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

Neuro-ophthalmology Afferent Efferent Other Anatomy Examination Diagnoses Tests

Afferent Anatomy Anatomy Examination Diagnoses Tests

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

Afferent Anatomy

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

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

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

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

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

Normal sagittal scan

Post-op visual fields

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

HVF

MRI

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

Before Temporal Lobectomy

After temporal lobectomy

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

GVF

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

GVF

MRI

32 yo man

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

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

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

Giant Cell Arteritis, Day 2

Giant Cell Arteritis, Day 4

65 year old man

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

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

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

Fundi

Goldmann Visual Fields

IVFA

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

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

Fundi

GVF

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

1 week later

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

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