Approach to acute visual loss

11 : 2

Satish Khadilkar, Pramod Dhonde, Mumbai

Introduction
Acute visual loss is common in clinical practice. The symptom is very disturbing to patients and requires
rapid clinical analysis and therapy, as it can be associated with significant morbidity. Etiologies of acute
visual impairment range from retina to the occipital cortex, resulting in a plethora of presentations.
For example in a case of retinal disease patient may feel as if a curtain is rising or dropping and in
cases of occipital lesions, patients may give history of bumping into the objects without realization.
Also, patients tend to mistake diplopia for visual loss, which needs careful analysis.
Acute visual loss could either be monocular or binocular. Monocular loss is often related to local eye
pathologies as glaucoma, uveitis, retinitis or neurological diseases like optic neuritis (ON) and anterior
ischemic optic neuropathy (AION). Binocular diseases are usually associated with lesions affecting
structures such as optic chiasma, lateral geniculate body, occipital radiations and occipital cortex.
Pain is an important association of visual loss. Painful visual impairment is seen in ON, Tolosa Hunt
Syndrome, orbital apex syndrome, pituitary apoplexy and glaucoma. On the other hand, diseases of
the optic tracts, radiations, occipital cortex and AION tend to be painless. The natural history of the
visual impairment is helpful in the differential diagnosis. Transient visual deficit is seen in vascular
diseases, demyelinations, raised intracranial tension and hyper coagulable states. Lasting deficits are
seen with, strokes, tumors and AION.
Following cases will illustrate various clinical situations of acute visual impairment, encountered
by clinicians.
Case 1
32 yrs old female presented with right sided acute onset painful visual loss which progressed within
2-3 hours. Pain was worse with eye movements. On examination, the visual acuity was 6/60 with
cecocentral scotoma on field testing, right sided relative afferent papillary defect (RAPD) on swinging
flash light test, affected colour vision and normal fundus. Investigations revealed P100 latency of
145 ms on visual evoked potential (VEP), MRI Brain with orbital cuts with fat saturation revealed
right optic nerve enhancement with normal MRI spine. CSF study showed normal glucose with mild
raised proteins with pleocytosis. Patient was started on methylprednisolone (MP) for 5 days with
mild recovery over 3 weeks.
Discussion
The diagnosis is of right optic neuritis. Demyelinating optic neuritis is the most common non
glaucomatous optic neuropathy in young people. The incidence of ON is around 5.1 per 100,000 and
a prevalence of 115 per 100,000.1 Females are more commonly affected than males in approximately
3:1 ratio. Symptoms usually start with acute onset vision loss with progression over hours to days.
Pain is often associated with eye movements, but can be continuous. Pain is a consistent feature, seen
in almost 92% of cases which usually distinguishes ON from other optic neuropathies. Painful visual
loss can also be seen with orbital apex syndrome, but associated extraocular muscle involvement
differentiates this from ON. Dyschromatopsia is common, and patients often report that colors,

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Table 1 : Differentiating features between macular and optic
nerve lesions
Characteristics

Macular lesions

Table 2: Differences between papilloedema and papillitis

Papilloedema

Optic nerve lesions

Reduced visual acuity

Yes

Yes

Metamorphosis

Yes

No

Photostress test (bright Slow recovery

light for 10 seconds)

Rapid and significant recovery

Central scotoma

Yes

Reduced colour vision

Papillitis

Visual acuity

Preserved

Visual field

Enlargement of blind spot; Central scotoma

loss of peripheral vision

Reduced

Colour testing

Normal

Loss of red vision

Pupillary reflex Not affected

RAPD

Yes

Eye movements No pain on movements

Pain on movement

Moderate

Often severe

Laterality

Bilateral

Unilateral

Pain

No

Yes

Fundus

Absent of venous pulsation

Venous pulsations present

RAPD

No (Yes if severe)

Yes

normal and may show pallor later in the illness. Papilloedema

and papillitis can be differentiated as follows [Table 2]
The diagnosis of ON is mainly clinical and some ancillary
tests are required for further evaluation and prognostication.
VEP, CSF study and MRI brain with fat saturation images
with contrast with orbital cuts are the initial investigations
required. Sometimes papilloedema is difficult to differentiate
from papillitis. In such cases differences as shown in Table
2 helps. Optic neuritis is commonly the first demyelinating
event in Multiple sclerosis (MS). MRI of the brain can help
confirm the diagnosis of optic neuritis and helps to stratify the
risk of progression to MS. Those with a normal MRI finding
at the time of optic neuritis diagnosis have a 15% risk of
progression to MS at 5 years, 22% at 10 years, and 25% at
15 years; those with an abnormal brain MRI finding have a
42% risk of progression to MS at 5 years, 56% at 10 years,
and 72% at 15 years.3-5 Additionally aquaporin 4 antibody
testing can be considered for diagnosis of neuromyelitis optica
(NMO) as these can present with ON. Also MRI spine in MS
reveals usually short segment involvement while in NMO it
shows long segment involvement. Treatment options include
methylprednisolone for the acute event and interferons in case
of MS and plasmapheresis in case of NMO.

Fig. 1: Swinging flash light test. In this example, the left optic
nerve is not functioning properly, and there is a paradoxic dilation
of the left pupil as the light is directed into it (left relative afferent
pupillary defect).2

Case 2

particularly red, appear less intense in the affected eye. Visual

acuity may range from 6/6 to complete blindness. Visual field
testing varies from enlargement of blind spot to cecocentral
scotoma to complete blindness. RAPD ipsilateral to visual
loss, seen with optic neuropathy or severe retinal disease (in
which case retina looks abnormal on fundus examination) is
the most important clue to differentiate from other diseases.
ON closely mimics maculopathy and can be differentiated as
follows (Table 1).

A 74 year old male presented with fever, malaise, weight loss,

progressive headache since 8 weeks, diminution of vision
and inability to open mouth since 5 days. On examination
patient had stable vitals with necrotic patches over scalp, lip
and tongue. Visual acuity revealed only hand movement in
both eyes. Fundus showed left eye anterior ischaemic optic
neuropathy. There was bilateral scalp tenderness with non
pulsatile tender temporal arteries (Fig 1).

RAPD is tested by the swinging flashlight test. This simple

and useful clinical test depicted in Figure 1.

Investigations showed thrombocytosis (8.32 lakhs/cmm), ESR

was 108 mm and C-reactive protein was increased. Temporal
artery biopsy confirmed giant cell arteritis. Discussion

A close clinical differential diagnosis of optic neuritis is

papilloedema. When the disc head is inflamed, the changes
can look close to rise of intracranial pressure. On the other
hand, in cases of retrobulbar neuritis the optic head is often

Optic nerve ischemia occurs most frequently at the optic nerve

head, where structural crowding of nerve fibers and reduction
of the vascular supply may combine to impair perfusion to
a critical degree and produce optic disc edema. The most

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Fig 2: Showing thickened temporal artery with scalp necrosis and AION.
Table 2: Comparison between arteritic and nonarteritic
AION.7
Feature

Arteritic AION

Nonarteritic AION

Age (mean yrs)

70

60

Sex ratio

F>M

M=F

Associated symptoms

Headache, scalp tender- Pain occasionally noted

ness, jaw claudication

Visual acuity

Upto
76%
20/200(6/60)

Disc

Pale > hyperemic oede- Hyperemic

ma
oedema
Cup small
Cup normal

Mean ESR (mm/hr)

70

Natural history

Improvement rare
Improvement in upto
Fellow eye in upto 95% 43%
Fellow eye in < 30%

Treatment

Corticosteroids

females 2 to 6 times more commonly than males. Clinical

criteria most strongly suggestive of giant cell arteritis include
AION, jaw claudication, C-reactive protein above 2.45 mg/
dl, neck pain, and an erythrocyte sedimentation rate of 47
mm/hour or more, in that order. C-reactive protein tend to be
a little more sensitive (100%) than erythrocyte sedimentation
rate (92%) for detection of giant cell arteritis; erythrocyte
sedimentation rate combined with C-reactive protein give the
best specificity (97%).9 American college of rheumatology
(1990) suggested three or more of the following five criteria
to have a specificity of 93.5% and sensitivity of 91.2%.

< Upto 61% > 20/200

(6/60)
>

pale

1. Age of onset greater than 50 years

20-40

2. Onset of new headaches

3. Temporal artery abnormalities (tenderness or reduced
pulsation)

None proved

common such syndrome is AION.6

AION has been divided into arteritic (associated with Giant
cell arteritis) and nonarteritic. Table 2 points out the differences
in the arteritic and non arteritic types.
AION presents with rapid onset of painless, unilateral visual
loss manifested by decreased visual acuity, visual field, or
both. The level of visual acuity impairment varies widely, from
minimal loss to no light perception, and the visual field loss
may conform to any pattern of deficit related to the optic disc.
An altitudinal field defect is most common, but generalized
depression, broad arcuate scotomas, and cecocentral defects
are also uncommonly seen. A relative afferent pupillary defect
invariably is present with monocular optic neuropathy. The
optic disc is edematous at onset, and edema occasionally
precedes visual loss by weeks to months.8
Giant cell arteritis presents in later decades of life, affecting

4. Elevated erythrocyte sedimentation rate (>50 mm/h using the Westergren method)
5. Positive temporal artery biopsy.
Corticosteroids started at the earliest, form the mainstay of
treatment. One may choose not to wait for the biopsy when
visual compromise is seen, as it can lead to permanent visual
loss and neurological complications like strokes. Therapy is
to be continued for at least 1-2 years depending on the clinical
condition. Scalp necrosis seen in the present case is a rare
phenomenon in GCA.
In 9095% of cases, AION is unrelated to temporal arteritis.
The nonarteritic form (NAION) of the disease occurs in a
relatively younger age group (mean age of 60 years) and usually
is associated with less severe visual loss. Frequently, visual
impairment is reported upon awakening, possibly related to
nocturnal systemic hypotension.10 NAION is associated with

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Fig 3: Right occipital infarct

Fig 4: Bilateral occipital subcortical white matter changes suggestive of PRES.

carotid occlusive disease, CNS small vessel disease, diabetes

and hypertension. Diabetic papillopathy characterized by
optic disc edema (unilateral in approximately 60%), only mild
optic nerve dysfunction and type 1 diabetes is of particular
importance in ischaemic optic neuropathies. NAION has
no proven therapy. Lebers hereditary optic neuropathy
(LHON) can also present acutely with vision loss like AION.
LHON progress over a period of time and associated cardiac
abnormalities can be seen with this condition. Fundus in acute
conditions is normal, but presence of telangiectasias favors
LHON. Diagnosis of LHON is achieved by genetic analysis.

Case 3
48 yrs old male, working in a color factory, returned home and
rang the doorbell. When his wife opened the door, he did not
recognize her, apologized and started climbing down. Wife
called him and he immediately recognized her by voice. On
examination his visual acuity was normal, but he was not
able to identify famous personalities. He was not able to mix
and match colors too, which was his job of many years. Final
diagnosis of acute onset prosopagnosia with color agnosia
made. Patient was found to be hypertensive. MRI study
confirmed a right parieto occipital infarct (Fig. 3).

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pressure was 180/110 mm of Hg with normal fundus and

papillary examination. On confrontation she was having left
homonymous hemianopia and MRI Brain was suggestive
of bilateral posterior subcortical white matter changes with
normal angio sequences (Fig 4). Final diagnosis of Antons
syndrome was made as patient was denying blindness as
well as confabulating things, due to posterior reversible
encephalopathy syndrome (PRES). Patient recovered over
2-3 weeks with complete resolution of MRI changes.

Table 3: Cerebral visual loss

Mechanism

Etiologies of vision loss

Vascular

Vertebrobasilar ischemia (PCA territory)

Cerebral anoxia
Cerebral venous thrombosis (superior sagittal sinus)
Hypertensive encephalopathy (posterior reversible encephalopathy syndrome)
Eclampsia

Head trauma

Occipital lobe injury

Occipital mass

Tumor
Abscess
Vascular (aneurysm, arteriovenous malformation)
Hemorrhage

Discussion
PRES is a clinico radiological entity, more prevalent in
women and presents with headache, visual disturbances
(eg, blurry vision, hemianopsia, visual neglect, cortical
blindness), seizures, altered mental status and occasionally
hemiparesis.12 It is usually seen in the setting of moderate
to severe hypertension. Preeclampsia, immunosuppressants
(Tacrolimus, Cyclosporine A), autoimmune diseases (eg,
lupus), sepsis and shock are some of the precursors of
PRES.12-14 Complete recovery of the MRI changes as well as
clinical disability over 2-3 weeks, is common. Compromise
of autoregulatory mechanisms of cerebral blood flow is the
most accepted theory for PRES.14

Demyelinating disease Multiple sclerosis

Infection

Occipital abscess
Meningitis
Progressive multifocal
(PML)
Creuzfeldt-Jacob disease
SSPE

Toxic

Cyclosporine
Tacrolimus
Methanol

Metabolic

Hypoglycemia
Porphyria
Hepatic encephalopathy

leucoencephalopathy

Migraine

Migranous visual aura

Seizure

Occipital lobe seizures [and its causes]

Discussion
In this case, patients inability for face recognition suggested
prosopagnosia. Prosopagnosia is defined as an inability
to recognize faces in the absence of loss of visual acuity
and general intellectual functions. It usually results from
bilateral occipitotemporal infarcts and sometimes right sided
involvement only. Some studies have suggested for object
recognition, left occipitotemporal lobe is activated while for
face recognition, right occipitotemporal lobe is activated.11 In
such cases, prognosis is good with significant recovery over
3-4 months. Cerebral disturbances of vision can be caused
by affection of visual association areas or subcortical white
matter. Alzheimers disease, posterior cortical atrophy and
Creuzfeldt-Jacob disease (Heidenhain variant) can cause
visual agnosias of insidious evolution.
Case 4
A 30 years old pregnant female with history of preeclampsia
on day 3 after uneventful delivery, presented with new onset
headaches and difficulty in vision. She denied blindness
and made up visual sequences [confabulations]. She was
feeling persistence of images after removing her gaze
from objects (palinopsia). She was seeing flashes of lights
(photopsias) and saw relatives in front of her, in their absence
(formed visual hallucinations). On examination her blood

Cortical blindness usually occurs with bilateral PCAocclusions.

Basilar artery thrombosis and vertebral artery dissection tend
to result in such visual loss. Table 3 enumerates etiologies
for cerebral visual loss with underlying mechanisms. The
management of such cases depends on the underlying
etiologies.
Lastly, non organic visual loss, not too uncommon,
needs a special mention.15 There are various clinical and
ophthalmological tests to differentiate it from organic causes.
For example, the patient is instructed to look straight ahead
at the mirror and the mirror is rotated from side to side. Eye
movements indicate that the patient can see his or her own
image in the mirror. Another test is a person who is truly blind
can touch the tips of the fingers properly and a person with
nonorganic visual loss is often unable to touch the fingers
properly. Also positive optokinetic nystagmus indicates
vision of at least 20/400 vision in tested eye.16 Stereopsis,
tangent screen testing and visual evoked potential can aid
in diagnosing nonorganicity. Vision funnels in organic and
tunnels in nonorganic vision loss.
Concluding remarks
As seen from the above discussion, acute vision loss can
be a result of lesions involving anterior or posterior visual
pathways. History and clinical examination are the most
important aspects to help reach the localization. Relevant
hematological tests, followed by neuroimaging help further
differential diagnosis. As visual loss results in decline of
quality of life, early recognition and therapy is of paramount

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importance in the total outlook of recovery.

Examination should start with pin hole testing; if it improves
vision then refractive error is most likely. Visual acuity
testing, confrontation visual testing, pupillary reactions,
ophthalmoscopy, external examination of the eye with a
torchlight and tonometry to measure the intraocular pressure
should be performed in all cases. The following flow diagram
gives the approach towards the visual loss.

Painless

Optic Neuritis

AION

Orbital apex syndrome

Toxic amblyopia

Pituitary apoplexy

LHON
Methanol poisoning

6. ArnoldA.C.:Ischemic optic neuropathies. Ophthalmol Clin North

Am2001;14:83-98.
8. HayrehS.S.:Anterior ischemic optic neuropathy. V. Optic disk
edema an early sign.Arch Ophthalmol1981;99:1030-1040.

Posterior (spares
pupils in most of the
cases)

9. Hayreh SS. Giant cell arteritis: validity and reliability of various

diagnostic criteria. Am J Ophthalmol 1997;123:285-96.

Painful

10. Hayreh SS, Zimmerman BM, Podhajsky PA, Alward WLM. Nocturnal arterial hypotension and its role in optic nerve head and
ocular ischemic disorders. Am J Ophthalmol 1994;117:60324.

9
Painful

5. Optic Neuritis Study Group. Multiple sclerosis risk after optic

neuritis: final optic neuritis treatment trial follow-up. Arch Neurol
2008; 65:72732.

7. Yanoff and Duker Ophthalmology 3rd edition.970.

Approach to acute
vision loss

Anterior (involves
pupils in many
cases)

4. Optic Neuritis Study Group. High- and low-risk profiles for the
development of multiple sclerosis within 10 years after optic neuritis: experience of the optic neuritis treatment trial. Arch Ophthalmol 2003; 121:9449.

Pituitary
9
apoplexy
(Rare, If
postfixed
chiasmal
aection)

Painless

11. Nakamura K, Kawashima R, Sato N et al.Functional delineation

of human occiptotemporal areas related to face and scene processing. A PET study Brain 2000;123:1903-1912.

Hemianopia and
cortical blindness due
to various etiologies
given in Table 3.

12. Hinchey J, Chaves C, Appignani B et al.A reversible posterior leucoencephalopathy syndrome. N Engl J Med 1996;334:494e500.

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