Typical Optic Neuritis Atypical Optic Neuritis
Pathophysiology Inflammation of the optic nerve
Primary destruction of the
myelin sheath
Related conditions Idiopathic autoimmune process
affecting central
nervous system white matter.
History or evidence of
multiple sclerosis in 1/3
Age 15-45 (20-50 years)
mean age 30-35 years
Gender F>M=3:1
(75% female)
Symptoms 2 major symptoms :
 Acute monocular central
vision loss
 Periocular pain (92%)
exacerbated by eye
movement.
Precede or occur
concurrently with visual loss
Other symptoms:
 Photopsia up to 30%):
spontaneous flashing black
squares, flashes of light, or
showers of sparks, sometimes
precipitated by eye movement
or certain sounds.
 Pulfrich phenomenon:
misjudging the position of
moving objects (caused by
difference in the speed of
AAION
Secondary destruction of the
myelin sheath
 bacterial or fungal
infections
 presumed viral infections
or vaccination
 connective tissue diseases
 sarcoidosis
 choroiditis or retinitis
NAION
Circulatory insufficiency within the optic nerve head
5,7% of AION
Giant cell arteritis (GCA) (71-
83%)
>65
mean age of onset = 70
rare occurrence <60
 F>M
 Most common in
Caucasian
 Unusual in African-
American, Hispanic
 Headache (the most
common symptom)
 Jaw claudicatio, scalp
tenderness (the most
specific)
 Periocular pain (-)
95% of AION
 Systemic hypertension (34-
50%)
 Diabetes (5-25%)
 OSAS
 Other vascular events :
stoke, myocardial infarction
 Other vascular risk factors :
smoking,
hypercholesterolemia,
hyperhomosysteinemia,
prothrombotic factors
>50
range = 45-70 years
 F=M
 Higher in Caucasian
 Vision loss is most
frequently reported upon
awakening
 Pain with eye
movement : (-)
 Periocular discomfort in
8-12%
 Headache (-)
 Jaw claudication, scalp
tenderness (-)
 conduction in the 2 optic
nerves)
 Uhthoff symptom :
movement-induced photopsias
or transient loss of vision with
overheating or exercise ; most
common in patients with other
evidence of MS
Hypotheses:
(1) elevation of body
temperature interferes directly
with axon conduction
(2) exercise or a rise in body
temperature changes the
metabolic
environment of the axon,
which, in turn, interferes with
conduction.
Binocular involvement : more Binocular involvement : Binocular involvement : Binocular involvement :
common in children. Usually one eye up to 50% with interval often < 1 up to 20% but interval rarely <6
Usually one eye week (54-95%) months (12-19%)
Degree of visual loss : Degree of visual loss : severe Degree of visual loss: variable,
55% = VA >20/200 (20/25 – (VA <20/200 in >60%, frequently but milder than arteritic (VA
20/200) 1/300/worse) >20/200 in 66%,
11% = VA >=20/20; 3% = NLP >20/60 in 50%)
Visual field defects: Visual field defects may follow
Vary from mild to severe, diffuse any pattern related to ON
or focal, can involve central or damage, (altitudinal loss, usually
peripheral field. inferior, in 55-80%)
 50% diffuse
 20% focal nerve fiber
bundle defect (altitudinal,
arcuate, nasal step)
 8% central/cecocentral
defect
 5% hemianopia defects
Color visual loss is often Color visual loss tends to
disproportionately greater than parallel visual acuity loss.
visual acuity loss.
Transient visual loss: Transient visual loss:
preceded the persistent visual (-)
loss in 7-18% (shorter duration)
SYSTEMIC SYMPTOMS SYSTEMIC SYMPTOMS

Signs RAPD (+) in unilateral cases
Slit lamp examination:
Normal
Very mild anterior/posterior
uveitis may be observed.
Optic disc normal
(edema in 1/3 cases)
Disc swelling is more prevalent
in optic neuritis patients of
Asian, African, and Caribbean
origin.
Over approximately 4-6 weeks, the
optic disc may become pale, even as
the visual acuity and other
parameters of vision improve. The
pallor may be diffuse or sectoral,
most often in the temporal region
Disc or peripapillary
hemorrhages and segmental
disc swelling are less common.
 Polymyalgia rheumatic No associated symptoms
(PMR) complex : present
in >=50%
(malaise, anorexia, weight
loss, fever, proximal joint
arthralgia, mylagia)
 Up to 20% : occult GCS
(visual loss without overt
systemic symptoms, without
abnormal blood testing)
RAPD (+) in unilateral cases
Slit lamp examination:
cellular reaction is extensive (
sarcoidosis? infectious causes?)
Pallid optic disc edema  Optic disc edema :
Optic disc edema typically resolve diffuse/segmental,
over 4-8 weeks, with resultant optic pale/hyperemic
atrophy and generalized attenuation (pallid optic disc edema less
of retinal arterioles.
frequently)
Optic disc become visibly
atrophic (sectoral/diffuse)
usually within 4-6 weeks.
 Focal hyperemic
telangiectatic vessels on
the optic disc
Excavation of the optic disc Excavation of the optic disc :
occurs more frequently after unusual
AAION (‘pseudoglaucomatous
cupping’)
May see retinal cotton-wool Retinal cotton wool spots (-)
spots
(indicative of concurrent retinal
ischemia)
Retinal arterial occlusion may Peripapillary retinal
occur simultaneously (especially hemorrhages are common
cilioretinal artery occlusion) (72%)
Retinal arterioles are focally
narrowed in peripapillary
region in 68%

Ancillary testing Brain MRI :
Other ancillary testing ½ normal
½ have signal abnormalities
typical of MS (40-60%)
Diagnostic testing should be
performed on a case-by-case
basis.
Unaffected eye : normal
diameter, normal physiologic
cup
ECR : elevated in >80% (mean
70mm/hr, often >100mm/hr)
CRP : elevated in >80%
(N : <0.5mg/dL)
Unaffected eye : small in
diameter, cupless disc (‘disk at
risk’)
ECR, CRP : normal
Fibrinogen, thrombocyt :
normal

Abnormalities in MRI: Fibrinogen : elevated

 optic nerve enlargement
 T2 hyperintensity
 and/or enhancement with
gadolinium
contrast
(nonspecific)
The most important
application of MRI :
 identification of signal
abnormalities in the white
matter of the brain,
usually in the
periventricular region,
consistent with
demyelination
Lumbar puncture
help define a very low risk
population for MS if both CSF
and MRI are normal.
CSF analysis:
 Oligoclonal banding in
development of MS
 to rule out another
inflammatory or infectious
disorder.
Treatments Systemic CS :
 MP IV 1gr/day for the first
3 days
 Oral prednisone
1mg/kg/day for 11 days
Treatment underlying disease
Systemic CS
Thrombocytosis (up to 50%
GCA)
FFA : FFA :
 Disc delay  Disc delay
 Reduced choroidal  Normal choroidal perfusion
perfusion (choroid delay)
Orbital color Doppler imaging : Orbital color Doppler imaging :
reduced arterial flow normal arterial flow
Temporal artery biopsy : (+) in Temporal artery biopsy : (-)
95%, very few false positives
Histopathologic studies:
vasculitis of the short posterior
ciliary vessels, in addition
superficial temporal,
ophthalmic, choroidal, and
central retinal arteries
Systemic CS : None proven effective therapy
 MP IV 1gr/day for the first
3-5 days (particularly in
acute phase)
 Oral prednisone at least
 1mg/kg/day (after IV
therapy or initially if the IV
route is not used) 
tapered slowly,
maintained for at least 6-9
months, often up to a year
and longer
Antiplatelet therapy :
may be benefit in conjunction
with CS
Effect of CS treatments: Effect of CS treatments :
 Prompt recovery of PMR (-)
symptoms
 Normalization of acute
phase reactans

Natural history Improvement (+) Rarely improve 16-72% improve

Untreated: Untreated, NAION generally
 step worsening within 1 remains stable, most cases
week showing no significant
 begins to recover (85%) in improvement or deterioration
2 weeks over time.
 reaches maximum
recovery within 30 days
(70% >=20/20; 93%
>=20/40)
Treated:
 No difference from
untreated, except
recovery is faster within
first 2 weeks

Recurrent attacks
 10-year ONTT recurrence
rate = 35%.
 The risk of recurrence was
increased (41%) in group
treated with oral steroids
compared with those
receiving intravenous
steroids or placebo (25%)
Note McDonald Criteria for MS The diagnosis atypical optic
(below) neuritis depends on two
features:
 Baseline clinical or imaging
evidence of a disease other
than MS that could cause
optic nerve inflammation.
 A clinical course that differs
from that of typical optic
neuritis.
 Papillitis/anterior optic neuritis = swollen optic disc
 Retrobulbar optic neuritis = optic disc appears normal
 Neuroretinitis = inflammatory ONH + peripapillary retina (optic
disc swelling + macular star)
 Optic perineuritis = inflammatory optic nerve sheath, without
inflammation of the nerve itself.
Tentative diagnosis of GCA:
Age + typical clinical symptoms +
elevated ESR/CRP
Guideline for upper level of
normal ESR:
 M = age/2
 F = (age+10)/2
Confirmed diagnosis : positive
temporal artery biopsy
To reduce false-negative in
temporal artery biopsy:
 Minimum 3-6 cm of
specimen
 Bilateral biopsy
Occurrence in the second eye
produces the clinical appearance
of the “pseudo-Foster Kennedy
syndrome”, in which the
previously affected eye is
atrophic and the currently
involved nerve head is
edematous.
True Foster Kennedy syndrome :
 Disc edema due to
elevated intracranial
pressure
 Does not produce visual
loss acutely in the
edematous eye.
An attack is defined as : any subjective or objective neurologic disturbance of 24-hour duration occurring 30 days apart, in the absence of fever or infection.