Optic nerve

Optic Neuritis, Papilledema and Optic Atrophy

DR ; HASAN ASLAM
SENIOR REGISTRAR
Department of Ophthalmology
MITH
2
3
 Optic nerve
• 2nd craneal nerve.
• ptic nerve enters the bony skull through
the optic foramen and travels within the
optic canal to reach the middle cranial
fossa.
• An outgrowth of brain.
• About 47 to 50mm in length.

4
5
6
Intra ocular part of nerve fiber

7
Normal optic disc

8
9
10
 Retinal vessels
• Emerge on medial side of the cup, slightly
supro nasally
• Temporal artries make arcuate course as
they leave the disc.
• Course or artries and veins addintical.

11
12
Arrangements of optic nerve
fibers

13
Signs of optic nerve dysfunction
• Reduced va
• Rapd
• Visual field defect
• Dischrometopsia
• Deminished light/contrast sensitivity

14
 Learning Objectives
• At the end of the class, students shall be able to
• Define and classify optic neuritis.
• Understand the aetiology and principles of
management of optic neuritis.
• Understand the stages and importance of
papilloedema.
• Differentiate between various types of optic atrophy.

15
Question
• The disc in question is
of a 60 year old myope
who is instilling timolol
eye drops since the
past 5 years.

• Is this a normal optic

disc?

16
 Optic Neuritis
Definition: Inflammation of the optic nerve,
impairing nerve conduction.
Secondary to demyelination, infection or
autoimmune pathology.

17
 Classification
A. Papillitis
B. Retrobulbar neuritis
– Acute
– Chronic (toxic
amblyopia)
C. Neuroretinitis
D. Perineuritis

18
 Aetiology
• Idiopathic
• Demyelinating disorders

• Multiple Sclerosis
– Presenting feature in 25% patients
– 70% cases occur in established disease
– Recurs in same/ opposite eye in 25% patients
– Uhthoff”s phenomenon: impairment of vision more
with increased body temperature
– Pulfrich phenomenon: altered perception of moving
objects
19
 Aetiology
• Neuromyelitis optica (of Devic): acute, bilateral optic
neuritis in young patient with paraplegia
• Post-viral: mumps, measles, chicken pox, whooping
cough

• Metabolic/Nutritional deficiency:

B1, B6, B12, B2, Folic acid deficiency

Thyroid dysfunction, diabetes

• Hereditary optic neuritis (Leber's disease)

20
 Aetiology
• Toxic amblyopia:
Chloroquine, Ethambutol
Tobacco, Ethyl alcohol, methyl alcohol
Lead, Arsenic.

• Ischaemic: Giant cell arteritis, Takayasu's disease,

PAN, SLE

• Granulomatous inflammation:
Sarcoidosis, tuberculosis, syphilis
21
 Symptoms
• Idiopathic/demyelinating : 20-40 years of age

• Viral: children

• Uniocular sudden/rapid diminution of vision

• Visual loss, usually maximum by end of second week,

improves by 1-4 weeks
• Discomfort/pain behind eyeball especially when moved
superiorly

22
 Signs

• Visual Acuity: Usually 6/60 or less

• Local tenderness

• Pupillary reaction: Sluggish, ill-sustained or RAPD

• Impaired coloured vision: hue, brightness

• Impaired contrast sensitivity

• Delayed dark adaptation

• Visual Field: central, centrocaecal or paracentral scotoma,

more pronounced for coloured fields
23
 Ophthalmoscopic findings
• Optic neuritis: MC in children,
engorged, oedematous optic disc
with obliteration of optic cup, small
haemorrhages on disc

• Retrobulbar neuritis: MC in adults

• Neuroretinitis:
Optic neuritis+ macular star

24
 Differential diagnosis
• Papilloedema
• Pseudopapillitis
High hypermetropia,
Myelinated nerve fibres,
Optic nerve head drusen
(blurred margin, disc not significantly
elevated, no vascular changes, stationary)

25
 Investigations
• MRI: demyelinating
lesions, SOL
• VEP: reduced
amplitude and delayed
transmission time
(P100 latency
increased)

Right optic neuritis

26
 Course and prognosis
• Recovery takes 4-6 weeks
• 90% recover normal VA, but colour vision
defects may persist
• No correlation between initial visual loss
and final visual outcome
• 10% secondary or post-neuritic optic
atrophy
• Better outcome in young, unilateral cases
27
 Treatment
• Of cause e.g. anti-infective therapy

• Intravenous methyl prednisolone 20 mg/kg/day(250

mg QID) for 3 consecutive days followed by oral
prednisolone 1-1.5 mg/kg
• Dexamethasone 200 mg OD pulse for 3-5 days is a
cheaper alternative
• Supportive therapy like B1,B6,B12 for 3 wks
28
 Papilloedema

• Definition: Bilateral,

• non-inflammatory passive swelling of optic

disc due to raised Intracranial pressure.
• Does not develop if optic nerve is atrophic

• May be asymmetrical

29
30
pathophysiology

31
32
Foster-Kennedy syndrome : contralateral
papilloedema with ipsilateral pressure atrophy of
optic nerve
Due to - frontal lobe tumour, olfactory meningioma

33
 Aetio-Pathogenesis
• Elevated Intracranial pressure due to any cause

• Prelaminar Optic Nerve is affected by changes in tissue

pressure, IOP and CSF pressure
• Increased CSF pressure increases tissue pressure
hampering axoplasmic flow
• This further increases pressure on pre-laminar capillaries and
small veins causing vasodilatation and tortuosity
• Venous drainage compromise further increases congestion

34
 General Symptoms

• Headache, made worse by coughing or

straining
• Vomiting

• Focal neurological deficit with/without

changes in level of consciousness

35
 Ocular symptoms
• Systemic symptoms. include headaches, nausea, vomiting, and
pulsatile tinnitus. Headache characteristics typically are positional
and worse in the mornings and when laying down.
• Visual symptoms. include transient visual obscurations.

36
 Signs
• Pupillary reactions are normal until
secondary atrophy sets in
• Early:
– Blurring of nasal>superior>inferior margins of
disc
– Disc hyperemia and dilated capillaries
– Spontaneous venous pulsation absent
– Splinter haemorrhages at/just off disc margin
– Normal optic cup preserved
37
38
39
40
 Established papilloedema
• Margins indistinct and cup
obliterated
• Surface elevated upto more than
+3 D with direct ophthalmoscope
• Flame-shaped haemorrhages,
cotton-wool spots
• Venous engorgement and
peripapillary oedema
• Paton's Lines-radial lines
cascading from optic disc
• Macular star
41
 With progression
• Chronic papilloedema
– Central cup remains
obliterated
– Haemorrhagic and exudative
components resolve gradually
– 'Champagne cork' appearance

42
• Atrophic papilloedema
– Retinal vessels attenuated
with perivascular sheathing
– Dirty white colour due to
reactive gliosis
– Leads to secondary optic
atrophy

43
 Visual fields
• Early-no changes
• Established stage- enlarged blind spot
• Chronic- peripheral constriction of field
with nerve fibre bundle defects
• Finally- total loss of visual field

44
Fundus photo, FFA , OCT

45
 Papilloedema Optic neuritis
History Headache, vomiting Rapid DV preceded by
fever/respiratory infection
Laterality usually bilateral usually unilateral

VA normal till late stage severely reduced <6/60

Pain/tenderness of eyeball absent may be present

Pupil reaction normal RAPD (Marcus-Gunn's pupil)

Disc swelling >+3 D in established +2D to +3D

Haemorrhage, exudates More, in established relatively less

Visual fields Enlarged blind spot, Central or centrocaecal scotoma

later gradual
constriction
Colour vision No effect Affected

CT/MRI SOL Demyelinating disorder

Recovery of vision May not be complete Usually complete after adequate

46
even after treatment treatment
 Treatment
• Conservative management
– Weight reduction: a recommended amount of weight loss was found to be (5%-
10%) to improve the symptoms and signs of high ICP .
– Avoid precipitating medications
– Control underlying risk factors ( e.g. thyroid disease, or obstructive sleep apnea
to name a few)

• Medical management
• acetazolamide (a carbonic anhydrase inhibitor)

Surgical decompression of optic nerve to preserve vision

47
 Optic Atrophy
Optic nerve shrinkage from any process that
produce degeneration of axons in the
ant.visual (Retinogeniculate) pathway

48
49
 Classification-Aetiological
Primary Optic Atrophy
• No local disturbance,
associated with CNS disease
or no discoverable cause
• Commonest cause – Multiple
Sclerosis
• Leber's optic atrophy
• Nerve compression:
Tumour, hydrocephalus
• Injury to retrobulbar optic
nerve
50
 Cavernous type of Primary Optic
Atrophy
• Deep excavated
cup with
undermined
edges
– Glaucomatous
optic atrophy

51
 Aetiological classification
• Secondary optic atrophy: preceded by
swelling of optic disc- papilledema, optic neuritis,
neuroretinitis

• Consecutive optic atrophy:

follows extensive disease of the retina -
Retinitis pigmentosa

Long-standing retinal detachment

52
 Anatomical classification
• Ascending: Lesion in retina, terminates at lateral
geniculate body –
• Eg: RP, CRAO

• Descending: Disease involving optic nerve fibres

anterior to LGB, terminates at optic disc –
• Eg: chiasmal compression

53
 Symptoms

• Gradual/rapid loss of central/peripheral

vision
• Impairment of colour vision

54
 Signs
• Visual Acuity impaired in proportion to
death of optic nerve fibres
• RAPD in unilateral Optic Atrophy
Ultimately pupil dilated and immobile

55
 Primary OA ophthalmoscopy
• Pale disc, classically
paper white in colour
• Margins sharply defined
• Minimal atrophic cupping
• Blood vessels
attenuated with marked
reduction of small blood
vessels on ONH to <6

56
Secondary OA ophthalmoscopy
• Pale disc with dirty-grey
colour, blurred margins
• Physiological cup is full,
lamina cribrosa obscured
• Narrowing of blood vessels
with sheathing
• Gliosis over disc surface
extending towards
peripapillary retina
57
 Primary OA Secondary OA
Appearance chalky white dirty grey
Margins sharply defined blurred
Cup deep obliterated
Laminar dots visible not visible
Glial proliferation absent marked
Vessels no sheathing sheathing
Previous disc oedema absent present

58
 Consecutive optic atrophy
• Yellowish-waxy pallor of the disc
• Margins less sharply defined
• Marked narrowing, even obliteration of
retinal blood vessels

59
60
 Investigations

• Visual field: In partial OA, central vision is

depressed with concentric contraction of the
visual field
• FFA of Optic nerve head

• VEP especially in children

• Neurological evaluation
61
 Hereditary optic atrophy
• Autosomal recessive/dominant
• LHON: Leber's hereditary Optic
atrophy

62
 Treatment
• Treat the cause
• Gene therapy is
emerging
• Community based
rehabilitation in
bilateral cases as
prognosis is poor

Low-vision aids

63
Thank you

64