Optical coherence tomography and investigation of optic neuropathies
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Optical Coherence Tomography and Investigation of Optic Neuropathies. Dina Mohammed Abdulmannan Umm Al- Qura University. Optic Neuropathies. Demyelinating Compressive Ischemic Toxic/Nutritional Traumatic Hereditary Inflammatory/Infectious. Optic Neuropathies and OCT.

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Optical coherence tomography and investigation of optic neuropathies

Optical Coherence Tomography and Investigation of Optic Neuropathies

Dina Mohammed Abdulmannan

Umm Al-Qura University


Optic neuropathies

Optic Neuropathies

  • Demyelinating

  • Compressive

  • Ischemic

  • Toxic/Nutritional

  • Traumatic

  • Hereditary

  • Inflammatory/Infectious


Optic neuropathies and oct

Optic Neuropathies and OCT

  • To monitor loss of retinal nerve fiber layer (RNFL) thickness

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


Case 1 anterior ischemic optic neuropathy

Case 1: Anterior Ischemic Optic Neuropathy

  • 70-year old gentleman

  • “Blurred vision” in the right eye upon awakening

  • No Giant Cell Arteritis Symptoms

  • Vascular risk factors: Diabetes, Hypertension, and Dyslipidemia


Case 1 aion

Case 1: AION

  • Visual Acuity:20/40 OD

    20/25 OS

  • Pupils:RAPD OD

  • IOP:19 mm Hg in both eyes

  • Fundus:Optic disc edema in the right eye. Absent physiological cup in the left eye


Case 1 aion clinical course

Case 1: AION Clinical Course

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

  • No Symptoms of GCA

  • ESR = 6 mm per hour


Fundus photos

OS

OD

Fundus Photos

Optic disc

edema (OD)


Case 1 aion and vf

OS

OD

Case 1: AION and VF

  • MD -25.80 dB

  • Greater loss in superior field

  • MD -3.60 dB


Fast rnfl thickness 3 4

Fast RNFL Thickness (3.4)

3.4 mm

1.92 seconds


Case 1 rnfl findings

Case 1: RNFL findings

Mean RNFL = 52 µ

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

  • Greatest loss in inferior region correlating with superior VF loss

Mean RNFL = 82 µ


Case 1 anterior ischemic optic neuropathy1

Case 1: Anterior Ischemic Optic Neuropathy

  • OCT done 6 months following ischemic onset

  • What is timeline for axon loss to occur?

  • Does timeline differ across other optic neuropathies?


Case 2 recurrent optic neuritis

Case 2: Recurrent Optic Neuritis

  • 20-year old young woman

  • Developed “blurring” in the right eye

  • Pain with extraocular movements


Case 2 recurrent optic neuritis1

Case 2: Recurrent Optic Neuritis

Past Medical History

  • Bilateral optic neuritis in 1993

    • treated with IV steroids

    • Cranial MRI scan normal

    • CSF analysis normal

    • No antecedent illness

    • Residual vision loss 20/40 OD and color vision deficit


Optical coherence tomography and investigation of optic neuropathies

Case 2: Recurrent Optic Neuritis

  • Recurrent optic neuritis in the right eye in 2000

    • Repeat MRI scan normal

    • Vision recovered to baseline

  • Recurrence of optic neuritis in right eye in spring 2003


Case 2 recurrent optic neuritis2

Case 2: Recurrent Optic Neuritis

  • Present Visual Acuity:

    20/40 OD20/20 OS

  • Pupils: Right RAPD

  • Fundus: Bilateral optic atrophy (temporal pallor)


Case 2 visual fields

OD

OS

Case 2: Visual Fields

  • MD -3.81 dB

  • MD -10.39 dB

  • Central loss greater in OD


Rnfl thickness

OD

OS

RNFL Thickness

  • Bilateral RNFL thinning

  • worse in OD


Case 1 recurrent optic neuritis

Case 1: Recurrent Optic Neuritis

  • 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 3 optic neuritis

Case 3: Optic Neuritis

  • 32-year old woman

  • Diagnosed with MS in 1992

  • Reported 6 recurrent bouts of optic neuritis affecting both eyes

  • Denied active optic neuritits at the time of examination


Case 3 optic neuritis1

Case 3: Optic Neuritis

  • Visual Acuity: 20/25 OU

  • Pupils: left RAPD

  • Color Vision: 9/16 Ishihara plates in the right eye, and 6/16 plates in the left eye

  • Fundus: Bilateral optic atrophy


Case 3 optic neuritis2

OD

OS

Case 3: Optic Neuritis

MD -4.31 dB

MD -3.03 dB


Case 3 optic neuritis3

OS

OD

Case 3: Optic Neuritis

  • Residual central field depression OU

  • Greater in left eye


Rnfl thickness1

OD

RNFL Thickness

OS

  • RNFL thinning in the left eye relative to the right


Rnfl vs visual field loss

RNFL vs Visual Field Loss

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


Case 4 compressive optic neuropathy

Case 4: Compressive Optic Neuropathy

  • 18 year old male

  • Developed headache and vision loss in both eyes

  • Diagnosed with TB Meningitis

    • Large suprasellar tuberculoma

    • Causing compression of right ON and optic chiasm


Case 4 compressive optic neuropathy1

Case 4: Compressive Optic Neuropathy

  • Visual Acuity: CF OD

    20/25 OS

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

  • Ocular Motility: Right third nerve palsy with aberrant renervation

  • Fundi: Bilateral optic atrophy


Case 4 compressive optic neuropathy2

OS

OD

Case 4: Compressive Optic Neuropathy

  • Dense central scotoma OD

  • Temporal cut OS


Optical coherence tomography and investigation of optic neuropathies

Case 4: Compressive Optic Neuropathy

MRI: T1-weighted, post Gd

Cystic Suprasellar Mass


Case 4 compressive optic neuropathy3

Case 4: Compressive Optic Neuropathy

Mean=40µ

  • profound bilateral RNFL thinning

  • worse in the right eye

  • OCT findings correlated well functional measures of visual integrity

Mean=53µ


Case 5 optic neuritis

Case 5: Optic Neuritis?

  • 41-year old woman

  • Developed sudden onset vertigo and nausea in the fall of 2002

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


Case 5 optic neuritis1

Case 5: Optic Neuritis?

  • Visual acuity measured 20/20 in both eyes

  • Right RAPD

  • Color Vision 16/16 Ishihara plates in both eyes

  • Fundi: Examination normal


Case 5 optic neuritis2

Case 5: Optic Neuritis?

  • The visual field defect persisted

  • Cranial MRI scan normal

  • Orbital CT scan normal

  • CSF analysis normal


Case 5 optic neuritis3

Case 5: Optic Neuritis?

OS

OD

  • Mean Deviation – 0.31dB

  • Normal field

  • Mean Deviation -7.55 dB

  • Nasal superior and inferior visual field loss


Case 5 multifocal ergs

Case 5: Multifocal ERGs

mERG Trace Arrays

Retinal

view

Retinal

view

OD

OS

  • 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 ergs1

Case 5: Multifocal ERGs

Statistical Probability Maps

OD

OS

SPM

85 normal

eyes

The Patient

  • 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


Optical coherence tomography and investigation of optic neuropathies

RNFL

  • RNFL thickness within normal limits OU

  • Good RNFL symmetry between eyes


Case 5 optic neuritis4

Case 5: Optic Neuritis?

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

    Summary:

    • Atypical Optic Neuritis

    • MS work-up negative

    • Lack of optic disc pallor

    • Persistent visual field defect

    • Positive visual phenomena and floaters in both eyes


Case 5 optic neuritis5

Case 5: Optic Neuritis?

  • Diagnosis: Acute Zonal Occult Outer Retinopathy


Case 5 optic neuritis6

Case 5: Optic Neuritis?

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

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


Case 6 traumatic optic neuropathy

Case 6: Traumatic Optic Neuropathy?

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

  • Developed chronic headaches

  • Noted inferior visual field loss in the left eye

  • Referred to the Neuro-Ophthalmology Clinic in January 2003


Case 6 traumatic optic neuropathy1

Case 6: Traumatic Optic Neuropathy?

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

  • Pupils: Equal with no RAPD

  • Color Vision: 15/16 Ishihara Plates in the right eye, and 12/16 plates in the left eye

  • Fundi: Normal


Case 6 traumatic optic neuropathy2

Case 6: Traumatic Optic Neuropathy?

  • Serology Studies- Normal

  • Cranial/Orbital CT scan- Normal

  • Orbital Ultrasound- Normal


Case 6 traumatic optic neuropathy3

Case 6: Traumatic Optic Neuropathy?

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

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


Case 6 traumatic optic neuropathy4

MD -6.91 dB

MD -0.64 dB

OD

OS

Case 6: Traumatic Optic Neuropathy

  • Visual Fields Obtained in March 2003

  • OD-Normal

  • OS-defects in superior and inferior


Case 6 traumatic optic neuropathy5

MD -7.81 dB

MD -2.41 dB

OS

OD

Case 6: Traumatic Optic Neuropathy?

  • Visual Fields obtained in April 2003

  • OD- now developing VF defects

  • OS- visual defects worsened


Case 6 traumatic optic neuropathy6

Case 6: Traumatic Optic Neuropathy?

Repeat Examination

  • Visual acuity: 20/25 in both eyes

  • Pupils: equal with no RAPD

  • Color Vision: 12/16 Ishihara Plates in the right eye, and 7.5/16 plates in the left eye

  • Fundi: Normal


Case 6 traumatic optic neuropathy7

Case 6: Traumatic Optic Neuropathy?

mERG Trace Arrays

Retinal

view

Retinal

view

OD

OS

  • 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 optic neuropathy8

Case 6: Traumatic Optic Neuropathy

Statistical Probability Maps

OD

OS

  • 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


Optical coherence tomography and investigation of optic neuropathies

RNFL

  • RNFL thickness within normal limits OU

  • Good RNFL symmetry between eyes


Case 6 traumatic optic neuropathy9

Case 6: Traumatic Optic Neuropathy?

  • Diagnosis: Acute Zonal Occult Outer Retinopathy

  • Para-neoplastic work-up was recommended by Retinal Specialist


Final observations and conclusions

Final Observations and Conclusions

  • Cases 5 & 6 demonstrate the utility of OCT and mERG in differentiation of optic nerve vs retinal insults

  • OCT measurement of RNFL

    • Reproducibility of 10-20 µ

    • Adequate for long-term follow-up of progressive RNFL damage

  • OCT may prove useful in compressive disease in predicting the likelihood of visual recovery based upon remaining RNFL available at time of diagnosis


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