A Review of Optic Neuritis

Digital Journal of Ophthalmology 1997

Volume 3, Number 4
May 1, 1997

Ken Graham, M.D. | Massachusetts Eye and Ear Infirmary, Harvard Medical School
Joseph Rizzo, M.D. | Massachusetts Eye and Ear Infirmary, Harvard Medical School

ABSTRACT INTRODUCTION DISCUSSION REFERENCES CLINICAL PICTURES

Abstract
Objective
Optic neuritis is inflammation of the optic nerve. The cause of the inflammation and the most
appropriate treatment is a subject of debate. This article will review a typical case of optic
neuritis, the clinicalmanifestations of optic neuritis, and the findings of the Optic Neuritis
Treatment Trial.
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Introduction
Optic neuritis is a serious condition which typically involves theyoung adult population. Years
ago it was thought that one third ofoptic neuritis patients would go on to have neurological
symptoms frommultiple sclerosis (MS). More recent long term studies indicate that over decades
the risk of developing MS is much higher.(1) Ophthalmologists must be alert to the symptoms of
optic neuritis andthey must be aware of the more serious complications to which these symptoms
are linked

CASE PRESENTATION

A 30 year old caucasian man presented with 2 weeks of gradually worsening vision in his left
eye.

History of present illness:

The patient had been seen once by a neurologist 2 years previously for flashes. At that time a
head CT was normal. The patient was lost to follow up with the neurologist, but the flashes had
continued for the 2 year period. The patient has a history of color blindness. The patient also
reported posterior pressure and tightness with left eye movement for the past week. The patient
reported his vision in the left eye worsened when showering. The patient did not experience
visual changes with activity or movement. Patient denied a history of trauma, redness, discharge
or headache.

Past medical history: negative

Social history: negative

Medications: none

No known drug allergies

Examination:
The exam revealed a vision of 20/20 OD and 20/30 OS. The patient was only able to get the
control plate correct on the color plates in both eyes consistent with his history of color
blindness. The external exam revealed no ptosis, and no resistance to retropulsion. His pupils
were reactive to light, changing FROM 4 to 2mm symmetrically. There was a left afferent
pupillary defect. Hertel measurement with a base of 102 was 19 OD and 18 OS. Extraocular
movements were full OU, though the patient reported a "tight" feeling on OS abduction. Tension
was 15 OU. Slit lamp exam was normal OU. Dilated exam was normal OU with no disc edema.
Humphrey visual fields showed an inferior altitudinal defect OS ( Fig 1and 2).

One week follow up:

The patient reported continued decreasing vision OS. The exam revealed visual acuity of 20/20
OD and 20/400 OS. The OS afferent pupillary defect remained. The rest of the exam was the
same. Goldmann visual fields were done and showed a central scotoma OS (slide 3).

A MRI was done at this time and showed inflammation of the left optic nerve (slide 4), and
white matter hyperintensity of the left occipital (slide 5) and right frontal(slide 6) lobes. Possible
treatment with corticosteroids was discussed with the patient. He chose to be observed with no
corticosteroid treatment.

One month follow up:

The patient reported marked recovery of vision OS, but had some blurring in bright sunlight. The
patient was on no medications. His vision was 20/20 OU. There was no afferent pupillary defect
and no "tightness" on eye movement. The rest of the exam was unchanged. A Humphrey visual
field showed improvement in the central scotoma .
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Discussion
CLINICAL MANIFESTATION

Optic neuritis typically presents with a triad of symptoms: loss of vision, dyschromatopsia and
eye pain. The initial attack is unilateral in 70% of adult patients and bilateral in 30%. The mean
age of onset of optic neuritis is in the third decade of life, but can occur FROM the first to the
seventh decades. The annual incidence of optic neuritis ranges FROM 1.4 to 6.4 new cases per
100,000 population. (2,3) Associated visual symptoms are reduced perception of light intensity
and Uhthoff's symptom (visual deficit induced by exercise or increased body temperature).(4)

The visual loss may be subtle or profound. Complete loss of vision may be caused by a single
plaque. In some cases the vision may be 20/20 with the only symptoms being blurred vision on
exertion or other isolated symptoms.(5) The rate of visual decline varies. Visual loss may occur
over hours (rarely) to days (most commonly). The nadir is usually about 1 week after the
onset.(6)

The prognosis for visual recovery is usually good. The majority of patients (65-80%) recover
visual acuity of 20/30 or better.(7,8) Most cases will recover visual acuity in a few months,
although the patients will often report some residual visual defect. There have been reported
residual abnormalities in contrast sensitivity, color vision, visual field loss, and light
brightness.(9-12)

In a patient with optic neuritis, the vision may improve, but the risk for development of multiple
sclerosis is high. This risk does not decrease over time.(1) In the past a number of patients have
been treated with oral or intravenous corticosteroids in the hope of improving visual recovery
and decreasing the incidence of multiple sclerosis. To help resolve the controversy of
corticosteroids, the Optic Neuritis Treatment Trial was formed.

OPTIC NEURITIS TREATMENT TRIAL

The Optic Neuritis Treatment Trial (ONTT) was a multicenter study in which 389 patients with
acute optic neuritis were randomly assigned to one of three treatment groups. The first GROUP
was given oral prednisone (1 mg/kg daily) for 14 days. The second GROUP was given
intravenous methylprednisolone, 250 mg four times daily for three days, followed by oral
prednisone for 14 days. The third GROUP was given an oral placebo for 14 days.

The eligibility criteria for the ONTT selected for patients with typical clinical features of optic
neuritis. These criteria were: age range of 18 to 46 years; acute unilateral optic neuritis with
visual symptoms of 8 days or less; a relative afferent pupillary defect and a visual field defect in
the affected eye; no previous episodes of optic neuritis in the affected eye; no previous
corticosteroid treatment for optic neuritis or multiple sclerosis; and no systemic disease other
than multiple sclerosis that might be associated with optic neuritis.(13)

All patients in the ONTT had blood testing to exclude collagen vascular disease (antinuclear
antibody), syphilis (FTA-ABS), and a chest x-ray to detect sarcoidosis. A lumbar puncture was
optional and was performed in 141 patients. Antinuclear antibody was positive in a titer 1:320 or
greater in 3% of patients. Of these patients, only one developed a connective tissue disease in the
first two years of follow up. FTA-ABS was positive in 1.3% of the patients, and none were
judged to have syphilis. A chest x-ray did not SHOW evidence of sarcoidosis in any patients.
CSF analysis never yielded unsuspected information. Based on these results, chest x-ray, blood
tests, and lumbar puncture are deemed not to be necessary in evaluating patients with typical
clinical features of optic neuritis.

The efficacy of corticosteroid therapy in optic neuritis has been controversial. The ONTT
showed that intravenous methylprednisolone followed by oral prednisone speeds the recovery of
visual loss, but there were no significant differences in visual acuity comparing the three groups
at 6 months. Unexpectedly, oral prednisone was found to increase the risk of recurrent optic
neuritis. Thus, treatment with oral prednisone in standard doses is no longer advised.
The ONTT also evaluated the relationship between optic neuritis and multiple sclerosis. A
standardized detailed neurologic examination was performed at study entry, at 6 months, at 1
year and then yearly. Clinically definite multiple sclerosis was diagnosed when new neurologic
symptoms developed that were attributable to demyelination in one or more regions of the
central nervous system. Treatment with the intravenous followed by oral corticosteroid regimen
reduced the rate of development of MS during the first 2 years. This was particularly evident in
patients with three or more brain MRI signal abnormalities consistent with demyelination in
locations characteristic of MS. By 3 years, this treatment effect had subsided.

The ONTT therefore recommends obtaining a brain MRI to assess the risk of developing MS.
The MRI is used to assist in the decision of whether to use IV steroids. It is unknown if repeat IV
steroids at 2 years would continue to affect the rate of development of MS. Other prospective
studies using beta interferon are currently in progress.(14)
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References
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A long term prospective study. Neurology 38:185-190, 1988.

2. Brewis M, Poskanzer DC, Rolland C, et al: Neurological disease in an English city. Acta
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3. Wray SH: Optic Neuritis. Principles and Practice of Ophthalmology. Volume 4, 2539-2568.

4. Percy AK, Nobrega FT, Kurland LT: Optic neuritis and multiple sclerosis. Arch Ophthalmol
87:135,1972.

5. Nikoskelainen E: Symptoms, signs and early course of optic neuritis. Acta Ophthalmol
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6. Lillie WI: The clinical significance of retrobulbar and optic neuritis. Am J Ophtahlmol 17:110,
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7. Perkin GD, Rose CF: Optic Neuritis and its Differential Diagnosis. Oxford, Oxford University
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11. Van Dalen JTW, Greve EL: Visual field defects in multiple sclerosis. Neuro-ophthalmology
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12. Hess RF, Plant GT: The psychophysical loss in optic neuritis: spatial and temporal aspects. In
Optic Neuritis. Cambridge, Cambridge University Press, 1986, p 109.

13. Beck RW, Cleary PA, et al: Optic Neuritis Treatment Trial. Arch Ophthalmol 3:773-775,
1993.

14. Beck RW, Trobe JD: What we have learned FROM the Optic Neuritis Treatment Trial.
Ophthalmology 10:1504-1509, 1995.