OPTIC NEURITIS

MODERATOR: DR PRANESH KULKARNI

PRESENTER: DR SHRADDHA SUDARSHAN
DEFINITION

OPTIC NEURITIS IS AN INFLAMMATORY INFECTIVE OR

DEMYELINATING PROCESS AFFECTING THE OPTIC NERVE
CLASSIFICATION OF OPTIC NEURITIS

ACCORDING TO OPHTHALMOSCOPIC APPEARANCE

RETROBULBAR NEURITIS
PAPILLITIS
NEURORETINITIS
ACCORDING TO ETIOLOGY

DEMYELINATING
PARAINFECTIOUS
INFECTIOUS
NON INFECTIOUS
 ACCORDING TO OPHTHALMOSCOPIC APPEARANCE

RETROBULBAR PAPILLITIS NEURORETINITIS

NEURITIS

● Most common type in ● Most common type in ● Papillitis +

adults children inflammation of RNFL
● Associated with ● Hyperemia and edema and macular star
MULTIPLE SCLEROSIS of disc a/w figure
● Optic disc appears peripapillary flame ● Least common
normal- as optic nerve shaped haemorrhages ● Rarely a manifestation
head is not involved ● Cells may be seen in of demyelination
the posterior vitreous
Normal optic disc, primary optic atrophy. The condition may be truly described
as ‘ the patient sees nothing and the doctor sees nothing.’
Retrobulbar neuritis
PAPILLITIS :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
Neuroretinitis
Macular star
(Exudates in a star-shaped pattern
radiating from the macula)
 ACCORDING TO ETIOLOGY

PARAINFECTIOUS INFECTIOUS NON

DEMYELINATING
INFECTIOUS
MOST COMMON FOLLOWING VIRAL SINUS RELATED
INFECTION OR CATSCRATCH DX SARCOIDOSIS
CAUSE
IMMUNIZATION LYMES DISEASE SLE
SYPHILIS POLYARTERITIS
CRYPTOCOCCAL NODOSA
MENINGITIS VASCULITIDIS
HERPES ZOSTER
 DEMYELINATING OPTIC NEURITIS

DEMYELINATION: PATHOLOGICAL PROCESS IN WHICH NORMALLY

MYELINATED NERVE FIBRES LOSE THEIR INSULATING MYELIN LAYER.

Myelin phagocytosed by microglia and macrophages

Astrocytes lay down fibrous tissue in plaques

● Disrupts nervous conduction within the white matter tracts of the brain,
brainstem and spinal cord
Demyelinating conditions involving the visual system
1. ISOLATED OPTIC NEURITIS
With no evidence of generalized demyelination- although it may develop subsequently

2. MULTIPLE SCLEROSIS
Most common demyelinating disease

3. DEVIC DISEASE (NEUROMYELITIS OPTICA)

● Rare autoimmune disease
● Antibodies against------------ Aquaporin 4 protein in the cell membrane of astrocytes
● Can occur at any age
● Characterised by ----- BILATERAL OPTIC NEURITIS

Followed by

TRANSVERSE MYELITIS
4. SCHILDER DISEASE
● Rare
● Progressive
● Autoimmune
● Onset <10 years of age, death in 1-2 years
● BILATERAL OPTIC NEURITIS WITHOUT SUBSEQUENT IMPROVEMENT
MULTIPLE SCLEROSIS

IDIOPATHIC DEMYELINATING DISEASE INVOLVING THE CENTRAL

NERVOUS SYSTEM WHITE MATTER
EPIDEMIOLOGY

● More common in whites

● 2-3 times more common in women

● Uncommon in <10 years of age

● Most common in the age of 25-40 years

GENETIC FACTORS

● Risk of MS is significantly increased in 1st degree relatives

● Identical twins------- 10 fold increased risk

● HLA association -HLA DRB1

HLA DR15

HLA DQ6
COURSE AND PROGNOSIS

● 90% - relapsing/ remitting course - episodes separated by months

or years
● With in 10 years 50%- SECONDARY PROGRESSIVE DISEASE
● 10-20% - PRIMARY PROGRESSIVE DISEASE
● 5-10%- relatively benign course without serious disability or
reduction of lifespan.
SYSTEMIC FEATURES

Spinal cord Weakness

Stiffness
Sphincter disturbance
Sensory loss.

Brainstem Diplopia
Nystagmus
Dysarthria
Dysphagia
○
Cerebral Hemiparesis
Hemianopia
Dysphasia

Psychological Intellectual decline

Depression
Euphoria.
TRANSIENT FEATURES

LHERMITTE SIGN : ELECTRICAL SENSATION ON FLEXION OF NECK

UHTHOFF PHENOMENON : SUDDEN WORSENING OF VISION OR

OTHER SYMPTOMS ON EXERCISE OR INCREASE IN TEMPERATURE.
OPHTHALMOLOGICAL FEATURES

○ Common. Optic neuritis (usually retrobulbar), internuclear

ophthalmoplegia, nystagmus

○ Uncommon. Skew deviation, ocular motor nerve palsies,

hemianopia.

○ Rare. Intermediate uveitis and retinal periphlebitis.

OCULAR MOTILITY DISTURBANCES IN MULTIPLE SCLEROSIS

● Diplopia is a frequent MS symptom.

● Motility abnormalities resulting from MS are typically localized to the
supranuclear, nuclear, and fascicular portions of the ocular motor
system.
● Internuclear ophthalmoplegia, especially when bilateral, may present
with exotropia in primary position and bilaterally impaired adduction
(ie, wall-eyed, bilateral internuclear ophthalmoplegia, or WEBINO)
● It is highly suggestive of MS in someone under age 50 years.
● Complete or partial paralysis of horizontal or vertical gaze or a skew
deviation (vertical misalignment not attributable to single CN or
muscle dysfunction).
● CN VI involvement is most commonly reported, but CN III or CN IV
paresis has also been described.
● Nystagmus is frequently present in MS. It may be horizontal, rotary, or
vertical, and both pendular and jerk types may occur
ASSOCIATION BETWEEN OPTIC NEURITIS AND MS

Overall 15 year risk of developing MS following attack of optic neuritis -----

50%

MRI lesions-- strong predictive factor

With no MRI Lesion- 25%

One or more MRI lesions - 70%

Optic neuritis is the presenting feature of MS in upto 30%

Optic neuritis occurs at some point in 50% of patients with MS

CLINICAL FEATURES
SYMPTOMS

● Subacute monocular visual impairment

● 20-50 years of age
● Tiny white coloured flashes- phosphenes
● Discomfort or pain around eye exacerbated on ocular movement
- precede or accompany visual loss
● Frontal headache and tenderness of globe
SIGNS

● Visual acuity 6/18-6/60 or rarely worse

● Signs of optic nerve dysfunction
Relative afferent pupillary defect
Dyschromatopsia - RED-GREEN
Diminished light brightness sensitivity
Diminished contrast sensitivity
● RETROBULBAR NEURITIS- NORMAL OPTIC DISC
● Others PAPILLITIS - hyperemia, edema, peripapillary flame shaped
haemorrhages
● Temporal disc pallor in fellow eye - indicative of previous attack
● Visual field defects - diffuse depression in central 30 degree
COURSE

VISIONS - WORSENS - several days to 3 weeks

IMPROVES - INITIAL - rapid recovery

6-12 months- Gradually

PROGNOSIS

● >90% recover to vision of 6/9 or better

● colour vision may remain abnormal
● Mild RAPD may persist
● Temporal disc pallor or marked optic atrophy
● 10% - Chronic optic neuritis - slowly progressive/ stepwise vision
loss
INVESTIGATION

● Lumbar puncture shows oligoclonal bands on protein electrophoresis of

cerebrospinal fluid in 90–95%.

● MRI almost always shows characteristic white matter lesions (PLAQUES)

● VEPs are abnormal (conduction delay and a reduction in amplitude) in up

to 100% of patients with clinically definite MS.

MRI

● Although MS is classically considered a demyelinating disease, axonal

damage does occur early and is an integral part of the disease process.
● This axonal loss manifests as “black holes” on T1-weighted MRI
sequences
● Myelin destruction is associated with local perivascular mononuclear cell
infiltration, myelin removal by macrophages, and astrocytic proliferation
with production of glial fibrils.
● The term multiple sclerosis stems from the presence of these numerous
gliotic (sclerotic) plaque lesions. Plaques are often situated in the white
matter at the ventricular margins, the optic nerves and chiasm, the
corpus callosum, and the spinal cord and throughout the brainstem and
cerebellar peduncles.
A gadolinium-enhanced
fat-saturated T1-
weighted scan will show
a bright enhancement
of the inflamed optic
nerve (upper left red
arrow). Additionally,
bright spots
characteristic of multiple
sclerosis may also be
seen in the brain (lower
right red arrow).
MANAGEMENT

80-85%- RELAPSING COURSE

UNTREATED- Neurological diability over time

Disease modifying therapies- prevent relapses and reduce

neurological diability
INDICATION OF STEROID TREATMENT IN OPTIC NEURITIS

● VA in 1st week of onset - 6/12 or less

● Treatment may speed up recovery by 2-3 weeks and delay the onset of

clinical MS

● Therapy does not influence the final visual outcome

STEROID REGIMEN

IV Methyl prednisolone 1g daily x 3 days

Followed by

Oral 1mg/kg methyl prednisolone for 11 days

Tapered over 3 days

DISEASE MODIFYING THERAPIES FOR MS

INTERFERON BETA : Given in early stages , intramuscular weekly regimen.

Causes Transient flu like symptoms

MONOCLONAL ANTIBODIES : causes oppourtunistic infections, derranged

liver function

a) NATALIZUMAB : Highly active relapsing or remitting MS in patients not

responding to interferon beta
b) OCRELIZUMAB : Highly active disease oe primary progressive disease
Causes progressive multifocal leuko encephalopathy

c) ALEMTUZUMAB : Cost effective

APPROACH TO TREATMENT

● MULTIDICIPLINARY APPROACH

● 1ST Attack ----- INTERFERON BETA OR GLATIRAMER ACETATE

● Relapsing MS --------a) Inactive disease -- monitoring

● b) Active and highly active disease :

disease modifying therapy

● Progressive MS ---------- OCRELIZUMAB

PARAINFECTIOUS OPTIC NEURITIS
● ASSOCIATED WITH VIRAL INFECTION : measles, rubella, mumps,
whooping cough , glandular fever
● FOLLOWING IMMUNIZATION
● Children > Adults
● 1-3 weeks after viral infection
● ACUTE SEVERE BILATERAL VISUAL LOSS
● BILATERAL PAPILLITIS
● Occasionally neuroretinitis or normal disc
● Spontaneous recovery- treatment not required in majority
● SEVERE AND BILATERAL IV steroids under antiviral cover
● INVOLVES ONLY SEEING EYE
INFECTIOUS OPTIC NEURITIS

1. SINUS RELATED

Recurrent attacks of unilateral vision loss associated with severe

headache and sphenoethmoidal sinusitis

Mechanism : direct spread, occlusive vasculitis, mucocoele

Rx : systemic antibiotics, surgical drainage

2. CAT SCRATCH FEVER

Benign lymphoreticulosis
Organism : BARTONELLA HENSELAE
Neuroretinitis

3. SYPHILIS

Acute papilltis or neuroretinitis in primary or secondary stages

4. LYMES DISEASE - BORRELIOSIS

BORRELIA BURGDORFERI, Tick bite

Neuroretinitis
Retrobulbar neuritis with other neurological features- mimics MS

5. CRYPTOCOCCAL MENINGITIS a/w AIDS

Acute b/l optic neuritis

NON INFECTIOUS
1. SARCOIDOSIS
● 1-5%
● Optic nerve head - lumpy appearance - granulomatous infiltration
● Associated vitritis ‘
● Steroid response- rapid
● Methotrexate: Adjunct to steroids / monotherapy
2. AUTOIMMUNE

● Retrobulbar neuritis/ AION

● Some patients- slowly progressive vision loss -- COMPRESSION
● Rx Systemic steroids , immunosuppressants
NEURORETINITIS

Combination of optic neuritis and signs of retinal, usually macular

inflammation

60% - CAT SCRATCH FEVER

25% - IDIOPATHIC - Leber idiopathic stellate neuroretinitis

Others : Syphilis, Lymes disease, mumps, leptospirosis

 DIAGNOSIS
SYMPTOMS
● Painless unilateral visual impairment usually gradually worsening over a week
SIGNS
● Varying levels of visual loss : severe due to macular involvement
● Optic nerve dysfunction : mild / absent
● Papillitis a/w peripapillary and macular edema
● MACULAR STAR ----Disc edema spreads in the outer plexiform layer along
papillomacular bundle

As edema resolves

Lipid precipitates in radial pattern in henles layer

 ● Venous engorgement and splinter haemorrhages
● Fellow eye involvement : occasionally

INVESTIGATION
1. OCT - Subretinal and intraretinal fluid
2. FA - diffuse leakage from superficial disc vessels
3. Serology : Bartonella

TREATMENT
Specific to cause --- antibiotics
Recurrent idiopathic : steroids/ immunosuppressants
THANK YOU