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Optical Coherence Tomography and Investigation of Optic Neuropathies. Mohammed Al- Naqeeb Umm Al- Qura University. Optic Neuropathies. Demyelinating Compressive Ischemic Toxic/Nutritional Traumatic Hereditary Inflammatory/Infectious. Optic Neuropathies and OCT. - PowerPoint PPT Presentation
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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
Case 2: Recurrent Optic Case 2: Recurrent Optic NeuritisNeuritis
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)
Case 2: Recurrent Optic Case 2: Recurrent Optic NeuritisNeuritis
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
Case 3: Optic NeuritisCase 3: Optic Neuritis
OS OD
Case 3: Optic NeuritisCase 3: Optic Neuritis
MD -4.31 dB
MD -3.03 dB
OS OD
Case 3: Optic NeuritisCase 3: Optic Neuritis
•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
Case 4: Compressive Case 4: Compressive Optic NeuropathyOptic Neuropathy
OS OD
Case 4: Compressive Case 4: Compressive Optic NeuropathyOptic Neuropathy
•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
Case 4: Compressive Case 4: Compressive Optic NeuropathyOptic Neuropathy
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
Case 5: Optic Neuritis?Case 5: Optic Neuritis?
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
Case 5: Optic Neuritis?Case 5: Optic Neuritis?
OS OD
•Mean Deviation -7.55 dB•Nasal superior and inferior visual field loss
•Mean Deviation – 0.31dB
•Normal field
Case 5: Optic Neuritis?Case 5: Optic Neuritis?
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°10°15°20°25°30°Total Response:2.78 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
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°10°15°20°25°30°Total Response:5.57 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
Case 5: Optic Neuritis?Case 5: Optic Neuritis?
Diagnosis: Acute Diagnosis: Acute Zonal Occult Outer Zonal Occult Outer RetinopathyRetinopathy
Case 5: Optic Neuritis?Case 5: Optic Neuritis?
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
Case 5: Optic Neuritis?Case 5: Optic Neuritis?
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
Case 6: Traumatic Case 6: Traumatic Optic Neuropathy?Optic Neuropathy?
Serology Studies- Serology Studies- NormalNormal
Cranial/Orbital CT scan- Cranial/Orbital CT scan- NormalNormal
Orbital Ultrasound- Orbital Ultrasound- NormalNormal
Case 6: Traumatic Case 6: Traumatic Optic Neuropathy?Optic Neuropathy?
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
Case 6: Traumatic Case 6: Traumatic Optic Neuropathy?Optic Neuropathy?
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
Case 6: Traumatic Case 6: Traumatic Optic Neuropathy?Optic Neuropathy?
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
Case 6: Traumatic Case 6: Traumatic Optic Neuropathy?Optic Neuropathy?
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 Neuropathy?Optic Neuropathy?
Case 6: Traumatic Case 6: Traumatic Optic NeuropathyOptic Neuropathy
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°10°15°20°25°30°Total Response:5.05 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
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°10°15°20°25°30°Total Response:3.82 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
Case 6: Traumatic Case 6: Traumatic Optic Neuropathy?Optic Neuropathy?
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