RETROBULBAR

NEURITIS
Classification of optic neuritis

According to ophthalmoscopic appearance

1.Retrobulbar neuritis: in which the optic disc appears normal, at least

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).
2.Papillitis is characterized by hyperaemia and oedema of the optic
disc, which may be associated with peripapillary flame-shaped
haemorrhages. Cells may be seen in the posterior vitreous. Papillitis is
the most common type of optic neuritis in children, but can also affect
adults.
3.Neuroretinitis is characterized by papillitis in association with
inflammation of the retinal nerve fibre layer and a macular star figure.
It is the least common type and is only rarely a manifestation of
demyelination.
According to aetiology

1.Demyelinating. This is by far the most common cause.

2.Parainfectious. following a viral infection or immunization.
3.Infectious. This may be sinus-related, or associated with conditions
such as cat-scratch disease, syphilis, Lyme disease, cryptococcal
meningitis and herpes zoster.
4.Non-infectious causes include sarcoidosis and systemic autoimmune
diseases such as systemic lupus erythematosus, polyarteritis nodosa
and other vasculitides.
Demyelinating optic neuritis
Demyelination is a pathological process in which 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 in plaques. Demyelinating disease
disrupts nervous conduction within the white matter tracts of the brain,
brainstem and spinal cord.
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.
• Multiple sclerosis (MS)
• Devic disease (neuromyelitis optica), a very rare disease that may
occur at any age, characterized by bilateral optic neuritis and the
subsequent development of transverse myelitis (demyelination of the
spinal cord) within days or weeks.
• Schilder disease, a very rare relentlessly 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
Multiple sclerosis
Multiple sclerosis (MS) is an 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.
Systemic features may include:
Spinal cord e.g. weakness, stiffness, sphincter disturbance, sensory loss.
Brainstem e.g. diplopia, nystagmus.
Cerebral e.g. hemiparesis, hemianopia.
Psychological e.g. intellectual decline, depression, euphoria.
Transient features e.g. the Lhermitte sign (electrical sensation on neck
flexion) and the Uhthoff phenomenon (sudden worsening of vision or
other symptoms on exercise or increase in body temperature).
Ophthalmic features
Optic neuritis (usually retrobulbar)
internuclear ophthalmoplegia
nystagmus.
ocular motor nerve palsies
hemianopia.
Investigation
Lumbar puncture shows oligoclonal bands on protein electrophoresis of
cerebrospinal fluid in 90–95%.
MRI almost always shows characteristic white matter lesions.
VEPs are abnormal (conduction delay and a reduction in amplitude) in
up to 100% of patients with clinically definite MS.
Clinical features of demyelinating optic neuritis

Symptoms
Subacute monocular visual impairment.
Usual age range 20–50 years (mean around 30).
Some patients experience tiny white or coloured flashes or sparkles
(phosphenes).
Discomfort or pain in or around the eye is present in over 90% and
typically 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 may also be present.
Signs
Visual acuity (VA) is usually 6/18–6/60, but may rarely be worse.
Other signs of optic nerve dysfunction, particularly 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.
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.
Focal defects are frequently accompanied by an element of
superimposed generalized depression.
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.
Prognosis
More than 90% of patients recover visual acuity to 6/9 or better.
Subtle parameters of visual function, such as 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.
Treatment
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 over the short term.
This may be relevant in the patients with poor vision in the fellow eye
or those with occupational requirements, but the limited benefit must
be balanced against the risks of high-dose steroids. Therapy does not
influence the eventual visual outcome and the great majority of
patients do not require treatment.
Steroid regimen. Intravenous methylprednisolone 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.
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. A decision should be individualized,
based on risk profile – particularly the presence of brain lesions – and
patient preference; most do not commence IMT until a second episode
of clinical demyelination has occurred.