atypical optic neuritis -red flags

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Red Flags in Optic Red Flags in Optic Neuritis Neuritis Raed Bbehbehani , MD, ABO Raed Bbehbehani , MD, ABO

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Optic neuritis is a common presentation of MS. Physicians need to be aware of the typical presentation of ON and also be aware of the atypical signs and features that should incite a search for alternative diagnosis.

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Page 1: Atypical Optic Neuritis -Red Flags

Red Flags in Optic Red Flags in Optic Neuritis Neuritis

Raed Bbehbehani , MD, ABORaed Bbehbehani , MD, ABO

Page 2: Atypical Optic Neuritis -Red Flags

PearlsPearls

• History : Pain ( 92 % mild) and vision loss.History : Pain ( 92 % mild) and vision loss.

• Diagnosis : Pupil + Color vision.Diagnosis : Pupil + Color vision.

• MS PrognosisMS Prognosis

• Neuro-imaging : MRI brain/orbit ( T1 fat Neuro-imaging : MRI brain/orbit ( T1 fat suppressed views of the orbit with Gad).suppressed views of the orbit with Gad).

Page 3: Atypical Optic Neuritis -Red Flags

Optic neuritisOptic neuritis

• Young, femaleYoung, female

• Pain ( dull-aching , periocular headache , Pain ( dull-aching , periocular headache , worse with EOM)worse with EOM)

• Visual acuity can be normal.Visual acuity can be normal.

• RAPD RAPD

• Visual field defectVisual field defect

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RAPD

• In the absence of contralateral optic nerve damage.

• Swinging Flash Test.

• Neural Density Filter (0.3-1.2 log)

Page 5: Atypical Optic Neuritis -Red Flags

Signs

•Dyschromatopsia• Hardy Rand Ritter

• Ishihara pseudo=isochomatic plates

• Fans-worth Munsell 100-hue Test

Page 6: Atypical Optic Neuritis -Red Flags

Signs

• Utohff’s phenomenon : visual blurring with heat or exhaustions .

• Vision comes back with cool down (5 min - 24 hours)

• Also seen in Compressive , Toxic , LHON , Inflammatory optic neuritis.

Page 7: Atypical Optic Neuritis -Red Flags

Signs

• Contrast Sensiitivty

• Pelli Robson

• Sloan Low Contrast acuity

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Visual Field Defact

• In ONTT : Central field > peripheral

• Focal defect (42%) : Arcuate , Altitudinal , Nasal

Page 9: Atypical Optic Neuritis -Red Flags

OCT in ON

Acute RNFL thickeningLate RNFL loss

Page 10: Atypical Optic Neuritis -Red Flags

Disc Appearance

•Normal – ⅔•Oedematous - ½ •Temporal pallor – 10% , suggestive of preceding attack•Disc Oedema is without Hemorrages / lipids/ cotton wool

Page 11: Atypical Optic Neuritis -Red Flags
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MRI in MS

•Brain lesions : > 3mm, oval, in peri-ventricular white matter, radially oriented towards the ventricular spaces on a unenhanced MRI• In Optic neuritis always get – •Optic nerve – STIR signals (fat suppression)•Gadolinium enhancements•Single white matter lesion in AMON – high risk for M.S (ONTT study)

Page 13: Atypical Optic Neuritis -Red Flags

MRI in optic neuritisMRI in optic neuritis

Page 14: Atypical Optic Neuritis -Red Flags

MRI brainMRI brain

Page 15: Atypical Optic Neuritis -Red Flags

The predictive value of MRI to The predictive value of MRI to develop MS (10 year ONTT develop MS (10 year ONTT

Data)Data)

• 22% if normal initial MRI.22% if normal initial MRI.

• 56% if >= 1 baseline lesion (3mm diameter).56% if >= 1 baseline lesion (3mm diameter).

• Risk does not increase appreciably with Risk does not increase appreciably with increasing lesions.increasing lesions.

Page 16: Atypical Optic Neuritis -Red Flags

ONTT 15 years

Page 17: Atypical Optic Neuritis -Red Flags

Should I order Blood work Should I order Blood work up ? up ?

• Optic neuritis treatment trial (ONTT).Optic neuritis treatment trial (ONTT).

• ANA < 1:320 in 13% , >1:320 in 3 %.ANA < 1:320 in 13% , >1:320 in 3 %.

• Only 1 out of 457 was eventually diagnosed with Only 1 out of 457 was eventually diagnosed with collagen vascular disease !collagen vascular disease !

• FTA-ABS positive in 6 patients but none had FTA-ABS positive in 6 patients but none had syphilis.syphilis.

• CXR normal in all patients.CXR normal in all patients.

• No lab studies required for typical optic neuritis.No lab studies required for typical optic neuritis.

Page 18: Atypical Optic Neuritis -Red Flags

ONTT 10 year DataONTT 10 year Data• No cases of CDMS if normal MRI and any of :No cases of CDMS if normal MRI and any of :

1)1) Severe disc edema (n=22)Severe disc edema (n=22)

2)2) Painless (n=18)Painless (n=18)

3)3) Disc hemorrhages (n= 16)Disc hemorrhages (n= 16)

4)4) Retinal exudates (n=8)Retinal exudates (n=8)

5)5) NLP visual acuity (n=6)NLP visual acuity (n=6)

• Low risk for CDMS (5%) if :Low risk for CDMS (5%) if :

- Male- Male

- Disc edema- Disc edema

- normal MRI- normal MRI

Page 19: Atypical Optic Neuritis -Red Flags

Course of optic Course of optic neuritisneuritis

• Vision recovery starts within 2 weeks.Vision recovery starts within 2 weeks.

• ONTT : at 3 months, visual acuity was ONTT : at 3 months, visual acuity was >=20/40 in 93 %.>=20/40 in 93 %.

• 35 % recurrence in the affected or fellow eye 35 % recurrence in the affected or fellow eye ( 10 year ONTT)( 10 year ONTT)

• Recurrence twice more common in MS Recurrence twice more common in MS patients than non-MS patients.patients than non-MS patients.

Page 20: Atypical Optic Neuritis -Red Flags

Mimickers of Typical Optic Mimickers of Typical Optic NeuritisNeuritis

• Ischemic (AION, PION).Ischemic (AION, PION).

• Neuromyelitis Optica (NMO)Neuromyelitis Optica (NMO)

• Compressive.Compressive.

• Infectious/ para-infectious.Infectious/ para-infectious.

• Inflammatory and infiltrative.Inflammatory and infiltrative.

• Leber’s optic neuropathy.Leber’s optic neuropathy.

• Auto-immune.Auto-immune.

• Paraneoplastic.Paraneoplastic.

Page 21: Atypical Optic Neuritis -Red Flags

Atypical optic neuritisAtypical optic neuritis“Red Flags”“Red Flags”

• Age <12 years or >50 yearsAge <12 years or >50 years

• Severe loss of vision (NLP) , Bilateral onset in an adult, no Severe loss of vision (NLP) , Bilateral onset in an adult, no improvement after 6 weeks , progressive course.improvement after 6 weeks , progressive course.

• No pain.No pain.

• Ocular findings : severe disc edema , marked hemorrhages, Ocular findings : severe disc edema , marked hemorrhages, uveitis, exudate, retinitis, phelbitisuveitis, exudate, retinitis, phelbitis

• Recurrences within a short interval or during steroid taper.Recurrences within a short interval or during steroid taper.

• Pre-existing diagnosis of a systemic disease ( Cancer, CT Pre-existing diagnosis of a systemic disease ( Cancer, CT disease, Vasculitis, immunosuppression)disease, Vasculitis, immunosuppression)

• Systemic Symptoms : Arm or leg weakness, bowel or Systemic Symptoms : Arm or leg weakness, bowel or bladder Sx, Arththralgia ,skin rash, feverbladder Sx, Arththralgia ,skin rash, fever

Page 22: Atypical Optic Neuritis -Red Flags

Non-Arteritic Anterior Non-Arteritic Anterior Ischemic Optic Neuropathy Ischemic Optic Neuropathy

(NAION)(NAION)

Page 23: Atypical Optic Neuritis -Red Flags

Non-Arteritic Anterior Non-Arteritic Anterior Ischemic Optic Nuropathy Ischemic Optic Nuropathy

(NAION)(NAION)• Age> 40.Age> 40.

• Unilateral visual acuity/field loss.Unilateral visual acuity/field loss.

• Disc edema ( initially pallid ) , can be sectoral or Disc edema ( initially pallid ) , can be sectoral or diffuse.diffuse.

• Small cup/disc ratio (anamolous disc).Small cup/disc ratio (anamolous disc).

• Vascular risk factors (diabetes,hypertension, Vascular risk factors (diabetes,hypertension, smoking, hypercholesterolemia).smoking, hypercholesterolemia).

• Usually remains static but can improve in 42.7 % or Usually remains static but can improve in 42.7 % or progress over several weeks in 25 %.progress over several weeks in 25 %.

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NAIONNAION

Hemorrhage

Anamolousdisc

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NAIONNAION

Narrowed arterioles

Segmental pallor

Page 26: Atypical Optic Neuritis -Red Flags

Neuromyelitis Optica• Inflammatory optic neuritis

• Median age : 35-44 years ; children : 4.4 years

• Less common than demyelinating (Asia , African , West Indies 50% of demyelination)

• Diagnostic Criteria

• Optic neuritis

• Transverse Myelitis

• At least 2 of 3

• LETM ( 3 contiguous veterbal segments)

• NMO IgG (70% sensitive , 100% specific)

• Brain lesions not compatible with MS

Page 27: Atypical Optic Neuritis -Red Flags

NMO• NMO disease spectrum ( seropositive optic

neuritis without TM or vice versa)

• Optic neuritis tend to be more severe

• Less visual recovery

• More than 50% of NMO will develop ON in 5 years of Dx

• ON and TM can simultaneous or separated.

Page 28: Atypical Optic Neuritis -Red Flags

When to Suspect

• Severe vision loss

• poor visual recovery

• Severe RNFL loss by OCT

• MRI posterior ON , chiasmal or posterior visual pathway involvement

• CSF - pleocytosis (PMN + eosinophils)

Page 29: Atypical Optic Neuritis -Red Flags

Auto-immune Optic Auto-immune Optic NueropathyNueropathy

• ANA + , anticardiolipin antibody + (89 % IgM). ANA + , anticardiolipin antibody + (89 % IgM).

• Does not meet criteria of collagen vascular Does not meet criteria of collagen vascular disease.disease.

• Skin biopsy 92% abnormal ( 67% Skin biopsy 92% abnormal ( 67% immunofloresence)immunofloresence)

• Multiple recurrences.Multiple recurrences.

• Treatment : Corticosteroids +- Treatment : Corticosteroids +- immunosuppressants.immunosuppressants.

Page 30: Atypical Optic Neuritis -Red Flags

Neuro-retinitisNeuro-retinitis• Cat scratch (Bartonella henselae).Cat scratch (Bartonella henselae).

• TBTB

• SyphilisSyphilis

• SarcoidosisSarcoidosis

• EBV / CMV / HSV / HZV / MumpsEBV / CMV / HSV / HZV / Mumps

• LymeLyme

• ToxoplasmosisToxoplasmosis

Page 31: Atypical Optic Neuritis -Red Flags

Neuro-retinitisNeuro-retinitis

http://medstat.med.utah.edu/NOVEL

Page 32: Atypical Optic Neuritis -Red Flags

Syphilis

• Optic neuritis, uveitis.

• Immunocompromised or HIV.

• Can occur at any stage of disease.

• Positive serum and CSF-VDRL.

• Suspect if reactive CSF and negative serum and CSF VDRL.

Page 33: Atypical Optic Neuritis -Red Flags

Sarcoid Optic Sarcoid Optic NeuropathyNeuropathy

• CXR and ACE are positive in 70 %.CXR and ACE are positive in 70 %.

• MRI and Galium scan are positive in 80%-90%.MRI and Galium scan are positive in 80%-90%.

• Evolving role of PET scan for occult lesions for Evolving role of PET scan for occult lesions for biopsy.biopsy.

• CSF : high protein and lymphocytosis (non-CSF : high protein and lymphocytosis (non-specific)specific)

• Responds to steroids.Responds to steroids.

Page 34: Atypical Optic Neuritis -Red Flags

SarcoidosisSarcoidosis

http://medstat.med.utah.edu/NOVEL

Page 35: Atypical Optic Neuritis -Red Flags

Neuro-sarcoidosisNeuro-sarcoidosis

Page 36: Atypical Optic Neuritis -Red Flags

Leber’s Heriditary Optic Neuropathy

• Mitochondrial optic neuropathy (Maternal Inheritance)

• Males (85% , females 15%) , age 10-80 years.

• Sequential vision loss.

• Acute or chronic presentation.

Page 37: Atypical Optic Neuritis -Red Flags

LHON• Fundus

• Swollen RNFL with circumperipapillary telangectiasia

• Atrophy

Page 38: Atypical Optic Neuritis -Red Flags

LHON

• Rule out cardiac conduction defects (EKG).

• Diagnosis is by mitochondrial genetic testing (m.11778G>A, m.3460G>A or m.14484T>C)

• Treatment : Co-enzyme 10 , Idebenone

Page 39: Atypical Optic Neuritis -Red Flags

Suggested Blood Work up for Atypical Optic Neuritis

Test Disease

CBC with Differntial, ESR, CRPInfections,

Inflammatory

Serum CSF-VDRL, FTA-Abs Syphilis

ACE Sarcoid

ANA, Anti-DNA SLE

NMO IgG NMO

C-ACNA, anti-pretinase 3

PPD TB

Bartoenlla Hensellae Serology Cat Scratch

LHON genetic testing LHON

Page 40: Atypical Optic Neuritis -Red Flags

Additional Work up

• Tissue biopsy of lesions of conjunctiva , ocular adnexa , sinus mucosa and sometimes optic nerve sheath.

• Radiologic studies : must include MRI of the brain and orbit with fat-suppression and gadolinium enhancement of the optic nerve sheath.

• PET/CT imaging, galluim scan.

Page 41: Atypical Optic Neuritis -Red Flags

CaseCase

• VA : 20/30 both eyes.VA : 20/30 both eyes.

• Color : full both eyes.Color : full both eyes.

• Pupils : small right RAPD.Pupils : small right RAPD.

• Inferior visual field defect right eye.Inferior visual field defect right eye.

Page 42: Atypical Optic Neuritis -Red Flags

CaseCase

Page 43: Atypical Optic Neuritis -Red Flags

CaseCase• ANA, C-ANCA, P-ANCA, Lyme titers, FTA-ABS and ACE levels ANA, C-ANCA, P-ANCA, Lyme titers, FTA-ABS and ACE levels

normal. normal.

• Chest x-ray normal.Chest x-ray normal.

• VA decreased to 20/80 right eye and color vision to 2/6 over VA decreased to 20/80 right eye and color vision to 2/6 over 3 months despite oral steroids.3 months despite oral steroids.

• LP: normal CSF.LP: normal CSF.

• Vitreous cells seen.Vitreous cells seen.

• CT of chest and abdomen: normal.CT of chest and abdomen: normal.

• PET scan: normal.PET scan: normal.

Page 44: Atypical Optic Neuritis -Red Flags

12/03

3/04

MRI

Page 45: Atypical Optic Neuritis -Red Flags

Optic Nerve Optic Nerve Sheath BiopsySheath Biopsy

Positive CD20 and CD45

Page 46: Atypical Optic Neuritis -Red Flags

SummarySummary• RAPD and dyschromatopsia are the hallmarks of RAPD and dyschromatopsia are the hallmarks of

optic neuropathy.optic neuropathy.

• Typical optic neuritis is a clinical diagnosis.Typical optic neuritis is a clinical diagnosis.

• Low risk of MS if normal MRI, disc edema and Low risk of MS if normal MRI, disc edema and male.male.

• Suspect atypical optic neuritis (bilateral, painless, Suspect atypical optic neuritis (bilateral, painless, uveitis, no improvement after 6/52, severe disc uveitis, no improvement after 6/52, severe disc edema and hemorrhages).edema and hemorrhages).

• Atypical optic neuritis should be investigated Atypical optic neuritis should be investigated more intensively (serology, LP, biopsy).more intensively (serology, LP, biopsy).