Symposium

Management of optic neuritis

Vimla Menon, Rohit Saxena, Ruby Misra, Swati Phuljhele

Optic neuritis is an inflammatory condition of the optic nerve characterized by a sudden onset of unilateral Access this article online
visual loss, usually affecting young females. Demyelination associated with multiple sclerosis (MS) is Website:
the most common cause in regions where MS is prevalent; while in other places, there are a substantial www.ijo.in
proportion of cases where infective or autoimmune causes are seen. Optic Neuritis Treatment Trial (ONTT) DOI:
was the first major study that provided information on the natural history, role of steroids in treatment 10.4103/0301-4738.77020
and risk of development of MS. Subsequently, numerous clinical trials have evaluated different modalities PMID:
of management of optic neuritis and MS. The Controlled High-Risk Subjects Avonex Multiple Sclerosis *****

Prevention Study (CHAMPS); the Prevention of Relapses and Disability by Interferon β-1a Subcutaneously Quick Response Code:
in Multiple Sclerosis (PRISMS) Trial; and, most recently, the Betaferon in Newly Emerging Multiple Sclerosis
for Initial Treatment (BENEFIT) Study have provided large amount of information on the natural history
of optic neuritis and management options available. However, due to the low prevalence of MS reported in
Asian studies, high cost of therapy and indefinite time period of treatment, it may not be cost effective to
start interferon therapy in most cases.

Key words: Disc edema, multiple sclerosis, optic neuritis, papillitis

Indian J Ophthalmol: 2011;59:117-122

Optic neuritis is an inflammatory condition affecting the optic of patients have disc edema (papillitis), particularly in this part
nerve, usually affecting young adults, especially females, of the world.[5,6] A variety of visual field defects ranging from
between 18 and 45 years of age. Although it has been reported commonly seen diffuse depression and centrocecal scotoma
from almost all parts of the world, regions with the highest to rarely seen quadrantic defects and altitudinal defects are
incidence include northern Europe, southern Australia present in patients with optic neuritis. Subnormal color vision
and middle part of North America.[1,2] Most of the cases are or contrast sensitivity is noted in the affected eye and at times
idiopathic in nature; however, it could be associated with in the fellow eye and is suggestive of subclinical involvement
demyelinating lesions, of which multiple sclerosis (MS) is the of the fellow eye. Recovery of vision usually begins within the
most common cause. Other less common etiologies include first month. In the ONTT, 79% of the participants had started
infectious and para-infectious causes, inflammatory and para to improve by 3 weeks; and 93%, by 5 weeks.[7] Improvement
vaccination immunological responses [Fig. 1]. may continue after this, especially in patients with poor vision,
up to a period of 12 months. Though good functional visual
Clinical Features recovery is seen in most patients, around 5% to 10% of patients
Typical optic neuritis in adults usually presents as acute fail to recover fully. Atypical features include absence of pain,
monocular loss of vision progressing over several hours to which may be seen in only 8% of the patients with typical optic
days, often associated with ocular pain that worsens on eye neuritis; marked swelling of the nerve with retinal exudates
movement. Presenting vision can range from 20/20 with and peripapillary hemorrhages; severe visual loss to no light
mild visual defects to no light perception. The Optic Neuritis perception; progression of visual loss or pain for more than
Treatment Trial (ONTT) reported a vision of 20/20 or better 2 weeks; and lack of recovery after 3 weeks.[8,9] Bilateral optic
at presentation in 10.5% of the patients of optic neuritis, and neuritis may occur, either simultaneously or sequentially,[10]
no perception of light in 3.1% of the cases.[3] Although vision which would also be an unusual feature in typical optic
loss is usually monocular, involvement of both eyes can occur, neuritis.[11] Patients with atypical optic neuritis are at lower
usually in children.[4] A relative afferent pupillary defect is risk of developing MS and should be extensively evaluated
present in almost all unilateral cases, but absence of the defect for other causes of optic neuropathy.
suggests a pre-existing or coincident optic neuropathy in the
fellow eye. Although retrobulbar optic neuritis (normal disc Asian MS has traditionally been thought of as a distinct
appearance) is more common,[3] quite a substantial proportion entity different from that seen in the west, characterized by
high incidence of visual involvement at onset, prevalence of
recurrent acute transverse myelitis [considered as an optico-
Dr. R. P. Centre for Ophthalmic Sciences, All India Institute of Medical spinal MS (OSMS)] and a high degree of overlap with neuron
Sciences, New Delhi, India myelitis optica (NMO).[12,13] In a prospective study done in
Correspondence to: Dr. Rohit Saxena, Squint and Neuro- Chandigarh by Jain et al.,[14] 42 patients of optic neuritis were
Ophthalmology Section, Dr. R. P. Centre for Ophthalmic Sciences, followed up for a period up to 6 months. In 20 (29.4%) eyes, the
All India Institute of Medical Sciences, New Delhi - 110 029, India. appearance of the optic disc was normal, indicating retrobulbar
E-mail: rohitsaxena80@yahoo.com neuritis; whereas 38 (56%) eyes showed blurring of the disc
Manuscript received: 13.03.10; Revision accepted: 10.11.10 margins with or without edema of the disc, suggestive of
118 Indian Journal of Ophthalmology Vol. 59 No. 2

Optic Neuritis

Demyelinating Infectious and Others

para-infectious causes
- Multiple sclerosis - Post-vaccination
- Neuromyelitis optica - Viral (adenovirus, coxsackievirus, measles, - Inflammation (sarcoidosis;
mumps, rubella, varicella-zoster) vasculitidis, e.g.,SLE;
- Shilder’s disease
polyarteritis nodosa)
- Encephalitis periaxialis - Bacterial (syphilis, tuberculosis, Lyme disease,
concentrica cat-scratch disease, β-hemolytic streptococcal - Miscellaneous (Gullain-Barre
infection, brucellosis, typhoid fever, meningococcal syndrome, birdshot
infection, Whipple’s disease) retinochoroidopathy,
intraocular nematode infection,
toxoplasmosis, toxocariasis)

Figure 1: Causes of optic neuritis

papillitis or anterior retrobulbar neuritis. Sixty-two percent

had bilateral involvement of the discs. Only 3 (7.1%) patients An attack of optic neuritis
had some neurological deficit, and a provisional diagnosis of
MS was made. Similar clinical profile has been reported in Baseline MRI
the other studies from Asian region.[5,6,15,16] Higher incidence
of involvement of optic nerve head, less incidence of pain, No demyelinating lesion Demyelinating
less brain magnetic resonance imaging (MRI) abnormalities,
severe visual loss and poorer visual outcome are common
findings among the population from Asia. The incidence of MS Regular - Recurrent attack of optic neuritis
follow-up
reported in these studies was much lower than that reported - Appearance of demyelinating
lesions on MRI
in the western literature. In a retrospective study of Taiwanese
patients with acute optic neuritis,[17] it was found that the 5-year - Appearance of neurological Refer to neurologist
symptoms
cumulative probability of conversion to MS was 14.28%.
Discuss interferon
Epidemiology therapy

Region-wise estimates of the incidence of demyelinating optic Figure 2: Management protocol for patients of optic neuritis
neuritis are unavailable. Optic neuritis is reported to have an
incidence of 1-5 cases per 100,000/year; higher the latitude,
higher was found to be the incidence of optic neuritis.[18-20] patient of appropriate age with the clinical features mentioned
While the estimated prevalence of MS in the United States and above. Although no investigations are necessary for confirming
England is 46 per 100,000 and 93 per 100,000, respectively,[2] the diagnosis, investigations are needed to assess the risk of
the prevalence in eastern countries varies from 0.77 to 1.8
developing MS and to rule out other disorders. In atypical cases,
per 100,000,[21,22] suggesting that the profile of optic neuritis
e.g., those of patients older than 50 years, or cases with a history
patients is different in the eastern and western parts of the
suggestive of a secondary etiology, an additional workup to
world. The optico-spinal variant of MS, which is characterized
identify the etiology should be considered. Since tuberculosis
by involvement of only the optic nerves and spinal cord with
is common in our country, a baseline chest x-ray is done in all
no brain lesions, is more prevalent in this part of the world and
patients prior to starting treatment with steroids. Serology for
can be easily misdiagnosed as NMO, which again is widespread
syphilis, bartonella and toxoplasmosis; Mantoux test; blood
in this region.[13,23] Pandit et al.[24] found 47% of their MS cases
culture; cerebrospinal fluid (CSF) examination; and blood
to have clinical attacks confined to the optic nerve and spinal
tests should be done to rule out infective and inflammatory
cord. There are no large-scale epidemiological studies from
cases. The choice of investigations depends upon the clinical
India on the incidence and prevalence of MS. Indian studies
picture, for example, MRI of the optic nerves in suspected cases
have shown that MS constitutes 0.32% to 1.58% of neurology
of compressive optic neuropathy; computerized tomography
admissions in hospitals,[25-28] and a prevalence of approximately
(CT) of the orbits in bony orbital lesions; orbital ultrasound for
1.33/100,000 was reported by Singhal et al. in the mid-eighties
posterior scleritis; optical coherence tomography, fluorescein
from the west coast of India.[29] However, the incidence of NMO
has been reported to be 9.5% in a recent study in India.[24] angiography, and electroretinography for retinal diseases.
Specialized tests, e.g., toxin screens and serum B12 for toxic
Diagnosis / Ancillary Testing and optic neuropathy; markers for autoimmune diseases; antibody
to aquaporin-4 and MRI spine in NMO; genetic analysis for
Management [Fig. 2] mitochondrial mutation in cases of Leber’s hereditary optic
The diagnosis of optic neuritis is a clinical one, made in a neuropathy, are required in suspected conditions.
 Menon, et al.: Management of optic neuritis
March - April 2011 119

Though MacDonald’s criteria recommend MRI of brain and group, 94% of the intravenous steroid group and 91% of the
spinal cord to diagnose MS, the diagnosis of MS in India has oral steroid group at 1 year.[36] After15 years, 72% of the eyes
relied heavily on clinical criteria owing to lack of facilities and/ affected with optic neuritis had visual acuity of ≥ 20/20, and
or financial constraints in the general population. Radiological 66% of the patients had ≥ 20/20 acuity in both eyes.[37]
and other paraclinical tests are not afforded by all patients. In
The interesting finding was that patients with oral regimen
addition to the above facts, the clinical presentation of MS in
had a twofold greater rate of recurrent optic neuritis. Of
the form of OSMS, which is similar to that of NMO, confuses
the patients treated with oral steroids, 30% experienced a
the scenario.
recurrence, either in the fellow eye or in the affected eye in
the first 2 years of follow-up, compared to only 13% in the
Recommendations for MRI intravenous steroid group and 16% in the placebo group.[38] In
In populations with a high incidence of MS, all patients the first 2 years of follow-up, intravenous steroids reduced the
with acute monosymptomatic demyelinating optic neuritis risk of developing MS compared to the other 2 groups. At 2
should undergo gadolinium-enhanced MRI of the brain years, 8% of the patients treated with intravenous steroids had
and spine to determine if they are at a high risk for the clinically definite MS, whereas 18% of the placebo group and
subsequent development of clinically definite multiple sclerosis 16% of the oral steroid group developed MS. Among the patients
(CDMS).[30] MRI imaging becomes essential in recurrent optic who had not developed multiple sclerosis at 5 years after study
neuritis, in patients keen to know about long-term prognosis, enrollment, the probability of being diagnosed as having MS
in patients with a history or evidence of other neurological between 5 and 10 years was 7% in 142 patients with no lesions
involvement, in atypical cases[30] and in acute optic neuritis in on MRI and 27% in the 89 patients with 1 or more lesions.
children.[31] The presence of demyelinating lesions on brain MRI
at the time of clinical presentation is the strongest predictor However, it should be noted that the baseline MRI scans in
for developing CDMS. MRI showing ≥2 white matter lesions this study were performed before availability of gadolinium
(≥3 mm in diameter, at least 1 lesion periventricular or ovoid) enhancement and advanced inversion recovery imaging. The
indicates a high risk for CDMS.[32,33] overall high rate of 32% for conversion to MS between years
10 and 15 suggests that patients should be followed up for a
On follow-up of the patients of the ONTT group after a long period of time. Among the patients who had no lesions
period of 15 years, it was seen that the cumulative probability on MRI, it was found that male gender and optic disc swelling
for developing MS after the first episode of optic neuritis was were associated with a lower risk of MS, as was the presence
50%, the risk being highest in the first 5 years. If the MRI was of the following atypical features: no light perception vision;
negative at baseline, the overall risk of MS was 25%. Presence absence of pain; and ophthalmoscopic findings of severe optic
of a single lesion doubled the 15-year risk to 50%, while three disc edema, peripapillary hemorrhages, or retinal exudates.
or more lesions caused a threefold increase in the risk, viz.,
Based on the findings of the ONTT, the study group made
to 78%.[34]
several recommendations:[39]
• Chest x-ray, blood tests and lumbar puncture are not
Role of steroids
indicated for typical cases of optic neuritis
The most reliable information concerning long-term outcome • Treatment with oral prednisolone in conventional doses
of optic neuritis comes from the ONTT, [35] which was a alone, is contraindicated
multi-centered randomized trial involving 454 patients from • Consider treatment with intravenous steroids when 3 or
1988 to 2006, mainly undertaken to evaluate the efficacy more signal abnormalities are present on MRI to reduce
of corticosteroid treatment for acute optic neuritis and to 2-year risk of developing MS, or in patients requiring
investigate the relationship between optic neuritis and MS. The expedited recovery of vision (i.e., monocular patients,
cases included in the study had a clinical picture consistent with employment demands, bilateral involvement and patients
unilateral optic neuritis, visual loss lasting 8 days or less with desiring intervention).
no previous episodes of optic neuritis in the affected eye, no
previous steroid treatment for MS or optic neuritis and no other Studies with higher doses of oral corticosteroids
systemic disease except MS as a cause of optic neuritis. The (methylprednisolone) vs. placebo have been conducted, but
patients were randomized into 3 different treatment groups: no statistically significant benefit could be seen on a long-term
oral placebo for 14 days; intravenous methylprednisolone basis or in the relapse rate.[40] An observational study conducted
(250 mg, 6 hourly) for 3 days, followed by oral prednisolone at our center to evaluate the efficacy and safety profile of
(1 mg\kg\d) for 11 days and a 3-day tapering of prednisolone intravenous dexamethasone showed that the intravenous
thereafter; oral prednisolone (1 mg\kg\d) for 14 days, followed pulse dexamethasone led to rapid recovery of vision in acute
by a 3-day tapering. optic neuritis, without any serious side effects.[41] Later on, a
case-control study[42] was done to compare the efficacies of
The results showed that the intravenous steroid group intravenous mega-dose methylprednisolone and intravenous
recovered vision faster compared to those treated with oral dexamethasone in terms of visual recovery, as well as to evaluate
administration or placebo, but the difference in the rate of their side effects. Intravenous dexamethasone was found to be
recovery subsided within 1 month. Intravenous steroids as effective as mega-dose intravenous methylprednisolone
afforded a short-term but statistically significant benefit in therapy recommended by the ONTT study, with the added
contrast sensitivity, color vision and visual field but not in advantage of being easier to administer and less costly (costing
visual acuity at 6 months. At the 1-year follow-up, there was no one sixth of injection methylprednisolone). At our center, we
statistically significant difference in visual function among the routinely use 200 mg intravenous dexamethasone for 3 days,
groups. Visual acuity was 20/ 40 or better in 95% of the placebo followed by oral prednisolone.
120 Indian Journal of Ophthalmology Vol. 59 No. 2

A recent study [43] regarding the use of intravenous Similarly, intravenous immunoglobulin treatment tried
methylprednisolone given at regular intervals has recently has been found to reduce the rate of conversion to
been completed. A single monthly infusion of 500 mg MS and decrease accumulation of several MRI abnormalities
methylprednisolone with a 3-day oral tapering can reduce over a 1-year period after CIS (clinically isolated syndrome).[50]
inflammatory disease activity in patients with relapsing-
Our current approach regarding initiation of
remitting MS, without clinically relevant side effects. There was
immunomodulator therapy is based on the following facts:
a reduction in the number of gadolinium-enhanced lesions and
(1) low prevalence of MS in Asian countries; (2) expense of
T2 lesion load over a 6-month follow-up period.
Rs. 6,000 per injection for Avonex®, which would amount to
Rs. 312,000 per year; (3) no end point to the duration of the
Role of immunomodulators
treatment; (4) disease-modifying drugs are only partially
At present the immunomodulating drugs that have been effective in the short-term; besides, prevention of disability
shown to reduce the development and severity of CDMS in the long-term is unproven; moreover, with the prolonged
include interferon β-1a (Avonex®, Rebif®), interferon β-1b treatment, it is hard to distinguish whether a favorable outcome
(Betaseron®) and Glatiramer acetate.[44] The various mechanisms reflects a favorable natural history or successful treatment in
proposed include reduced antigen presentation, inhibition of an individual patient, especially if treatment is started without
pro-inhibitory cytokines and autoreactive T cells, induction a period of observation; (5) MS often has a favorable natural
of immunosuppressive cytokines and decreased migration history.
of cells in the central nervous system (CNS). Another
antineoplastic, immunomodulatory agent, viz., Mitoxantrone Patients who have a normal MRI of the brain are kept under
(Novantrone®),[45] a synthetic anthracenedione derivative, when regular follow-up. These patients should undergo surveillance
given intravenously has been shown to improve neurological MRI (at least annually at first) to look for the development of
disability and result in delayed progression of MS in patients white matter lesions, as the ONTT showed even this cohort has
with worsening relapsing-remitting (RR) or secondary- a 22% risk of developing MS. Patients with 1 or more lesions are
progressive (SP) disease. counseled regarding the pros and cons of immunomodulator
therapy and kept under close follow-up with serial monitoring
CHAMPS (Controlled High-Risk Subjects Avonex Multiple of MRI scans [Fig. 1].
Sclerosis Prevention Study)[46] was a randomized, double-
blind trial involving 383 patients with an initial, acute We avoid treating those with a greater chance of benign
monosymptomatic demyelinating event (unilateral optic course (i.e., those with a low expanded disability status scale
neuritis, incomplete transverse myelitis, or brainstem/ (EDSS)[51] score at 5 years and/ or a low attack rate and little
cerebellar) and at least 2 silent T2 lesions on brain MRI. The accumulation of MRI lesions early in the disease course), a low
patients were randomized to weekly intramuscular interferon- chance of benefit (i.e., patients with established progressive
β1a (Avonex®, Biogen Idec) or placebo. The treatment group disease without clinical or radiological markers of ongoing
experienced a 44% reduction in the rate of development of inflammatory disease); and those with an indeterminate
CDMS compared with the placebo group over 3 years of prognosis (e.g., CIS or early relapsing-remitting MS with
follow-up. There were statistically significant beneficial effects infrequent mild attacks and a favorable prognostic profile).
on all MRI parameters for the treatment group, including The risk of MS is much lower in children than in adults.
decrease in T2 lesion development, gadolinium-enhancing One large, retrospective study found the cumulative risk of
lesions and T2 lesion volume. developing MS (the study predated the McDonald criteria)
The 10-year follow-up showed that patients treated was 13% at 10 years and 19% by 20 years.[52] Besides, use of
immediately after their first episode had a significantly lesser immunomodulatory therapies to reduce the risk of MS has
chance of experiencing a second attack compared to those who not been well studied in children. Hence children with optic
had delayed treatment (after about 30 months). This showed neuritis, most often bilateral and of viral etiology, are treated
the advantages of initiating early treatment with interferon-β1a. with intravenous steroids.

The most common side effects associated with Avonex are Prognosis
flu-like symptoms, including myalgia, fever, fatigue, headache,
The long-term visual prognosis of idiopathic optic neuritis
chills, nausea, vomiting, pain and asthenia.[47]
remains good. More than 90% of the patients recover a visual
The PRISMS[48] (Prevention of Relapses and Disability by acuity of 20/40 or better by 6 months, as seen in the ONTT. A
Interferon β-1a Subcutaneously in Multiple Sclerosis) Trial cut-off level of vision ≤ 20/50 (6/15), contrast sensitivity of <1.0
assessed efficacy of interferon (IFN)-β1a in dosages of 22 μg log units and a visual field mean deviation of ≤ − 15 dB after 1
and 44 μg s.c. given subcutaneously compared to placebo in month in the ONTT were predictive of a poor visual outcome
RRMS (relapsing-remitting multiple sclerosis) patients, and it at 6 months, a prediction that could not be made with any
was seen that both the treatment groups had fewer relapses. certainty at baseline.[53] On the other hand, the OSMS form of
MS that is prevalent in the Asian region is associated with poor
The most recent study has been the Betaferon in Newly
visual recovery.[54] However, it should be kept in mind that the
Emerging Multiple Sclerosis for Initial Treatment (BENEFIT)
other causes of optic neuritis, specifically NMO and infective,
study, which included patients with a single neurologic event
which are more prevalent in this region, may skew the data
and at least 2 clinically silent MRI lesions. In a 24-month study
regarding the outcome of optic neuritis.
period, standard dose of interferon-β1b (Betaseron®, Bayer
Health Care Pharmaceuticals) was seen to reduce the risk of Despite the relatively good visual outcome, most patients
MS by 50%.[49] show a degree of long-lasting damage to the optic nerve,
 Menon, et al.: Management of optic neuritis
March - April 2011 121

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Source of Support: Nil, Conflict of Interest: None declared.
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