2. Mohammed Al-Naqeeb Umm Al-Qura University Optical Coherence Tomography and Investigation of Optic Neuropathies

3. Demyelinating Demyelinating Compressive Compressive Ischemic Ischemic Toxic/Nutritional Toxic/Nutritional Traumatic Traumatic Hereditary Hereditary Inflammatory/Infectious Inflammatory/Infectious Optic Neuropathies

4. To monitor loss of retinal nerve fiber layer (RNFL) thickness To monitor loss of retinal nerve fiber layer (RNFL) thickness RNFL thickness is a reflection of axonal integrity in response to injury RNFL thickness is a reflection of axonal integrity in response to injury Optic Neuropathies and OCT

5. 70-year old gentleman 70-year old gentleman “Blurred vision” in the right eye upon awakening “Blurred vision” in the right eye upon awakening No Giant Cell Arteritis Symptoms No Giant Cell Arteritis Symptoms Vascular risk factors: Diabetes, Hypertension, and Dyslipidemia Vascular risk factors: Diabetes, Hypertension, and Dyslipidemia Case 1: Anterior Ischemic Optic Neuropathy

6. Visual Acuity:20/40 OD Visual Acuity:20/40 OD 20/25 OS 20/25 OS Pupils: RAPD OD Pupils: RAPD OD IOP: 19 mm Hg in both eyes IOP: 19 mm Hg in both eyes Fundus: Optic disc edema in the right eye. Absent physiological cup in the left eye Fundus: Optic disc edema in the right eye. Absent physiological cup in the left eye Case 1: AION

7. Vision worsened in the right eye after a week - Count fingers Vision worsened in the right eye after a week - Count fingers No Symptoms of GCA No Symptoms of GCA ESR = 6 mm per hour ESR = 6 mm per hour Case 1: AION Clinical Course

8. Fundus Photos OD OS Optic disc edema (OD)

9. OS OD MD -3.60 dB MD -25.80 dB Greater loss in superior field Case 1: AION and VF

10. Fast RNFL Thickness (3.4) 3.4 mm 1.92 seconds

11. 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 findings

12. OCT done 6 months following ischemic onset OCT done 6 months following ischemic onset What is timeline for axon loss to occur? What is timeline for axon loss to occur? Does timeline differ across other optic neuropathies? Does timeline differ across other optic neuropathies? Case 1: Anterior Ischemic Optic Neuropathy

13. 20-year old young woman 20-year old young woman Developed “blurring” in the right eye Developed “blurring” in the right eye Pain with extraocular movements Pain with extraocular movements Case 2: Recurrent Optic Neuritis

14. Past Medical History Bilateral optic neuritis in 1993 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

15. Recurrent optic neuritis in the right eye in 2000 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 Recurrence of optic neuritis in right eye in spring 2003 Case 2: Recurrent Optic Neuritis

16. Present Visual Acuity: Present Visual Acuity: 20/40 OD20/20 OS 20/40 OD20/20 OS Pupils: Right RAPD Pupils: Right RAPD Fundus: Bilateral optic atrophy (temporal pallor) Fundus: Bilateral optic atrophy (temporal pallor) Case 2: Recurrent Optic Neuritis

17. OD OS MD -10.39 dB Central loss greater in OD MD -3.81 dB Case 2: Visual Fields

18. RNFL Thickness OD OS Bilateral RNFL thinning worse in OD

19. 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 Optic Neuritis

20. 32-year old woman 32-year old woman Diagnosed with MS in 1992 Diagnosed with MS in 1992 Reported 6 recurrent bouts of optic neuritis affecting both eyes Reported 6 recurrent bouts of optic neuritis affecting both eyes Denied active optic neuritits at the time of examination Denied active optic neuritits at the time of examination Case 3: Optic Neuritis

21. Visual Acuity: 20/25 OU Visual Acuity: 20/25 OU Pupils: left RAPD Pupils: left RAPD Color Vision: 9/16 Ishihara plates in the right eye, and 6/16 plates in the left eye Color Vision: 9/16 Ishihara plates in the right eye, and 6/16 plates in the left eye Fundus: Bilateral optic atrophy Fundus: Bilateral optic atrophy Case 3: Optic Neuritis

22. OSOD Case 3: Optic Neuritis MD -4.31 dBMD -3.03 dB

23. OS OD Case 3: Optic Neuritis Residual central field depression OU Greater in left eye

24. RNFL Thickness OS OD RNFL thinning in the left eye relative to the right

25. RNFL vs Visual Field Loss How well does the pattern of RNFL loss reflect or correlate with visual field loss in these patients? How well does the pattern of RNFL loss reflect or correlate with visual field loss in these patients?

26. 18 year old male 18 year old male Developed headache and vision loss in both eyes Developed headache and vision loss in both eyes Diagnosed with TB Meningitis Diagnosed with TB Meningitis –Large suprasellar tuberculoma –Causing compression of right ON and optic chiasm Case 4: Compressive Optic Neuropathy

27. Visual Acuity: CF OD Visual Acuity: CF OD 20/25 OS Pupils: Fixed pupil on right, with right RAPD (by reverse testing) Pupils: Fixed pupil on right, with right RAPD (by reverse testing) Ocular Motility: Right third nerve palsy with aberrant renervation Ocular Motility: Right third nerve palsy with aberrant renervation Fundi: Bilateral optic atrophy Fundi: Bilateral optic atrophy Case 4: Compressive Optic Neuropathy

28. OS OD Case 4: Compressive Optic Neuropathy Dense central scotoma OD Temporal cut OS

29. Case 4: Compressive Optic Neuropathy MRI: T1-weighted, post Gd Cystic Suprasellar Mass

30. Mean=40µ profound bilateral RNFL thinning worse in the right eye OCT findings correlated well functional measures of visual integrity Case 4: Compressive Optic Neuropathy Mean=53µ

31. 41-year old woman 41-year old woman Developed sudden onset vertigo and nausea in the fall of 2002 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 Developed vision loss (nasal) and a floater and a “sparkle” in the right eye Case 5: Optic Neuritis?

32. Visual acuity measured 20/20 in both eyes Visual acuity measured 20/20 in both eyes Right RAPD Right RAPD Color Vision 16/16 Ishihara plates in both eyes Color Vision 16/16 Ishihara plates in both eyes Fundi: Examination normal Fundi: Examination normal Case 5: Optic Neuritis?

33. The visual field defect persisted The visual field defect persisted Cranial MRI scan normal Cranial MRI scan normal Orbital CT scan normal Orbital CT scan normal CSF analysis normal CSF analysis normal Case 5: Optic Neuritis?

34. OS OD Mean Deviation -7.55 dB Nasal superior and inferior visual field loss Mean Deviation – 0.31dB Normal field Case 5: Optic Neuritis?

35. Case 5: Multifocal ERGs ODOS Retinal view Retinal view 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

36. Case 5: Multifocal ERGs OD OS Statistical Probability Maps The Patient 85 normal eyes 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

37. RNFL RNFL thickness within normal limits OU RNFL thickness within normal limits OU Good RNFL symmetry between eyes Good RNFL symmetry between eyes

38. In May, 2003 the patient reported new “sparkles” in the left eye In May, 2003 the patient reported new “sparkles” in the left eyeSummary: –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 Neuritis?

39. Diagnosis: Acute Zonal Occult Outer Retinopathy Diagnosis: Acute Zonal Occult Outer Retinopathy Case 5: Optic Neuritis?

40. The clinical distinction between a retinal versus an optic nerve problem may be difficult 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 Ancillary studies such as OCT and mERG can be very useful in this regard Case 5: Optic Neuritis?

41. 61-year old woman was hit with a tennis ball in the left temple in October 2002 61-year old woman was hit with a tennis ball in the left temple in October 2002 Developed chronic headaches Developed chronic headaches Noted inferior visual field loss in the left eye Noted inferior visual field loss in the left eye Referred to the Neuro- Ophthalmology Clinic in January 2003 Referred to the Neuro- Ophthalmology Clinic in January 2003 Case 6: Traumatic Optic Neuropathy?

42. Visual Acuity: 20/20 in the right eye and 20/25 in the left eye Visual Acuity: 20/20 in the right eye and 20/25 in the left eye Pupils: Equal with no RAPD Pupils: Equal with no RAPD Color Vision: 15/16 Ishihara Plates in the right eye, and 12/16 plates in the left eye Color Vision: 15/16 Ishihara Plates in the right eye, and 12/16 plates in the left eye Fundi: Normal Fundi: Normal Case 6: Traumatic Optic Neuropathy?

43. Serology Studies- Normal Serology Studies- Normal Cranial/Orbital CT scan- Normal Cranial/Orbital CT scan- Normal Orbital Ultrasound- Normal Orbital Ultrasound- Normal Case 6: Traumatic Optic Neuropathy?

44. In April, 2003 the patient developed new visual field loss in the right eye 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 In May, 2003 she noted sparkles, flashes, and floaters in both eyes Case 6: Traumatic Optic Neuropathy?

45. 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 Optic Neuropathy

46. 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 Optic Neuropathy?

47. 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 Neuropathy?

48. ODOS Retinal view Retinal view 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 Optic Neuropathy?

49. Case 6: Traumatic Optic Neuropathy 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

50. RNFL RNFL thickness within normal limits OU RNFL thickness within normal limits OU Good RNFL symmetry between eyes Good RNFL symmetry between eyes

51. Diagnosis: Acute Zonal Occult Outer Retinopathy Diagnosis: Acute Zonal Occult Outer Retinopathy Case 6: Traumatic Optic Neuropathy?  Para-neoplastic work-up was recommended by Retinal Specialist

52. Final Observations and Conclusions Cases 5 & 6 demonstrate the utility of OCT and mERG in differentiation of optic nerve vs retinal insults Cases 5 & 6 demonstrate the utility of OCT and mERG in differentiation of optic nerve vs retinal insults OCT measurement of RNFL 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 OCT may prove useful in compressive disease in predicting the likelihood of visual recovery based upon remaining RNFL available at time of diagnosis