Updates on Optic Neuritis

Briar Sexton
Neuro-ophthalmology Clinical Day
Friday, November 18, 2005
 Introduction
Optic neuritis
Atypical optic neuritis
Treatment of optic neuritis
Optic neuritis and MS
 Optic Neuritis: Epidemiology
Incidence: 1-5 per 100 000 per year

Highest incidence in
Caucasians
Countries with high latitudes: genetics?
Springtime
Ages 20-49
Women
 Optic Neuritis
Sub-acute, monocular visual loss
Painful extraocular movements
RAPD
Dyschromatopsia
Decreased contrast
sensitivity
VF deficits
Fundus Signs of Optic Neuritis
 Investigations
Based on ONTT results for typical optic neuritis

Demyelination is the most common cause

No need for laboratory investigation

i.e. ESR, ANA

Need to do MRI of the brain

Assess MS risk
 Atypical Optic Neuritis
Atypical symptoms
Unusual tempo of onset
Absence of pain
Co-morbidity
Atypical signs
Progressive decline in vision > 2/52
Severe/hemorrhagic disc edema
Uveitis: vitritis, retinitis, choroiditis
Persistent ON sheath enhancement on MRI
Fundus Photos: Atypical ON
 Corticosteroid Dependent Optic
Neuritis
Another atypical optic neuritis

Response to steroids

Vision falls with taper

Requires investigation
Atypical Optic Neuritis: Work-up
Laboratory investigations
CBC, ESR, ANA, MHA-ATP, ACE
Lyme, Baronella, TB skin test
CXR
Consider LP
Make sure MRI images optic nerve/orbits
Visual Fields
Central scotomas

Paracentral scotomas

Altitudinal defects
 Neuroimaging
MRI
FLAIR sequencing
Gadolinium enhancement
Optic nerve sheath enhancement with gad

Periventricular white matter lesions on

FLAIR
 MRI: Nerve Sheath Enhancement

MRI: White Matter Lesions

 The Optic Neuritis Treatment Trial
(ONTT)
Objective: to evaluate the role of
corticosteroids in the treatment of unilateral
optic neuritis

Inclusion criteria: unilateral optic neuritis

 The ONTT: Methods
Randomization to one of 3 groups
1. IV steroids: 250 mg methylprednisolone qid x
3 days, oral prednisone (1mg/kg) x 11 days

2. Oral steroids: prednisone 1mg/kg/day x 14

days

3. Oral placebo: 14 days

 ONTT: Results
IV steroids
More rapid recovery but
same endpoint
Protective v. placebo at 2
years, not 3

Oral prednisone
Higher rate of new ON
attacks at 1 year
Highest rate of relapse at 5
years
 The ONTT and Oral Prednisone
Routing vs. Dose?
Probably dose: Greater CD4 than CD8 effect
 Prognosis
Natural history: worsening over days to weeks
followed by spontaneous recovery
79% of patients begin to recover by 3/52
93% of patients show improvement by 5/52

Ongoing clinical improvement to 1 year

VEP latency improves to 2 years

 Prognosis
Severity of initial
visual loss is related to
final visual outcome

Most recover well

74% 20/20
92% 20/40
 Visual Sequelae
Optic nerve head pallor will develop

VF deficits may persist

Uhtoffs phenomenon

Pulfrich phenomenon
 Optic Neuritis Recurrence
From the ONTT
35% of patients experienced recurrence in the
previously affected eye or an attack in the fellow
eye at 10 years

Recurrence rate was double in those with CDMS

Recurrence rate highest in the oral steroid group

 Sub-clinical Optic Neuritis
Not all optic neuritis attacks are clinically
evident
Sisto et al 2005
VEP abnormalities in 54.4% of CD-MS patients
asymptomatic for visual impairment
Vidovic et al 2005
70% of visually asymptomatic MS patients had
GVF defects consistent with optic neuritis
 Optic Neuritis and MS
Clinical diagnosis
2 demyelinating attacks separated in time and
space
Sequential optic neuritis in one eye than the
other meets the criteria
Discrete attacks in the same eye meets the
criteria
Radiologic: Mac Donald Criteria
 Optic Neuritis and MS
Lessell et al. 1988: 58% of optic neuritis at
15 years in initially isolated cases

38-50% of all CDMS develops optic

neuritis at some point
 Radiologic Predictors of MS
10 year ONTT data
White matter lesions on MRI

Risk is 22% if no baseline brain lesions

Risk is 56% if 1 baseline lesion
Risk increases with increasing lesions
 Clinical Predictors of MS
ONTT 10 year data
Low risk if no MRI lesions and
Male gender
Optic disc swelling
No CDMS in subset with above and one of
No pain
Severe disc edema
Peripapillary hemorrhages
Retinal exudates
 Managing Optic Neuritis and MS
Positive MRI
Consider immunomodulatory therapy ie
interferon or glatiramer acetate

Patients should be seen by neurology

 CHAMPS Study
Effect of Interferon B 1a treatment in
patients with optic neuritis and MRI
changes compatible with MS
Significantly less CDMS
Less progression of MRI lesions
 Conclusions
Patients must be investigated for
demyelination

Remember the atypical optic neuritis