excellent presentation on detailed workup and management of optic neuritis

2. OPTIC NEURITIS
Presenter : Dr Usman Tariq
(Resident Ophthalmology)

3. DEFINITION
 Optic neuritis refers to
inflammation of the optic
nerve and is characterized by
acute, unilateral decreased vision
and associated optic nerve
dysfunction.
 Occurs in about 50 % of patients
with multiple sclerosis and is
presenting feature in 30% of MS.

4. EPIDEMIOLOGY
 1/1,00,000
 3:2- female:male ratio
 Young age (20-40 years old)

5. CLASSIFICATION:
Can beclassified on the basis of both
ophthalmoscopic appearnce and etiology :
⚫ Retrobulbarneuritis
⚫ Papillitis
⚫ Neuroretinitis
⚫ Demyelinating
⚫ Parainfectious
(rubella,mumps&chicken pox)
⚫ Infectious
(sinusitis.syphilis,cat-scratch fever)
⚫ Autoimmune
OPHTHALMOSCOPICALLY
ETIOLOGICALLY

6. Ophthalmoscopical Classification
 the optic disc appears
normal, at least initially,
because the optic nerve
head is not involved
 The optic disc appears normal
 The condition may be truly
described as ‘ the patient sees
nothing and the doctor sees
nothing.’
 is the most common
type in adults and is
frequently associated
with multiple sclerosis
Retrobulbar neuritis Papillitis Neuroretinitis
 Optic nerve head affected  papillitis in association
with inflammation of the
retinal nerve fibre layer
and a macularstar figure
 Hyperaemia and oedema of
the optic disc
 May be a/w peripapillary
flame-shaped haemorrhages
 Cells may be seen in the
posteriorvitreous.
 the mostcommon type
of optic neuritis in
children, but can also
affectadults.
 Macular star
(exudates that form
around the macula give
the appearance of the
star)
 It is the leastcommon
type
 only rarely a
manifestation of
demyelination

7. Normal optic disc, primary optic atrophy. The condition may be truly described as ‘
the patient sees nothing and the doctor sees nothing.’
Retrobulbar neuritis

8. 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

9. Macularstar
(Exudates in a star-shaped
pattern radiating from the
macula)
Neuroretinitis

10. According to aetiology
⚫Demyelinating:-
This is by farthe mostcommon cause.
⚫Parainfectious:-
following aviral infectionor immunization.
⚫Infectious:-
This may besinus-related, orassociated with conditions
such as cat-scratch disease, syphilis, Lyme disease,
cryptococcal meningitis and herpeszoster.
⚫ Non-infectious :-
sarcoidosis
systemic autoimmune diseases such as systemic lupus
erythematosus, polyarteritis nodosa and other
vasculitides.

11. TYPICAL OPTIC NEURITIS
 Predominantly affects females
 15-45 years
 Unilateral
 Acute , painful vision loss over hours to days

12. ATYPICAL OPTIC NEURITIS
 Painless visual loss
 Extremes of age
 Bilateral
 Disc hemorrhage , cotton wool
spots
 Progression of visual loss beyond 2
weeks
 Fails to improve with treatment

13. Differential Diagnosis:-
• Ischemic Optic Neuropathy
• Acute Papilledema
• Severe Systemic HTN
• Orbital Tumors
• Intracranial Mass
• LHON
• Toxic or Metabolic Optic Neuropathy

14. Demyelinating optic neuritis
⚫Demyelination :-
Apathological process in which normally myelinated nerve
fibres lose their insulating myelin layer.
The myelin is phagocytosed by microglia and macrophages,
Subsequently astrocytes laydown fibrous tissue in plaques.
Demyelinating diseasedisrupts nervous conduction within the
white mattertracts of the brain, brainstem and spinal cord.

15. PATHOGENESIS
 Perivascular infiltrate of
inflammatory cells
 Destruction of myelin
 Removal of disintegrated myelin
by phagocytic cells
 Proliferative gliosis

16. Demyelinating conditions that may involve the visual system
⚫ • Isolated optic neuritis:-
noclinical evidence of generalized demyelination, although in a
high proportion of cases this subsequentlydevelops.
⚫ • Multiple sclerosis (MS):-
by farthe mostcommon demyelinating disease .
⚫ • Devic disease (neuromyelitisoptica) :-
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) withindays orweeks.
⚫ • Schilderdisease:-
avery rare relentlessly progressive generalized disease with an
onset prior to the ageof 10 yearsand death within 1–2 years.
Bilateral optic neuritis withoutsubsequent improvement may occur.

17. 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 toan unremitting pattern.

18. • Systemic features may include:
⚫○ Spinal cord e.g. weakness,
stiffness, sphincterdisturbance,
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).

19. Ophthalmic features
⚫○ Common.
Optic neuritis (usuallyretrobulbar)
Internuclearophthalmoplegia
Nystagmus
⚫ ○ Uncommon
Skew deviation
Ocular motor nerve palsies
Hemianopia
⚫○ Rare
Intermediate uveitisand retinal periphlebitis

20. Association between optic neuritis and multiple sclerosis
⚫• Theoverall 15-yearrisk of developing MS following an
acuteepisodeof optic neuritis isabout 50%;
⚫with no lesionson MRI the risk is 25%,
⚫butover 70% in patientswithoneor more lesionson
MRI;
⚫the presence of MRI lesions is therefore a very strong
predictive factor.

21. Clinical features of demyelinating optic neuritis
Symptoms :-
○ Subacute monocularvisual impairment.
○ Usual age range 20–50 years (mean around 30).
○ Some patientsexperience tinywhiteorcoloured flashes
or sparkles (phosphenes).
○ Discomfort or pain in or around the eye is present in over
90% and typicallyexacerbated byocular movement; it
may precedeoraccompany thevisual lossand usually
lastsa few days.

22. Clinical features of demyelinating optic neuritis
Symptoms :-
○ Frontal headache and tendernessof theglobe mayalso be
present.

23. Signs
⚫○ Visual acuity (VA):-
usually 6/18–6/60, but may rarely
be worse.
⚫○ Othersigns of optic nerve
dysfunction :-
particularly impaired colour
vision and a relativeafferent
pupillarydefect.
⚫○ Theopticdisc is normal in the
majority of cases (retrobulbar
neuritis);
the remaindershow papillitis.
papillitis
Temporal discpallor

24. Signs
papillitis
Temporal discpallor
○ Temporal disc pallor may be
seen in the felloweye,
indicative of previousoptic
neuritis.

25. • Investigation
MRI:-
Almostalways shows characteristic
white matter lesions.
Imaging can show some broad
differences between etiologies of ON
or MS-ON.
Bilateral involvement of the optic
nerves is more common in NMOSD-
ON and MOG-ON.

26. • Investigation
MRI:-
Retrobulbar optic nerve involvement
is seen more often in MOG-ON, and
intracranial involvement is seen
more often in NMOSD-ON.
MOG-ON may be associated with
optic nerve sheath and surrounding
orbital fat enhancement.
.

27. • Investigation
○ Lumbar puncture :-
oligoclonal bands on protein
electrophoresisof cerebrospinal
fluid in 90–95%.
○ VEPs :-
abnormal (conduction delay
and a reduction in amplitude) in
up to 100% of patients with
clinically definite MS.

28. • Investigation
○ Trans Orbital Sonography :
To enhance diagnostic accuracy
for ON by measuring differences
in optic nerve sheath diameter.
68% sensitivity and 88%
specificity .

29. • Investigation
○ Blood Tests :
CBC & ESR
Serum AQP4 IGg levels
Anti MOG Abs

30. Visual field defects
⚫○ Diffusedepression of
sensitivity in theentire
central 30° is the most
common.
⚫○ Altitudinal/arcuatedefects
focal central/centrocaecal
scotomasare also frequent.

31. Course:-
⚫Vision worsensoverseveral days to 3 weeksand
then begins to improve .
⚫Initial recovery is fairly rapid and then slower
over 6–12 months.

32. Prognosis
○ More than 90% of patients recovervisual acuity to 6/9 or
better.
○ Subtleparameters of visual function, such as colour
vision, may remain abnormal.
○ A mild relativeafferent pupillarydefect may persist.
○ Temporal opticdisc palloror more marked opticatrophy may
ensue.
○ About 10%developchronic optic neuritiswith slowly
progressiveorstepwisevisual loss.

33. Treatment - Demyelinating optic
neuritis
• Indications forsteroid treatment:
Whenvisual acuitywithin the firstweek 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.

34. Treatment following demyelinating
optic neuritis
Therapy does not influence the eventual visual outcome and
the great majority of patients do not require treatment.
Intravenous methylprednisolone sodium succinatedaily
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.

35. Immunomodulatory treatment
(IMT)
⚫Reduces the risk of progression to clinical MS in some
patients, with the optionsavailable which include
interferon beta, glatiramer and monoclonal
antibodies(Natalizumab , ocrelizumab and alemtuzumab)
⚫Based on risk profile – particularly the presenceof
brain lesions – and patient preference;
⚫ most do not commence IMT until a second episode
of clinical demyelination has occurred

36. Parainfectious optic neuritis
⚫Associated with viral infections such as measles, mumps,
chickenpox, rubella, whooping coughand glandularfever,
and mayalsooccur following immunization.
⚫ Children areaffected much more frequentlythan adults.
⚫usually 1–3 weeksafteraviral infection, with acutesevere
visual lossgenerally involving botheyes.

37. Parainfectious optic neuritis
⚫Bilateral papillitis is the rule; (occasionally neuroretinitis may
occur or thediscs may be normal).
⚫The prognosis forspontaneousvisual 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 whereappropriate.

38. Infectious optic neuritis
⚫Sinus-related optic neuritis
⚫Cat-scratch fever(benign lymphoreticulosis)
neuroretinitis.
⚫Syphilis maycauseacute papillitisor neuroretinitis
during theprimaryorsecondarystages.
⚫ Lymedisease (borreliosis)
⚫ Cryptococcal meningitis.
⚫•Varicella zoster virus may cause papillitis by spread
from contiguous retinitis (i.e. acute retinal necrosis,
progressive retinal necrosis) orassociated with herpes
zosterophthalmicus.

39. Non-infectious optic neuritis
Sarcoidosis
⚫Optic neuritisaffects 1–5%.
⚫The response to steroid therapy
is often rapid, thoughvision may
decline if treatment is tapered or
stopped prematurely, and some
patients require long-term low-
dose therapy.
⚫Methotrexate mayalso be used
as an adjunct tosteroidsoras
monotherapy in steroid-
intolerant patients.

40. The optic nerve head may exhibit a lumpy appearance
suggestive of granulomatous infiltration and there may be
associated vitritis

41. Autoimmune
⚫Autoimmuneoptic nerve involvement may take the
form of retrobulbar neuritis or anterior ischaemic
optic neuropathy.
⚫Some patients mayalsoexperience slowlyprogressive
visual loss suggestiveof compression.
⚫Treatment is with systemicsteroidsand other
immunosuppressants.

42. Neuroretinitis
⚫Neuroretinitis refers to thecombinationof 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 notablecauses includesyphilis, Lymedisease,
mumpsand leptospirosis.

43. Neuroretinitis
⚫ Symptoms:
Painless unilateral visual impairment, usually
graduallyworsening overaboutaweek.
⚫ Signs :
○ VA is impaired toavariabledegree.
○ Signs of optic nervedysfunctionare usually mild orabsent,
as visual loss is largelydue to macular involvement.

44. Neuroretinitis
○ Venousengorgementand splinter haemorrhages may be
present in severecase.
○ Felloweye involvementoccasionallydevelops.

45. Papillitis associated with
peripapillary and macular
oedema
A macularstartypically
appears as disc swelling
settles; the macularstar
resolves with a return to
normal or near-normal visual
acuityover 6–12 months

46. INVESTIGATIONS
⚫OCT demonstratessub- and intraretinal fluid to a
variableextent.
⚫FA shows diffuse leakage from superficial disc
vessels.
⚫ Blood tests may include serology for Bartonella and
other causes according to clinical suspicion .

47. Treatment :
This is specific to thecause, and often consistsof
antibiotics.
Recurrent idiopathiccases may require treatment
with steroidsand/orother immunosuppressants.

48. Take Home Message :
• ON should be the diagnosis when other causes
have been ruled out especially retrobulbar
neuritis which is the commonest type and
where there is normal fundus exam and its
higher association with MS demands prompt
referal to neurologist.

49. Take Home Message :
•94% of cases are self resolving and do not need
treatment , steroid therapy if started has to be
implemented judiciuosly because oral
prednisolone alone has higher recurrence rate
than either IV plus oral regimen or placebo
regimen . Optic Neuritis Treatment Trial