinical Electrophysiology ofVision (ISCEV) [4 5] Stimulus 06 log units stronger thanthe ISCEV maximum standard flash was also used betterto demonstrate the dark adapted a-wave (DA 110) Visualevoked potentials to a pattern stimulus (PVEP) and (EOG)were also recorded according to ISCEV standards [6 7]

According to the results PERG P50 amplitude as anindex of macular function was reduced in the right eyebut was within normal limits in the left eye indicatingright eye macular dysfunction (Figure 3(a)) The PVEP P

100

component was of reduced amplitude and normal peak timein the right eye (possibly secondary to macular dysfunctionrather than reflecting primary optic nerve disease) whileresponses in the left eye were recorded within normal range

Rod specific ERG (DA 001) was of reduced amplitude inthe right eye only Bright flash dark adapted ERGs (DA 110)were of normal a-wave amplitude bilaterally while b-wavewas of reduced amplitude in the right eye and subnormal inthe left eye indicating a ldquonegative ERGrdquo in the right eye (a-wave amplitudes 302 120583V and 305 120583V in the right and left eyeresp b-wave amplitudes 276120583V and 345 120583V in the right andleft eye resp) (Figure 3(b)) Photopic single flash ERGs (LA30) responses were subnormal in the right eye only (a-waveamplitudes 25 120583V and 385 120583V in the right and left eye resp

b-wave amplitudes 115 120583V and 200 120583V in the right and lefteye (Figure 3(c)) Light adapted 30Hz flicker ERG (LA 30Hz)was delayed in the right eye but within normal limits in theleft eye (implicit time of 31msec and 26msec in the right andleft eyes resp) (Figure 3(d)) EOG light rise and the Ardenratio were within normal limits bilaterally (Arden ratio 42and 39 in the right and left eyes resp) (Figure 4)

The electrophysiological findings thus indicated general-ized retinal dysfunction (affecting both cone and rod systemsand inner retina) associated with macular involvement in theright eye only The patient was diagnosed with AZOOR dueto typical clinical visual field and ERG findings despite thenormal EOG findings

Two months after first visit patient evaluation wasrepeated Vision in her right eye had deteriorated to 2063while in the left eye it was stable at 2020 Visual field testingwas also repeated and revealed further depression of sensitiv-ity in the right eye and involvement of the left eye (Figure 5)The patient was followed up for 8 additional months andher symptoms and clinical findings were stabilized bilaterallyIn the course of follow-up she also underwent magneticresonance imaging of head and abdomen without findings

3 Discussion

Acute zonal occult outer retinopathy is a rare disease ofunknown etiology affecting mostly young female adults [1]There are only a few studies and case reports in the literatureof this condition describing electroretinography as the criticaltest for confirmation of diagnosis in addition to the clinicalfindings and the visual field defect [1ndash3] To the best of ourknowledge this case is the first described in the literaturewithnontypical electrophysiology due to the normal findings inEOG

Our case presented with vision deterioration in one eyeand photopsias bilaterally Photopsias are described as asymptom at presentation in the vast majority of the casesof AZOOR [1] Visual acuity is not always affected sincemost of eyes retain a visual acuity of 2020 Still there are

Case Reports in Medicine 5

Single field analysis Eye rightName ID DOB 01-01-1989

Central 30-2 threshold testFixation monitor blind spotFixation target centralFixation losses 017False POS errors 0False NEG errorsTest duration 0901

Fovea off

Date 30-11-2012Time 1307Age 23

Pupil diameterVisual acuityRX DS DC X

Stimulus III whiteBackground 315 ASBStrategy SITA standard

GHTOutside normal limits

MD

PSD

lt5lt2

lt5lt2

lt1lt05

lt1lt05

Totaldeviation

Patterndeviation

3030

lt0lt0

lt0

lt0

lt0

lt0

lt0lt0

lt0 lt0

lt0

lt0

lt0

lt0

lt0

lt0

lt0lt0lt0lt0

lt0lt0

lt0lt0

lt0lt0

lt0 lt0

lt0 lt0

lt0 lt0 lt0lt0

lt0

lt0

lt0

lt0

lt0

lt0

lt0 lt0

lt0

lt0

lt0 lt0

lt0

lt0

lt0 lt0

lt0

lt0 lt0

1

1

1

1

1

1

7 3

5

3

3

2

3

1

0

00

0

5

0

0

8

0

0

0

0

0

0

0

0 0 0

011

15

10

11

9

minus2

minus4

minus2

minus2

minus2

minus2

minus2

minus2

minus2

minus6

minus6

minus6 minus7

minus7minus7

minus7

minus3

minus3

minus3

minus3

minus3minus3

minus3

minus4

minus6

minus5

minus29

minus29

minus29

minus29

minus22

minus28

minus27

minus34minus34minus34

minus34 minus34

minus34 minus34

minus34

minus34minus34

minus34

minus34 minus34

minus34

minus35minus35

minus35

minus35

minus36minus36minus36

minus36minus37

minus30

minus36

minus29minus29

minus21

minus25minus19

minus30

minus31minus31

minus31

minus31

minus31

minus31

minus31

minus30

minus31

minus31

minus31minus31

minus31

minus31

minus33

minus33

minus33minus33minus33

minus33

minus32

minus32

minus33

minus33

minus33

minus33minus33

minus33

minus33

minus33

minus32

minus32minus32

minus32

minus32

minus31

minus5

minus5 minus5

minus4

minus4

minus4

minus4 minus4

minus4minus4minus4

minus4

minus4

minus4minus4

minus4

minus4minus3

minus1

minus1

minus1

minus1

minus1

minus1

minus3 minus3

minus3

minus3

minus1 minus1minus2minus2

minus2

NA

copy 1994-2000 Humphrey SystemsHFA II 740-11145-3232

minus3214 dB P lt 05

448dB P lt 05

(a)

Figure 5 Continued

6 Case Reports in Medicine

Single field analysis Eye leftName ID DOB 01-01-1989

Central 30-2 threshold testFixation monitor offFixation target centralFixation losses 00False POS errors 0False NEG errorsTest duration 0659

Fovea off

Date 30-11-2012Time 1319Age 23

Pupil diameterVisual acuityRX DS DC X

Stimulus III whiteBackground 315 ASBStrategy SITA standard

GHTOutside normal limits

MDPSD

lt5lt2

lt5lt2lt1

lt05

lt1lt05

Totaldeviation Pattern

deviation

3030

lt0

lt0lt0

lt0

lt0

1

1

1 1

1

0

0

0

0

0

0

0

0

00

0

0

0

18

18 19

11

11

12

1417

1713 18

26 23

26 26 26

24

26

26

26

24 24

27 28

28

28

28

27

27

31

30

31

31

31

31 31

3132

32

32

33 32

32

323230

3034

30

30

30

30

3128

28

28

28

25

25

25

25

29

29

20

20

29 29

33

69

minus2minus2

minus2

minus2

minus2

minus2

minus2 minus2

minus2

minus2minus2

minus2

minus4

minus2

minus2

minus2

minus2

minus2minus2

minus2minus3minus12

minus19

minus19 minus18

minus14

minus14

minus14 minus14

minus16

minus12

minus15

minus15

minus15

minus15 minus20

minus20

minus20

minus26minus26

minus26minus23 minus25minus22 minus21minus12 minus12minus21

minus11

minus26 minus10

minus3

minus3

minus3

minus4minus4

minus4

minus4 minus4

minus4

minus4

minus4

minus5

minus5minus5minus5

minus33minus33

minus32minus27minus33

minus33minus33minus33minus33

minus5minus16minus11

minus11

minus6

minus5

minus4

minus9minus9minus9 minus9

minus8minus8

minus3 minus1

minus1

minus1 minus1 minus1 minus1

minus1

minus1

minus1

minus1

minus1

minus1minus6 minus1 minus1 minus1 minus1

minus1

minus1

minus1

minus1

minus1minus1

minus3

minus3

minus3

minus3minus3

minus3minus3

minus3

minus3minus3

minus3

minus3 minus3

minus1

minus1

minus2

minus2

minus2

6

copy 1994-2000 Humphrey SystemsHFA II 740-11145-3232

minus771dB P lt 051077dB P lt 05

(b)

Figure 5 Visual field of right eye (a) and left eye (b) demonstrating deterioration of the right eye and involvement of the left

Case Reports in Medicine 7

a percentage of patients with impaired visual acuity In aseries of patients presented by Gass et al CDVA was 2040or more in 76 of patients at presentation and final visualacuity was 2040 or more in 68 of patients [2] Slit lampbiomicroscopy and fundus imaging examination (FA andICG) were unremarkable in our case which according tothe literature is a common condition in diagnosed AZOORcases since 50 of published cases present with no relatedfindings in FA [1] Optical coherence tomography (OCT) ofthe macula was normal According to the literature OCTmay show disruptions in the inner segmentouter segmentjunction line and in the outer segment tip lines of thephotoreceptors especially in the late phase of the disease [8]

Visual field defect is also one of themain findings in casesof AZOOR Several types of visual field defects have beenobserved such as blind spot enlargement constriction of theperipheral visual field and central scotomas [1] In our casethe patient presented with a full scotoma of the central visualfield in the initially affected eye which was not related toany other optic nerve pathology The course of our patient(deterioration of the most affected eye in the first monthsand involvement of the contralateral eye) was also typical ofAZOOR

Electrophysiology is the critical testing for confirmationof diagnosis in cases of AZOOR [1ndash3] Gass et al [2] reportedabnormal findings in ERG of all 51 patients in their series andmost of the published cases in the literature report abnormalERGs [1] Common findings are depressed scotopic andorphotopic amplitudes in one eye (the most severely affected)or both eyes [2] A negative amplitude is not common butwas also reported in the literature in one case by Gass et al[2] and in one case by Piao et al where dysfunction of theinner retina as well as the outer retina was proposed in suchcases [9] Our patient also presented with a ldquonegativerdquo ERGin his right eye Macular involvement was also demonstratedin that eye

In a retrospective series of 28 patients Francis et al[3] identified a pattern of electrophysiological anomalies inAZOOR cases where all affected eyes demonstrated delayedimplicit time of the 30Hz cone flicker ERG Our case alsodisplayed delayed implicit time in his right eye which is inaccordance with these criteria In the aforementioned caseseries [3] all of the patients had a marked reduction in theEOG light rise indicating a generalized dysfunction of theretinal pigment epithelium Accordingly case reports in thepublished literature that include EOG testing of the affectedeyes report abnormal results of EOG [1] In our case EOGlight rise and the Arden ratio were within normal limitsbilaterally despite the fact that the rest of the test results weresuggestive of AZOOR

In all reported cases and series of patients in the literaturethere is no definite description of the disease Even thepathophysiology of AZOOR is not clear since it is either con-sidered part of the white dot disease spectrum or triggered byinflammatory disorders such as punctuate inner choroiditisand multifocal inner choroiditis [3] Differential diagnosisof cases of AZOOR with such findings at presentation as inour case includes other conditions such as cancer associatedretinopathy and melanoma associated retinopathy [1] Our

patient had clear medical history and was referred to forcomplete evaluation for possible occultmalignancy includingclinical examination blood testing and chest and abdominalMRI which were not suggestive of such condition

4 Conclusion

In conclusion our patients had typical clinical findings ofAZOOR with photopsia and visual field defect Howeverit represents an unusual case of AZOOR as she presentedwith a ldquonegativerdquo ERG indicating an abnormal inner andouter retina dysfunction and with normal EOG recordingsThe latter may indicate that involvement of retinal pigmentepithelium as demonstrated by EOG abnormality is notimperative in the course of AZOOR

Conflict of Interests

The authors declare that there is no conflict of interestsregarding the publication of this paper

References

[1] D M Monson and J R Smith ldquoAcute zonal occult outerretinopathyrdquo Survey of Ophthalmology vol 56 no 1 pp 23ndash352011

[2] J D Gass A Agarwal and I U Scott ldquoAcute zonal occult outerretinopathy a long-term follow-up studyrdquo American Journal ofOphthalmology vol 134 no 3 pp 329ndash339 2002

[3] P J Francis A Marinescu F W Fitzke A C Bird and G EHolder ldquoAcute zonal occult outer retinopathy towards a set ofdiagnostic criteriardquo The British Journal of Ophthalmology vol89 no 1 pp 70ndash73 2005

[4] M Bach M G Brigell M Hawlina et al ldquoISCEV standardfor clinical pattern electroretinography (PERG) 2012 updaterdquoDocumenta Ophthalmologica vol 126 no 1 pp 1ndash7 2013

[5] M F Marmor A B Fulton G E Holder Y Miyake MBrigell and M Bach ldquoISCEV Standard for full-field clinicalelectroretinography (2008 update)rdquoDocumenta Ophthalmolog-ica vol 118 no 1 pp 69ndash77 2009

[6] J V Odom M Bach M Brigell G E Holder D L McCullochand A P Tormene ldquoISCEV standard for clinical visual evokedpotentials (2009 update)rdquo Documenta Ophthalmologica vol120 no 1 pp 111ndash119 2010

[7] M F Marmor M G Brigell D L McCulloch C A Westalland M Bach ldquoISCEV standard for clinical electro-oculography(2010 update)rdquo Documenta Ophthalmologica vol 122 no 1 pp1ndash7 2011

[8] T Wakazono S Ooto M Hangai and N Yoshimura ldquoPho-toreceptor outer segment abnormalities and retinal sensitivityin acute zonal occult outer retinopathyrdquo Retina vol 33 no 3pp 642ndash648 2013

[9] C Piao M Kondo S Ishikawa S Okinami and H TerasakildquoA case of unusual retinopathy showing features similar toacute zonal occult outer retinopathy associated with negativeelectroretinogramsrdquo Japanese Journal of Ophthalmology vol 51no 1 pp 69ndash71 2007

Submit your manuscripts athttpwwwhindawicom

Stem CellsInternational

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

MEDIATORSINFLAMMATION

of

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Behavioural Neurology

EndocrinologyInternational Journal of

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Disease Markers

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

BioMed Research International

OncologyJournal of

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Oxidative Medicine and Cellular Longevity

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

PPAR Research

The Scientific World JournalHindawi Publishing Corporation httpwwwhindawicom Volume 2014

Immunology ResearchHindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Journal of

ObesityJournal of

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Computational and Mathematical Methods in Medicine

OphthalmologyJournal of

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Diabetes ResearchJournal of

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Research and TreatmentAIDS

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Gastroenterology Research and Practice

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Parkinsonrsquos Disease

Evidence-Based Complementary and Alternative Medicine

Volume 2014Hindawi Publishing Corporationhttpwwwhindawicom

2 Case Reports in Medicine

Single field analysis Eye rightNameID

DOB 01-01-1989

Central 30-2 threshold testFixation monitor offFixation target centralFixation losses 00False POS errors 1False NEG errors 66Test duration 0922

Fovea off

Date 27-09-2012Time 1442Age 23

Pupil diameterVisual acuityRX DS DC X

Stimulus III whiteBackground 315 ASBStrategy SITA standard

GHTOutside normal limitsPattern deviation not

shown for severelydepressed fields Referto total deviation

Pattern deviation notshown for severelydepressed fields Referto total deviation

Pattern deviation

VFI 24

MDPSD

Ophthalmiatrio AthinonGlaukoma Department26 Panepistimiou stTel 3623191-2

lt5lt2

lt1lt05

copy 2007 Carl Zeiss MeditecHFA II 750-9124-41422

14

12

12

1525

25

24

25

30 30

26 26

12

12

19

19 1913

17

13

13

14

14

10

10

10

lt0

lt0

lt0

lt0

lt0

lt0

lt0

lt0

lt0

lt0

lt0

lt0

lt0lt0

lt0lt0

lt0

lt0

lt0

lt0

lt0

lt0

lt0

lt0

lt0

lt0

8

8 8

7

7

1

9 3

9

6 6

4

4

4

3

5

5

5

0

0

0

0

0

0

0

0

minus14

minus18

minus13minus15

minus15

minus15

minus19

minus18

minus19

minus31 minus31

minus31

minus31

minus31

minus31minus31

minus31minus31

minus33

minus33minus33

minus33

minus33

minus33

minus33

minus33

minus33minus32

minus32

minus30

minus30

minus33minus34

minus31

minus36

minus37

minus36

minus32

minus31

minus31

minus21

minus21

minus22

minus22

minus23

minus23

minus27

minus27minus25

minus25

minus26

minus26

minus26minus28

minus24

minus24

minus24

minus23

minus20

minus20

minus28

minus27minus20minus20

minus22

minus29

minus29

minus29

minus8

minus8

minus8

minus9

minus6minus7

Total deviation

minus2447dB P lt 05989dB P lt 05

Figure 1 Visual field of the patientrsquos right eye demonstrating a generalized depression

Case Reports in Medicine 3

OD

(a)

OS

(b)

Figure 2 Optical coherence tomography of the macula without any abnormal findings

Right eye Left eye25 120583Vdiv

+

+

+

+

+

+

N35 N35

P50N95

N95

NormalP50

20msdiv

5 120583Vdiv

++++++++++++++++++

++++++++++++

+++++++++N35

P50NN95

(a)

+

+

+

+

+

+N b

a

N b

a

+

+

N

b

a

250120583Vdiv

20msdiv

(b)

+

++

++

N

ba

bb

aa

250120583Vdiv

20msdiv

+++++++++

++++++++++++

Na

(c)

+

+

+

+

+

+

N1N1 N1

P1

P1P1

100120583Vdiv

20msdiv

(d)

Figure 3 (a) Pattern electroretinogram (PERG) showing reduction of the P50 amplitude in the right eye (b) Bright flash dark adapted ERGs(DA 110) demonstrating a ldquonegativerdquo ERG in the right eye as b-wave is of a lower amplitude than a-wave (c) Photopic single flash ERGs(LA 30) responses were subnormal in the right eye (d) Light adapted 30Hz flicker ERG (LA 30Hz) demonstrating implicit time delay in theright eye

4 Case Reports in Medicine

Right

Peak

1998400div

Dark (15998400) Light (15998400)

Trough

(a)

Left

Peak

Trough

1998400div

Dark (15998400) Light (15998400)

Peak

Troughggg

(b)

Figure 4 EOG recordings demonstrating a normal light rise and Arden ratio bilaterally

inspection of anterior and posterior segments The patientsrsquoCDVA was 2050 in the right eye and 2020 in the lefteye Intraocular pressure was 14mmHg in the right eye and15mmHg in the left eye The patient had no history ofother ophthalmic or systemic diseases Anterior andposteriorsegments examination was unremarkableThe patient under-went visual field testing that revealed a large central scotomain the right eye and was normal in the left eye (Figure 1)

Fluorescein angiography and indocyanine green angiog-raphy were unremarkable bilaterally Optical coherencetomography (OCT) (Spectralis SD-OCT Heidelberg Engi-neering Inc Germany) also revealed no macular pathology(Figure 2)

Additionally the patient underwent PERG and ERGelectroretinograms according to the protocols recommendedby the International Society for Clinical Electrophysiology ofVision (ISCEV) [4 5] Stimulus 06 log units stronger thanthe ISCEV maximum standard flash was also used betterto demonstrate the dark adapted a-wave (DA 110) Visualevoked potentials to a pattern stimulus (PVEP) and (EOG)were also recorded according to ISCEV standards [6 7]

According to the results PERG P50 amplitude as anindex of macular function was reduced in the right eyebut was within normal limits in the left eye indicatingright eye macular dysfunction (Figure 3(a)) The PVEP P

100

component was of reduced amplitude and normal peak timein the right eye (possibly secondary to macular dysfunctionrather than reflecting primary optic nerve disease) whileresponses in the left eye were recorded within normal range

Rod specific ERG (DA 001) was of reduced amplitude inthe right eye only Bright flash dark adapted ERGs (DA 110)were of normal a-wave amplitude bilaterally while b-wavewas of reduced amplitude in the right eye and subnormal inthe left eye indicating a ldquonegative ERGrdquo in the right eye (a-wave amplitudes 302 120583V and 305 120583V in the right and left eyeresp b-wave amplitudes 276120583V and 345 120583V in the right andleft eye resp) (Figure 3(b)) Photopic single flash ERGs (LA30) responses were subnormal in the right eye only (a-waveamplitudes 25 120583V and 385 120583V in the right and left eye resp

b-wave amplitudes 115 120583V and 200 120583V in the right and lefteye (Figure 3(c)) Light adapted 30Hz flicker ERG (LA 30Hz)was delayed in the right eye but within normal limits in theleft eye (implicit time of 31msec and 26msec in the right andleft eyes resp) (Figure 3(d)) EOG light rise and the Ardenratio were within normal limits bilaterally (Arden ratio 42and 39 in the right and left eyes resp) (Figure 4)

The electrophysiological findings thus indicated general-ized retinal dysfunction (affecting both cone and rod systemsand inner retina) associated with macular involvement in theright eye only The patient was diagnosed with AZOOR dueto typical clinical visual field and ERG findings despite thenormal EOG findings

Two months after first visit patient evaluation wasrepeated Vision in her right eye had deteriorated to 2063while in the left eye it was stable at 2020 Visual field testingwas also repeated and revealed further depression of sensitiv-ity in the right eye and involvement of the left eye (Figure 5)The patient was followed up for 8 additional months andher symptoms and clinical findings were stabilized bilaterallyIn the course of follow-up she also underwent magneticresonance imaging of head and abdomen without findings

3 Discussion

Acute zonal occult outer retinopathy is a rare disease ofunknown etiology affecting mostly young female adults [1]There are only a few studies and case reports in the literatureof this condition describing electroretinography as the criticaltest for confirmation of diagnosis in addition to the clinicalfindings and the visual field defect [1ndash3] To the best of ourknowledge this case is the first described in the literaturewithnontypical electrophysiology due to the normal findings inEOG

Our case presented with vision deterioration in one eyeand photopsias bilaterally Photopsias are described as asymptom at presentation in the vast majority of the casesof AZOOR [1] Visual acuity is not always affected sincemost of eyes retain a visual acuity of 2020 Still there are

Case Reports in Medicine 5

Single field analysis Eye rightName ID DOB 01-01-1989

Central 30-2 threshold testFixation monitor blind spotFixation target centralFixation losses 017False POS errors 0False NEG errorsTest duration 0901

Fovea off

Date 30-11-2012Time 1307Age 23

Pupil diameterVisual acuityRX DS DC X

Stimulus III whiteBackground 315 ASBStrategy SITA standard

GHTOutside normal limits

MD

PSD

lt5lt2

lt5lt2

lt1lt05

lt1lt05

Totaldeviation

Patterndeviation

3030

lt0lt0

lt0

lt0

lt0

lt0

lt0lt0

lt0 lt0

lt0

lt0

lt0

lt0

lt0

lt0

lt0lt0lt0lt0

lt0lt0

lt0lt0

lt0lt0

lt0 lt0

lt0 lt0

lt0 lt0 lt0lt0

lt0

lt0

lt0

lt0

lt0

lt0

lt0 lt0

lt0

lt0

lt0 lt0

lt0

lt0

lt0 lt0

lt0

lt0 lt0

1

1

1

1

1

1

7 3

5

3

3

2

3

1

0

00

0

5

0

0

8

0

0

0

0

0

0

0

0 0 0

011

15

10

11

9

minus2

minus4

minus2

minus2

minus2

minus2

minus2

minus2

minus2

minus6

minus6

minus6 minus7

minus7minus7

minus7

minus3

minus3

minus3

minus3

minus3minus3

minus3

minus4

minus6

minus5

minus29

minus29

minus29

minus29

minus22

minus28

minus27

minus34minus34minus34

minus34 minus34

minus34 minus34

minus34

minus34minus34

minus34

minus34 minus34

minus34

minus35minus35

minus35

minus35

minus36minus36minus36

minus36minus37

minus30

minus36

minus29minus29

minus21

minus25minus19

minus30

minus31minus31

minus31

minus31

minus31

minus31

minus31

minus30

minus31

minus31

minus31minus31

minus31

minus31

minus33

minus33

minus33minus33minus33

minus33

minus32

minus32

minus33

minus33

minus33

minus33minus33

minus33

minus33

minus33

minus32

minus32minus32

minus32

minus32

minus31

minus5

minus5 minus5

minus4

minus4

minus4

minus4 minus4

minus4minus4minus4

minus4

minus4

minus4minus4

minus4

minus4minus3

minus1

minus1

minus1

minus1

minus1

minus1

minus3 minus3

minus3

minus3

minus1 minus1minus2minus2

minus2

NA

copy 1994-2000 Humphrey SystemsHFA II 740-11145-3232

minus3214 dB P lt 05

448dB P lt 05

(a)

Figure 5 Continued

6 Case Reports in Medicine

Single field analysis Eye leftName ID DOB 01-01-1989

Central 30-2 threshold testFixation monitor offFixation target centralFixation losses 00False POS errors 0False NEG errorsTest duration 0659

Fovea off

Date 30-11-2012Time 1319Age 23

Pupil diameterVisual acuityRX DS DC X

Stimulus III whiteBackground 315 ASBStrategy SITA standard

GHTOutside normal limits

MDPSD

lt5lt2

lt5lt2lt1

lt05

lt1lt05

Totaldeviation Pattern

deviation

3030

lt0

lt0lt0

lt0

lt0

1

1

1 1

1

0

0

0

0

0

0

0

0

00

0

0

0

18

18 19

11

11

12

1417

1713 18

26 23

26 26 26

24

26

26

26

24 24

27 28

28

28

28

27

27

31

30

31

31

31

31 31

3132

32

32

33 32

32

323230

3034

30

30

30

30

3128

28

28

28

25

25

25

25

29

29

20

20

29 29

33

69

minus2minus2

minus2

minus2

minus2

minus2

minus2 minus2

minus2

minus2minus2

minus2

minus4

minus2

minus2

minus2

minus2

minus2minus2

minus2minus3minus12

minus19

minus19 minus18

minus14

minus14

minus14 minus14

minus16

minus12

minus15

minus15

minus15

minus15 minus20

minus20

minus20

minus26minus26

minus26minus23 minus25minus22 minus21minus12 minus12minus21

minus11

minus26 minus10

minus3

minus3

minus3

minus4minus4

minus4

minus4 minus4

minus4

minus4

minus4

minus5

minus5minus5minus5

minus33minus33

minus32minus27minus33

minus33minus33minus33minus33

minus5minus16minus11

minus11

minus6

minus5

minus4

minus9minus9minus9 minus9

minus8minus8

minus3 minus1

minus1

minus1 minus1 minus1 minus1

minus1

minus1

minus1

minus1

minus1

minus1minus6 minus1 minus1 minus1 minus1

minus1

minus1

minus1

minus1

minus1minus1

minus3

minus3

minus3

minus3minus3

minus3minus3

minus3

minus3minus3

minus3

minus3 minus3

minus1

minus1

minus2

minus2

minus2

6

copy 1994-2000 Humphrey SystemsHFA II 740-11145-3232

minus771dB P lt 051077dB P lt 05

(b)

Figure 5 Visual field of right eye (a) and left eye (b) demonstrating deterioration of the right eye and involvement of the left

Case Reports in Medicine 7

a percentage of patients with impaired visual acuity In aseries of patients presented by Gass et al CDVA was 2040or more in 76 of patients at presentation and final visualacuity was 2040 or more in 68 of patients [2] Slit lampbiomicroscopy and fundus imaging examination (FA andICG) were unremarkable in our case which according tothe literature is a common condition in diagnosed AZOORcases since 50 of published cases present with no relatedfindings in FA [1] Optical coherence tomography (OCT) ofthe macula was normal According to the literature OCTmay show disruptions in the inner segmentouter segmentjunction line and in the outer segment tip lines of thephotoreceptors especially in the late phase of the disease [8]

Visual field defect is also one of themain findings in casesof AZOOR Several types of visual field defects have beenobserved such as blind spot enlargement constriction of theperipheral visual field and central scotomas [1] In our casethe patient presented with a full scotoma of the central visualfield in the initially affected eye which was not related toany other optic nerve pathology The course of our patient(deterioration of the most affected eye in the first monthsand involvement of the contralateral eye) was also typical ofAZOOR

Electrophysiology is the critical testing for confirmationof diagnosis in cases of AZOOR [1ndash3] Gass et al [2] reportedabnormal findings in ERG of all 51 patients in their series andmost of the published cases in the literature report abnormalERGs [1] Common findings are depressed scotopic andorphotopic amplitudes in one eye (the most severely affected)or both eyes [2] A negative amplitude is not common butwas also reported in the literature in one case by Gass et al[2] and in one case by Piao et al where dysfunction of theinner retina as well as the outer retina was proposed in suchcases [9] Our patient also presented with a ldquonegativerdquo ERGin his right eye Macular involvement was also demonstratedin that eye

In a retrospective series of 28 patients Francis et al[3] identified a pattern of electrophysiological anomalies inAZOOR cases where all affected eyes demonstrated delayedimplicit time of the 30Hz cone flicker ERG Our case alsodisplayed delayed implicit time in his right eye which is inaccordance with these criteria In the aforementioned caseseries [3] all of the patients had a marked reduction in theEOG light rise indicating a generalized dysfunction of theretinal pigment epithelium Accordingly case reports in thepublished literature that include EOG testing of the affectedeyes report abnormal results of EOG [1] In our case EOGlight rise and the Arden ratio were within normal limitsbilaterally despite the fact that the rest of the test results weresuggestive of AZOOR

In all reported cases and series of patients in the literaturethere is no definite description of the disease Even thepathophysiology of AZOOR is not clear since it is either con-sidered part of the white dot disease spectrum or triggered byinflammatory disorders such as punctuate inner choroiditisand multifocal inner choroiditis [3] Differential diagnosisof cases of AZOOR with such findings at presentation as inour case includes other conditions such as cancer associatedretinopathy and melanoma associated retinopathy [1] Our

patient had clear medical history and was referred to forcomplete evaluation for possible occultmalignancy includingclinical examination blood testing and chest and abdominalMRI which were not suggestive of such condition

4 Conclusion

In conclusion our patients had typical clinical findings ofAZOOR with photopsia and visual field defect Howeverit represents an unusual case of AZOOR as she presentedwith a ldquonegativerdquo ERG indicating an abnormal inner andouter retina dysfunction and with normal EOG recordingsThe latter may indicate that involvement of retinal pigmentepithelium as demonstrated by EOG abnormality is notimperative in the course of AZOOR

Conflict of Interests

The authors declare that there is no conflict of interestsregarding the publication of this paper

References

[1] D M Monson and J R Smith ldquoAcute zonal occult outerretinopathyrdquo Survey of Ophthalmology vol 56 no 1 pp 23ndash352011

[2] J D Gass A Agarwal and I U Scott ldquoAcute zonal occult outerretinopathy a long-term follow-up studyrdquo American Journal ofOphthalmology vol 134 no 3 pp 329ndash339 2002

[3] P J Francis A Marinescu F W Fitzke A C Bird and G EHolder ldquoAcute zonal occult outer retinopathy towards a set ofdiagnostic criteriardquo The British Journal of Ophthalmology vol89 no 1 pp 70ndash73 2005

[4] M Bach M G Brigell M Hawlina et al ldquoISCEV standardfor clinical pattern electroretinography (PERG) 2012 updaterdquoDocumenta Ophthalmologica vol 126 no 1 pp 1ndash7 2013

[5] M F Marmor A B Fulton G E Holder Y Miyake MBrigell and M Bach ldquoISCEV Standard for full-field clinicalelectroretinography (2008 update)rdquoDocumenta Ophthalmolog-ica vol 118 no 1 pp 69ndash77 2009

[6] J V Odom M Bach M Brigell G E Holder D L McCullochand A P Tormene ldquoISCEV standard for clinical visual evokedpotentials (2009 update)rdquo Documenta Ophthalmologica vol120 no 1 pp 111ndash119 2010

[7] M F Marmor M G Brigell D L McCulloch C A Westalland M Bach ldquoISCEV standard for clinical electro-oculography(2010 update)rdquo Documenta Ophthalmologica vol 122 no 1 pp1ndash7 2011

[8] T Wakazono S Ooto M Hangai and N Yoshimura ldquoPho-toreceptor outer segment abnormalities and retinal sensitivityin acute zonal occult outer retinopathyrdquo Retina vol 33 no 3pp 642ndash648 2013

[9] C Piao M Kondo S Ishikawa S Okinami and H TerasakildquoA case of unusual retinopathy showing features similar toacute zonal occult outer retinopathy associated with negativeelectroretinogramsrdquo Japanese Journal of Ophthalmology vol 51no 1 pp 69ndash71 2007

Submit your manuscripts athttpwwwhindawicom

Stem CellsInternational

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

MEDIATORSINFLAMMATION

of

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Behavioural Neurology

EndocrinologyInternational Journal of

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Disease Markers

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

BioMed Research International

OncologyJournal of

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Oxidative Medicine and Cellular Longevity

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

PPAR Research

The Scientific World JournalHindawi Publishing Corporation httpwwwhindawicom Volume 2014

Immunology ResearchHindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Journal of

ObesityJournal of

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Computational and Mathematical Methods in Medicine

OphthalmologyJournal of

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Diabetes ResearchJournal of

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Research and TreatmentAIDS

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Gastroenterology Research and Practice

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Parkinsonrsquos Disease

Evidence-Based Complementary and Alternative Medicine

Volume 2014Hindawi Publishing Corporationhttpwwwhindawicom

Case Reports in Medicine 3

OD

(a)

OS

(b)

Figure 2 Optical coherence tomography of the macula without any abnormal findings

Right eye Left eye25 120583Vdiv

+

+

+

+

+

+

N35 N35

P50N95

N95

NormalP50

20msdiv

5 120583Vdiv

++++++++++++++++++

++++++++++++

+++++++++N35

P50NN95

(a)

+

+

+

+

+

+N b

a

N b

a

+

+

N

b

a

250120583Vdiv

20msdiv

(b)

+

++

++

N

ba

bb

aa

250120583Vdiv

20msdiv

+++++++++

++++++++++++

Na

(c)

+

+

+

+

+

+

N1N1 N1

P1

P1P1

100120583Vdiv

20msdiv

(d)

Figure 3 (a) Pattern electroretinogram (PERG) showing reduction of the P50 amplitude in the right eye (b) Bright flash dark adapted ERGs(DA 110) demonstrating a ldquonegativerdquo ERG in the right eye as b-wave is of a lower amplitude than a-wave (c) Photopic single flash ERGs(LA 30) responses were subnormal in the right eye (d) Light adapted 30Hz flicker ERG (LA 30Hz) demonstrating implicit time delay in theright eye

4 Case Reports in Medicine

Right

Peak

1998400div

Dark (15998400) Light (15998400)

Trough

(a)

Left

Peak

Trough

1998400div

Dark (15998400) Light (15998400)

Peak

Troughggg

(b)

Figure 4 EOG recordings demonstrating a normal light rise and Arden ratio bilaterally

inspection of anterior and posterior segments The patientsrsquoCDVA was 2050 in the right eye and 2020 in the lefteye Intraocular pressure was 14mmHg in the right eye and15mmHg in the left eye The patient had no history ofother ophthalmic or systemic diseases Anterior andposteriorsegments examination was unremarkableThe patient under-went visual field testing that revealed a large central scotomain the right eye and was normal in the left eye (Figure 1)

Fluorescein angiography and indocyanine green angiog-raphy were unremarkable bilaterally Optical coherencetomography (OCT) (Spectralis SD-OCT Heidelberg Engi-neering Inc Germany) also revealed no macular pathology(Figure 2)

Additionally the patient underwent PERG and ERGelectroretinograms according to the protocols recommendedby the International Society for Clinical Electrophysiology ofVision (ISCEV) [4 5] Stimulus 06 log units stronger thanthe ISCEV maximum standard flash was also used betterto demonstrate the dark adapted a-wave (DA 110) Visualevoked potentials to a pattern stimulus (PVEP) and (EOG)were also recorded according to ISCEV standards [6 7]

According to the results PERG P50 amplitude as anindex of macular function was reduced in the right eyebut was within normal limits in the left eye indicatingright eye macular dysfunction (Figure 3(a)) The PVEP P

100

component was of reduced amplitude and normal peak timein the right eye (possibly secondary to macular dysfunctionrather than reflecting primary optic nerve disease) whileresponses in the left eye were recorded within normal range

Rod specific ERG (DA 001) was of reduced amplitude inthe right eye only Bright flash dark adapted ERGs (DA 110)were of normal a-wave amplitude bilaterally while b-wavewas of reduced amplitude in the right eye and subnormal inthe left eye indicating a ldquonegative ERGrdquo in the right eye (a-wave amplitudes 302 120583V and 305 120583V in the right and left eyeresp b-wave amplitudes 276120583V and 345 120583V in the right andleft eye resp) (Figure 3(b)) Photopic single flash ERGs (LA30) responses were subnormal in the right eye only (a-waveamplitudes 25 120583V and 385 120583V in the right and left eye resp

b-wave amplitudes 115 120583V and 200 120583V in the right and lefteye (Figure 3(c)) Light adapted 30Hz flicker ERG (LA 30Hz)was delayed in the right eye but within normal limits in theleft eye (implicit time of 31msec and 26msec in the right andleft eyes resp) (Figure 3(d)) EOG light rise and the Ardenratio were within normal limits bilaterally (Arden ratio 42and 39 in the right and left eyes resp) (Figure 4)

The electrophysiological findings thus indicated general-ized retinal dysfunction (affecting both cone and rod systemsand inner retina) associated with macular involvement in theright eye only The patient was diagnosed with AZOOR dueto typical clinical visual field and ERG findings despite thenormal EOG findings

Two months after first visit patient evaluation wasrepeated Vision in her right eye had deteriorated to 2063while in the left eye it was stable at 2020 Visual field testingwas also repeated and revealed further depression of sensitiv-ity in the right eye and involvement of the left eye (Figure 5)The patient was followed up for 8 additional months andher symptoms and clinical findings were stabilized bilaterallyIn the course of follow-up she also underwent magneticresonance imaging of head and abdomen without findings

3 Discussion

Acute zonal occult outer retinopathy is a rare disease ofunknown etiology affecting mostly young female adults [1]There are only a few studies and case reports in the literatureof this condition describing electroretinography as the criticaltest for confirmation of diagnosis in addition to the clinicalfindings and the visual field defect [1ndash3] To the best of ourknowledge this case is the first described in the literaturewithnontypical electrophysiology due to the normal findings inEOG

Our case presented with vision deterioration in one eyeand photopsias bilaterally Photopsias are described as asymptom at presentation in the vast majority of the casesof AZOOR [1] Visual acuity is not always affected sincemost of eyes retain a visual acuity of 2020 Still there are

Case Reports in Medicine 5

Single field analysis Eye rightName ID DOB 01-01-1989

Central 30-2 threshold testFixation monitor blind spotFixation target centralFixation losses 017False POS errors 0False NEG errorsTest duration 0901

Fovea off

Date 30-11-2012Time 1307Age 23

Pupil diameterVisual acuityRX DS DC X

Stimulus III whiteBackground 315 ASBStrategy SITA standard

GHTOutside normal limits

MD

PSD

lt5lt2

lt5lt2

lt1lt05

lt1lt05

Totaldeviation

Patterndeviation

3030

lt0lt0

lt0

lt0

lt0

lt0

lt0lt0

lt0 lt0

lt0

lt0

lt0

lt0

lt0

lt0

lt0lt0lt0lt0

lt0lt0

lt0lt0

lt0lt0

lt0 lt0

lt0 lt0

lt0 lt0 lt0lt0

lt0

lt0

lt0

lt0

lt0

lt0

lt0 lt0

lt0

lt0

lt0 lt0

lt0

lt0

lt0 lt0

lt0

lt0 lt0

1

1

1

1

1

1

7 3

5

3

3

2

3

1

0

00

0

5

0

0

8

0

0

0

0

0

0

0

0 0 0

011

15

10

11

9

minus2

minus4

minus2

minus2

minus2

minus2

minus2

minus2

minus2

minus6

minus6

minus6 minus7

minus7minus7

minus7

minus3

minus3

minus3

minus3

minus3minus3

minus3

minus4

minus6

minus5

minus29

minus29

minus29

minus29

minus22

minus28

minus27

minus34minus34minus34

minus34 minus34

minus34 minus34

minus34

minus34minus34

minus34

minus34 minus34

minus34

minus35minus35

minus35

minus35

minus36minus36minus36

minus36minus37

minus30

minus36

minus29minus29

minus21

minus25minus19

minus30

minus31minus31

minus31

minus31

minus31

minus31

minus31

minus30

minus31

minus31

minus31minus31

minus31

minus31

minus33

minus33

minus33minus33minus33

minus33

minus32

minus32

minus33

minus33

minus33

minus33minus33

minus33

minus33

minus33

minus32

minus32minus32

minus32

minus32

minus31

minus5

minus5 minus5

minus4

minus4

minus4

minus4 minus4

minus4minus4minus4

minus4

minus4

minus4minus4

minus4

minus4minus3

minus1

minus1

minus1

minus1

minus1

minus1

minus3 minus3

minus3

minus3

minus1 minus1minus2minus2

minus2

NA

copy 1994-2000 Humphrey SystemsHFA II 740-11145-3232

minus3214 dB P lt 05

448dB P lt 05

(a)

Figure 5 Continued

6 Case Reports in Medicine

Single field analysis Eye leftName ID DOB 01-01-1989

Central 30-2 threshold testFixation monitor offFixation target centralFixation losses 00False POS errors 0False NEG errorsTest duration 0659

Fovea off

Date 30-11-2012Time 1319Age 23

Pupil diameterVisual acuityRX DS DC X

Stimulus III whiteBackground 315 ASBStrategy SITA standard

GHTOutside normal limits

MDPSD

lt5lt2

lt5lt2lt1

lt05

lt1lt05

Totaldeviation Pattern

deviation

3030

lt0

lt0lt0

lt0

lt0

1

1

1 1

1

0

0

0

0

0

0

0

0

00

0

0

0

18

18 19

11

11

12

1417

1713 18

26 23

26 26 26

24

26

26

26

24 24

27 28

28

28

28

27

27

31

30

31

31

31

31 31

3132

32

32

33 32

32

323230

3034

30

30

30

30

3128

28

28

28

25

25

25

25

29

29

20

20

29 29

33

69

minus2minus2

minus2

minus2

minus2

minus2

minus2 minus2

minus2

minus2minus2

minus2

minus4

minus2

minus2

minus2

minus2

minus2minus2

minus2minus3minus12

minus19

minus19 minus18

minus14

minus14

minus14 minus14

minus16

minus12

minus15

minus15

minus15

minus15 minus20

minus20

minus20

minus26minus26

minus26minus23 minus25minus22 minus21minus12 minus12minus21

minus11

minus26 minus10

minus3

minus3

minus3

minus4minus4

minus4

minus4 minus4

minus4

minus4

minus4

minus5

minus5minus5minus5

minus33minus33

minus32minus27minus33

minus33minus33minus33minus33

minus5minus16minus11

minus11

minus6

minus5

minus4

minus9minus9minus9 minus9

minus8minus8

minus3 minus1

minus1

minus1 minus1 minus1 minus1

minus1

minus1

minus1

minus1

minus1

minus1minus6 minus1 minus1 minus1 minus1

minus1

minus1

minus1

minus1

minus1minus1

minus3

minus3

minus3

minus3minus3

minus3minus3

minus3

minus3minus3

minus3

minus3 minus3

minus1

minus1

minus2

minus2

minus2

6

copy 1994-2000 Humphrey SystemsHFA II 740-11145-3232

minus771dB P lt 051077dB P lt 05

(b)

Figure 5 Visual field of right eye (a) and left eye (b) demonstrating deterioration of the right eye and involvement of the left

Case Reports in Medicine 7

a percentage of patients with impaired visual acuity In aseries of patients presented by Gass et al CDVA was 2040or more in 76 of patients at presentation and final visualacuity was 2040 or more in 68 of patients [2] Slit lampbiomicroscopy and fundus imaging examination (FA andICG) were unremarkable in our case which according tothe literature is a common condition in diagnosed AZOORcases since 50 of published cases present with no relatedfindings in FA [1] Optical coherence tomography (OCT) ofthe macula was normal According to the literature OCTmay show disruptions in the inner segmentouter segmentjunction line and in the outer segment tip lines of thephotoreceptors especially in the late phase of the disease [8]

Visual field defect is also one of themain findings in casesof AZOOR Several types of visual field defects have beenobserved such as blind spot enlargement constriction of theperipheral visual field and central scotomas [1] In our casethe patient presented with a full scotoma of the central visualfield in the initially affected eye which was not related toany other optic nerve pathology The course of our patient(deterioration of the most affected eye in the first monthsand involvement of the contralateral eye) was also typical ofAZOOR

Electrophysiology is the critical testing for confirmationof diagnosis in cases of AZOOR [1ndash3] Gass et al [2] reportedabnormal findings in ERG of all 51 patients in their series andmost of the published cases in the literature report abnormalERGs [1] Common findings are depressed scotopic andorphotopic amplitudes in one eye (the most severely affected)or both eyes [2] A negative amplitude is not common butwas also reported in the literature in one case by Gass et al[2] and in one case by Piao et al where dysfunction of theinner retina as well as the outer retina was proposed in suchcases [9] Our patient also presented with a ldquonegativerdquo ERGin his right eye Macular involvement was also demonstratedin that eye

In a retrospective series of 28 patients Francis et al[3] identified a pattern of electrophysiological anomalies inAZOOR cases where all affected eyes demonstrated delayedimplicit time of the 30Hz cone flicker ERG Our case alsodisplayed delayed implicit time in his right eye which is inaccordance with these criteria In the aforementioned caseseries [3] all of the patients had a marked reduction in theEOG light rise indicating a generalized dysfunction of theretinal pigment epithelium Accordingly case reports in thepublished literature that include EOG testing of the affectedeyes report abnormal results of EOG [1] In our case EOGlight rise and the Arden ratio were within normal limitsbilaterally despite the fact that the rest of the test results weresuggestive of AZOOR

In all reported cases and series of patients in the literaturethere is no definite description of the disease Even thepathophysiology of AZOOR is not clear since it is either con-sidered part of the white dot disease spectrum or triggered byinflammatory disorders such as punctuate inner choroiditisand multifocal inner choroiditis [3] Differential diagnosisof cases of AZOOR with such findings at presentation as inour case includes other conditions such as cancer associatedretinopathy and melanoma associated retinopathy [1] Our

patient had clear medical history and was referred to forcomplete evaluation for possible occultmalignancy includingclinical examination blood testing and chest and abdominalMRI which were not suggestive of such condition

4 Conclusion

In conclusion our patients had typical clinical findings ofAZOOR with photopsia and visual field defect Howeverit represents an unusual case of AZOOR as she presentedwith a ldquonegativerdquo ERG indicating an abnormal inner andouter retina dysfunction and with normal EOG recordingsThe latter may indicate that involvement of retinal pigmentepithelium as demonstrated by EOG abnormality is notimperative in the course of AZOOR

Conflict of Interests

The authors declare that there is no conflict of interestsregarding the publication of this paper

References

[1] D M Monson and J R Smith ldquoAcute zonal occult outerretinopathyrdquo Survey of Ophthalmology vol 56 no 1 pp 23ndash352011

[2] J D Gass A Agarwal and I U Scott ldquoAcute zonal occult outerretinopathy a long-term follow-up studyrdquo American Journal ofOphthalmology vol 134 no 3 pp 329ndash339 2002

[3] P J Francis A Marinescu F W Fitzke A C Bird and G EHolder ldquoAcute zonal occult outer retinopathy towards a set ofdiagnostic criteriardquo The British Journal of Ophthalmology vol89 no 1 pp 70ndash73 2005

[4] M Bach M G Brigell M Hawlina et al ldquoISCEV standardfor clinical pattern electroretinography (PERG) 2012 updaterdquoDocumenta Ophthalmologica vol 126 no 1 pp 1ndash7 2013

[5] M F Marmor A B Fulton G E Holder Y Miyake MBrigell and M Bach ldquoISCEV Standard for full-field clinicalelectroretinography (2008 update)rdquoDocumenta Ophthalmolog-ica vol 118 no 1 pp 69ndash77 2009

[6] J V Odom M Bach M Brigell G E Holder D L McCullochand A P Tormene ldquoISCEV standard for clinical visual evokedpotentials (2009 update)rdquo Documenta Ophthalmologica vol120 no 1 pp 111ndash119 2010

[7] M F Marmor M G Brigell D L McCulloch C A Westalland M Bach ldquoISCEV standard for clinical electro-oculography(2010 update)rdquo Documenta Ophthalmologica vol 122 no 1 pp1ndash7 2011

[8] T Wakazono S Ooto M Hangai and N Yoshimura ldquoPho-toreceptor outer segment abnormalities and retinal sensitivityin acute zonal occult outer retinopathyrdquo Retina vol 33 no 3pp 642ndash648 2013

[9] C Piao M Kondo S Ishikawa S Okinami and H TerasakildquoA case of unusual retinopathy showing features similar toacute zonal occult outer retinopathy associated with negativeelectroretinogramsrdquo Japanese Journal of Ophthalmology vol 51no 1 pp 69ndash71 2007

Submit your manuscripts athttpwwwhindawicom

Stem CellsInternational

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

MEDIATORSINFLAMMATION

of

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Behavioural Neurology

EndocrinologyInternational Journal of

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Disease Markers

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

BioMed Research International

OncologyJournal of

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Oxidative Medicine and Cellular Longevity

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

PPAR Research

The Scientific World JournalHindawi Publishing Corporation httpwwwhindawicom Volume 2014

Immunology ResearchHindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Journal of

ObesityJournal of

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Computational and Mathematical Methods in Medicine

OphthalmologyJournal of

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Diabetes ResearchJournal of

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Research and TreatmentAIDS

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Gastroenterology Research and Practice

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Parkinsonrsquos Disease

Evidence-Based Complementary and Alternative Medicine

Volume 2014Hindawi Publishing Corporationhttpwwwhindawicom

4 Case Reports in Medicine

Right

Peak

1998400div

Dark (15998400) Light (15998400)

Trough

(a)

Left

Peak

Trough

1998400div

Dark (15998400) Light (15998400)

Peak

Troughggg

(b)

Figure 4 EOG recordings demonstrating a normal light rise and Arden ratio bilaterally

inspection of anterior and posterior segments The patientsrsquoCDVA was 2050 in the right eye and 2020 in the lefteye Intraocular pressure was 14mmHg in the right eye and15mmHg in the left eye The patient had no history ofother ophthalmic or systemic diseases Anterior andposteriorsegments examination was unremarkableThe patient under-went visual field testing that revealed a large central scotomain the right eye and was normal in the left eye (Figure 1)

Fluorescein angiography and indocyanine green angiog-raphy were unremarkable bilaterally Optical coherencetomography (OCT) (Spectralis SD-OCT Heidelberg Engi-neering Inc Germany) also revealed no macular pathology(Figure 2)

Additionally the patient underwent PERG and ERGelectroretinograms according to the protocols recommendedby the International Society for Clinical Electrophysiology ofVision (ISCEV) [4 5] Stimulus 06 log units stronger thanthe ISCEV maximum standard flash was also used betterto demonstrate the dark adapted a-wave (DA 110) Visualevoked potentials to a pattern stimulus (PVEP) and (EOG)were also recorded according to ISCEV standards [6 7]

According to the results PERG P50 amplitude as anindex of macular function was reduced in the right eyebut was within normal limits in the left eye indicatingright eye macular dysfunction (Figure 3(a)) The PVEP P

100

component was of reduced amplitude and normal peak timein the right eye (possibly secondary to macular dysfunctionrather than reflecting primary optic nerve disease) whileresponses in the left eye were recorded within normal range

Rod specific ERG (DA 001) was of reduced amplitude inthe right eye only Bright flash dark adapted ERGs (DA 110)were of normal a-wave amplitude bilaterally while b-wavewas of reduced amplitude in the right eye and subnormal inthe left eye indicating a ldquonegative ERGrdquo in the right eye (a-wave amplitudes 302 120583V and 305 120583V in the right and left eyeresp b-wave amplitudes 276120583V and 345 120583V in the right andleft eye resp) (Figure 3(b)) Photopic single flash ERGs (LA30) responses were subnormal in the right eye only (a-waveamplitudes 25 120583V and 385 120583V in the right and left eye resp

b-wave amplitudes 115 120583V and 200 120583V in the right and lefteye (Figure 3(c)) Light adapted 30Hz flicker ERG (LA 30Hz)was delayed in the right eye but within normal limits in theleft eye (implicit time of 31msec and 26msec in the right andleft eyes resp) (Figure 3(d)) EOG light rise and the Ardenratio were within normal limits bilaterally (Arden ratio 42and 39 in the right and left eyes resp) (Figure 4)

The electrophysiological findings thus indicated general-ized retinal dysfunction (affecting both cone and rod systemsand inner retina) associated with macular involvement in theright eye only The patient was diagnosed with AZOOR dueto typical clinical visual field and ERG findings despite thenormal EOG findings

Two months after first visit patient evaluation wasrepeated Vision in her right eye had deteriorated to 2063while in the left eye it was stable at 2020 Visual field testingwas also repeated and revealed further depression of sensitiv-ity in the right eye and involvement of the left eye (Figure 5)The patient was followed up for 8 additional months andher symptoms and clinical findings were stabilized bilaterallyIn the course of follow-up she also underwent magneticresonance imaging of head and abdomen without findings

3 Discussion

Acute zonal occult outer retinopathy is a rare disease ofunknown etiology affecting mostly young female adults [1]There are only a few studies and case reports in the literatureof this condition describing electroretinography as the criticaltest for confirmation of diagnosis in addition to the clinicalfindings and the visual field defect [1ndash3] To the best of ourknowledge this case is the first described in the literaturewithnontypical electrophysiology due to the normal findings inEOG

Our case presented with vision deterioration in one eyeand photopsias bilaterally Photopsias are described as asymptom at presentation in the vast majority of the casesof AZOOR [1] Visual acuity is not always affected sincemost of eyes retain a visual acuity of 2020 Still there are

Case Reports in Medicine 5

Single field analysis Eye rightName ID DOB 01-01-1989

Central 30-2 threshold testFixation monitor blind spotFixation target centralFixation losses 017False POS errors 0False NEG errorsTest duration 0901

Fovea off

Date 30-11-2012Time 1307Age 23

Pupil diameterVisual acuityRX DS DC X

Stimulus III whiteBackground 315 ASBStrategy SITA standard

GHTOutside normal limits

MD

PSD

lt5lt2

lt5lt2

lt1lt05

lt1lt05

Totaldeviation

Patterndeviation

3030

lt0lt0

lt0

lt0

lt0

lt0

lt0lt0

lt0 lt0

lt0

lt0

lt0

lt0

lt0

lt0

lt0lt0lt0lt0

lt0lt0

lt0lt0

lt0lt0

lt0 lt0

lt0 lt0

lt0 lt0 lt0lt0

lt0

lt0

lt0

lt0

lt0

lt0

lt0 lt0

lt0

lt0

lt0 lt0

lt0

lt0

lt0 lt0

lt0

lt0 lt0

1

1

1

1

1

1

7 3

5

3

3

2

3

1

0

00

0

5

0

0

8

0

0

0

0

0

0

0

0 0 0

011

15

10

11

9

minus2

minus4

minus2

minus2

minus2

minus2

minus2

minus2

minus2

minus6

minus6

minus6 minus7

minus7minus7

minus7

minus3

minus3

minus3

minus3

minus3minus3

minus3

minus4

minus6

minus5

minus29

minus29

minus29

minus29

minus22

minus28

minus27

minus34minus34minus34

minus34 minus34

minus34 minus34

minus34

minus34minus34

minus34

minus34 minus34

minus34

minus35minus35

minus35

minus35

minus36minus36minus36

minus36minus37

minus30

minus36

minus29minus29

minus21

minus25minus19

minus30

minus31minus31

minus31

minus31

minus31

minus31

minus31

minus30

minus31

minus31

minus31minus31

minus31

minus31

minus33

minus33

minus33minus33minus33

minus33

minus32

minus32

minus33

minus33

minus33

minus33minus33

minus33

minus33

minus33

minus32

minus32minus32

minus32

minus32

minus31

minus5

minus5 minus5

minus4

minus4

minus4

minus4 minus4

minus4minus4minus4

minus4

minus4

minus4minus4

minus4

minus4minus3

minus1

minus1

minus1

minus1

minus1

minus1

minus3 minus3

minus3

minus3

minus1 minus1minus2minus2

minus2

NA

copy 1994-2000 Humphrey SystemsHFA II 740-11145-3232

minus3214 dB P lt 05

448dB P lt 05

(a)

Figure 5 Continued

6 Case Reports in Medicine

Single field analysis Eye leftName ID DOB 01-01-1989

Central 30-2 threshold testFixation monitor offFixation target centralFixation losses 00False POS errors 0False NEG errorsTest duration 0659

Fovea off

Date 30-11-2012Time 1319Age 23

Pupil diameterVisual acuityRX DS DC X

Stimulus III whiteBackground 315 ASBStrategy SITA standard

GHTOutside normal limits

MDPSD

lt5lt2

lt5lt2lt1

lt05

lt1lt05

Totaldeviation Pattern

deviation

3030

lt0

lt0lt0

lt0

lt0

1

1

1 1

1

0

0

0

0

0

0

0

0

00

0

0

0

18

18 19

11

11

12

1417

1713 18

26 23

26 26 26

24

26

26

26

24 24

27 28

28

28

28

27

27

31

30

31

31

31

31 31

3132

32

32

33 32

32

323230

3034

30

30

30

30

3128

28

28

28

25

25

25

25

29

29

20

20

29 29

33

69

minus2minus2

minus2

minus2

minus2

minus2

minus2 minus2

minus2

minus2minus2

minus2

minus4

minus2

minus2

minus2

minus2

minus2minus2

minus2minus3minus12

minus19

minus19 minus18

minus14

minus14

minus14 minus14

minus16

minus12

minus15

minus15

minus15

minus15 minus20

minus20

minus20

minus26minus26

minus26minus23 minus25minus22 minus21minus12 minus12minus21

minus11

minus26 minus10

minus3

minus3

minus3

minus4minus4

minus4

minus4 minus4

minus4

minus4

minus4

minus5

minus5minus5minus5

minus33minus33

minus32minus27minus33

minus33minus33minus33minus33

minus5minus16minus11

minus11

minus6

minus5

minus4

minus9minus9minus9 minus9

minus8minus8

minus3 minus1

minus1

minus1 minus1 minus1 minus1

minus1

minus1

minus1

minus1

minus1

minus1minus6 minus1 minus1 minus1 minus1

minus1

minus1

minus1

minus1

minus1minus1

minus3

minus3

minus3

minus3minus3

minus3minus3

minus3

minus3minus3

minus3

minus3 minus3

minus1

minus1

minus2

minus2

minus2

6

copy 1994-2000 Humphrey SystemsHFA II 740-11145-3232

minus771dB P lt 051077dB P lt 05

(b)

Figure 5 Visual field of right eye (a) and left eye (b) demonstrating deterioration of the right eye and involvement of the left

Case Reports in Medicine 7

a percentage of patients with impaired visual acuity In aseries of patients presented by Gass et al CDVA was 2040or more in 76 of patients at presentation and final visualacuity was 2040 or more in 68 of patients [2] Slit lampbiomicroscopy and fundus imaging examination (FA andICG) were unremarkable in our case which according tothe literature is a common condition in diagnosed AZOORcases since 50 of published cases present with no relatedfindings in FA [1] Optical coherence tomography (OCT) ofthe macula was normal According to the literature OCTmay show disruptions in the inner segmentouter segmentjunction line and in the outer segment tip lines of thephotoreceptors especially in the late phase of the disease [8]

Visual field defect is also one of themain findings in casesof AZOOR Several types of visual field defects have beenobserved such as blind spot enlargement constriction of theperipheral visual field and central scotomas [1] In our casethe patient presented with a full scotoma of the central visualfield in the initially affected eye which was not related toany other optic nerve pathology The course of our patient(deterioration of the most affected eye in the first monthsand involvement of the contralateral eye) was also typical ofAZOOR

Electrophysiology is the critical testing for confirmationof diagnosis in cases of AZOOR [1ndash3] Gass et al [2] reportedabnormal findings in ERG of all 51 patients in their series andmost of the published cases in the literature report abnormalERGs [1] Common findings are depressed scotopic andorphotopic amplitudes in one eye (the most severely affected)or both eyes [2] A negative amplitude is not common butwas also reported in the literature in one case by Gass et al[2] and in one case by Piao et al where dysfunction of theinner retina as well as the outer retina was proposed in suchcases [9] Our patient also presented with a ldquonegativerdquo ERGin his right eye Macular involvement was also demonstratedin that eye

In a retrospective series of 28 patients Francis et al[3] identified a pattern of electrophysiological anomalies inAZOOR cases where all affected eyes demonstrated delayedimplicit time of the 30Hz cone flicker ERG Our case alsodisplayed delayed implicit time in his right eye which is inaccordance with these criteria In the aforementioned caseseries [3] all of the patients had a marked reduction in theEOG light rise indicating a generalized dysfunction of theretinal pigment epithelium Accordingly case reports in thepublished literature that include EOG testing of the affectedeyes report abnormal results of EOG [1] In our case EOGlight rise and the Arden ratio were within normal limitsbilaterally despite the fact that the rest of the test results weresuggestive of AZOOR

In all reported cases and series of patients in the literaturethere is no definite description of the disease Even thepathophysiology of AZOOR is not clear since it is either con-sidered part of the white dot disease spectrum or triggered byinflammatory disorders such as punctuate inner choroiditisand multifocal inner choroiditis [3] Differential diagnosisof cases of AZOOR with such findings at presentation as inour case includes other conditions such as cancer associatedretinopathy and melanoma associated retinopathy [1] Our

patient had clear medical history and was referred to forcomplete evaluation for possible occultmalignancy includingclinical examination blood testing and chest and abdominalMRI which were not suggestive of such condition

4 Conclusion

In conclusion our patients had typical clinical findings ofAZOOR with photopsia and visual field defect Howeverit represents an unusual case of AZOOR as she presentedwith a ldquonegativerdquo ERG indicating an abnormal inner andouter retina dysfunction and with normal EOG recordingsThe latter may indicate that involvement of retinal pigmentepithelium as demonstrated by EOG abnormality is notimperative in the course of AZOOR

Conflict of Interests

The authors declare that there is no conflict of interestsregarding the publication of this paper

References

[1] D M Monson and J R Smith ldquoAcute zonal occult outerretinopathyrdquo Survey of Ophthalmology vol 56 no 1 pp 23ndash352011

[2] J D Gass A Agarwal and I U Scott ldquoAcute zonal occult outerretinopathy a long-term follow-up studyrdquo American Journal ofOphthalmology vol 134 no 3 pp 329ndash339 2002

[3] P J Francis A Marinescu F W Fitzke A C Bird and G EHolder ldquoAcute zonal occult outer retinopathy towards a set ofdiagnostic criteriardquo The British Journal of Ophthalmology vol89 no 1 pp 70ndash73 2005

[4] M Bach M G Brigell M Hawlina et al ldquoISCEV standardfor clinical pattern electroretinography (PERG) 2012 updaterdquoDocumenta Ophthalmologica vol 126 no 1 pp 1ndash7 2013

[5] M F Marmor A B Fulton G E Holder Y Miyake MBrigell and M Bach ldquoISCEV Standard for full-field clinicalelectroretinography (2008 update)rdquoDocumenta Ophthalmolog-ica vol 118 no 1 pp 69ndash77 2009

[6] J V Odom M Bach M Brigell G E Holder D L McCullochand A P Tormene ldquoISCEV standard for clinical visual evokedpotentials (2009 update)rdquo Documenta Ophthalmologica vol120 no 1 pp 111ndash119 2010

[7] M F Marmor M G Brigell D L McCulloch C A Westalland M Bach ldquoISCEV standard for clinical electro-oculography(2010 update)rdquo Documenta Ophthalmologica vol 122 no 1 pp1ndash7 2011

[8] T Wakazono S Ooto M Hangai and N Yoshimura ldquoPho-toreceptor outer segment abnormalities and retinal sensitivityin acute zonal occult outer retinopathyrdquo Retina vol 33 no 3pp 642ndash648 2013

[9] C Piao M Kondo S Ishikawa S Okinami and H TerasakildquoA case of unusual retinopathy showing features similar toacute zonal occult outer retinopathy associated with negativeelectroretinogramsrdquo Japanese Journal of Ophthalmology vol 51no 1 pp 69ndash71 2007

Submit your manuscripts athttpwwwhindawicom

Stem CellsInternational

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

MEDIATORSINFLAMMATION

of

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Behavioural Neurology

EndocrinologyInternational Journal of

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Disease Markers

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

BioMed Research International

OncologyJournal of

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Oxidative Medicine and Cellular Longevity

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

PPAR Research

The Scientific World JournalHindawi Publishing Corporation httpwwwhindawicom Volume 2014

Immunology ResearchHindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Journal of

ObesityJournal of

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Computational and Mathematical Methods in Medicine

OphthalmologyJournal of

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Diabetes ResearchJournal of

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Research and TreatmentAIDS

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Gastroenterology Research and Practice

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Parkinsonrsquos Disease

Evidence-Based Complementary and Alternative Medicine

Volume 2014Hindawi Publishing Corporationhttpwwwhindawicom

Case Reports in Medicine 5

Single field analysis Eye rightName ID DOB 01-01-1989

Central 30-2 threshold testFixation monitor blind spotFixation target centralFixation losses 017False POS errors 0False NEG errorsTest duration 0901

Fovea off

Date 30-11-2012Time 1307Age 23

Pupil diameterVisual acuityRX DS DC X

Stimulus III whiteBackground 315 ASBStrategy SITA standard

GHTOutside normal limits

MD

PSD

lt5lt2

lt5lt2

lt1lt05

lt1lt05

Totaldeviation

Patterndeviation

3030

lt0lt0

lt0

lt0

lt0

lt0

lt0lt0

lt0 lt0

lt0

lt0

lt0

lt0

lt0

lt0

lt0lt0lt0lt0

lt0lt0

lt0lt0

lt0lt0

lt0 lt0

lt0 lt0

lt0 lt0 lt0lt0

lt0

lt0

lt0

lt0

lt0

lt0

lt0 lt0

lt0

lt0

lt0 lt0

lt0

lt0

lt0 lt0

lt0

lt0 lt0

1

1

1

1

1

1

7 3

5

3

3

2

3

1

0

00

0

5

0

0

8

0

0

0

0

0

0

0

0 0 0

011

15

10

11

9

minus2

minus4

minus2

minus2

minus2

minus2

minus2

minus2

minus2

minus6

minus6

minus6 minus7

minus7minus7

minus7

minus3

minus3

minus3

minus3

minus3minus3

minus3

minus4

minus6

minus5

minus29

minus29

minus29

minus29

minus22

minus28

minus27

minus34minus34minus34

minus34 minus34

minus34 minus34

minus34

minus34minus34

minus34

minus34 minus34

minus34

minus35minus35

minus35

minus35

minus36minus36minus36

minus36minus37

minus30

minus36

minus29minus29

minus21

minus25minus19

minus30

minus31minus31

minus31

minus31

minus31

minus31

minus31

minus30

minus31

minus31

minus31minus31

minus31

minus31

minus33

minus33

minus33minus33minus33

minus33

minus32

minus32

minus33

minus33

minus33

minus33minus33

minus33

minus33

minus33

minus32

minus32minus32

minus32

minus32

minus31

minus5

minus5 minus5

minus4

minus4

minus4

minus4 minus4

minus4minus4minus4

minus4

minus4

minus4minus4

minus4

minus4minus3

minus1

minus1

minus1

minus1

minus1

minus1

minus3 minus3

minus3

minus3

minus1 minus1minus2minus2

minus2

NA

copy 1994-2000 Humphrey SystemsHFA II 740-11145-3232

minus3214 dB P lt 05

448dB P lt 05

(a)

Figure 5 Continued

6 Case Reports in Medicine

Single field analysis Eye leftName ID DOB 01-01-1989

Central 30-2 threshold testFixation monitor offFixation target centralFixation losses 00False POS errors 0False NEG errorsTest duration 0659

Fovea off

Date 30-11-2012Time 1319Age 23

Pupil diameterVisual acuityRX DS DC X

Stimulus III whiteBackground 315 ASBStrategy SITA standard

GHTOutside normal limits

MDPSD

lt5lt2

lt5lt2lt1

lt05

lt1lt05

Totaldeviation Pattern

deviation

3030

lt0

lt0lt0

lt0

lt0

1

1

1 1

1

0

0

0

0

0

0

0

0

00

0

0

0

18

18 19

11

11

12

1417

1713 18

26 23

26 26 26

24

26

26

26

24 24

27 28

28

28

28

27

27

31

30

31

31

31

31 31

3132

32

32

33 32

32

323230

3034

30

30

30

30

3128

28

28

28

25

25

25

25

29

29

20

20

29 29

33

69

minus2minus2

minus2

minus2

minus2

minus2

minus2 minus2

minus2

minus2minus2

minus2

minus4

minus2

minus2

minus2

minus2

minus2minus2

minus2minus3minus12

minus19

minus19 minus18

minus14

minus14

minus14 minus14

minus16

minus12

minus15

minus15

minus15

minus15 minus20

minus20

minus20

minus26minus26

minus26minus23 minus25minus22 minus21minus12 minus12minus21

minus11

minus26 minus10

minus3

minus3

minus3

minus4minus4

minus4

minus4 minus4

minus4

minus4

minus4

minus5

minus5minus5minus5

minus33minus33

minus32minus27minus33

minus33minus33minus33minus33

minus5minus16minus11

minus11

minus6

minus5

minus4

minus9minus9minus9 minus9

minus8minus8

minus3 minus1

minus1

minus1 minus1 minus1 minus1

minus1

minus1

minus1

minus1

minus1

minus1minus6 minus1 minus1 minus1 minus1

minus1

minus1

minus1

minus1

minus1minus1

minus3

minus3

minus3

minus3minus3

minus3minus3

minus3

minus3minus3

minus3

minus3 minus3

minus1

minus1

minus2

minus2

minus2

6

copy 1994-2000 Humphrey SystemsHFA II 740-11145-3232

minus771dB P lt 051077dB P lt 05

(b)

Figure 5 Visual field of right eye (a) and left eye (b) demonstrating deterioration of the right eye and involvement of the left

Case Reports in Medicine 7

a percentage of patients with impaired visual acuity In aseries of patients presented by Gass et al CDVA was 2040or more in 76 of patients at presentation and final visualacuity was 2040 or more in 68 of patients [2] Slit lampbiomicroscopy and fundus imaging examination (FA andICG) were unremarkable in our case which according tothe literature is a common condition in diagnosed AZOORcases since 50 of published cases present with no relatedfindings in FA [1] Optical coherence tomography (OCT) ofthe macula was normal According to the literature OCTmay show disruptions in the inner segmentouter segmentjunction line and in the outer segment tip lines of thephotoreceptors especially in the late phase of the disease [8]

Visual field defect is also one of themain findings in casesof AZOOR Several types of visual field defects have beenobserved such as blind spot enlargement constriction of theperipheral visual field and central scotomas [1] In our casethe patient presented with a full scotoma of the central visualfield in the initially affected eye which was not related toany other optic nerve pathology The course of our patient(deterioration of the most affected eye in the first monthsand involvement of the contralateral eye) was also typical ofAZOOR

Electrophysiology is the critical testing for confirmationof diagnosis in cases of AZOOR [1ndash3] Gass et al [2] reportedabnormal findings in ERG of all 51 patients in their series andmost of the published cases in the literature report abnormalERGs [1] Common findings are depressed scotopic andorphotopic amplitudes in one eye (the most severely affected)or both eyes [2] A negative amplitude is not common butwas also reported in the literature in one case by Gass et al[2] and in one case by Piao et al where dysfunction of theinner retina as well as the outer retina was proposed in suchcases [9] Our patient also presented with a ldquonegativerdquo ERGin his right eye Macular involvement was also demonstratedin that eye

In a retrospective series of 28 patients Francis et al[3] identified a pattern of electrophysiological anomalies inAZOOR cases where all affected eyes demonstrated delayedimplicit time of the 30Hz cone flicker ERG Our case alsodisplayed delayed implicit time in his right eye which is inaccordance with these criteria In the aforementioned caseseries [3] all of the patients had a marked reduction in theEOG light rise indicating a generalized dysfunction of theretinal pigment epithelium Accordingly case reports in thepublished literature that include EOG testing of the affectedeyes report abnormal results of EOG [1] In our case EOGlight rise and the Arden ratio were within normal limitsbilaterally despite the fact that the rest of the test results weresuggestive of AZOOR

In all reported cases and series of patients in the literaturethere is no definite description of the disease Even thepathophysiology of AZOOR is not clear since it is either con-sidered part of the white dot disease spectrum or triggered byinflammatory disorders such as punctuate inner choroiditisand multifocal inner choroiditis [3] Differential diagnosisof cases of AZOOR with such findings at presentation as inour case includes other conditions such as cancer associatedretinopathy and melanoma associated retinopathy [1] Our

patient had clear medical history and was referred to forcomplete evaluation for possible occultmalignancy includingclinical examination blood testing and chest and abdominalMRI which were not suggestive of such condition

4 Conclusion

In conclusion our patients had typical clinical findings ofAZOOR with photopsia and visual field defect Howeverit represents an unusual case of AZOOR as she presentedwith a ldquonegativerdquo ERG indicating an abnormal inner andouter retina dysfunction and with normal EOG recordingsThe latter may indicate that involvement of retinal pigmentepithelium as demonstrated by EOG abnormality is notimperative in the course of AZOOR

Conflict of Interests

The authors declare that there is no conflict of interestsregarding the publication of this paper

References

[1] D M Monson and J R Smith ldquoAcute zonal occult outerretinopathyrdquo Survey of Ophthalmology vol 56 no 1 pp 23ndash352011

[2] J D Gass A Agarwal and I U Scott ldquoAcute zonal occult outerretinopathy a long-term follow-up studyrdquo American Journal ofOphthalmology vol 134 no 3 pp 329ndash339 2002

[3] P J Francis A Marinescu F W Fitzke A C Bird and G EHolder ldquoAcute zonal occult outer retinopathy towards a set ofdiagnostic criteriardquo The British Journal of Ophthalmology vol89 no 1 pp 70ndash73 2005

[4] M Bach M G Brigell M Hawlina et al ldquoISCEV standardfor clinical pattern electroretinography (PERG) 2012 updaterdquoDocumenta Ophthalmologica vol 126 no 1 pp 1ndash7 2013

[5] M F Marmor A B Fulton G E Holder Y Miyake MBrigell and M Bach ldquoISCEV Standard for full-field clinicalelectroretinography (2008 update)rdquoDocumenta Ophthalmolog-ica vol 118 no 1 pp 69ndash77 2009

[6] J V Odom M Bach M Brigell G E Holder D L McCullochand A P Tormene ldquoISCEV standard for clinical visual evokedpotentials (2009 update)rdquo Documenta Ophthalmologica vol120 no 1 pp 111ndash119 2010

[7] M F Marmor M G Brigell D L McCulloch C A Westalland M Bach ldquoISCEV standard for clinical electro-oculography(2010 update)rdquo Documenta Ophthalmologica vol 122 no 1 pp1ndash7 2011

[8] T Wakazono S Ooto M Hangai and N Yoshimura ldquoPho-toreceptor outer segment abnormalities and retinal sensitivityin acute zonal occult outer retinopathyrdquo Retina vol 33 no 3pp 642ndash648 2013

[9] C Piao M Kondo S Ishikawa S Okinami and H TerasakildquoA case of unusual retinopathy showing features similar toacute zonal occult outer retinopathy associated with negativeelectroretinogramsrdquo Japanese Journal of Ophthalmology vol 51no 1 pp 69ndash71 2007

Submit your manuscripts athttpwwwhindawicom

Stem CellsInternational

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

MEDIATORSINFLAMMATION

of

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Behavioural Neurology

EndocrinologyInternational Journal of

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Disease Markers

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

BioMed Research International

OncologyJournal of

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Oxidative Medicine and Cellular Longevity

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

PPAR Research

The Scientific World JournalHindawi Publishing Corporation httpwwwhindawicom Volume 2014

Immunology ResearchHindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Journal of

ObesityJournal of

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Computational and Mathematical Methods in Medicine

OphthalmologyJournal of

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Diabetes ResearchJournal of

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Research and TreatmentAIDS

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Gastroenterology Research and Practice

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Parkinsonrsquos Disease

Evidence-Based Complementary and Alternative Medicine

Volume 2014Hindawi Publishing Corporationhttpwwwhindawicom

6 Case Reports in Medicine

Single field analysis Eye leftName ID DOB 01-01-1989

Central 30-2 threshold testFixation monitor offFixation target centralFixation losses 00False POS errors 0False NEG errorsTest duration 0659

Fovea off

Date 30-11-2012Time 1319Age 23

Pupil diameterVisual acuityRX DS DC X

Stimulus III whiteBackground 315 ASBStrategy SITA standard

GHTOutside normal limits

MDPSD

lt5lt2

lt5lt2lt1

lt05

lt1lt05

Totaldeviation Pattern

deviation

3030

lt0

lt0lt0

lt0

lt0

1

1

1 1

1

0

0

0

0

0

0

0

0

00

0

0

0

18

18 19

11

11

12

1417

1713 18

26 23

26 26 26

24

26

26

26

24 24

27 28

28

28

28

27

27

31

30

31

31

31

31 31

3132

32

32

33 32

32

323230

3034

30

30

30

30

3128

28

28

28

25

25

25

25

29

29

20

20

29 29

33

69

minus2minus2

minus2

minus2

minus2

minus2

minus2 minus2

minus2

minus2minus2

minus2

minus4

minus2

minus2

minus2

minus2

minus2minus2

minus2minus3minus12

minus19

minus19 minus18

minus14

minus14

minus14 minus14

minus16

minus12

minus15

minus15

minus15

minus15 minus20

minus20

minus20

minus26minus26

minus26minus23 minus25minus22 minus21minus12 minus12minus21

minus11

minus26 minus10

minus3

minus3

minus3

minus4minus4

minus4

minus4 minus4

minus4

minus4

minus4

minus5

minus5minus5minus5

minus33minus33

minus32minus27minus33

minus33minus33minus33minus33

minus5minus16minus11

minus11

minus6

minus5

minus4

minus9minus9minus9 minus9

minus8minus8

minus3 minus1

minus1

minus1 minus1 minus1 minus1

minus1

minus1

minus1

minus1

minus1

minus1minus6 minus1 minus1 minus1 minus1

minus1

minus1

minus1

minus1

minus1minus1

minus3

minus3

minus3

minus3minus3

minus3minus3

minus3

minus3minus3

minus3

minus3 minus3

minus1

minus1

minus2

minus2

minus2

6

copy 1994-2000 Humphrey SystemsHFA II 740-11145-3232

minus771dB P lt 051077dB P lt 05

(b)

Figure 5 Visual field of right eye (a) and left eye (b) demonstrating deterioration of the right eye and involvement of the left

Case Reports in Medicine 7

a percentage of patients with impaired visual acuity In aseries of patients presented by Gass et al CDVA was 2040or more in 76 of patients at presentation and final visualacuity was 2040 or more in 68 of patients [2] Slit lampbiomicroscopy and fundus imaging examination (FA andICG) were unremarkable in our case which according tothe literature is a common condition in diagnosed AZOORcases since 50 of published cases present with no relatedfindings in FA [1] Optical coherence tomography (OCT) ofthe macula was normal According to the literature OCTmay show disruptions in the inner segmentouter segmentjunction line and in the outer segment tip lines of thephotoreceptors especially in the late phase of the disease [8]

Visual field defect is also one of themain findings in casesof AZOOR Several types of visual field defects have beenobserved such as blind spot enlargement constriction of theperipheral visual field and central scotomas [1] In our casethe patient presented with a full scotoma of the central visualfield in the initially affected eye which was not related toany other optic nerve pathology The course of our patient(deterioration of the most affected eye in the first monthsand involvement of the contralateral eye) was also typical ofAZOOR

Electrophysiology is the critical testing for confirmationof diagnosis in cases of AZOOR [1ndash3] Gass et al [2] reportedabnormal findings in ERG of all 51 patients in their series andmost of the published cases in the literature report abnormalERGs [1] Common findings are depressed scotopic andorphotopic amplitudes in one eye (the most severely affected)or both eyes [2] A negative amplitude is not common butwas also reported in the literature in one case by Gass et al[2] and in one case by Piao et al where dysfunction of theinner retina as well as the outer retina was proposed in suchcases [9] Our patient also presented with a ldquonegativerdquo ERGin his right eye Macular involvement was also demonstratedin that eye

In a retrospective series of 28 patients Francis et al[3] identified a pattern of electrophysiological anomalies inAZOOR cases where all affected eyes demonstrated delayedimplicit time of the 30Hz cone flicker ERG Our case alsodisplayed delayed implicit time in his right eye which is inaccordance with these criteria In the aforementioned caseseries [3] all of the patients had a marked reduction in theEOG light rise indicating a generalized dysfunction of theretinal pigment epithelium Accordingly case reports in thepublished literature that include EOG testing of the affectedeyes report abnormal results of EOG [1] In our case EOGlight rise and the Arden ratio were within normal limitsbilaterally despite the fact that the rest of the test results weresuggestive of AZOOR

In all reported cases and series of patients in the literaturethere is no definite description of the disease Even thepathophysiology of AZOOR is not clear since it is either con-sidered part of the white dot disease spectrum or triggered byinflammatory disorders such as punctuate inner choroiditisand multifocal inner choroiditis [3] Differential diagnosisof cases of AZOOR with such findings at presentation as inour case includes other conditions such as cancer associatedretinopathy and melanoma associated retinopathy [1] Our

patient had clear medical history and was referred to forcomplete evaluation for possible occultmalignancy includingclinical examination blood testing and chest and abdominalMRI which were not suggestive of such condition

4 Conclusion

In conclusion our patients had typical clinical findings ofAZOOR with photopsia and visual field defect Howeverit represents an unusual case of AZOOR as she presentedwith a ldquonegativerdquo ERG indicating an abnormal inner andouter retina dysfunction and with normal EOG recordingsThe latter may indicate that involvement of retinal pigmentepithelium as demonstrated by EOG abnormality is notimperative in the course of AZOOR

Conflict of Interests

The authors declare that there is no conflict of interestsregarding the publication of this paper

References

[1] D M Monson and J R Smith ldquoAcute zonal occult outerretinopathyrdquo Survey of Ophthalmology vol 56 no 1 pp 23ndash352011

[2] J D Gass A Agarwal and I U Scott ldquoAcute zonal occult outerretinopathy a long-term follow-up studyrdquo American Journal ofOphthalmology vol 134 no 3 pp 329ndash339 2002

[3] P J Francis A Marinescu F W Fitzke A C Bird and G EHolder ldquoAcute zonal occult outer retinopathy towards a set ofdiagnostic criteriardquo The British Journal of Ophthalmology vol89 no 1 pp 70ndash73 2005

[4] M Bach M G Brigell M Hawlina et al ldquoISCEV standardfor clinical pattern electroretinography (PERG) 2012 updaterdquoDocumenta Ophthalmologica vol 126 no 1 pp 1ndash7 2013

[5] M F Marmor A B Fulton G E Holder Y Miyake MBrigell and M Bach ldquoISCEV Standard for full-field clinicalelectroretinography (2008 update)rdquoDocumenta Ophthalmolog-ica vol 118 no 1 pp 69ndash77 2009

[6] J V Odom M Bach M Brigell G E Holder D L McCullochand A P Tormene ldquoISCEV standard for clinical visual evokedpotentials (2009 update)rdquo Documenta Ophthalmologica vol120 no 1 pp 111ndash119 2010

[7] M F Marmor M G Brigell D L McCulloch C A Westalland M Bach ldquoISCEV standard for clinical electro-oculography(2010 update)rdquo Documenta Ophthalmologica vol 122 no 1 pp1ndash7 2011

[8] T Wakazono S Ooto M Hangai and N Yoshimura ldquoPho-toreceptor outer segment abnormalities and retinal sensitivityin acute zonal occult outer retinopathyrdquo Retina vol 33 no 3pp 642ndash648 2013

[9] C Piao M Kondo S Ishikawa S Okinami and H TerasakildquoA case of unusual retinopathy showing features similar toacute zonal occult outer retinopathy associated with negativeelectroretinogramsrdquo Japanese Journal of Ophthalmology vol 51no 1 pp 69ndash71 2007

Submit your manuscripts athttpwwwhindawicom

Stem CellsInternational

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

MEDIATORSINFLAMMATION

of

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Behavioural Neurology

EndocrinologyInternational Journal of

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Disease Markers

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

BioMed Research International

OncologyJournal of

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Oxidative Medicine and Cellular Longevity

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

PPAR Research

The Scientific World JournalHindawi Publishing Corporation httpwwwhindawicom Volume 2014

Immunology ResearchHindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Journal of

ObesityJournal of

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Computational and Mathematical Methods in Medicine

OphthalmologyJournal of

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Diabetes ResearchJournal of

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Research and TreatmentAIDS

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Gastroenterology Research and Practice

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Parkinsonrsquos Disease

Evidence-Based Complementary and Alternative Medicine

Volume 2014Hindawi Publishing Corporationhttpwwwhindawicom

Case Reports in Medicine 7

a percentage of patients with impaired visual acuity In aseries of patients presented by Gass et al CDVA was 2040or more in 76 of patients at presentation and final visualacuity was 2040 or more in 68 of patients [2] Slit lampbiomicroscopy and fundus imaging examination (FA andICG) were unremarkable in our case which according tothe literature is a common condition in diagnosed AZOORcases since 50 of published cases present with no relatedfindings in FA [1] Optical coherence tomography (OCT) ofthe macula was normal According to the literature OCTmay show disruptions in the inner segmentouter segmentjunction line and in the outer segment tip lines of thephotoreceptors especially in the late phase of the disease [8]

Visual field defect is also one of themain findings in casesof AZOOR Several types of visual field defects have beenobserved such as blind spot enlargement constriction of theperipheral visual field and central scotomas [1] In our casethe patient presented with a full scotoma of the central visualfield in the initially affected eye which was not related toany other optic nerve pathology The course of our patient(deterioration of the most affected eye in the first monthsand involvement of the contralateral eye) was also typical ofAZOOR

Electrophysiology is the critical testing for confirmationof diagnosis in cases of AZOOR [1ndash3] Gass et al [2] reportedabnormal findings in ERG of all 51 patients in their series andmost of the published cases in the literature report abnormalERGs [1] Common findings are depressed scotopic andorphotopic amplitudes in one eye (the most severely affected)or both eyes [2] A negative amplitude is not common butwas also reported in the literature in one case by Gass et al[2] and in one case by Piao et al where dysfunction of theinner retina as well as the outer retina was proposed in suchcases [9] Our patient also presented with a ldquonegativerdquo ERGin his right eye Macular involvement was also demonstratedin that eye

In a retrospective series of 28 patients Francis et al[3] identified a pattern of electrophysiological anomalies inAZOOR cases where all affected eyes demonstrated delayedimplicit time of the 30Hz cone flicker ERG Our case alsodisplayed delayed implicit time in his right eye which is inaccordance with these criteria In the aforementioned caseseries [3] all of the patients had a marked reduction in theEOG light rise indicating a generalized dysfunction of theretinal pigment epithelium Accordingly case reports in thepublished literature that include EOG testing of the affectedeyes report abnormal results of EOG [1] In our case EOGlight rise and the Arden ratio were within normal limitsbilaterally despite the fact that the rest of the test results weresuggestive of AZOOR

In all reported cases and series of patients in the literaturethere is no definite description of the disease Even thepathophysiology of AZOOR is not clear since it is either con-sidered part of the white dot disease spectrum or triggered byinflammatory disorders such as punctuate inner choroiditisand multifocal inner choroiditis [3] Differential diagnosisof cases of AZOOR with such findings at presentation as inour case includes other conditions such as cancer associatedretinopathy and melanoma associated retinopathy [1] Our

patient had clear medical history and was referred to forcomplete evaluation for possible occultmalignancy includingclinical examination blood testing and chest and abdominalMRI which were not suggestive of such condition

4 Conclusion

In conclusion our patients had typical clinical findings ofAZOOR with photopsia and visual field defect Howeverit represents an unusual case of AZOOR as she presentedwith a ldquonegativerdquo ERG indicating an abnormal inner andouter retina dysfunction and with normal EOG recordingsThe latter may indicate that involvement of retinal pigmentepithelium as demonstrated by EOG abnormality is notimperative in the course of AZOOR

Conflict of Interests

The authors declare that there is no conflict of interestsregarding the publication of this paper

References

[1] D M Monson and J R Smith ldquoAcute zonal occult outerretinopathyrdquo Survey of Ophthalmology vol 56 no 1 pp 23ndash352011

[2] J D Gass A Agarwal and I U Scott ldquoAcute zonal occult outerretinopathy a long-term follow-up studyrdquo American Journal ofOphthalmology vol 134 no 3 pp 329ndash339 2002

[3] P J Francis A Marinescu F W Fitzke A C Bird and G EHolder ldquoAcute zonal occult outer retinopathy towards a set ofdiagnostic criteriardquo The British Journal of Ophthalmology vol89 no 1 pp 70ndash73 2005

[4] M Bach M G Brigell M Hawlina et al ldquoISCEV standardfor clinical pattern electroretinography (PERG) 2012 updaterdquoDocumenta Ophthalmologica vol 126 no 1 pp 1ndash7 2013

[5] M F Marmor A B Fulton G E Holder Y Miyake MBrigell and M Bach ldquoISCEV Standard for full-field clinicalelectroretinography (2008 update)rdquoDocumenta Ophthalmolog-ica vol 118 no 1 pp 69ndash77 2009

[6] J V Odom M Bach M Brigell G E Holder D L McCullochand A P Tormene ldquoISCEV standard for clinical visual evokedpotentials (2009 update)rdquo Documenta Ophthalmologica vol120 no 1 pp 111ndash119 2010

[7] M F Marmor M G Brigell D L McCulloch C A Westalland M Bach ldquoISCEV standard for clinical electro-oculography(2010 update)rdquo Documenta Ophthalmologica vol 122 no 1 pp1ndash7 2011

[8] T Wakazono S Ooto M Hangai and N Yoshimura ldquoPho-toreceptor outer segment abnormalities and retinal sensitivityin acute zonal occult outer retinopathyrdquo Retina vol 33 no 3pp 642ndash648 2013

[9] C Piao M Kondo S Ishikawa S Okinami and H TerasakildquoA case of unusual retinopathy showing features similar toacute zonal occult outer retinopathy associated with negativeelectroretinogramsrdquo Japanese Journal of Ophthalmology vol 51no 1 pp 69ndash71 2007

Submit your manuscripts athttpwwwhindawicom

Stem CellsInternational

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

MEDIATORSINFLAMMATION

of

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Behavioural Neurology

EndocrinologyInternational Journal of

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Disease Markers

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

BioMed Research International

OncologyJournal of

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Oxidative Medicine and Cellular Longevity

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

PPAR Research

The Scientific World JournalHindawi Publishing Corporation httpwwwhindawicom Volume 2014

Immunology ResearchHindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Journal of

ObesityJournal of

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Computational and Mathematical Methods in Medicine

OphthalmologyJournal of

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Diabetes ResearchJournal of

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Research and TreatmentAIDS

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Gastroenterology Research and Practice

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Parkinsonrsquos Disease

Evidence-Based Complementary and Alternative Medicine

Volume 2014Hindawi Publishing Corporationhttpwwwhindawicom

Submit your manuscripts athttpwwwhindawicom

Stem CellsInternational

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

MEDIATORSINFLAMMATION

of

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Behavioural Neurology

EndocrinologyInternational Journal of

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Disease Markers

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

BioMed Research International

OncologyJournal of

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Oxidative Medicine and Cellular Longevity

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

PPAR Research

The Scientific World JournalHindawi Publishing Corporation httpwwwhindawicom Volume 2014

Immunology ResearchHindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Journal of

ObesityJournal of

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Computational and Mathematical Methods in Medicine

OphthalmologyJournal of

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Diabetes ResearchJournal of

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Research and TreatmentAIDS

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Gastroenterology Research and Practice

Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014

Parkinsonrsquos Disease

Evidence-Based Complementary and Alternative Medicine

Volume 2014Hindawi Publishing Corporationhttpwwwhindawicom