Optical Coherence Tomography and Investigation of Optic Neuropathies

Mohammed Al-NaqeebMohammed Al-NaqeebUmm Al-Qura UniversityUmm Al-Qura University

Optical Coherence Optical Coherence Tomography and Tomography and Investigation of Optic Investigation of Optic NeuropathiesNeuropathies

DemyelinatingDemyelinating CompressiveCompressive IschemicIschemic Toxic/NutritionalToxic/Nutritional TraumaticTraumatic HereditaryHereditary Inflammatory/InfectiousInflammatory/Infectious

Optic NeuropathiesOptic Neuropathies

To monitor loss of retinal To monitor loss of retinal nerve fiber layer (RNFL) nerve fiber layer (RNFL) thicknessthickness

RNFL thickness is a RNFL thickness is a reflection of axonal integrity reflection of axonal integrity in response to injuryin response to injury

Optic Neuropathies Optic Neuropathies and OCTand OCT

70-year old gentleman70-year old gentleman ““Blurred vision” in the right eye Blurred vision” in the right eye

upon awakeningupon awakening No Giant Cell Arteritis No Giant Cell Arteritis

SymptomsSymptoms Vascular risk factors: Diabetes, Vascular risk factors: Diabetes,

Hypertension, and DyslipidemiaHypertension, and Dyslipidemia

Case 1: Anterior Case 1: Anterior Ischemic Optic Ischemic Optic NeuropathyNeuropathy

Visual AcuityVisual Acuity:: 20/40 OD20/40 OD 20/25 OS20/25 OS Pupils:Pupils: RAPD ODRAPD OD IOP:IOP: 19 mm Hg in both eyes19 mm Hg in both eyes Fundus:Fundus: Optic disc edema in Optic disc edema in

the right eye. Absent the right eye. Absent physiological cup in the left physiological cup in the left eyeeye

Case 1: AIONCase 1: AION

Vision worsened in the Vision worsened in the right eye after a week - right eye after a week - Count fingersCount fingers

No Symptoms of GCANo Symptoms of GCA ESR = 6 mm per hourESR = 6 mm per hour

Case 1: AION Clinical Case 1: AION Clinical CourseCourse

Fundus PhotosFundus Photos

OD OS

Optic disc edema (OD)

OS OD

•MD -3.60 dB •MD -25.80 dB

•Greater loss in superior field

Case 1: AION and VFCase 1: AION and VF

Fast RNFL Thickness Fast RNFL Thickness (3.4)(3.4)

3.4 mm

1.92 seconds

Mean RNFL = 52 µ

Mean RNFL = 82 µ

•RNFL thinning in the right eye relative to the left eye

•Greatest loss in inferior region correlating with superior VF loss

Case 1: RNFL findingsCase 1: RNFL findings

OCT done 6 months OCT done 6 months following ischemic onsetfollowing ischemic onset

What is timeline for axon What is timeline for axon loss to occur?loss to occur?

Does timeline differ Does timeline differ across other optic across other optic neuropathies?neuropathies?

Case 1: Anterior Case 1: Anterior Ischemic Optic Ischemic Optic NeuropathyNeuropathy

20-year old young woman20-year old young woman Developed “blurring” in Developed “blurring” in

the right eyethe right eye Pain with extraocular Pain with extraocular

movementsmovements

Case 2: Recurrent Case 2: Recurrent Optic NeuritisOptic Neuritis

Case 2: Recurrent Optic Case 2: Recurrent Optic NeuritisNeuritis

Past Medical HistoryPast Medical History Bilateral optic neuritis in 1993Bilateral optic neuritis in 1993

– treated with IV steroidstreated with IV steroids– Cranial MRI scan normalCranial MRI scan normal– CSF analysis normalCSF analysis normal– No antecedent illnessNo antecedent illness– Residual vision loss 20/40 OD and Residual vision loss 20/40 OD and

color vision deficitcolor vision deficit

Recurrent optic neuritis in the Recurrent optic neuritis in the right eye in 2000right eye in 2000– Repeat MRI scan normalRepeat MRI scan normal– Vision recovered to baselineVision recovered to baseline

Recurrence of optic neuritis in Recurrence of optic neuritis in right eye in spring 2003right eye in spring 2003


Present Visual Acuity:Present Visual Acuity: 20/40 OD20/40 OD 20/20 OS20/20 OS

Pupils:Pupils: Right RAPD Right RAPD Fundus:Fundus: Bilateral optic Bilateral optic

atrophy (temporal pallor)atrophy (temporal pallor)


ODOS•MD -10.39 dB

•Central loss greater in OD

•MD -3.81 dB

Case 2: Visual FieldsCase 2: Visual Fields

RNFL RNFL ThicknessThickness

OD

OS

•Bilateral RNFL thinning

• worse in OD

Few reserve axons remaining in OD– Following 3 bouts of optic

neuritis

What extent of axonal loss will predict a permanent deficit in vision?

Case 1: Recurrent Case 1: Recurrent Optic NeuritisOptic Neuritis

32-year old woman32-year old woman Diagnosed with MS in 1992Diagnosed with MS in 1992 Reported 6 recurrent bouts Reported 6 recurrent bouts

of optic neuritis affecting of optic neuritis affecting both eyesboth eyes

Denied active optic Denied active optic neuritits at the time of neuritits at the time of examinationexamination

Case 3: Optic NeuritisCase 3: Optic Neuritis

Visual Acuity:Visual Acuity: 20/25 OU 20/25 OU Pupils:Pupils: left RAPD left RAPD Color Vision:Color Vision: 9/16 Ishihara 9/16 Ishihara

plates in the right eye, and plates in the right eye, and 6/16 plates in the left eye6/16 plates in the left eye

Fundus:Fundus: Bilateral optic Bilateral optic atrophyatrophy


OS OD


MD -4.31 dB

MD -3.03 dB

OS OD


•Residual central field depression OU•Greater in left eye

RNFL RNFL ThicknessThickness

OS

OD

•RNFL thinning in the left eye relative to the right

RNFL vs Visual Field RNFL vs Visual Field LossLoss

How well does the pattern How well does the pattern of RNFL loss reflect or of RNFL loss reflect or correlate with visual field correlate with visual field loss in these patients?loss in these patients?

18 year old male18 year old male Developed headache and Developed headache and

vision loss in both eyesvision loss in both eyes Diagnosed with TB MeningitisDiagnosed with TB Meningitis

– Large suprasellar tuberculoma Large suprasellar tuberculoma – Causing compression of right Causing compression of right

ON and optic chiasmON and optic chiasm

Case 4: Compressive Case 4: Compressive Optic NeuropathyOptic Neuropathy

Visual Acuity:Visual Acuity: CF ODCF OD20/25 OS20/25 OS

Pupils:Pupils: Fixed pupil on right, Fixed pupil on right, with right RAPD (by reverse with right RAPD (by reverse testing)testing)

Ocular Motility:Ocular Motility: Right third Right third nerve palsy with aberrant nerve palsy with aberrant renervationrenervation

Fundi:Fundi: Bilateral optic atrophy Bilateral optic atrophy


OS OD


•Dense central scotoma OD•Temporal cut OS

Case 4: Compressive Optic Neuropathy

MRI: T1-weighted, post Gd

Cystic Suprasellar Mass

Mean=40µ • profound bilateral RNFL thinning

•worse in the right eye

•OCT findings correlated well functional measures of

visual integrity


Mean=53µ

41-year old woman 41-year old woman Developed sudden onset Developed sudden onset

vertigo and nausea in the vertigo and nausea in the fall of 2002fall of 2002

Developed vision loss Developed vision loss (nasal) and a floater and a (nasal) and a floater and a “sparkle” in the right eye “sparkle” in the right eye

Case 5: Optic Neuritis?Case 5: Optic Neuritis?

Visual acuity measured Visual acuity measured 20/20 in both eyes20/20 in both eyes

Right RAPDRight RAPD Color Vision 16/16 Ishihara Color Vision 16/16 Ishihara

plates in both eyesplates in both eyes Fundi: Examination normalFundi: Examination normal


The visual field defect The visual field defect persistedpersisted

Cranial MRI scan normal Cranial MRI scan normal Orbital CT scan normalOrbital CT scan normal CSF analysis normalCSF analysis normal


OS OD

•Mean Deviation -7.55 dB•Nasal superior and inferior visual field loss

•Mean Deviation – 0.31dB

•Normal field


Case 5: Multifocal Case 5: Multifocal ERGsERGs

200 nV

0 80 ms

200 nV

0 80 ms

OD OS

Retinalview

Retinalview

mERG Trace Arrays

•Multifocal ERG recordings from 61 regions in the central 45 degrees•mERG trace arrays appear reduced in inferior and superior temporal retina in OD•mERG trace arrays appear normal in OS

Case 5: Multifocal Case 5: Multifocal ERGsERGs

0 2 4 6 8 10 12 14 16 18 20 nV/deg^2

5°10°15°20°25°30°Total Response:7.83 nV/deg^2

0 2 4 6 8 10 12 14 16 18 20 nV/deg^2


-5 -4 -3 -2 -1 0 1 2 3 4 5 StDev

5°10°15°20°25°30°

0 2 4 6 8 10 12 14 16 18 20 nV/deg^2


0 2 4 6 8 10 12 14 16 18 20 nV/deg^2


-5 -4 -3 -2 -1 0 1 2 3 4 5 StDev

5°10°15°20°25°30°OD OS

Statistical Probability Maps

The Patient85 normaleyes

SPM

•Statistical probability mapping of response density was normal in OS•Reduced response density in inferior and superior temporal retina in OD•Correlates with visual field defect in OD

RNFLRNFL

RNFL RNFL thickness thickness within normal within normal limits OUlimits OU

Good RNFL Good RNFL symmetry symmetry between between eyeseyes

In May, 2003 the patient reported In May, 2003 the patient reported new “sparkles” in the left eyenew “sparkles” in the left eye

Summary:Summary:– Atypical Optic NeuritisAtypical Optic Neuritis– MS work-up negativeMS work-up negative– Lack of optic disc pallorLack of optic disc pallor– Persistent visual field defectPersistent visual field defect– Positive visual phenomena Positive visual phenomena

and floaters in both eyesand floaters in both eyes


Diagnosis: Acute Diagnosis: Acute Zonal Occult Outer Zonal Occult Outer RetinopathyRetinopathy


The clinical distinction The clinical distinction between a retinal versus between a retinal versus an optic nerve problem an optic nerve problem may be difficultmay be difficult

Ancillary studies such as Ancillary studies such as OCT and mERG can be OCT and mERG can be very useful in this regardvery useful in this regard


61-year old woman was hit with 61-year old woman was hit with a tennis ball in the left temple in a tennis ball in the left temple in October 2002October 2002

Developed chronic headachesDeveloped chronic headaches Noted inferior visual field loss in Noted inferior visual field loss in

the left eyethe left eye Referred to the Neuro-Referred to the Neuro-

Ophthalmology Clinic in January Ophthalmology Clinic in January 20032003

Case 6: Traumatic Case 6: Traumatic Optic Neuropathy?Optic Neuropathy?

Visual Acuity:Visual Acuity: 20/20 in the right 20/20 in the right eye and 20/25 in the left eyeeye and 20/25 in the left eye

Pupils:Pupils: Equal with no RAPD Equal with no RAPD Color Vision:Color Vision: 15/16 Ishihara 15/16 Ishihara

Plates in the right eye, and Plates in the right eye, and 12/16 plates in the left eye12/16 plates in the left eye

Fundi:Fundi: Normal Normal


Serology Studies- Serology Studies- NormalNormal

Cranial/Orbital CT scan- Cranial/Orbital CT scan- NormalNormal

Orbital Ultrasound- Orbital Ultrasound- NormalNormal


In April, 2003 the patient In April, 2003 the patient developed new visual field developed new visual field loss in the right eyeloss in the right eye

In May, 2003 she noted In May, 2003 she noted sparkles, flashes, and sparkles, flashes, and floaters in both eyesfloaters in both eyes


OS

MD -6.91 dB

OD

MD -0.64 dB

•Visual Fields Obtained in March 2003•OD-Normal•OS-defects in superior and inferior

Case 6: Traumatic Case 6: Traumatic Optic NeuropathyOptic Neuropathy

•Visual Fields obtained in April 2003•OD- now developing VF defects•OS- visual defects worsened

OS

MD -7.81 dB

OD

MD -2.41 dB


Repeat ExaminationRepeat Examination– Visual acuity:Visual acuity: 20/25 in both 20/25 in both

eyeseyes– Pupils:Pupils: equal with no RAPD equal with no RAPD– Color Vision:Color Vision: 12/16 Ishihara 12/16 Ishihara

Plates in the right eye, and Plates in the right eye, and 7.5/16 plates in the left eye7.5/16 plates in the left eye

– Fundi:Fundi: Normal Normal


200 nV

0 80 ms

200 nV

0 80 ms

OD OS

Retinalview

Retinalview

mERG Trace Arrays

•Multifocal ERGs from 61 regions in the central 45 degrees•mERGs from OD are diminished centrally extending to superior nasal retina•mERGs from OS show multiple patchy areas of abnormality


Case 6: Traumatic Case 6: Traumatic Optic NeuropathyOptic Neuropathy

0 2 4 6 8 10 12 14 16 18 20 nV/deg^2


0 2 4 6 8 10 12 14 16 18 20 nV/deg^2


-3 -2 -1 0 1 2 3 StDev

5°10°15°20°25°30°

0 2 4 6 8 10 12 14 16 18 20 nV/deg^2


0 2 4 6 8 10 12 14 16 18 20 nV/deg^2


-3 -2 -1 0 1 2 3 StDev

5°10°15°20°25°30°

•Statistical probability mapping shows areas of significantly reduced response density centrally and in superior nasal retina in OD•OS shows much greater involvement in response density reduction in inferior and superior retina

Statistical Probability Maps

OD OS

RNFLRNFL

RNFL RNFL thickness thickness within normal within normal limits OUlimits OU

Good RNFL Good RNFL symmetry symmetry between between eyeseyes

Diagnosis: Acute Zonal Diagnosis: Acute Zonal Occult Outer Occult Outer RetinopathyRetinopathy


Para-neoplastic work-up was recommended by Retinal Specialist

Final Observations and Final Observations and ConclusionsConclusions Cases 5 & 6 demonstrate the utility Cases 5 & 6 demonstrate the utility

of OCT and mERG in differentiation of of OCT and mERG in differentiation of optic nerve vs retinal insultsoptic nerve vs retinal insults

OCT measurement of RNFL OCT measurement of RNFL – Reproducibility of 10-20 Reproducibility of 10-20 µµ– Adequate for long-term follow-up of Adequate for long-term follow-up of

progressive RNFL damageprogressive RNFL damage OCT may prove useful in compressive OCT may prove useful in compressive

disease in predicting the likelihood of disease in predicting the likelihood of visual recovery based upon remaining visual recovery based upon remaining RNFL available at time of diagnosisRNFL available at time of diagnosis

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Optical Coherence Tomography and Investigation of Optic Neuropathies

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