This document discusses various causes of visual loss including transient or persistent visual loss, monocular or binocular visual loss, and various pathologies affecting the media, retina, or neural visual pathway. Specific conditions covered include cerebrovascular disease, ocular issues, vasculitis, migraine, corneal keratitis, corneal edema, hyphema, crystalline lens abnormalities, vitreous hemorrhage, uveitis, retinal vascular occlusions, retinal detachment, acute maculopathy, optic neuritis, giant cell arteritis, malignancy infiltration, and dominant optic neuropathy. Diagnostic criteria and characteristics are provided for many of these conditions.

1. Dr. Parag Moon
Senior resident
Dept. of Neurology
GMC, Kota.

2.  Transient visual loss (<24hr)
 Persistent visual loss(>24 hr)
◦ Sudden, painless
◦ Gradual, painless
◦ Painful
 Monocular: anterior to chiasm
 Binocular: posterior to chiasm

3. 1. Embolic cerebrovascular disease
2. Ocular (intermittent angle closure
glaucoma, hyphema, impending central
retinal vein occlusion, optic disc edema)
3. Vasculitis (GCA)
4. Migraine/vasospasm
5. Other (Uhthoff phenomenon, idiopathyic,
nonorganic)

4. Pathologically can be broadly divided into
three major categories:
 Media problems
 Retinal problems
 Neural visual pathway problems

5.  Keratitis
• Infectious or non-infectious
 Corneal edema
• Acute glaucoma (primarily)
 Hyphema
• Spontaneous or traumatic
 Alterations in crystalline lens
• Thickening, clouding or dislocation
 Vitreous hemorrhage
• Spontaneous or traumatic Uveitis

6.  Inflammation of cornea due to trauma, abrasive
exposure, allergy or infection.
 Marked by cloudiness, irregularity or loss of
epithelial or sub-epithelial corneal tissues.
 Typically eye is tearing, red, painful or irritated.
 Loss of epithelial cells demonstrated by corneal
uptake of fluorescein dye, creating a focal or
diffuse green glow under a cobalt blue light.
 Deeper corneal disease may be visible as a focal or
diffuse white opacity, or by dulling of usually
distinct reflection of light off of cornea (corneal
light reflex).

8.  Loss of corneal clarity.
 Dulling of corneal light reflex or frank grey or white
color to substance of cornea.
 Acute angle-closure glaucoma.
 Typically has nausea, vomiting, and may see coloured
halos around lights.
 Eye is tearing, red, and extremely painful, often with
ipsilateral brow ache.
 Pupil may be fixed in mid-dilated position
 Slit lamp examination- a shallow anterior chamber.
 IOP is often dangerously elevated (usually 40 to 80
mmHg).
 Suspected by noting hardness to palpation in
comparison to fellow eye.

9.  Blood in anterior chamber.
 May result from blunt trauma or may occur
spontaneously marked by abnormal growth
or fragility of iris blood vessels (often in
chronic poorly controlled diabetes).
 Biomicroscopic examination reveals red blood
cells circulating and/or layered in anterior
chamber.
 Intraocular pressure may become
dangerously elevated.

11.  Changes in size, clarity, or positioning of the
crystalline lens-alter focus of light onto retina.
 Trauma or a variety of congenital conditions can
lead to lens dislocation and resultant vision loss.
 Lens clouding (cataract)-generally chronic.
 Elevated blood glucose can cause increased lens
tumescence, altering refractive error. If change is
great enough, patients may perceive vision loss.
 Vision impairment typically resolves within days
to weeks of normalization of blood glucose

12.  Can occur in setting of trauma, spontaneous
retinal tear, spontaneous vitreous detachment or
in any condition with retinal neovascularization
(poorly controlled diabetes).
 Reduction in vision directly proportional to
amount of blood in vitreous.
 Hemorrhage if dense enough, there may be a
decreased red reflex (reddish orange reflection
off subretinal layers when examining eye with an
ophthalmoscope), or retina may not be visible
with ophthalmoscopy.

14.  Inflammation of anterior structures of eye-red,
painful light sensitivity
 Isolated inflammation of intermediate and posterior
structures-normal general appearance of eye with
decrease in red reflex and/or complaint of new
floaters.
 Endophthalmitis- serious bacterial or fungal infection
of all intraocular tissues, caused by surface
pathogens (usually in recent ocular surgery) or
blood-borne agents.
 Eye is tearing, red, and painful.
 Biomicroscopic examination-white blood cells in
anterior chamber, vitreous space, or both.
 Hypopyon, corneal edema and decreased red reflex.

16. Retina problems
 Retinal vascular occlusion (CRAO, CRVO)
 Retinal detachment
 Acute maculopathy

17.  Thrombosis, embolism, or arteritis of central
retinal artery results in retinal ganglion cell
damage, leading to severe, sudden, painless,
central or paracentral visual loss.
 Within minutes to hours-only abnormality noted
on ophthalmoscopy may be vascular narrowing.
 Embolus visible in 20 percent with CRAO.
 After several hours-inner layer of retina becomes
ischemic, turning milky white, except in fovea,
which appears as a cherry-red spot.
 Afferent papillary defect typically present.

19.  Thrombosis of central retinal vein-venous stasis
 Disc swelling, diffuse nerve fiber layer and pre-
retinal hemorrhage, and cotton wool spots called
"the blood and thunder" fundus.
 While vision loss may be severe, the onset is
typically subacute in contrast to sudden visual
loss typical of CRAO.
 When venous stasis is severe, infarction may
occur due to slowed retinal blood flow on the
arterial side. In this setting, a relative afferent
papillary defect is often present.

21.  May occur spontaneously or trauma.
 Most common form-due to a tear or break in
retina.
 Sudden onset of new floaters or black dots in
their vision, often accompanied by flashes of
light (photopsias).
 Not painful and does not cause a red eye.
 Dulling of red reflex and ophthalmoscopic
examination may reveal the retina to be elevated
with folds.
 If extensive, there may be a relative afferent
pupillary defect.

23.  Associated with a central blind spot
(scotoma), blurred vision, or visual distortion.
 May be due to fluid leakage, bleeding,
infection, or can occur de novo or as an acute
worsening of a chronic disease (eg, new
edema in previously dry diabetic retinopathy)
 Diagnosis requires detailed ophthalmoscopy
with a high magnification lens through
pharmacologically dilated pupils.

27.  Acute onset
 Central, cecocentral, arcuate pattern of visual
loss
 75% normal disc
 Neurological signs of brain stem (retrobulbar)
(diplopia, ataxia, weakness) or spinal cord
involvement (leg weakness, bladder
symptoms, paresthesias, abnormal MRI)
 90% recovery with steroids

29.  First described by Dutton and colleagues in 1982.
 Prone to recurrent optic neuritis events that respond
to corticosteroid therapy.
 Vision loss may manifest during a steroid taper
 More frequent in women.
 Optic nerve may appear mildly edematous during
acute events
 79% have abnormal antinuclear antibody (ANA),82% -
elevated anticardiolipin antibody titers.
 Skin biopsy may show leukocytoclastic or
lymphohistiocytic vasculitis (or both) and
immunereactant deposition

30.  Nonspecific inflammation of optic nerve
similar to typical optic neuritis
 Infiltration of optic nerve or sheaths
mimicking mass lesions, such as optic nerve
glioma or meningioma
 Mass effect due to direct compression of
nerve
 Parachiasmal involvement

32.  Typically affects young, healthy adults
 Two-thirds-antecedent viral prodrome.
 Cecocentral and central type visual field
defect.
 Present with stellate maculopathy and optic
disc edema,
 May be associated with exudative detachment
in peripapillary region.

33.  Multiple focal yellow-white retinal lesions
may appear,
 Optic disc edema associated with
neuroretinitis typically begins to abate in 2
weeks, and resolve within 3 months.
 Macular star may be present for up to a year.
 Catscratch disease (Bartonella henselae),
syphilis(treponema pallidum), Lymes
disease(B. Burgdorfelia)

35.  Presence of severe disc edema with hemorrhages
characteristic
 Usually over 50 years old with Vascular risk
factors such as diabetes, hypertension, and
smoking.
 in young patients (below 45 years) associated
with hypercholesterolemia and
hyperhomocysteinemia
 Visual acuity can be normal or severely affected.
 Usually inferonasal arcuate or altitudinal.
 Optic nerve head in the other eye is often has
small cup-to-disc ratio (0.1 or less).

36.  Visual acuity usually remains static or improves
slightly in majority of patients
 In a small subset of patients, visual acuity may
actually worsen over the fi rst few weeks
(progressive NAION).
 Rarely lasts more than 3–4 weeks
 Bilateral simultaneous involvement can occur
during a hypotensive episode such as blood loss
or over-dosing of anti-hypertensive medications.
 Recurrence risk-5% in same eye, in follow eye
within 5 years-15%

38.  Over 60 year old with features of ischaemic
neuropathy strongly consider possibility of giant
cell arteritis (GCA).
 Careful medical History inquiring about temporal
pain, jaw claudications, transient visual or
diplopia, fever, weight loss, myalgias and fatigue.
 Cup-to-disk ratio of greater than 0.2 in other
eye,
 Early massive or bilateral simultaneous visual
loss
 Markedly pallid disk edema often described as
“chalky-white” swelling in 68.7% of cases

39.  Choroidal infarcts.
 End-stage optic disc appearance characterized by
marked cupping with pallor.
 Complete blood count, erythrocyte sedimentation
rate (ESR), and C-reactive protein (CRP).
 Steroid treatment either oral (80–120 mg
prednisone) or intravenous (1 gram
methyleprdnisolone) for 3–5 days followed by
oral steroids.
 Defi nitive diagnosis by temporal artery biopsy
and histopathological confirmation

42.  Optic nerve can be infiltrated in systemic
malignancies such as lymphoma, leukemia, multiple
myeloma, and carcinoma
 Optic disc can be swollen or normal in appearance.
 MRI of brain and orbit may show meningeal and optic
nerve enhancement.
 Spinal tap recommended in suspected CNS
malignancy but more than one spinal tap may be
needed to detect malignant cells
 In case of localized optic nerve infiltration with no
evidence of systemic disease, histopathological
diagnosis by direct optic nerve sheath biopsy

44.  Dominant optic neuropathy (Kjers’ type)
 Present in first decade of life
 Bilateral central or cecocentral scotomas.
 Color vision deficit along tritan (blue-yellow)
axis.
 Optic disc-temporal pallor and in some cases
severe excavation and cupping.
 Recessive optic neuropathy rare
 First year of life
 Associated with diabetes mellitus, diabetes
inspidus, and deafness (Wolfram syndrome).

45.  Acute unilateral, painless, visual loss.
 Sequential bilateral involvement may occur
weeks or months later.
 Visual field defects-central or cecocentral as
papillo-macular bundle is first and most
severely affected
 Fundoscopy may show disk swelling,
thickening of the peripapillary retinal nerve
fiber layer and peripapillary retinal
telangectatic vessels which do not leak on
flourescin angiography.

46.  MRI-optic nerve enhancement and white
matter lesions,
 LHON has 4 primary mitochondrial genome
mutations; G11778A, G3460A and T14484C
and T10663C.
 Male:female ratio-2.5:1 for G11778A and
G3460A mutation
 6:1 ratio for T14484C mutation.

47.  Present with vision loss, months or years
following history of radiation exposure to
brain or orbit.
 Risk increased with concomitant
chemotherapy .
 Mechanism-ischemia caused by endothelial
cell injury from radiation.
 Optic disc is usually normal but can be
swollen.
 MRI-optic nerve enhancement with
gadolinium

48.  May also have radiation retinopathy with
retinal hemorrhages, cotton wool spots,
exudates and macular edema.
 Visual prognosis is poor with 45% ending with
no light perception visual acuity.

49.  Usually had suffered craniofacial trauma but
occasionally mild orbital or eye injury.
 RAPD-main clue to diagnosis.
 CT scan of orbit recommended detect any
bony fractures, fractures of optic canal, and
acute orbital hemorrhages.
 High-dose steroid therapy-within 8 hrs of
injury

50.  Small cell and non-small cell lung carcinoma
of lung
 Often have bilateral disc swelling and
progressive visual loss before diagnosis of
systemic malignancy made.
 Collapsing response-mediating protein
(CRMP- 5) found to be useful marker with
lung carcinoma.

51.  Classically diagnosed by presence of
progressive optic nerve cupping with
concurrent progressive VF loss (arcuate nasal
scotomas).
 Diagnosis aided by presence of risk factors
such as elevated intraocular pressure (IOP),
positive family history, predisposed race,
advanced age and thin central corneal
thickness

52.  Notching of the rim.
 Verticalization of the optic cup.
 Acquired optic pit.
 Baring of a circumlinear vessel.
 Vessel bayoneting at the optic rim (indicating bean-
pot cupping).
 Nasalization of vessels.
 Disc hemorrhage (Drance hemorrhage).
 Abnormally large or atypical pattern of peripapillary
atrophy (beta zone atrophy).
 Nerve not exhibiting rim pallor.

54.  History
◦ Timing — distinction between sudden onset of visual
loss and sudden discovery of preexisting visual loss.
◦ Laterality — Bilateral loss often suggests a retrochiasmal
visual pathway disorder.
◦ Quality — Monocular or binocular
◦ Pain — Keratitis -sharp superficial pain, acute
glaucoma-deep brow ache with nausea and vomiting,
endophthalmitis- deep boring pain, optic neuritis-pain
worse with eye movement.
◦ Redness — keratitis, acute glaucoma, and uveitis
◦ Associated symptoms-diplopia, floaters, coloured halos,
neurological signs
◦ Trauma

55.  Past medical history
 Vascular disease — diabetes, coronary artery disease,
hypertension, hypercoagulability, or vascular risk
factors
 Refractive status
 Contact lens wear —microbial keratitis
 Eye surgery .
 Medications-
◦ Anticholinergics: loss of accommodation, angle closure
glaucoma
◦ Bisphosphonates: uveitis
◦ Digoxin-yellow vision
◦ Rifabutin: uveitis
◦ Sildenafil- blue vision, ischemic optic neuropathy
◦ Topiramate: angle closure glaucoma

56.  Physical examination :
◦ General inspection — noting erythema, tearing,
light sensitivity
◦ Visual acuity — to be tested with glasses, one eye
at time
◦ RAPD
◦ Colour vision
◦ Evaluation of extraocular movement
◦ Confrontation visual fields

57. ◦ Pupils — symmetry, reactivity to light, pupillary reflex
◦ Fluorescein application
◦ Intraocular pressure testing (by tonometry or palpation)
◦ Slit lamp exam
◦ Ophthalmoscopic examination
◦ Visual field testing (manual kinetic or automatic static )
◦ Visual evoked potentials
◦ Pattern ERG
◦ Fluroscein angiography
◦ Optical coherence tomography (OCT)

59. Thank you

60.  Diagnostic Approach to Vision Loss; Nancy
Newman,Vale’rie Biousse; Continuum
(Minneap Minn) 2014;20(4):785–815
 Inflammatory Optic Neuropathies; Fiona
Costello;Continuum Aug-2014
 Approach to acute visual loss; Satish
Khadilkar, Pramod Dhonde;Medicine Update
2012;Vol. 22
 Clinical approach to optic neuropathies;
Clinical Ophthalmology 2007:1(3) 233–246
 www.update.com