4. • Retrobulbar neuritis
Optic nerve is affected more posteriorly with no disc swelling.
• Papillitis
Affect optic nerve head (hyperaemia & oedema of optic disc) +
peri-papillary flame-shaped haemorrhages
• Neuroretinitis
Characterized by papillitis with inflammation of the retinal nerve
fiber layer. A macular star figure composed of hard exudates
may present. Least common and most frequently associated with
viral infections and cat-scratch fever, Syphilis and Lyme disease.
Usually self-limiting which resolves 6-12 months.
Neuroretinitis is rarely a manifestation of demyelination.
5. Ophthalmoscopical Classification
Retrobulbar neuritis Papillitis Neuroretinitis
Optic nerve affected
behind the eyeball
Optic nerve head affected Concomitant swelling of
optic nerve head
(papillitis) and macula
(inflammation of retinal
nerve fibre layer)
The optic disc appears
normal
The condition may be truly
described as ‘ the patient
sees nothing and the
doctor sees nothing.’
Hyperaemia and oedema
of the optic disc
May be a/w peripapillary
flame-shaped
haemorrhages
Macular star
(exudates that form
around the macula give
the appearance of the
star)
Most frequent type in
adults
Frequently a/w multiple
sclerosis (MS)
Most common type in
children
Least common type
Rarely a manifestation of
demyelination
Due to viral infection,cat-
6. Normal optic disc, primary optic atrophy. The condition may be truly described
as ‘ the patient sees nothing and the doctor sees nothing.’
Retrobulbar neuritis
7. 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
8. Macular star
(Exudates in a star-shaped
pattern radiating from the
macula)
Neuroretinitis
Diffuse Unilateral Subacute
Neuroretinitis - Nematode
9. Aetiological Classification (causes)
1. Demyelinating
Pathological process: the normally myelinated nerve fibres lose
their insulating myelin layer (The myelin is phagocytosed by
microglia and macrophages,subsequent to which astrocytes lay
down fibrous tissue (plaque) -> distrupts nerve conduction w/in
white matter tracts( brain,brainstem,spinal cord)
Ages: 20-50 years old, F:M = 3:2
Most common cause (eg. Multiple sclerosis)
Optic disc is normal (retrobulbar neuritis)
10. DEMYLINATING DISEASE CAUSES OCULAR PROBLEMS:
1. Isolated optic neuritis
2. Multiple sclerosis
3. Devic disease (neuromyelitis optica) : bilateral optic neuritis -> transverse
myelitis
4. Schilder disease : bilateral; progressive generalised disease (onset prior to
10y/o death within 1-2yrs)
a) visual p/way-> lesion commonly involve optic nerves and cause optic
neuritis
b) Brainstem-> lesions result in gaze palsies, ocular motor cranial nerve
palsies, trigeminal , facial nerve palsies and nystagmus
12. 2. Parainfectious
Common in children
Occurs d/t exposure of antigens of certain infectious agents
eg. measles, mumps, chickenpox, rubella,whooping
cough,glandular fever
Following viral infection, immunization
Usually 1-3 weeks following viral infection with acute severe
visual loss
Usually bilateral papillitis occur
(occasionally: neuroretinitis or the discs may be normal)
Spontaneous recovery within few weeks
May be a/w other neurological features eg. headache,
seizures or ataxia (meningoencephalitis)
13. 3. Infectious
Sinus-related optic neuritis
a/w Cat-scratch fever, syphilis, Lyme disease, cryptococcal
meningitis in patients with AIDS and herpes zoster.
4. Non-infectious
Sarcoidosis- optic nerve head exhibit lumpy appearance,
inflammatory reaction in vitreous
Systemic autoimmune diseases –retrobulbar neuritis
eg. SLE, polyarteritis nodosa, other vasculitides (vasculitis)
15. Clinical Presentations
• Usu. women aged btw 20-40 (mean around 30 years)
• Acute unilateral loss of vision, may progress over a few days,
then slowly improve
• Pain exacerbated on eye movement (retrobulbar neuritis) b/c rectus
contraction pulls on the optic nerve sheath; painless (papillitis)
• Dyschromatopsia (change in color perception) in affected eye
• Flashes of light/ phosphenes (tiny white or coloured flashes or
sparkles) induced by eye movement or loud sounds
• Relapsing( most common)/ Progressive (10%)
16. • A preceding H/O viral illness in some cases
• Vision loss exacerbated by heat or exercise (Uhthoff
phenomenon).
• 40-70% of patients will have other neurological symptoms
(such as parasthesia, weakness, incontinence, Lhermitte sign)
to suggest a diagnosis of demyelination (multiple sclerosis)
18. Diagnosis
• The diagnosis of optic neuritis is clinical, based on the history
and physical findings.
• Investigations
▫ Brain MRI
▫ Serological tests: ESR, syphilis, antinuclear antibody
(ANA), antineutrophil cytoplasmic antibodies (ANCA)
▫ CSF analysis (lumbar puncture) : Presence of oligoclonal
band, elevated IgG level suggest MS.
19. Brain MRI
• MRI of the brain and orbits with gadolinium contrast has
become the cornerstone of the evaluation in patients with optic
neuritis
▫ To confirm the clinical diagnosis (look for additional
plaques of demyelination)
▫ Offers very strong prognostic information about the risk of
future demyelinating events and MS
▫ For treatment decision
20. 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).
White matter lesions in
brain MRI denote a higher
risk of developing MS
22. Management: Demyelinating Optic
Neuritis
• In most cases (mild vision loss, no significant visual field
loss, no enhancing lesions on brain MRI) - no treatment
• Vision slowly recovers over several weeks even w/o treatment
(spontaneously)
• Treatment Indicated
▫ Visual acuity worsen than 6/12
▫ Treatment hasten the recovery process, but no effect on
long-term visual outcome
23. Regimen
▫ IV corticosteroid (eg. methylprednisolone)
1g daily for 3 days followed by oral steroid as prednisone
(1mg/kg/day) for 11 days and then tapered for 3 days
▫ IM interferon beta-1a
Reducing the development of clinical MS over the
following 3 years in patients at high risk (based on the
presence of subclinical brain MRI lesion)
NB. Oral prednisolone alone is contraindicated b/c it does not affect
the speed of recovery and is a/w a significantly higher recurrence rate.
Corticosteroids S/E
• Mood changes
• Facial flushing
• Weight gain
• Dyspepsia
Notes de l'éditeur
The papilla (the blind spot) is the spot there the optic nerve leaves the retina
A swollen disc with blurring and hyperaemia of disc margin
Usually bilateral papillitis (occasionally: neuroretinitis or the discs may be normal)
Parainfectious- both eyes acute severe visual loss, bilateral papillitis, spontaneous recovery.
Sinus – recurrent, unilateral, sinusitis. Antibiotics; surgical drainage.
Cat scratch- Bartonella henselae/quintana. Neuroretinitis etc
Syphilis- acute papillitis or neuroretinitis. Primary or secondary stage.
Autoimmune- retrobulbar neuritis
Sarcoidosis - A systemic granulomatous disease of unknown cause, especially involving the lungs with resulting interstitial fibrosis, but also involving lymph nodes, skin, liver, spleen, eyes, phalangeal bones, and parotid glands; granulomas are composed of epithelioid and multinucleated giant cells with little or no necrosis
sarcoidosis
Phosphenes – sensation of light produced by mechanical or electrical stimulation for the peripheral or central optic pathway of nervous system
Lhermitte sign= electric sensation on neck flexion
Interferon beta balances the expression of pro- and anti-inflammatory agents in the brain, and reduces the number of inflammatory cells that cross the blood brain barrier.[4] Overall, therapy with interferon beta leads to a reduction of neuron inflammation.[4] Moreover, it is also thought to increase the production of nerve growth factor and consequently improve neuronal survival.[4]
IM interferon β-1a - immunosuppresant medication may help to prevent or delay its onset. These medications are given as subcutaneous injections. They include interferon β-1a (Avonex, Rebif) and interferon β-1b (Betaseron). Also associated with a wide range of side effects, such as flu-like symptoms, depression, liver damage, reduced immunity and tissue damage at the site of injection.