03sexton.ppt
TRANSCRIPT
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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 > 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 groups1. 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
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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
• Uhtoff’s 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