OPTIC NEURITIS

Chikita Artia Sari / I 11109014

Definition
Optic neuritis, or primary inflammation of the
optic nerve, is referred to as papillitis when the
optic disc is swollen and retrobulbar neuritis
when the disc appears normal.
The most common form of optic neuritis is acute
demyelinating optic neuritis.
Epidemiology
The annual incidence of optic neuritis, as
estimated in population-based studies, is
approximately 35 per 100,000 per year, while
the prevalence is 115 per 100,000.
The majority of patients who develop optic
neuritis are between the ages of 20 and 50
years.
77% of the patients were women, 85% were
white, and the mean age was 32 7 years.
Symptoms
Painless loss of vision over hours to days. Vision
loss can be subtle or profound.
Reduced visual acuity, colour and contrast
vision.
Usually unilateral, but may rarely be bilateral.
More often affecting females aged between 18-
45.
Orbital pain usually associated with eye
movement.
May have other focal neurological symptoms.
Ocular Manifestasions
Loss of vision in patients with acute
demyelinating optic neuritis is usually abrupt and
occurs over several hours to days.
Progression for more than one week or failure of
recovery to begin within four weeks is possible
but suggests an alternative underlying cause.
Visual loss is usually monocular, although
occasionally both eyes are affected
simultaneously, particularly in children.

Ocular Manifestasions
Mild pain in or around the eye is present in more
than 90% of patients. Such pain may precede or
occur concomitantly with visual loss, is usually
exacerbated by eye movement, and generally
lasts no more than a few days.
The presence of pain, particularly on eye
movement, is a helpful (although not definitive)
clinical feature that differentiates acute
demyelinating optic neuritis from nonarteritic
anterior ischemic optic neuropathy (AION)

Ocular Manifestasions
The severity of visual loss varies from a mild
visual field defect to severe loss of central acuity
(3% of ONTT participants had no light
perception, and 90% described at least some
loss of central acuity).

Severe loss of visual acuity is more common in
children.

Color vision and contrast sensitivity are impaired
in almost all cases, often out of proportion to
visual acuity.
Visual field loss, which may be diffuse (48%) or
focal (i.e. nerve fiber bundle defects, central or
cecocentral scotomas, hemianopic defects), is
also common in acute optic neuritis.
Ocular Manifestasions
Altitudinal defects (focal visual field loss above or
below the horizontal meridian) are less common
and should prompt consideration of a diagnosis
of anterior ischemic optic neuropathy (AION).

Low-contrast letter acuity has recently emerged
as a very sensitive test for optic neuropathy.
An afferent pupillary defect (APD) is detected in
almost all unilateral cases of optic neuritis. If an
APD is not present, a pre-existing optic
neuropathy in the fellow eye must be suspected.
Ocular Manifestasions
In fact, asymptomatic visual dysfunction is fairly
common among fellow eyes of patients who
have apparent unilateral optic neuritis.
The optic disc appears normal in approximately
two thirds of adults with acute demyelinating
optic neuritis (retrobulbar optic neuritis), while
disc swelling is present in about one third of adult
cases (papillitis); children with optic neuritis
experience optic disc swelling more frequently
than do adults.


Signs
Relative Afferent Pupillary Defect (RAPD)
Decreased visual acuity.
Decreased colour vision.
+/- Patchy visual field defects.
+/-Swollen optic disc.
May have other focal neurological signs.
Funduscopic features of optic disc swelling
include elevation of the optic nerve head, disk
hyperemia, blurring of the disc margins, and
edema of the nerve fiber layer.

Diagnosis
Based on an appropriate history (typical versus
atypical course) and clinical signs and symptoms
as described above.
Diagnostic tests, including magnetic resonance
imaging (MRI), cerebrospinal fluid (CSF)
analysis, and serological studies, usually are
performed for the following reasons:
o To determine if the cause is noninflammatory
(such as a compressive lesion), or a
nonidiopathic inflammatory or infectious process
in cases that are not typical for acute
demyelinating optic neuritis.
o To determine the prognosis or risk for
subsequent development of MS in
monosymptomatic cases for which the history
and clinical signs are typical.
Investigation and Management
Complete ophthalmic and neurological
examination.
Blood count/Erythrocyte Sedimentation Rate
(ESR).
Urgent referral to ophthalmologist - immediate
consult by phone - may be indicated for further
MRI investigation and intravenous steroid
treatment may be required.
There are NO indications for oral cortico-steroids
as initial treatment.

Differential Diagnosis
Anterior ischemic optic
neuropathy
Tumor
Aneurysm
Vasculitis
Neuroretinitis
Metastatic carcinoma
Lymphoreticular
disorder

Sinusitis
Granulomatous
inflammation
Leber's hereditary optic
neuropathy (although
always bilateral, this
frequently presents
initially with visual loss
in only one eye)
Pathology
Although the exact underlying cause is unknown,
the pathophysiology of acute optic neuritis and
MS is that of primary inflammatory
demyelination.

Very little is written about the pathology of
isolated optic neuritis, and no autopsy data
have been reported.
The inflammatory response in MS plaques is
marked by perivascular cuffing, T cells, and
plasma cells.
Although MS, itself, previously was thought to be
exclusively a disease of myelin with sparing of
nerve axons, neuronal and axonal loss have
been demonstrated to occur pathologically
Idiopathic Demyelinative Optic
Neuritis
In adults, idiopathic demyelinative optic neuritis
is usually unilateral and occurs chiefly in women
(about 3:1), with onset mostly in the third or
fourth decade of life.
It has been reported to be associated with
multiple sclerosis in up to 85% of cases,
depending on several factors, including gender,
racial origin, and duration of follow-up.
Optic neuritis affects both eyes simultaneously
and produces papillitis more commonly in
children than in adults, but the risk of progression
to multiple sclerosis is lower in children.

Idiopathic Demyelinative Optic
Neuritis
Steroid therapy
intravenously (methylprednisolone, 1 g/d for 3
days with or without a subsequent tapering
course of oral prednisolone)
orally (methylprednisolone, 500 mg/d to 2 g/d for
35 days with or without subsequent oral
prednisolone, or prednisolone, 1 mg/kg/d tapered
over 1021 days)
retrobulbar injectionprobably accelerates
recovery of vision but does not influence the
ultimate visual outcome.

Idiopathic Demyelinative Optic
Neuritis
Without treatment, vision characteristically
begins to improve 23 weeks after onset and
sometimes recovers within a few days.
Improvement may continue slowly over many
months, with recovery of acuity to 20/40 or better
occurring in over 90% of cases at both 1 year
and 10 years from onset, providing that there are
no further episodes of optic neuritis.
Idiopathic Demyelinative Optic
Neuritis
Poorer vision during the acute episode is
correlated with poorer visual outcome, but even
loss of all perception of light can be followed by
recovery of acuity to 20/20.
A poor visual outcome is also associated with
longer lesions in the optic nerve, especially if
there is involvement of the nerve within the optic
canal.
In very severe or recurrent cases, a chalky white
disk with sharp outlines results, although disk
pallor does not necessarily correlate with poor
visual acuity.

Multiple Sclerosis
Multiple sclerosis is typically a chronic relapsing
and remitting demyelinating disorder of the
central nervous system.
Some patients develop a chronically progressive
form of the disease, either following a period of
relapses and remissions (secondary progressive)
or, less commonly, from the outset (primary
progressive).
Onset is usually in young adult life; this disease
rarely begins before 15 years or after 55 years of
age.
Multiple Sclerosis
There is a
tendency to involve
the optic nerves
and chiasm,
brainstem,
cerebellar
peduncles, and
spinal cord,
although no part of
the central nervous
system is
protected.
The peripheral
nervous system is
seldom involved.

Multiple Sclerosis
Steroid treatment, usually oral or intravenous
methylprednisolone, is useful in hastening
recovery from acute relapses but does not
influence the final disability or the frequency of
subsequent relapses.
Interferon and glatiramer acetate (copolymer 1)
reduce the rate and severity of relapses and slow
the progression of brain MRI abnormalities.
Mitoxantrone, a chemotherapeutic agent, and
monoclonal antibody therapy have produce
encouraging results for progressive and
recalcitrant relapsing-remitting disease.

Optic Neuropathy
Optic neuropathy in systemic lupus
erythematosus may be immune-mediated, with
features of inflammatory disease, or due to small
blood vessel occlusion, with features of ischemic
disease (see below).
Such disease occurring in individuals in whom no
evidence of sarcoidosis or other systemic
disease can be identified is known as idiopathic
granulomatous optic neuropathy or chronic
relapsing inflammatory optic neuropathy
(CRION).

Neuromyelitis Optica
Neuromyelitis optica (Devic's disease) is a rare
syndrome characterized by usually bilateral optic
neuritis and transverse myelitis.
Approximately 50% of patients progress to death
within the first decade due to the paraplegia, but
the remainder may have a prolonged remission
and, ultimately, a better prognosis than patients
with multiple sclerosis.



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