NEURO OPHTHALMOLOGY

1. INFLAMMATORY OPTIC NEUROPATHY
DR KUMAR SIDDHARTH
2ND YEAR PG, SCBMCH

2. ANATOMY OF THE OPTIC NERVE
1.2 million afferent nerve fibres which originate from retinal ganglion cell
Most synapse in Lateral geniculate body, while some reach other centres
notably Pretectal nuclei of the midbrain.
1/3rd of the fibres sub serve central 5 degree of the visual field
Subdivided into 600 bundles by fibrous septae derived from the piamater.
Covered by meninges, piamater, arachnoid and duramater

3. ANATOMIC SUBDIVISIONS
 50 mm long from globe to
chiasm, and is subdivided into 4
segments
INTRAOCULAR
1mm
INTRAORBITAL
25-30mm
Globe to optic foramen
INTRACANALICULAR
6mm
Segment traverses the optic canal
INTRACRANIAL
5-16mm
From optic canal to optic chiasma

4. SIGNS OF OPTIC NERVE DYSFUNCTION
Reduced visual acuity
Relative afferent
pupillary defect
Dyschromatopsia
Mainly affects red and green
Diminished light
brightness sensitivity
Persists even after return of
visual acuity to normal
Diminished contrast
sensitivity
Visual field defects
Type of visual field defects
vary with underlying
pathology

5. CLASSIFICATION OF OPTIC NEUROPATHY
Inflammatory
Ischaemic
Hereditary
Nutritional and toxic
Traumatic
Compressive/infiltrative
Papilloedematous
Glaucomatous

6. 1. INFLAMMATORY OPTIC NEUROPATHY
OPTIC NEURITIS
 Classification based on ophthalmological appearance
RETROBULBAR NEURITIS
• Optic disc
appears
PAPILLITIS
• Hyperaemia and oedema of the optic disc
• Peripapillary flame shaped haemorrhage
• Cells in posterior vitreous
• More common in children but also affects adults
NEURORETINITIS
• Papillitis with inflammation of retinal nerve fibre
layer
• Presence of macular star
• Least common type of optic neuritis

8. PATHOPHYSIOLOGY
In acute optic neuritis the lesions are sharply defined areas of myelin loss with
relative preservation of axons
Large number of foamy macrophages exist with cholesterol ester droplets and
abundant lymphocyte and plasma cell accumulations
In later stages the number of plasma cells and macrophages diminish, astrocytic
scar formation occurs
Little remyelination of the axons in lesions is associated with chronic multiple
sclerosis, although little remyelination attempts can also be seen in acute cases

9. OTHER FACTORS
Various genetic and environmental factors are presumed
to predispose patients to demyelination
HLA-DW2, HLA-DR2 are known risk factors for multiple
sclerosis and optic neuritis
Infections, stress, systemic antigens and metabolites have
been proposed as initiating events that result in
autoreactive antibodies leading to demyelination

10. COMMON SITES OF OPTIC NERVE
INVOLVEMENT
 Anterior (45%), abutting the optic disc
 Mid - intraorbital (61%)
 Intracanalicular (34%)
 Intracranial prechiasmatic (5%)
 Chiasmatic (2%)
Lesions usually involve more than one site

11. ETIOLOGICAL CLASSIFICATION OF OPTIC
NEURITIS
Demyelinating
Para infectious
Infectious
Non infectious

12. DEMYELINATING OPTIC NEURITIS
This is by far the most common cause of optic neuritis
Demyelination is a pathological process in which myelinated nerve fibre lose their myelin sheath
Myelin sheath is phagocytosed by microglia and macrophages
Astrocytes lay down fibrous tissue plaques
Demyelinating diseases disrupts nerve conduction within white matter of the brain, brainstem and
spinal cord

13. MULTIPLE SCLEROSIS
Most common demyelinating disease
Idiopathic demyelinating disease involving central nervous
system white matter
More common in women than men
Presents in 3rd – 4th decade of life
From relapsing remitting pattern, it later switches to unremitting
pattern
Rarely progressive from the outset

14. FEATURES OF MULTIPLE SCLEROSIS
SYSTEMIC FEATURES
•Spinal cord – weakness, stiffness, sphincter disturbance,
sensory loss
•Brainstem – diplopia, nystagmus, dysarthria, dysphagia
•Cerebral – hemiparesis, hemianopia, dysphasia
•Psychological – intellectual decline, depression, euphoria
•Lhermitte sign – electrical sensation on neck flexion
•Uhthoff phenomenon – sudden worsening of vision or
other symptoms on exercise or increase in body
temperature
OCULAR FEATURES
•Common
•Optic neuritis (most commonly retrobulbar neuritis)
•Internuclear ophthalmoplegia
•Nystagmus
•Uncommon
•Skew deviation
•Oculomotor nerve palsy
•Hemianopia
•Rare
•Intermediate uveitis
•Retinal periphlebitis

15. ASSOCIATION BETWEEN OPTIC NEURITIS AND
MULTIPLE SCLEROSIS
 Overall 15 year risk of developing MS following an acute episode of optic
neuritis is about 50%, risk is 70% with presence of one or more lesions on MRI
 Factors predicting lower chances of multiple sclerosis post optic neuritis
 No MRI lesions on the brain
 Male gender
 Absence of viral syndromes preceding the optic neuritis
 Absence of ophthalmoscopic features like disc oedema, haemorrhages and retinal
exudates
 Absence of periocular pain
 Optic neuritis is presenting feature of MS in 30% cases
 Optic neuritis occurs in 50% patients with established MS

16. WORK UP FOR CASES OF OPTIC NEURITIS
Routine haemogram
Chest x-ray
ANA, dsDNA
FTA-ABS and VDRL for syphilis
Serology and culture for Bartonella
Markers of viral infection
Serum electrolytes and fasting blood sugar
Contrast enhanced MRI
CSF tap
Blood culture
Visual evoked potential

17. INVESTIGATIONS FOR MULTIPLE SCLEROSIS
LUMBAR PUNCTURE
oligoclonal bands on protein electrophoresis of cerebrospinal
fluid
MRI
At least 3 lesions and 2 of the following should be present
Lesion abutting lateral ventricle
Lesions with diameter greater than 5 mm
Lesions present in the posterior fossa
VEP
conduction delay and reduction in amplitude

18. SYMPTOMS OF DEMYELINATING OPTIC NEURITIS
Subacute monocular visual impairment
Acute and often painful visual loss over hours to days
Peak visual loss within 2 within 2 weeks
Poor colour vision and contrast sensitivity
Age group 20-30 years
Tiny white or coloured flashes or sparkles (phosphenes)
Discomfort or pain in and around (90%), exacerbated by ocular movements
Frontal headache and tenderness of the globe may also be present

19. SIGNS OF DEMYELINATING OPTIC NEURITIS
Visual acuity is usually 6/18 – 6/60, rarely worse
Other signs of optic nerve dysfunction, specially impaired colour
vision and RAPD
Optic disc is normal in majority of cases (Retrobulbar neuritis),
remainder show papillitis. Almost never neuroretinitis
Temporal disc pallor maybe seen in the fellow eye, indicative of
previous optic neuritis
VER shows prolonged latency with normal or depressed amplitude

20. VISUAL FIELD DEFECTS IN DEMYELINATING
OPTIC NEURITIS
Diffuse depression of sensitivity in the entire
central 30 degrees is most common
Altitudinal/Arcuate defects and focal
central/centrocaecal scotomas are also frequent
Focal defects are frequently accompanied by an
element of superimposed generalised
depression

21. COURSE AND PROGNOSIS OF DEMYELINATING
OPTIC NEURITIS
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
More than 90% patients recover visual acuity to 6/9or better
Subtle parameters like colour vision may remain abnormal
Mild RAPD 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

22. TREATMENT OF DEMYELINATING OPTIC
NEURITIS
Indication of steroid treatment
• When visual acuity within first week of onset is worse than 6/12, treatment may speed up
recovery by 2 – 3 weeks and may delay onset of clinical MS over the short term
• Tis maybe relevant in patients with poor vision in the fellow eye or those with occupational
requirements
• Limited benefits must be balanced against risk of high dose steroid
• Therapy does not influence the eventual visual outcome and the great majority of cases do not
require treatment

23. STEROID REGIMEN FOR DEMYELINATING OPTIC
NEURITIS
Intravenous Methylprednisolone
sodium succinate 1g daily for 3
days
Followed by oral prednisolone
1mg/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

24. IMMUNOMODULATORY TREATMENT
 Reduces the risk of progression to clinical MS in some patients
 Benefit v/s risk has not been clearly defined with the options available
 Agents
 Interferon beta
 Teriflunomide
 Glatiramer
 Decision should be individualised, based on risk profile (brain lesion)
 Most do not commence IMT until a second episode of clinical
demyelination has occurred

25. ONTT: OPTIC NEURITIS TREATMENT TRIAL
IV methyl prednisolone followed by oral prednisolone speeds up recovery and results in better vision at 6
months, it also reduces 2 year recurrence rate
Oral prednisolone alone increase risk of recurrence
Most patients retain excellent vision in 5 years following attack of optic neuritis, even if the optic neuritis
recurred
Dyschromatopsias, defective stereoacuity, RAPD, delayed latencies on VER, Pulfrich and Uthoff
phenomenon and persistent field defects may remain as residual effects after an attack of optic neuritis
IV Dexamethasone has been found to be cheaper and effective alternative to Methylprednisolone with less
side effects

26. CHAMPS STUDY
Study was conducted in patients who have endured first acute demyelinating event
involving optic nerve, brainstem, cerebellum or spinal cord.
Study was undertaken with objective as to whether Interferon beta1a(Avonex) would
benefit these patients that have been previously predicted of high likelihood of having
MS like events in the future
At the end of 3 years the probability of clinically defined multiple sclerosis was 50% in
placebo treated group and interferon beta 1a (Avonex) treated group.

27. CHAMPIONS STUDY
This study compared the outcomes of those who had
been given the drug from the start of the CHAMPS study
versus those who had switched from placebo after 30
months
Immediate treatment group had significantly fewer
relapses and fewer MRI brain lesions than the delayed
treatment group, and that significantly fewer of its
members converted to definite MS

28. OTHER DEMYELINATING DISEASE INVOLVING
THE EYE
Isolated optic neuritis Devic’s disease
Optica)
Schilder’s disease
Encephalitis periaxialis
concentrica
Chronic relapsing optic
neuropathy

29. DEVIC’S DISEASE
Transverse ascending myelopathy in association with acute or subacute loss of visual acuity in one or both
eye (affects one eye first, second eye is affected within hours to days)
More frequently seen in children and young adults
Often preceded by prodrome of sore throat, fever, headache or vaccination
Ophthalmoscopic signs include mild swelling of the disc, venous dilatation and extensive peripapillary
exudation. Optic atrophy sets in as final sequelae
Although few patients develop permanent, severe visual loss, most patients show some visual recovery over
weeks to months
No specific treatment modality has been described in patients with neuromyelitis optica, but IV
corticosteroids and immunoglobulins have shown to reduce severity and accelerate recovery

30. SCHILDER’S DISEASE/ ENCEPHALITIS PERIAXIALIS
DIFFUSA
Seen in children and young adults
Visual loss in 60% patients resulting from damage to post chiasmal visual pathways
Cerebral blindness is an early feature, shows homonymous hemianopia / quadrantic defects
Optic neuritis is not a common feature
Associated change in personality imbalance, incontinence, muscle weakness, speech impairment and varying degrees of
paraparesis
Line of management is similar to that of multiple sclerosis

31. ENCEPHALITIS PERIAXIALIS CONCENTRICA
(CONCENTRIC SCLEROSIS OF BALO)
Visual loss is caused by damage to postgeniculate visual pathways, with homonymous field defects
and cerebral blindness
Optic nerve involvement is not a common feature
Cerebral white matter shows alternating bands of demyelination
Course is progressive, leading to death in few weeks to years if left untreated
Treatment with longterm corticosteroids and immunosuppressants may induce immediate or long
term remission

32. CHRONIC RELAPSING OPTIC NEUROPATHY
 Report of 15 patients over 10 years in which patients presented with
inflammatory bilateral neuropathy
 Associated with painful relapses and remissions
 Blood investigations and CSF was normal
 MRI of brain was normal, but MRI of optic nerve showed high signal
abnormalities
 Symptoms and signs responded well to corticosteroid treatment
 Relapses on steroid withdrawal were common
 Long term immunosuppressants were often necessary

33. PARA INFECTIOUS OPTIC NEURITIS
Optic neuritis maybe associated with
infections like viral infections such as
• Measles
• Mumps
• Chickenpox
• Rubella
• Whooping cough
• Glandular fever
Optic neuritis may also occur
following immunization
Most commonly with influenza, but
can occur with any vaccine
Onset is usually within 1-3 weeks

34. FEATURES OF PARA INFECTIOUS OPTIC NEURITIS
Children are affected much more than adults
Presentation is usually 1-3 weeks after infection
Acute severe visual loss, usually bilaterally
Bilateral papillitis is the rule, occasionally
neuroretinitis/retrobulbar neuritis
Prognosis is very good, with spontaneous
visual recovery, treatment is not needed in
most casesIf the visual loss is severe, or in one eyed
patient, intravenous steroid can be considered

35. INFECTIOUS OPTIC NEURITIS
Sinus related
•Uncommon
•Recurrent attacks of unilateral visual loss
•Severe headache and sphenoethmoidal sinusitis
•Possible mechanism include direct spread, occlusive vasculitis and mucocoele
•Treatment is with antibiotics and if appropriate, surgical drainage
Cat scratch (Benign lymphoreticulosis)
•Caused by Bartonella henselse, inoculated by cat scratch or bite
•Can cause neuroretinitis along with other ocular pathology
Syphilis
•Papillitis or neuroretinitis during primary or secondary stages

36. Lyme’s disease (Borelliosis)
• Spirochaetal infection caused by Borellia burgdorferi, transmitted by tick bite
• It may cause neuroretinitis and occasionally retrobulbar neuritis
• It can present with other neurological manifestation and can mimic MS
Cryptococcal meningitis
• In patients with AIDS
• Maybe associated with optic neuritis which maybe bilateral
Varicella zoster virus
• May cause papillitis
• Associated with hepes zoster ophthalmicus
• Primary optic neurits is rare but can occur in immunocompromised patients

37. NON INFECTIOUS OPTIC NEURITIS
SARCOIDOSIS Optic neuritis affect 1-5% of patients with neurosarcoid
Occasionally can be presenting feature of sarcoidosis but usually develops late during course of established
systemic disease
The optic nerve head may exhibit lumpy appearance suggestive of granulomatous infiltration, with
associated vitritis
Response to steroid therapy is rapid, but vision may decline if treatment is tapered or stopped prematurely
Some patients may require long term low dose therapy
Methotrexate may also be used as an adjunct to steroids or as monotherapy in steroid resistant cases
AUTOIMMUNE Treatment is with steroids and other immunosuppressants

38. NEURORETINITIS
Neuroretinitis refers to combination of papillitis and signs of retinal, usually macular
inflammation
Cat scratch fever is responsible for 60% cases
25% cases are idiopathic
Other notable causes include syphilis, Lyme disease, mumps and leptospirosis

39. FEATURES OF NEURORETINITIS
Painless unilateral visual impairment
Gradually worsening over about 1 week
Visual acuity impaired to a variable degree
Signs of optic nerve dysfunction are usually mild or absent, visual loss is largely due to macular involvement
Papillitis associated with peripapillary and macular oedema
Macular star, it resolves with a return to normal or near normal visual acuity over 6-12 months
Venous engorgement and splinter hemorrhages may be present in severe cases
Fellow eye involvement occasionally develops

41. MANAGEMENT OF NEURORETINITIS
Investigation
• Optical coherence tomography demonstrates
sub and intraretinal fluid to a variable extent
• Fluorescein angiography shows diffuse leakage
from superficial disc vessels
• Blood tests may include serology for Bartonella
and other causes according to clinical suspicion
Treatment
• Specific to the cause and often consists of
antibiotics
• Recurrent idiopathic cases may require
treatment with steroids and/or other
immunosuppressants

42. OPTIC NEURITIS IN CHILDREN
 Unique features differentiating it from optic neuritis in adult patients
 More often of anterior variety
 60% cases have an initial bilateral presentation
 It is seen to occur within 1-2 weeks after known presumed viral infection or
vaccination
 It is frequently associated with development of MS
 More steroid sensitive and steroid dependent