2. Headings
ā¢ Definition and aspects
ā¢ Approach to vision loss
ā¢ Patterns of vision loss
ā¢ Transient Monocular Vision Loss
ā¢ Persistent Monocular vision Loss
ā¢ Binocular vision loss
ā¢ Cerebral vision loss
ā¢ Functional vision loss
3. Aspects of Vision Loss
* Visual standards, Resolution of the International Council of Ophthalmology (2002)
4. Grades of Visual Impairment
* Visual standards, Resolution of the International Council of Ophthalmology (2002)
5. Causes of Vision Loss
Cause World South-Asia
Refractive error 20.62 (18.62 - 22.55) 36.76 (34.29 - 39.05)
Cataract 34.47 (25.69 - 43.35) 35.15 (27.18 - 43.16)
Age-related macular Degeneration 8.30 (2.85 - 15.42) 5.66 (2.05 - 10.35)
Glaucoma 5.64 (1.33 - 11.72) 2.40 (0.65 - 4.83)
Corneal opacity 3.46 (0.53 - 7.77) 2.64 (0.47 - 5.82)
Diabetic retinopathy 1.07 (0.15 - 2.44) 0.18 (0.03 - 0.38)
Trachoma 0.98 (0.80 - 1.16) 0.04 (0.01 - 0.07)
Other causes/unidentified 25.46 (9.82 - 44.20) 16.72 (5.97 - 30.58)
Percentage of blindness by cause for all ages in 2015
Flaxman, SR, Bourne, RRA, Resnikoff, S et al. Global causes of blindness and distance vision impairment
1990ā2020: a systematic review and meta-analysis. Lancet Glob Health. 2017
7. Approach to vision loss
ā¢ Age :
Degenerative and vascular disorders seen in adults
Neoplasms/ tumor types are age dependent
ā¢ Sex:
Optic neuritis and giant cell arteritis are more prevalent
in females
8. Approach to vision loss
ā¢ Is the visual loss monocular/ binocular ?
Monocular vision loss : abnormality in the eye itself or in
the optic nerve anterior to the chiasm
Binocular vision loss result from bilateral anterior lesions
or more likely chiasmal / retrochiasmal lesion
ā¢ Is the visual loss transient/ persistent ?
Neuro-Ophthalmology : Diagnosis and Management Grant T. Liu, Nicholas J. Volpe
9. Approach to vision loss
ā¢ What is the pattern and degree of vision loss ?
ā¢ What is the tempo of onset ?
ā¢ Is the visual loss static /progressive/ fluctuating/resolving?
ā¢ What are the associated symptoms/signs or if any triggers ?
10. Painful Vision Loss
Causes Characteristics and Associations
Vascular visual loss
ICA dissection( CRAO /AION)
Carotid occlusion
Neck Pain, Horner Syndrome
Ocular ischemic syndrome Orbital pain , Iris neovascularization
Iridocyclitis, retinal hemorrhages
Giant cell arteritis Jaw claudication, Systemic symptoms
Optic neuritis Periorbital/Pain on eye-movement
Angle-closure glaucoma Nausea,vomiting,conjunctival injection
Orbital apex syndrome Periorbital pain
Other cranial nerve involvement
Ocular trauma H/O Trauma
Pituitary Apoplexy Sudden temporal headache/Periorbital pain
,rapidly worsening visual loss
11. Visual field Interpretation- Pattern of vision loss
1. Monocular vs Binocular
2. Central vs Peripheral
3. Hemianopic or not
4. Congruous vs Incongruous Homonymous hemianopia
25. Amaurosis Fugax
ā¢ Acute onset, brief partial or complete monocular vision
loss
ā¢ Brief, usually <15 minutes and rarely > 30 minutes, most
patients are affected for only 1ā5 min
ā¢ Usually begins in the upper field :Altitudinal vision loss
with a shade /curtain effect seen in 15- 20%
Mungas JE, Baker WH. Amaurosis fugax. Stroke 1977
26. Amaurosis Fugax
Donders RC, Dutch TMB Study Group Clinical features of transient monocular blindness. J Neurol
Neurosurg Psychiatry. 2001
Clinical Feature Implication
Age > 45 years: Ischemic cause likely
< 40 years: Benign migrainous cause likely
Frequency of events ā¢ Isolated events may be d/t embolism
ā¢ Repeated events d/t hypoperfusion in arterial stenosis
Onset ā¢ Over seconds: more likely Embolic/ vasospastic
ā¢ Over minutes: Hypoperfusion events
Duration ā¢ Lasting seconds : ocular , orthostatic hypotension
ā¢ 2- 30 minutes: Ischemic event
ā¢ Minutes to hours: Vasospastic /Migrainous event
29. Hayreh S.S., Zimmerman M.B. Amaurosis fugax in ocular vascular occlusive disorders:
prevalence and pathogeneses. Retina. 2014
30. Amaurosis Fugax
ā¢ Comprises approximately 20-25% of TIAs
ā¢ Annual incidence of stroke was 2% / four times greater than a normal
population
ā¢ TMVL secondary to carotid artery stenosis, the 3-year ipsilateral
stroke rate (10%) was half that for hemisphere TIAs (20%) -NASCET
ā¢ The risk of death in patients with TMVL and atheromatous carotid
stenosis is around 4%/yr, mainly related to myocardial infarction
KIine LB. The natural history of patients with amaurosis fugax. Ophthalmol Clin North Am 1996
PooleCJM, RossRussell RW: Mortality and stroke after amaurosis fugax./ Neurol Neurosurg Psychiatry1985
32. 3-year absolute reduction of risk of stroke with carotid
endarterectomy in presence of 3 risk factors was 14.3%
Benavente O et al. Prognosis after transient monocular blindness associated with carotid-
artery stenosis. N Engl J med. 2001
34. Transient Binocular Vision Loss
Clinical Feature Migraine Occipital Seizure Vertebrobasilar TIA
Duration 20-30 minutes Variable Seconds to minutes
Headache Frequent headaches
Occur after vision loss
During or after vision
loss
During or after
vision loss
Typical visual
symptom
Hemifield marching Hemifield stationary Hemifield or total
Positive phenomena
ā¢Fortification scotoma
ā¢Formed images
Common
Uncommon
Rare
Common
Rare
Uncommon
Associated features Nausea, Photophobia
Paresthesias,Dysphasia
Eye deviation,
automatisms, loss of
consciousness
S/S Brainstem dysfn.
Dizziness, ataxia,
diplopia, numbness,
dysarthria
35. Sudden monocular vision loss: Clinical scenario
ā¢ 42 year old male presented with sudden onset of painless
blurring of vision in right eye since past 7 days
ā¢ He also complains of distortion of images and micropsia.
ā¢ On examination, visual acuity is 6/24 in right eye, with a
RAPD and central scotoma on visual field testing
38. Whether the visual loss a result of a lesion of the
Optic nerve or a lesion of the macula ?
SIMILARITIES
1. Decreased visual acuity
2. Central scotomas on visual fields
3. RAPD can occur in both*
4. Color vision can be affected in both*
39. Differences: ON vs Macula
Symptom Optic nerve abnormality Macular abnormality
Metamorphopsia Rare Common
Pain Usually present in optic neuritis Absent
Color vision/dyschromatopsia More affected
(for the degree of VA Deficit)
Less affected
Photopsia Rare Common
Darkening of vision Common Rare
Recognition of peripheral
field loss
Common Rare
Glare/ Light sensitivity Rare Sometimes
TVO Occasionally Rare
40. Differences
Sign/Investigations Optic nerve abnormality Macular abnormality
RAPD Common Rare
Ophthalmoscopy Swollen pale or normal optic
nerve
Macular abnormality
Pale optic nerve +/-
Visual field defects Central, Cecocentral, nasal,
arcuate, altitudinal
Positive Central
scotoma
Recovery following bright
light exposure
Normal Abnormal
ERG/OCT Normal
Nerve fibre layer thinning +/-
Abnormal
VEP Large latency delay Small latency delay
42. RAPD
ā¢ Result of consensual and bilateral nature of the light reflex
ā¢ Unilateral or asymmetrical bilateral disruption of the afferent
limb of light reflex
ā¢ Detected by swinging flash light test
ā¢ Can quantitate the severity of retinal ganglion cell and optic
nerve damage
44. ā¢ The relative afferent pupillary defect (RAPD) was measured in ten
patients, each of whom had a dense cataract in one eye only.
ā¢ All patients with mature or nuclear cataracts had a measureable
RAPD in the other eye
ā¢ May be due to increased intraocular scatter of light by the cataract
ā¢ RAPD in the same eye as a unilateral cataract, likely to be a major
defect of the anterior visual pathway in that eye.
45. ā¢ The ābetterā eyes had optic nerve or retinal dysfunction.
ā¢ The eyes with worse visual acuity but no afferent pupillary
defect had an abnormality of the ocular media.
47. Sudden Monocular Vision Loss with Progression
Optic neuritis
Leberās hereditary optic neuropathy
Anterior ischemic optic neuropathy ( arteritic/nonarteritic)
48.
49. CRAO
ā¢ Painless sudden monocular vision loss; usually embolic
etiology (commonly Carotid artery atherosclerotic disease )
ā¢ Prudent to rule out giant cell arteritis ( if also age>50 yrs)
ā¢ Vascular emergency ; evaluate on lines of cerebral infarction
ā¢ Fundus may be normal in acute stages; repeat examination
necessary
53. ā¢ Annual risk of stroke in patients with visible asymptomatic retinal
cholesterol emboli :
8.5% vs 0.8% controls ( RR 9.9; 95% CI (2.3 to 43.1); P = 0.002)
ā¢ Stroke occurred in 15.0% RAO group vs 8.0% controls (P < 0.001).
RAO was associated with an increased risk of stroke occurrence
(hazard ratio, 1.78; 95% confidence interval, 1.32ā2.41)
Risk of stroke
Bruno A, Vascular outcome in men with asymptomatic retinal cholesterol emboli. A cohort
study. Ann Intern Med. 1995
Rim TH, et al. Retinal Artery Occlusion and the Risk of Stroke Development: Twelve-Year
Nationwide Cohort Study. Stroke. 2016
54. ā¢ Prognosis of CRAO is considered dismal, with some studies reporting as
few as 8% experiencing a recovery in visual acuity
ā¢ All patients with CRAO should be admitted for immediate workup and
initiation of secondary prevention. MR brain may detect concomitant
cerebral ischemia in 25% pts. Of CRAO
ā¢ Intraarterial thrombolysis or intravenous thrombolysis are of limited
benefit . ???
ā¢ In one metanalysis, systemic fibrinolysis (<4.5 hours onset) resulted in rate
of recovery is nearly 3 times that in the natural history cohort (Pā<ā.001),
with a 32.3% absolute RR and a NNT of 4.0
Management
Lee J, et al. Co-occurrence of acute retinal artery occlusion and acute ischemic stroke: diffusion-
weighted magnetic resonance imaging study. AM J Ophthalmol. 2014
Ahn SJ, : Efficacy and safety of intra-arterial thrombolysis in central retinal artery occlusion. Invest
Ophthalmol Vis Sci 2013
55. Classic features of a unilateral optic neuropathy
1. Central visual loss
2. Clear view through the ocular media to the optic nerve
3. Relative afferent pupillary defect
4. Swollen or pale optic nerve head
Exceptions ā
N-AION: Visual Acuity good despite altitudinal vision loss
Retrobulbar optic neuritis : Disc is normal for 4-6 weeks
56. Ischemic optic neuropathies
ā¢ Most common optic neuropathies in patients >50 yrs age
ā¢ Involvement of posterior ciliary arteries: atherosclerosis or vasculitis
ā¢ AION (90% of total cases) more common than PION
ā¢ Nonarteritic ischemic optic neuropathy is more common than
arteritic ION
Repka MX. Clinical profile and long-term implications of anterior ischemic optic neuropathy.
Am J Ophthalmol. 1983
57. Ischemic optic neuropathies
Parameter Arteritic AION NAION
Age (years) >65 45- 70
Sex F:M ( 3:1) M=F
Systemic symptoms
+ Headache/pain
>50% : Polymyalgia rheumatica
25% : Isolated visual s/s
Absent
Amaurosis Fugax Common ( 32%) Uncommon (2.5%)
ESR and CRP Raised
(ESR normal in 12%)
Normal
HayrehSS. Anterior ischaemic optic neuropathy: differentiation of arteritic from non-arteritic type
and its management.Eye.1990
58. Ischemic optic neuropathies
Parameter Arteritic AION NAION
Degree of vision loss Severe
70% < 6/60
Less severe
50% Better than 6/18
Arteriosclerotic Risk
Factors
According to Age Present
Binocular
involvement
30-50%
Interval often <1 week
20-30%
Interval rarely < 6 months
Improvement Rare
25% HM+ / worse
30% improvement
Ophthalmoscopy Pallid edema , Cotton wool spots
Disc hemorrhages
Disc edema, hemorrhages
Disk at risk
FFA Segmental Choroidal
hypoperfusion
Normal,
Delayed disc filling
60. 37-year-old woman
ā¢ Four weeks prior to presentation, she noticed painless decreased vision in
the inferior visual field in the right eye, which worsened over 4 to 5 days.
ā¢ She had a brain MRI that showed no optic nerve enhancement, but
showed one small nonenhancing periventricular T2 high signal lesion.
ā¢ She did not receive treatment. Her vision failed to improve during the
following 4 weeks.
ā¢ On neuro-ophthalmic examination, visual acuity was 6/6 left and 6/24 in
right eye and mild red desaturation was present in the right eye. RAPD +
Case vignette
63. NAION Vs Optic neuritis
Parameter NAION Optic neuritis
Age (years) , Sex >50, M=F <40, F>M
Pain Absent 90% of presentations
Onset Acute / One time event
Few cases have progression
Progressive
Recovery Static ( >2/3rd no improvement) Good recovery
Optic disc Pale disc, Disc at risk
Peripapillary hemorhages +
33% disc edema
Visual fields Altitudinal/nerve fibre bundle
defect
Central
FFA Delayed disc filling Normal
MR Brain No Optic nerve enhancement Enhancement + 84%
Rizzo JF, Lessell S. Optic Neuritis and Ischemic Optic Neuropathy Overlapping Clinical Profiles.
Arch Ophthalmol. 1991
64. ā¢ Altitudinal disc swelling and Hemorrhage on the swollen disc
was more common in AION than in ON
ā¢ AION was the clinical diagnosis
82% of the cases with altitudinal edema,
81% of the cases with disc hemorrhages
93% of the cases with pallid edema
90% of the cases with arterial attenuation
65. ā¢ Glaucoma, CRAO and ION were correctly identified by atleast
1/5 observers with accuracy >80%
Helpful features in differentiating entities:
1. Retinal arteriolar attenuation and sheathing in CRAO , AION
2. Segmental temporal pallor in hereditary (bilateral) and ON
(unilateral)
3. Pathological disc cupping for glaucoma
66. Optic Neuritis
Classical Features
Female , Age 77% , 32 +/- 6.7 years
Onset to peak Hours to days ( upto 2 weeks)
Ocular Pain 92% precedes vision loss , Usually lasts 3- 5 days
Swollen optic disc 35%
Visual Acuity 55% (6/7.5 to 6/60)
Recovery Untreated: Vision stops getting worse at 7 days, starts improving in
80% within 3 weeks; most recovery in 4 wks ; 70% recover 6/6
Treated: No difference ,except recovery faster within first 2 weeks
Recurrence Cumulative probability over 5 years:
19% : affected eye, 17% :unaffected eye, and 30% for either eye
Conversion to MS
(15 year)
No brain lesion: 25%, One or more lesions: 72%
Beck RW, Cleary PA, Anderson MM Jr, et al. A randomized, controlled trial of corticosteroids in the
treatment of acute optic neuritis. The Optic Neuritis Study Group. N Engl J Med 1992
67. Typical Vs Atypical ON
Optic Neuritis Atypical ON/ Red flags
Young Adults (Mean age 32 years)
Predominantly Females (77%)
Age > 45 years
Subacute visual loss over hours to days Progression of visual loss after 2 weeks
Presence of Pain (90%) Absence of Pain/ persistent pain
Predominantly Unilateral involvement Bilateral involvement
Optic disc edema (35%) Severe disc edema ,Vitreous cells and
hemorrhage, Macular star
Optic atrophy at presentation
Photopsia /phosphenes (1/3) Positive phenomena : Retinal mimic
Good recovery
Monophasic course
ā¢ Lack of partial recovery within 4 weeks
of onset of vision loss
ā¢ Relapse after stopping steroids
70. Case scenario
ā¢ 26 year female presented with C/C of right upper limb
radicular pain and neck pain 1.5 months PTA
ā¢ Right eye vision loss , inferior altitudinal at onset, 4 weeks
prior to presentation with complete loss 14 days post onset
ā¢ Horizontal diplopia, more on focussing to right side
ā¢ Painful left eye vision loss 2 weeks PTA with complete loss
7 days after onset
ā¢ Headache , bifrontal and moderate grade since 2 weeks PTA
73. Optic disc edema vs. Papilledema
Clinical features Optic disc edema/Papillitis Papilledema
Laterality Unilateral > Bilateral Bilateral, may be asymmetric
Early central vision loss
(visual acuity impaired)
Common Uncommon
Color vision Abnormal Preserved until late
Typical visual field defect Central or paracentral scotoma,
arcuate or altitudinal defect
Enlarged physiologic blind spot,
arcuate defect, nasal step,
inferotemporal loss,
concentric constriction
Friedman, D.I. Papilledema and idiopathic intracranial hypertension. Continuum (Minneap Minn). 2014
74. Optic disc edema vs. Papilledema
Clinical features Optic disc edema Papilledema
Spontaneous venous
pulsations
May be present Absent
Afferent pupillary defect Present if unilateral or asymmetric
vision loss
Usually absent unless
asymmetric visual loss
Disc leakage on
fluorescein angiogram
May be present Yes
Associated symptoms Pain on eye movement, other
symptoms specific to etiology
Headache, diplopia,
photophobia, nausea,
vomiting, meningismus
76. Papilledema
ā¢ Optic disc swelling secondary to raised ICP
ā¢ Secondary to blockage of axoplasmic flow in nerve fibres
ā¢ An intracranial mass lesion and malignant hypertension
should be excluded
ā¢ CSF opening pressure:
Abnormal values are >28 cm H20 in children and >25 cm H20
in adults (Normal of 6cm to 25cm H20 ā 95%CI)
1. Avery RA, Shah SS, Licht DJ, et al. Reference range for cerebrospinal fluid opening pressure in
children. N Engl J Med 2010.
2. Lee SC, Lueck CJ. Cerebrospinal fluid pressure in adults. J Neuroophthalmol 2014
77. Spontaneous venous pulsations
ā¢ Present in 90% of normal subjects
ā¢ SVPs occurred only in patients with CSF pressures below 19 cm H2O
and in the absence of optic disc edema
ā¢ In IIH ,CSF pressure often fluctuates and may even be normal at
times. SVPs may hence be present at times
Levin BE. The clinical significance of spontaneous pulsations of the retinal vein.
Arch Neurol 1978;35:37ā40
78. Transient Visual Obscurations
ā¢ Brief episodes (lasting seconds) of monocular or binocular
black-outs/gray-outs of vision in patients with optic disc edema
ā¢ Precipitated by postural changes / valsalva maneuvres/eye
movement
ā¢ TVOs may be the only symptom of raised ICP, which is their
most likely cause
ā¢ Due to transient decreased perfusion of the optic nerve head
Biousse V, Trobe JD. Transient monocular visual loss. Am J Ophthalmol 2005
79. Etiology of Papilledema
Common Causes Uncommon
IIH (44%) Dural sinus AV Malformation
Intracranial mass Lesion (21%) OSA
Hydrocephalus (17%) GBS
Venous sinus thrombosis (9%) CIDP
Intracranial Hemorrhage Spinal cord tumors
Meningitis Craniosynostosis
Neuro-Ophthalmology at a Tertiary Eye Care Centre in India. Sharma,Pradeep MD; Saxena, Rohit
MD, Journal of Neuro-Ophthalmology 2017
80. IIH: Clinical features
Features IIHT
(n=165)
AIIMS
(n= 89)
Mean Age 29(7.4) years 29.9(11)
Females 97.6% 82%
BMI 39.9(8.3) (88%obese) 27.1 Ā± 5.4 (67% obese)
Headache
TVO
Tinnitus
Diminution of vision
Binocular diplopia
84%
68%
52%
32%
18%
92%
53%
13%
62%
24%
Recent gain in weight 45% 11%
CSF Opening pressure;cm 34.3(8.6) 27.2(7.3)
M. Wall, The idiopathic intracranial hypertension treatment trial: clinical profile at baseline, JAMA Neurol. 71 (2014)
Agarwal A, Vibha D, Prasad K, Bhatia R, Singh MB, Garg A, et al. Predictors of poor visual outcome in patients with idiopathic
intracranial hypertension Clin Neurol Neurosurg. 2017
81.
82. Papilledema vs. Pseudopapilledema
Features Papilledema Pseudo-Papilledema
Disc colour Hyperemic Pink , yellowish pink
Disc Margins Indistinct early at superior and
inferior pole
Irregularly blurred, may be
lumpy
Vessels Normal distribution, Fullness
SVP Absent
Emanate from centre
Frequent anomalous pattern
SVP +/-
Nerve fiber layer Dull as a result of edema No edema
Hemorrhages Splinter Subretinal, retinal, vitreous
83.
84. Cerebral blindness
1. Complete loss of visual sensation, including light and
dark perception
2. Loss of reflex lid closure to bright illumination/
threatening gestures;
3. Retention of the pupillary light reaction and near
response;
4. Integrity of normal retinal structures;
5. Normal extraocular movements
Fraser JA, Newman NJ, Biousse V. Disorders of the optic tract, radiation and occipital lobe. Handb Clin
Neurol 2011
86. Deficit Localization
Achromatopsia/Dyschromatopsia Bilateral Lesions of Lingual Gyri
Apperceptive visual form agnosia Lateral occipital cortex
Associative visual agnosia Left or b/L lesions of the parahippocampal,
fusiform, and lingual gyri
Prosopagnosia Right or b/l fusiform gyri
Pure alexia Left occipitotemporal lesions
Ventral stream: Visuo-perception
94. ā¢ Prospective cohort study done in Department of Neurology at King
George Medical University, Uttar Pradesh, Lucknow over a span of
2 years (October 2011-September 2013)
ā¢ 64 patients were included in the study: 27 cases were male and 37
cases were female
95. DIAGNOSIS Percentage of patients
Demyelinating/inflammatory/ischemic
Optic neuropathy
37.5%
Chronically raised ICP 37.5%
Compressive Optic neuropathy 9.4%
Cortical vision impairment 15.6%
Distribution of etiology
96. Neuro-Ophthalmology at a Tertiary Eye Care Centre in India.
Sharma,Pradeep MD; Saxena, Rohit MD, Journal of Neuro-Ophthalmology
ā¢ Retrospective study (Jan 2015 ā December 2015) at RPC/AIIMS
ā¢ 1597 patients (5% of total) were referred for neuropthalmology
evaluation (out of which 1334 were deemed valid)
ā¢ Mean age of presentation was 30.8 Ā± 19.5 years (range:
3monthsā88 years)
ā¢ M:F : 2.02
ā¢ Sixteen percent (n = 263) of patients were incorrect referrals
including retinal dystrophy, maculopathy, cataract, and
refractive error
97. Neuro-Ophthalmology at a Tertiary Eye Care Centre in India
DIAGNOSIS Percentage of patients
Optic nerve disorders
Disc edema +
63.8% (1020)
33% (335)
Disc edema -- 67% (635)
Cranial nerve palsies 7%
Cortical visual impairment 6.5%
Others 6%
Incorrect referrals 16%
Sudden onset: vascular, inflammatory, infectious. Gradual: degenerative, compressive
Pattern: right/ left eye, field involved. Degree: complete, greyness, distortion
Progressive: compressive, Plateau/improve: vascular or inflammatory
Headache: migraine/ icp/compressive
10% for >50% stenosis in TMB vs 20% in hemispheric strokes
Pain: Giant cell arteritis/ Carotid dissection
Most common: Carotid artery atherosclerotic disease ; 50-70% of cases of CRAO
MRI and MRA 25% of patients with acute retinal ischemia (whether permanent or transient) will have concomitant acute
cerebral ischemia
CRAO: attenuated vessels, pale retina at posterior aspect, emboli esp. at bifurcation, and ofcourse cherry red spot
Pain: Giant cell arteritis/ Carotid dissection
Pain: Giant cell arteritis/ Carotid dissection
NAION: Painless acute, progress hours to days, RAPD+, altitudnal vision loss
Optic disc swelling, peripapillary hges, disk at risk, superior optic atrophy
NAION: Painless acute, progress hours to days, RAPD+, altitudnal vision loss
Optic disc swelling, peripapillary hges, disk at risk, superior optic atrophy