1. Neuro-ophthalmology Review First Hour— Afferent Visual System Thomas M. Bosley, MD Department of Ophthalmology King Saud University

2. Neuro-ophthalmology Afferent Efferent Other Anatomy Examination Diagnoses Tests

3. Afferent Anatomy Anatomy Examination Diagnoses Tests

4. Human Afferent Visual System Polyak, THE VERTEBRATE VISUAL SYSTEM, 1957

5. Afferent Anatomy

6. Afferent Visual System Anatomy Examination Diagnoses Tests Visual acuity Color vision Visual field Pupil examination Fundoscopy

7. Examination Visual acuity Color vision Visual field Pupil examination Fundoscopy Distance Near Ishihara AOC Confrontation Goldmann Humphrey Direct ophthalmoscopy Slit lamp and lens Indirect ophthalmoscopy Light Dark

8. Afferent Visual System Anatomy Examination Diagnoses Tests Compression Trauma Inflammation Ischemia Congenital and genetic problems

9. Afferent Diagnoses Compression Trauma Inflammation Ischemic Congenital and genetic problems Intraorbital ON Intracranial ON Optic chiasm Optic tract Posterior afferent system

10. 35 yo woman Reported 3 months of gradual visual loss OU 7 months pregnant Rapid visual loss OU over 1 week before N-O evaluation VA at presentation OD 20/40 and OS NLP

11. Normal sagittal scan

12. Post-op visual fields

13. 60 yo physician c/o mild visual blurring No DM, no HBP; impotence x 10 years Good visual acuity and color vision Mild temporal pallor OU Prolactin level of 11,000

14. HVF

15. MRI

16. Afferent Diagnoses Compression Trauma Inflammation Ischemic Congenital and genetic problems Globe Intraorbital ON Optic canal Optic chiasm Occipital lobe

17. Before Temporal Lobectomy

18. After temporal lobectomy

19. 27 yo man Driving his car at night 9 years before Hit a camel Substantial head trauma Hospitalized for 2 months Has noticed decreased vision in his right eye VA 20/40 OD and 20/20 OS Moderate bilateral optic atrophy

20. GVF

21. 18 yo boy Playing around with friends Fell over backwards, hitting his occiput with loss of consciousness for one hour Blurred vision OU after that VA 20/400 OU

22. GVF

23. MRI

24. 32 yo man

26. Afferent Diagnoses Compression Trauma Inflammation Ischemia Congenital and Genetic problems Optic neuritis Orbital pseudotumor Other

27. 27 yo woman Developed blurred vision OD and mild right periorbital pain VA 20/50 MRI abnormal Diagnosis multiple sclerosis

28. Afferent Diagnoses Compression Trauma Inflammation Ischemia Congenital and genetic problems Non-arteritic ischemic optic neuropathy Central retinal artery occlusion Other retinal emboli Giant cell arteritis with ION

29. Giant Cell Arteritis, Day 2

30. Giant Cell Arteritis, Day 4

31. 65 year old man

32. Afferent Diagnoses Compression Trauma Inflammation Ischemia Congenital and genetic problems Congenital retinal dystrophies Optic nerve hypoplasia Leber hereditary optic neuropathy Dominant and recessive optic atrophy Glaucoma

33. 70 yo man ChildhoodMild amblyopia OS PMH Seizures well controlled with Phenobarbital Family historyNon-contributory CC: Reduced vision OD for 3 days

34. Examination ODOS VACF 1’20/40 Pupils3+ APD AnteriorMild NS Lids8 mm

35. Fundi

36. Goldmann Visual Fields

37. IVFA

38. Evaluation ESR normal several times; VDRL and ANA normal CT of head unremarkable IVFA showed leakage at the right optic disk with small hemorrhages temporally DIAGNOSIS – NAION OD Vision stable thereafter, until 5 months later … when he c/o decreased vision OS x 2 days

39. Examination ODOS VACF 3’20/50 Pupils3+ APD Color0/104/10 GVFUnableInf alt defect

40. Fundi

41. GVF

42. Clinical Course ESR normal again Treated with IV steroids without improvement DIAGNOSIS – sequential NAION Subsequently …

43. 1 week later

44. Visual Acuity ODOS 2 mo laterCF 3’ 4 mo laterHM 2 yr laterLPHM 3 yr laterLP

45. Mitochondrial DNA Analysis Partial analysis of mitochondrial genome showed mutations at 4960 and 9957 –4960 mutation novel in our KSA analysis affects a poorly conserved nucleotide in NADH dehydrogenase subunit 2 (like secondary LHON mutations 4216 and 4917) –9957 mutation reported previously in association with MELAS note his history of seizures