The presentation was made under the wise guidance of my professor DR.(prof) P. Rawat (MGMMC & M.Y. HOSPITAL, INDORE).It covers the essential aspects of optic neuritis & optic atrophy.

1. Moderator: Dr. (prof.) P. RAWAT Ma’m
Presenter: Dr. SAMIKSHA

3. OPTIC NEURITIS

4. DEFINITION
An inflammation of the optic nerve is known as
OPTIC NEURITIS

5. AETIOLOGY

6. CLASSIFICATION
A) According to OPHTHALMOSCOPIC
APPEARANCE
 Retrobulbar neuritis
 Papillitis
 Neuroretinitis

7. B) According to the etiology
 Demyelinating (MOST COMMON)
 Parainfectious
 Infectious
 Non infectious

8. A.1 RETROBULBAR NEURITIS
 the optic disc appears normal, initially, because the
optic nerve head is not involved
 It is the most common type in adults and is
frequently associated with multiple sclerosis
(MS).

9. Demyelinating conditions that
may involve the visual system
include the following:
 Isolated optic neuritis with no clinical
evidence of generalized demyelination
although in a high proportion of cases this
subsequently develops

10.  Devic disease (neuromyelitis optica),
characterized by bilateral optic neuritis
and the subsequent development of
transverse myelitis (demyelination of the
spinal cord) within days or weeks.

11.  Schilder disease, a 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.

12.  idiopathic demyelinating disease involving
central nervous system white matter.
 It is more common in women than men.
 Presentation is 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.

13. Systemic features may include:
o Spinal cord
o Brainstem
o Cerebral
o Psychological
o Lhermitte sign (electrical sensation on neck
flexion)

14. Ophthalmic features:
 Common. Optic neuritis (usually retrobulbar)
 Uncommon. Skew deviation, ocular motor nerve
palsies, hemianopia.
 Rare. Intermediate uveitis and retinal
periphlebitis
 Uhthoff phenomenon (sudden worsening of vision or
other symptoms on exercise or increase in body
temperature)
 Pulfrich phenomenon (altered perception of moving
objects)

15. Investigation:.
○ Lumbar puncture shows oligoclonal bands on
protein electrophoresis of cerebrospinal fluid in
90–95% cases
○ MRI almost always shows characteristic white
matter lesions
○ VEPs are abnormal (conduction delay and
reduction in amplitude) in up to 100% of patients
with clinically definite MS

16. Multiple sclerosis. T1-weighted axial MR image
showing characteristic periventricular plaques

17. Oligoclonal bands on protein electrophoresis

18. Clinical features of
demyelinating optic neuritis
SYMPTOMS
○ Subacute monocular visual impairment.
○ age range 20–50 years
○ tiny white or coloured flashes or sparkles
(phosphenes).
○ Discomfort or pain in or around the eye,
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

19. SIGNS
○ Visual acuity (VA) is usually 6/18–6/60, but
may rarely be worse.
○ 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

20. VISUAL FIELD DEFECTS
○ Diffuse depression of sensitivity in the entire
central 30° is the most common.
○ Altitudinal/arcuate defects and focal
central/centrocaecal scotomas are also
frequent.

22. 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.

23. PROGNOSIS:
○ More than 90% of patients recover visual acuity
to 6/9 or better.
○ 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.

24. 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.
• Steroid regimen.
Intravenous methylprednisolone sodium succinate 1 g daily for 3
days, followed by oral prednisolone (1 mg/kg daily) for 11 days,
subsequently tapered over 3 days.
Oral prednisolone may increase the risk of recurrence of
optic neuritis if used without prior intravenous steroid.

25. • 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

26. A.2 PAPILLITIS
 It affects the optic nerve head (hyperemia &
oedema of optic disc) along with peripapillary
flame shaped haemorrhages
 The disc is at first hyperaemic; later the
margins become blurred, oedema ensue
which spread onto the retina, the retinal veins
become tortuous and extensively distorted,
exudates may accumulate upon the disc and
there are fine vitreous opacities

28. CLINICAL FEATURES:
 profound visual loss
 disturbance of colour vision
 decrease in contrast sensitivity
 diminished stereoacuity
 abnormal visual fields
 Pupillary reactions demonstrate a prominent
relative afferent pupillary defect

29. INFLAMMATION
MILD
INFECTION
RESOLVED
NORMAL
OPTIC DISC
SEVERE OR
PROLONGED
DESTRUCTION
OF NERVE
FIBRES
POST NEURITIC
ATROPHY

30. POST NEURITIC ATROPHY showing blurred disc margins,
dirty grey colour floor filled in with organized tissue which
extends onto the constricted arteries as perivascular sheaths

31. A.3 NEURO RETINITIS
 Inflammation of the optic disc with adjacent retinal
inflammation
 papillitis with retinal exudates which are in the retinal
nerve fibre layer are usually radially oriented forming
a macular fan or star.
 It is a combination of optic neuritis and signs of
retinal, usually macular, inflammation.
 60% of cases are due to CAT SCRATCH FEVER.
 25% of cases are idiopathic(Leber idiopathic stellate
neuroretinitis).
 Other causes include syphilis, Lyme disease, mumps
and leptospirosis

32. SYMPTOMS
Painless unilateral visual impairment, gradually
worsening over a week.
SIGNS
○ Impaired visual acuity
○ Signs of optic nerve dysfunction are usually mild or
absent, as visual loss is largely due to macular
involvement.
○ Papillitis associated with peripapillary and macular
oedema
○ A macular star appears as disc swelling settles; the
macular star resolves with a return to normal or near-
normal visual acuity over 6–12 months.
○ Venous engorgement and splinter haemorrhages
may be present in severe case.
○ Fellow eye involvement occasionally develops

33. Progression of neuroretinitis.
(A) Severe papillitis;
(B) later stage showing macular star

34. • Optical coherence tomography (OCT)
Shows sub- and intraretinal fluid to a
variable extent.
• Fluorescein angiography (FA)
shows diffuse leakage from superficial disc
vessels.
• Blood tests may include serology for
Bartonella and other causes according to
clinical suspicion

35. Treatment
 This is specific to the cause, and often
consists of antibiotics.
 Recurrent idiopathic cases may require
treatment with steroids

36. B.1 DEMYELINATING OPTIC NEURITIS
 Most common type
 Frequently associated with MULTIPLE
SCLEROSIS (discussed earlier)

37. B.2 PARAINFECTIOUS OPTIC NEURITIS
Optic neuritis may be associated with
1.viral infections such as measles,
mumps, chickenpox, rubella, whooping
cough and glandular Fever
2. following immunization
*Children>Adults

38. PRESENTATION
 usually 1–3 weeks after a viral infection
acute severe visual loss generally involving both eyes
 Bilateral papillitis is the rule
 occasionally there may be a neuroretinitis or the
discs may be normal.
PROGNOSIS
 spontaneous visual recovery is very good
 treatment is not required in the majority of patients
 when visual loss is severe and bilateral or involves
an only seeing eye, intravenous steroids should be
considered, antiviral cover where appropriate.

39. MEASLES
 Subacute sclerosing panencephalitis (SSPE) is
a late complication of measles infection,
manifesting with chronic progressive
neurodegenerative & usually fatal disease of
childhood
 Posterior uveitis is common, and may be the
presenting feature

40. Retinal involvement in subacute sclerosing
panencephalitis

41. RUBELLA
 ocular features of congenital rubella include
cataract, anterior uveitis, ‘salt and pepper’
pigmentary retinopathy, glaucoma and
microphthalmos

42. Rubella retinopathy

43. B.3 INFECTIOUS OPTIC NEURITIS
a) SINUS RELATED OPTIC NEURITIS
 is uncommon
 characterized by recurrent attacks of unilateral
visual loss
 associated with severe headache and spheno-
ethmoidal Sinusitis
 Possible mechanisms include direct spread of
infection, occlusive vasculitis and mucocoele
 Treatment : systemic antibiotics
surgical drainage

44. b) CAT SCRATCH FEVER
 caused by Bartonella henselae
 transmitted by the scratch (or bite) of an
apparently healthy cat
 One or more red papules at the site of
inoculation are followed by fever and regional
lymphadenopathy

45. Ocular features
 NEURORETINITIS is the most common
manifestation
 disc oedema with macular exudate in a star
conformation
 intermediate uveitis
 focal retinochoroiditis
 Vasculitis
 conjunctivitis with a 2–4 mm conjunctival
granuloma (Parinaud oculoglandular
Syndrome)

46. Cat-scratch disease – ulcerated papule on the
cheek caused by a cat scratch 2 weeks previously,
with enlargement of submandibular lymph nodes

48. Treatment
 Oral antibiotics, e.g. co-trimoxazole,
azithromycin, rifampicin or ciprofloxacin
(avoided in children)
 Steroids
 Treatment is not usually given for mild
systemic symptoms alone.

49. c) SYPHILIS
 caused by Treponema pallidum
 It may cause acute papillitis or neuroretinitis
during the primary or secondary stages

50. Ocular features
 Anterior uveitis
 Chorioretinitis is often multifocal bilateral and
associated with vitritis
 Acute syphilitic posterior placoid chorioretinopathy
(ASPPC) is characterized by large pale-yellowish
subretinal lesions in the posterior pole
 Retinitis has a ‘ground glass’ appearance
 Optic neuritis and neuroretinitis
 Other features include conjunctivitis, episcleritis and
scleritis, intermediate uveitis, glaucoma, cataract and
miscellaneous neuro-ophthalmic features related to
CNS involvement including Argyll Robertson pupils

51. ROSEOLAE

52. old multifocal chorioretinitis

53. acute posterior placoid chorioretinitis

54. NEURORETINITIS

55. d) LYME DISEASE
 Caused by Borrelia burgdorferi
 transmitted by a tick bite
 It may cause neuroretinitis and occasionally
acute retrobulbar neuritis, which may be
associated with other neurological
manifestations and can mimic MS.

56. Fundus photograph of patient having
Lyme disease

57. e) CRYPTOCOCCAL MENINGITIS
•Cryptococcal meningitis in patients with
acquired immunodeficiency syndrome (AIDS)
may be associated with acute optic neuritis,
which may be bilateral

58. Severe optic nerve head oedema in a patient with
CRYPTOCOCCAL MENINGITIS

59. Optic atrophy & severe visual loss after treatment of
cryptococcal meningitis

60. F) VARICELLA ZOSTER VIRUS
 may cause papillitis by spread from
contiguous retinitis or associated with herpes
zoster ophthalmicus.
 Primary optic neuritis is uncommon but may
occur in immunocompromised patients

61. A) SARCOIDOSIS
 The optic nerve head may exhibit a lumpy appearance
suggestive of granulomatous infiltration and there may
be associated vitritis
 The response to steroid therapy is often rapid, though
vision may decline if treatment is tapered or stopped
prematurely, 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.
B.4 NON INFECTIOUS OPTIC NEURITIS

62. Sarcoid granuloma of the optic nerve head with
overlying vitreous haze

63. B) AUTOIMMUNE
 Autoimmune optic nerve involvement may take the
form of retrobulbar neuritis or anterior ischaemic
optic neuropathy
 Some patients may also experience slowly
progressive visual loss suggestive of compression.
 Treatment is with systemic steroids and other
immunosuppressants.

66. PARASITIC INFESTATIONS OF
THE OPTIC NERVE
 Cysticercus cellulosae within the optic nerve
is rare.
 there is profound visual loss
 the condition may mimic optic neuritis,
papillitis, neuroretinitis or unilateral severe
disc oedema.
 the diagnosis is often delayed or missed as
it is often mistaken for an optic nerve
tumour on neuroimaging.

67. Fundus photograph showing severe disc oedema with inflammation

68. CT scan of the same patient shows a cystic lesion within
the optic nerve with an internal, hyperdense lesion suggestive of a
scolex.

69.  The cyst has a highly reflective pinhead lesion
within it representing the scolex which may
be detected by ultrasonography or careful
examination of CT or MRI scans performed
with 1 mm sections

70. TREATMENT
 high doses of STEROIDS to reduce
inflammation as the toxins released by the
dying parasite are believed to be responsible
for the visual loss.
 Medical treatment: ORAL ALBENDAZOLE
 surgical removal of the cyst (poor results)

71. DIFFERENTIAL DIAGNOSIS
 Ischemic optic neuropathy
 Papilloedema
 Leber hereditary optic neuropathy
 Toxic optic neuropathy
 metabolic optic neuropathy
 compressive space occupying lesion in the
orbit
 Compressive space occupying lesion
intracranially in the chiasmal region

72. CLINICAL WORKUP
 SYMPTOMS (discussed earlier)
 careful HISTORY TAKING
 AGE of the patient
 RAPIDITY OF ONSET
 occurrence of any PREVIOUS EPISODES
 presence of PAIN ON EYE MOVEMENTS

73. COMPLETE OPHTHALMIC EXAMINATION
 recording the VISUAL ACUITY
 COLOUR VISION
 assessing PUPILLARY REACTIONS
 RETINAL EXAMINATION to assess the optic nerve
 EVALUATION OF VITREOUS for cells.
 VISUAL FIELD testing
 VEPs recorded for both eyes, as asymptomatic
fellow eye abnormalities are common and can be
detected with these techniques

74. ADDITIONAL TESTS (performed for ‘atypical’
optic neuritis)
 complete blood count
 estimation of rapid plasma reagin
 CRP
 ESR
 fluorescent treponemal antibody absorption
(FTA-ABS) test
 antinuclear antibody (ANA) test.

75.  For the first episode and in every atypical case, MRI
of the brain and orbits with gadolinium
enhancement is recommended to predict the
likelihood of MS and ruling out a space-occupying
lesion.
 Patients with demyelination of the central nervous
system on MRI or an abnormal neurological
examination should be referred to a neurologist for
evaluation and management of possible multiple
sclerosis.

76. TREATMENT (based on ONTT)
1. profound visual loss + no previous history of optic
neuritis or multiple sclerosis seen early + MRI
shows at least one area of demyelination, I.V
METHYLPREDNISOLONE (250 mg intravenously slowly
over 30–60 minutes repeated 6 hourly for 3 days,
followed by oral prednisolone 1 mg/kg/day for 11
days)
Prednisolone is rapidly tapered off over the next 3
days.

77. If the MRI is normal, multiple sclerosis is
unlikely but cannot be entirely ruled out.
Pulsed intravenous steroid treatment may
still be used to shorten the period of visual
impairment, particularly in severe and
bilaterally affected cases.
Oral prednisolone, in conventional doses of 1
mg/kg/day, should never be used alone as
the recurrence rate is remarkably high

78. 2. If a patient has already been diagnosed to
have multiple sclerosis or has suffered from
prior episodes of optic neuritis,Observation is
the rule, unless faster visual recovery is
specifically required.

79. OPTIC ATROPHY

80. DEFINITION
 Optic atrophy refers to the late stage changes that
take place in the optic nerve resulting from axonal
degeneration in the pathway between the retina
and the lateral geniculate body, manifesting with
disturbance in visual function and in the
appearance of the optic nerve head.

81.  It can be classified in several ways, including
by whether axonal death is initiated in the
retina (ANTEGRADE) or more centrally
(RETROGRADE) & by cause
 A brain tumour will produce primary atrophy
if it presses upon the chiasma or optic nerve,
and a secondary optic atrophy if it causes
papilloedema due to increased intracranial
pressure.

82. 1) PRIMARY OPTIC ATROPHY
 occurs without antecedent swelling of
the optic nerve head.
 It may be caused by lesions affecting
the visual pathways at any point from
the retrolaminar portion of the optic
nerve to the lateral geniculate body.
 Lesions anterior to the optic chiasm
result in unilateral optic atrophy,
whereas those involving the chiasm
and optic tract will cause bilateral
changes.

83. • Signs
 Flat white disc with clearly delineated margins
 Reduction in the number of small blood vessels
on the disc surface.
 Attenuation of peripapillary blood vessels and
thinning of the retinal nerve fibre layer (RNFL).
 The atrophy may be diffuse or sectoral
depending on the cause and level of the lesion.
 Temporal pallor of the optic nerve head may
indicate atrophy of fibres of the papillomacular
bundle, classically seen following demyelinating
optic neuritis.
 Band atrophy is caused by involvement of the
fibres Entering the optic disc nasally and
temporally (occurs in lesions of the optic chiasm
or tract and gives nasal as well as temporal
pallor)

84. Primary optic atrophy due to compression

85. • Important causes
 Optic neuritis.
 Compression by tumours and aneurysms.
 Hereditary optic neuropathies.
 Toxic and nutritional optic neuropathies may
give temporal pallor
 Trauma.

86. primary optic atrophy due to nutritional neuropathy –
note predominantly temporal pallor;

87. Optic atrophy showing demyelination of the fibres
from the papillomacular bundle.

88. 2) SECONDARY OPTIC
ATROPHY
 Secondary optic atrophy is preceded by long-
standing swelling of the optic nerve head.

89. • SIGNS vary according to the cause and its course.
 Slightly or moderately raised white or greyish
disc with poorly delineated margins due to
gliosis
 Obscuration of the lamina cribrosa.
 Reduction in the number of small blood vessels
on the disc surface.
 Peripapillary circumferential retinochoroidal
folds, especially temporal to the disc (Paton lines)
 sheathing of arterioles and venous tortuosity may
be present.

90. secondary optic atrophy due to chronic papilloedema – note
prominent Paton lines

91. CAUSES
 chronic papilloedema
 anterior ischaemic optic neuropathy
 papillitis.
 Intraocular inflammatory causes of marked
disc swelling

92. 3) CONSECUTIVE OPTIC ATROPHY
 caused by disease of the inner retina or its
blood supply.
 The cause is usually obvious on fundus
examination, e.g. extensive retinal
photocoagulation, retinitis pigmentosa or
prior central retinal artery occlusion.
 The disc appears waxy.

93. consecutive optic atrophy due to vasculitis

95. 4) GLAUCOMATOUS OPTIC ATROPHY
A) SUBTYPES OF GLAUCOMATOUS DAMAGE
i) Focal ischaemic discs characterized by
 localized superior and/or inferior notching
 may be associated with localized field
defects

96. Focal ischaemic – inferior notch and disc haemorrhage

97. ii) Sclerotic discs are characterized by:
 a shallow saucerized cup and a gently sloping
NRR
 Variable peripapillary atrophy
 peripheral visual field loss
 associated with systemic vascular disease.

98. sclerotic

99.  iii) Myopic disc with glaucoma
 refers to a tilted (obliquely inserted) shallow
disc
 temporal crescent
 parapapillary atrophy

100. myopic

101. iv) Concentrically enlarging disc
 fairly uniform NRR thinning
 frequently associated with diffuse visual field
loss.

102. concentrically enlarging

103. NON SPECIFIC SIGNS OF GLAUCOMATOUS
CHANGE
 Disc haemorrhages
 Baring of circumlinear blood vessels
 Bayonetting
 Collaterals between two veins at the disc

104. Disc haemorrhages

105. baring of inferior circumlinear blood vessel

106. bayoneting of blood vessels

107. collateral vessels

108. loss of nasal neuroretinal rim and laminar dot sign

109. histological section of glaucomatous optic atrophy.

110. 5) TOTAL OPTIC ATROPHY
 pupils are dilated, not responding to
light
 In unilateral, the consensual reaction
to light is exaggerated.
 In partial optic atrophy, central vision
is depressed and there is concentric
contraction of the field, with or
without scotomata, relative or
absolute, depending upon the cause.

111.  No treatment is effective for optic atrophy
 Prognosis depends on the possibility of early
control of the causal factor.