Updates on Optic Neuritis

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Updates on Optic Neuritis

• Briar Sexton
• Neuro-ophthalmology Clinical Day
• Friday, November 18, 2005

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Introduction

• Optic neuritis
• Atypical optic neuritis
• Treatment of optic neuritis
• Optic neuritis and MS

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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

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Optic Neuritis

• Sub-acute, monocular visual loss
• Painful extraocular movements
• RAPD
• Dyschromatopsia
• Decreased contrast
• sensitivity
• VF deficits

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Fundus Signs of Optic Neuritis
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InvestigationsBased 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

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Atypical Optic Neuritis

• Atypical symptoms
• Unusual tempo of onset
• Absence of pain
• Co-morbidity
• Atypical signs
• Progressive decline in vision gt 2/52
• Severe/hemorrhagic disc edema
• Uveitis vitritis, retinitis, choroiditis
• Persistent ON sheath enhancement on MRI

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Fundus Photos Atypical ON
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Corticosteroid Dependent Optic Neuritis

• Another atypical optic neuritis
• Response to steroids
• Vision falls with taper
• Requires investigation

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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

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Visual Fields

• Central scotomas
• Paracentral scotomas
• Altitudinal defects

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Neuroimaging

• MRI
• FLAIR sequencing
• Gadolinium enhancement
• Optic nerve sheath enhancement with gad
• Periventricular white matter lesions on FLAIR

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MRI Nerve Sheath Enhancement

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MRI White Matter Lesions
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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

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The ONTT Methods

• Randomization to one of 3 groups
• IV steroids 250 mg methylprednisolone qid x 3
  days, oral prednisone (1mg/kg) x 11 days
• Oral steroids prednisone 1mg/kg/day x 14 days
• Oral placebo 14 days

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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

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The ONTT and Oral Prednisone

• Routing vs. Dose?
• Probably dose Greater CD4 than CD8 effect

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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

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Prognosis

• Severity of initial visual loss is related to
  final visual outcome
• Most recover well
• 74 20/20
• 92 20/40

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Visual Sequelae

• Optic nerve head pallor will develop
• VF deficits may persist
• Uhtoffs phenomenon
• Pulfrich phenomenon

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Optic Neuritis RecurrenceFrom 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

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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

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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

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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

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Radiologic Predictors of MS10 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

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Clinical Predictors of MSONTT 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

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Managing Optic Neuritis and MS

• Positive MRI
• Consider immunomodulatory therapy ie interferon
  or glatiramer acetate
• Patients should be seen by neurology

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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

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Conclusions

• Patients must be investigated for demyelination
• Remember the atypical optic neuritis