3. Ophthalmoscopical Classification
Retrobulbar neuritis Papillitis Neuroretinitis
the optic disc appears
normal, at least initially,
because the optic nerve
head is not involved
Optic nerve head affected papillitis in association
with inflammation of the
retinal nerve fibre layer
and a macular star figure
The optic disc appears normal
The condition may be truly
described as ‘ the patient sees
nothing and the doctor sees
nothing.’
Hyperaemia and oedema of
the optic disc
May be a/w peripapillary
flame-shaped haemorrhages
Cells may be seen in the
posterior vitreous.
Macular star
(exudates that form
around the macula give
the appearance of the
star)
is the most common
type in adults and is
frequently associated
with multiple sclerosis
the most common type
of optic neuritis in
children, but can also
affect adults.
It is the least common
type
only rarely a
manifestation of
4. Normal optic disc, primary optic atrophy. The condition may be truly described as ‘
the patient sees nothing and the doctor sees nothing.’
Retrobulbar neuritis
5. swollen disc with blurring and hyperaemia of disc margin; venous dilation and
engorgement ; vitreous haziness because of inflammatory exudates and cells
that invaded the vitreous( mild vitritis); Flame-shaped hemorrhages and cotton
wool spot (soft exudates) on and around the disc; secondary optic atrophy
Papillitis
7. According to aetiology
Demyelinating:-
This is by far the most common cause.
Parainfectious:-
following a viral infection or immunization.
Infectious:-
This may be sinus-related, or associated with conditions
such as cat-scratch disease, syphilis, Lyme disease,
cryptococcal meningitis and herpes zoster.
Non-infectious :-
sarcoidosis
systemic autoimmune diseases such as systemic lupus
erythematosus, polyarteritis nodosa and other
vasculitides.
8. Demyelinating optic neuritis
Demyelination :-
a pathological process in which normally myelinated nerve fibres
lose their insulating myelin layer.
The myelin is phagocytosed by microglia and macrophages,
subsequent to which astrocytes lay down fibrous tissue in plaques.
Demyelinating disease disrupts nervous conduction within the
white matter tracts of the brain, brainstem and spinal cord.
9. Demyelinating conditions that may involve the visual
system include the following:
• Isolated optic neuritis:-
no clinical evidence of generalized demyelination, although in a
high proportion of cases this subsequently develops.
• Multiple sclerosis (MS):-
by far the most common demyelinating disease .
• Devic disease (neuromyelitis optica) :-
a very rare disease that may occur at any age, characterized by
bilateral optic neuritis and the subsequent development of
transverse myelitis (demyelination of the spinal cord) within days
or weeks.
• Schilder disease:-
a very rare relentlessly progressive generalized disease with an
onset prior to the age of 10 years and death within 1–2 years.
Bilateral optic neuritis without subsequent improvement may occur.
10. Multiple sclerosis
an idiopathic demyelinating disease involving
central nervous system white matter.
It is more common in women than men.
typically in the third–fourth decades, generally
with relapsing/remitting demyelination that
may switch later to an unremitting pattern, and
less commonly with progressive disease from
the outset.
11. • Systemic features may include:
○ Spinal cord e.g. weakness,
stiffness, sphincter disturbance,
sensory loss.
○ Brainstem, e.g. diplopia,
nystagmus, dysarthria, dysphagia.
○ Cerebral, e.g. hemiparesis,
hemianopia, dysphasia.
○ Psychological, e.g. intellectual
decline, depression, euphoria.
○ Transient features, e.g.
the Lhermitte sign
(electrical sensation on neck flexion)
the Uhthoff phenomenon
(sudden worsening of vision or other
symptoms on exercise or increase in
body temperature).
13. • Investigation
○ Lumbar puncture :-
oligoclonal bands on protein
electrophoresis of cerebrospinal
fluid in 90–95%.
○ MRI:-
almost always shows characteristic
white matter lesions
○ VEPs :-
are abnormal (conduction delay
and a reduction in amplitude) in
up to 100% of patients with
clinically definite MS.
14. Association between optic neuritis and multiple sclerosis
• The overall 15-year risk of developing MS following an
acute episode of optic neuritis is about 50%;
with no lesions on MRI the risk is 25%,
but over 70% in patients with one or more lesions on
MRI;
the presence of MRI lesions is therefore a very strong
predictive factor.
15. • A substantially lower risk of developing MS when there are
no MRI lesions is conferred by the following factors, providing
critical support in deciding whether to commence
immunomodulatory MS-prophylactic treatment following an
optic neuritis episode:
○ Male gender.
○ Absence of a viral syndrome preceding the optic neuritis.
○ Absence of periocular pain.
○ Optic disc swelling, disc/peripapillary haemorrhages or
macular exudates.
○ Vision reduced to no light perception.
• Optic neuritis is the presenting feature of MS in up to 30%.
• Optic neuritis occurs at some point in 50% of patients with
established MS.
16. Clinical features of demyelinating optic neuritis
Symptoms :-
○ Subacute monocular visual impairment.
○ Usual age range 20–50 years (mean around 30).
○ Some patients experience tiny white or coloured flashes or
sparkles (phosphenes).
○ Discomfort or pain in or around the eye is present in over
90% and typically exacerbated by ocular movement; it may
precede or accompany the visual loss and usually lasts a few
days.
○ Frontal headache and tenderness of the globe may also be
present.
17. • Signs
○ Visual acuity (VA):-
usually 6/18–6/60, but may rarely
be worse.
○ Other signs of optic nerve
dysfunction :-
particularly impaired colour
vision and a relative afferent
pupillary defect.
○ The optic disc is normal in the
majority of cases (retrobulbar
neuritis);
the remainder show papillitis.
○ Temporal disc pallor may be
seen in the fellow eye, indicative
of previous optic neuritis.
papillitis
Temporal disc pallor
18. Visual field defects
○ Diffuse depression of
sensitivity in the entire
central 30° is the most
common.
○ Altitudinal/arcuate defects
focal central/centrocaecal
scotomas are also frequent.
○ Focal defects are
frequently accompanied by
an element of superimposed
generalized depression.
19. Course:-
Vision worsens over several days to 3 weeks and
then begins to improve.
Initial recovery is fairly rapid and then
slower over 6–12 months.
20. Prognosis
○ More than 90% of patients recover visual acuity to 6/9 or
better.
○ Subtle parameters of visual function, such as colour
vision, may remain abnormal.
○ A mild relative afferent pupillary defect may persist.
○ Temporal optic disc pallor or more marked optic atrophy may
ensue.
○ About 10%develop chronic optic neuritis with slowly
progressive or stepwise visual loss.
21. Treatment following demyelinating
optic neuritis
• Indications for steroid treatment:.
When visual acuity within the first week of onset is worse
than 6/12,
treatment may speed up recovery by 2–3 weeks and may
delay the onset of clinical MS over the short term.
This may be relevant in the patients with poor vision in the
fellow eye or those with occupational requirements, but the
limited benefit must be balanced against the risks of high-
dose steroids.
Therapy does not influence the eventual visual outcome and
the great majority of patients do not require treatment.
Intravenous methylprednisolone sodium succinate daily for 3
days, followed by oral prednisolone for 11 days
Oral prednisolone may increase the risk of recurrence of optic
neuritis if used without prior intravenous steroid.
22. Immunomodulatory treatment
(IMT)
reduces the risk of progression to clinical MS in
some patients, but the risk versus benefit ratio has
not yet been fully defined with the options available,
which include interferon beta, teriflunomide and
glatiramer.
based on risk profile – particularly the presence of
brain lesions – and patient preference;
most do not commence IMT until a second episode
of clinical demyelination has occurred,
23. Parainfectious optic neuritis
Ass. With viral infections such as measles, mumps,
chickenpox, rubella, whooping cough and glandular fever,
and may also occur following immunization.
Children are affected much more frequently than adults.
usually 1–3 weeks after a viral infection, with acute severe
visual loss generally involving both eyes.
Bilateral papillitis is the rule; (occasionally: neuroretinitis
or the discs may be normal).
The prognosis for spontaneous visual recovery is very good,
and treatment is not required in the majority of patients.
However, when visual loss is severe and bilateral or involves
an only seeing eye, intravenous steroids should be
considered, with antiviral cover where appropriate.
24. Infectious optic neuritis
Sinus-related optic neuritis
Cat-scratch fever (benign lymphoreticulosis)
neuroretinitis.
Syphilis may cause acute papillitis or neuroretinitis
during the primary or secondary stages.
Lyme disease (borreliosis)
Cryptococcal meningitis.
•Varicella zoster virus may cause papillitis by spread
from contiguous retinitis (i.e. acute retinal necrosis,
progressive retinal necrosis) or associated with herpes
zoster ophthalmicus.
Primary optic neuritis is uncommon but may occur in
immunocompromised patients, some of whom may
subsequently develop viral retinitis.
25. Non-infectious optic neuritis
Sarcoidosis
Optic neuritis affects 1–5%.
The response to steroid therapy
is often rapid, though vision may
decline if treatment is tapered or
stopped prematurely, and some
patients require long-term low-
dose therapy.
Methotrexate may also be used
as an adjunct to steroids or as
monotherapy in steroid-
intolerant patients.
26. The optic nerve head may exhibit a lumpy appearance
suggestive of granulomatous infiltration and there may be
associated vitritis
27. Autoimmune
Autoimmune optic nerve involvement may take the
form of retrobulbar neuritis or anterior ischaemic
optic neuropathy (NEXT LECTURE).
Some patients may also experience slowly progressive
visual loss suggestive of compression.
Treatment is with systemic steroids and other
immunosuppressants.
28. Neuroretinitis
Neuroretinitis refers to the combination of optic neuritis
and signs of retinal, usually macular, inflammation.
Cat-scratch fever is responsible for 60%of cases.
About 25% of cases are idiopathic (Leber idiopathic
stellate neuroretinitis).
Other notable causes include syphilis, Lyme disease,
mumps and leptospirosis.
29.
Diagnosis
• Symptoms.
Painless unilateral visual impairment, usually
gradually worsening over about a week.
• Signs :-
○ VA is impaired to a variable degree.
○ Signs of optic nerve dysfunction are usually mild or absent,
as visual loss is largely due to macular involvement.
○ Venous engorgement and splinter haemorrhages may be
present in severe case.
○ Fellow eye involvement occasionally develops.
30. Papillitis associated with
peripapillary and macular
oedema
A macular star typically
appears as disc swelling
settles; the macular star
resolves with a return to
normal or near-normal visual
acuity over 6–12 months
31. (OCT) demonstrates sub- and intraretinal fluid to
a variable extent.
(FA) shows diffuse leakage from superficial disc
vessels.
Blood tests may include serology for Bartonella
and other causes according to clinical suspicion .
Treatment
This is specific to the cause, and often consists of
antibiotics.
Recurrent idiopathic cases may require treatment with
steroids and/or other immunosuppressants.
Editor's Notes
The papilla (the blind spot) is the spot there the optic nerve leaves the retina
A swollen disc with blurring and hyperaemia of disc margin
