Optic neuritis Dr.K.Venkateswarlu M.D;D.M Prof. of Neurology Andhra Medical College

Why do we care? Prognosis for visual recovery Prognosis for MS Treatment for demyelination Differentiating from variants

Typical features F : M 3:1 Age range Pain on eye movement Visual loss to nadir in 2 wks Poor central visual acuity Poor color vision RAPD Normal / swollen disc Uthoff’s phenomenon

Typical Idiopathic Optic Neuritis Clinical characteristics Young women (14-45 years) (3F:1M) with: Unilateral (rarely bilateral) Acute to subacute onset (usually rapidly progressive over a few days- nadir in 2 wks) Decreased visual acuity (variable) Decreased color vision (usually pronounced) Pain with eye movements (in > 90% of cases) Exacerbation with heat or exercise (Uhthoff’s phenomenon)

Typical Idiopathic Optic Neuritis contd.. Examination Relative efferent pupillary defect (if unilateral or asymmetric optic neuropathy) Funduscopy: Normal (2/3 of cases ) of swollen (1/3 of cases) optic nerve Normal mucula and retina (no exudates, no hemorrhages) Optic disc pallor (if at least 4 to 6 weeks after onset) Visual field test: Often central scotoma Follow-up Spontaneous improvement of visual function within a few weeks in >90% of cases (absence of improvement should raise concern about another diagnosis) Risk of multiple sclerosis

Important Features of Idiopathic Optic Neuritis 1.The optic nerve appears normal in the acute phase in about two thirds of cases (retrobulbar optic neuritis) and is swollen in about one third of cases (anterior optic neuritis or papillitis) 2.Temporal pallor of the disc develops only after 4 to 6 weeks from the onset of visual loss. 3.Spontaneous visual recovery is nearly universal features of idiopathic acute optic neuritis, and the visual prognosis of these patients is usually excellent, regardless of treatment. 4.The risk of subsequent development of multiple sclerosis after an isolated attack of idiopathic optic neuritis has been estimated as high as 75% at 15 years

Prognosis Excellent 95% achieve 20/40 69% 20/20 at 12 months fup Improvement starts in 3-6 wks Residual dyschromatopsia, loss of stereovision, depth perception, alt. motion perception

Atypical features Bil. involvement Lack of improvement Subacute onset awareness Vitreous cells. Florid OD edema Older age (PION) Systemic features

ONTT trial ( Tr. Groups) Oral Pred.( 1mg/kg/day for 14 days) Oral placebo (14 days) IV MP( 250mg 6 th hrly for 3 days fup oral pred. 1 mg/kg/day 11 days 4 day taper

ONTT trial ( Tr. effects) IV /oral regimen – accelerated visual recovery, no long term benefit to vision Oral pred. alone – no benefit, but was associated with increased rate of new attacks of ON

Radiology

Features not associated with development of Multiple Sclerosis in ONTT FeaturesNumber of patients Absence of light perception in 6 the affected eye Absence of periocular pain18 Severe optic disc edema22 Peripapillary hemorrhage16 Retinal exudates 8

Features of High-Risk Optic Neuritis Patients Young white woman Visual acuity better than “no light perception” Periocular pain Normal-appearing optic nerve (acutely) No retinal abnormality Abnormal brain MRI (at least on T2 lesion suggesting demyelinating disease)

Treatment of ON Acute phase Steroids Prophylactic Immunomodulatory Interferons Glatiramer Mitoxantrone

CHAMPS IFN Beta reduced CDMS by 44%

ONTT 10 year conclusions MRI lesions >3mm in size – increase risk Even with abn. MRI – 40% do not develop MS Imp. of ophthalmological exam. in identifying low risk patients

Pediatric ON Mean age 9.8 yrs Females 60% Disc edema 2/3 Bil. Involvement 2/3 Preceding feb. illness in 2/3 VA worse, can be no PL Prognosis worse 23% with 20/50 or 20/200

Peadric ON Risk of Ms less 13% had MS in 10yrs, 19% in 20yrs Risk more if unilat. Visual prog. Better for unilat. ON Data inadequate on Tr Steroids and dis. Mod. Drugs if MRI is abn.

Neuromyelitis optica- Diag. criteria Absolute 1. Optic neuritis 2. Acute myelitis Supportive ( atleast 2) 1.Negative Brain MRI at onset 2.Spinal MRI contiguous T2W signal abn.Over 3 or more segments 3. NMO Ig G seropositivity

NMO spectrum disorders NMO Incomplete/partial forms Single or recurrent LTEM Recurrent ON with –ve brain MRI Asian optico-spinal MS ON/LTEM with systemic autoimmune disease ON/LTEM with distinct MRI patterns

Clinical course Recurrent attacks of ON / LTEM Simult./ isolation ON uni>bil. Monophasic in 20%, relapsing 80% 60% relapse in first year

Prognosis Worse 50% disabled in 5 yrs 30% dead in 5 yrs Due to resp. failure in cervical cord dis.

Treatment of NMO Acute phase Steroids, plasmapheresis Prophylactic Immunosuppression Azathioprine Mycophenlate IV Ig Rituximab

Optic Neuritis AtypicalTypical MRI brain Consider other cause of optic neuropathy NormalAbnormal (High-risk patient) MRI brain and orbits Blood tests as appropriate Consider lumbar puncture Chest x-ray Consider IV steroids Consider therapy with disease-modifying agents Close follow-up Consider IV steroids on a case-by-case basis

LHON Sub.ac seq. bil. Cental visual loss Age ( 1-80) M>F ( 80-90%) Progression in each eye over wks-months Interval bet. Eyes days/wks/months Acuty worse than 20/200 Color vision effected early Central/centrocecal defects Classic fundus appearance

LHON Spontaneous recovery Numerous anecdotal reports May occur years later 4-70% Depends on mt DNA mutation (50-90%)-worst prog.( 4% recover) (10-15%)- bad prog (15%) -good prog.( 70% recover)

LHON : associated findings Cardiac cond. Defects Skeletal abn. Minor neur. Abn MS like illness More severe neurological syn.

AIONONLHON AgeOlder >50Younger 15-45Younger GenderM=FF>M 3:1M>F 4:1 BilateralityNot acute(15%/5yrs) Not acute(30%/10yrs) 50% acute, 100% 1yr Visual lossAcuteRapid progSub ac. PainInfrequentFrequentNone Color visionMay be normalCommonly abn.Abn. early VF defectsAltitudinalCentralCentrocecal ODAc.Edema, smallc/d Late: seg. pallor N/edema Pallor Pseudoedema/N Pallor PrognosisVariableGoodPoor SystemicHTN,DM,r/o GCASubsequent MSNone in most

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