Clinical Approach to Optic Clinical Approach to Optic NeuritisNeuritis

Raed Bbehbehani , MD, ABORaed Bbehbehani , MD, ABO

PearlsPearls

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

Diagnosis : Pupil + Color vision.Diagnosis : Pupil + Color vision. MS PrognosisMS Prognosis Neuro-imaging : MRI brain/orbit ( T1 Neuro-imaging : MRI brain/orbit ( T1

fat suppressed views of the orbit fat suppressed views of the orbit with Gad).with Gad).

Optic neuritisOptic neuritis

Young, femaleYoung, female PainPain Visual acuity can be normal.Visual acuity can be normal. RAPD RAPD DyschromatopsiaDyschromatopsia Visual field defect.Visual field defect. MRIMRI

MRI in optic neuritisMRI in optic neuritis

3-year ONTT 3-year ONTT Beck et al. NEJM 1993Beck et al. NEJM 1993

IV + oral steroids speed visual IV + oral steroids speed visual recovery but does not provide long-recovery but does not provide long-term benefit.term benefit.

Oral steroids alone are associated with Oral steroids alone are associated with increased risk of recurrence of ON.increased risk of recurrence of ON.

IV+ oral steroids reduced the rate of IV+ oral steroids reduced the rate of CDMS in the first 2 years, but not CDMS in the first 2 years, but not after.after.

MR findings is the best predictor for MR findings is the best predictor for development of CDMS.development of CDMS.

MRI brainMRI brain

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 Only 1 out of 457 was eventually

diagnosed with collagen vascular diagnosed with collagen vascular disease !disease !

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

CXR normal in all patients.CXR normal in all patients. No lab studies required for typical No lab studies required for typical

optic neuritis.optic neuritis.

Should I do LP ?Should I do LP ?

ONTT : LP abnormalities did not add ONTT : LP abnormalities did not add any additional unsuspected any additional unsuspected diagnosis in 141 patients.diagnosis in 141 patients.

Normal CSF does not preclude future Normal CSF does not preclude future development of MS.development of MS.

Consider in atypical optic neuritis or Consider in atypical optic neuritis or if the MRI is normal.if the MRI is normal.

Should I do VEP ?Should I do VEP ?

Not necessary in classic optic Not necessary in classic optic neuritis.neuritis.

Does no alter diagnosis or treatment Does no alter diagnosis or treatment in classic optic neuritis.in classic optic neuritis.

May be useful to identify a second May be useful to identify a second site of involvement in a patient with site of involvement in a patient with MS to strengthen clinical diagnosis.MS to strengthen clinical diagnosis.

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 56% if >= 1 baseline lesion (3mm

diameter).diameter). Risk does not increase appreciably Risk does not increase appreciably

with increasing lesions.with increasing lesions.

ONTT 10 year DataONTT 10 year Data

No cases of CDMS if normal MRI and any No cases of CDMS if normal MRI and any of :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

Course of optic neuritisCourse of optic neuritis

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

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

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

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

What should I tell the patient ?What should I tell the patient ?Neurologic impairment 10 years after optic Neurologic impairment 10 years after optic

neuritisneuritis

ONTT Study group. Arch Neurol 1994ONTT Study group. Arch Neurol 1994 The degree of disability was unrelated to The degree of disability was unrelated to

whether the initial MRI was lesion-free or whether the initial MRI was lesion-free or showed lesions and to the number of showed lesions and to the number of lesions.lesions.

65% of patients having an Expanded 65% of patients having an Expanded Disability Status Scale score lower than Disability Status Scale score lower than 3.0. 3.0.

Most patients who develop CMD following Most patients who develop CMD following an initial episode of optic neuritis will have an initial episode of optic neuritis will have a relatively benign course for at least 10 a relatively benign course for at least 10 years. years.

Mimickers of Typical Optic Mimickers of Typical Optic NeuritisNeuritis

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

Atypical optic neuritisAtypical optic neuritis

Bilateral onset in an adult.Bilateral onset in an adult. No pain.No pain. Ocular findings : uveitis, exudate, retinitis.Ocular findings : uveitis, exudate, retinitis. Severe disc swelling.Severe disc swelling. Marked hemorrhages.Marked hemorrhages. No improvement after 6 weeks.No improvement after 6 weeks. Recurrences within a short interval or Recurrences within a short interval or

during steroid taper.during steroid taper. Age > 50 years.Age > 50 years. Pre-existing diagnosis of a systemic Pre-existing diagnosis of a systemic

disease.disease.

Non-Arteritic Anterior Ischemic Non-Arteritic Anterior Ischemic Optic Neuropathy (NAION)Optic Neuropathy (NAION)

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

Non-Arteritic Anterior Ischemic Non-Arteritic Anterior Ischemic Optic Nuropathy (NAION)Optic Nuropathy (NAION)

Age> 40.Age> 40. Unilateral visual acuity/field loss.Unilateral visual acuity/field loss. Disc edema ( initially pallid ) , can be Disc edema ( initially pallid ) , can be

sectoral or diffuse.sectoral or 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 Usually remains static but can improve in

42.7 % or progress over several weeks in 25 42.7 % or progress over several weeks in 25 %.%.

NAIONNAION

Hemorrhage

Anamolousdisc

NAIONNAION

Narrowed arterioles

Segmental pallor

CaseCase

66 year female with painless, 66 year female with painless, decreased vision over 1 month.decreased vision over 1 month.

PMH : Hypertension, and PMH : Hypertension, and hyperlipedemia.hyperlipedemia.

Meds : Lotrel, protonix, avocar, Meds : Lotrel, protonix, avocar, aspirin and biotin.aspirin and biotin.

No h/o fever, weight loss,rashes or No h/o fever, weight loss,rashes or joint pain. joint pain.

Case - ExamCase - Exam

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.

CaseCase

CaseCase

ANA, C-ANCA, P-ANCA, Lyme titers, FTA-ANA, C-ANCA, P-ANCA, Lyme titers, FTA-ABS and ACE levels normal. ABS and ACE levels normal.

Chest x-ray normal.Chest x-ray normal. VA decreased to 20/80 right eye and color VA decreased to 20/80 right eye and color

vision to 2/6 over 3 months despite oral vision to 2/6 over 3 months despite oral steroids.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.

12/03

3/04

MRI

Optic Nerve Optic Nerve Sheath BiopsySheath Biopsy

Positive CD20 and CD45

Behbehani et al.Am J Ophthalmol. 2005;139:1128-30.

Neuro-retinitisNeuro-retinitis

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

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

Sarcoid optic neuopathySarcoid optic neuopathy

CXR and ACE are positive in 70 %.CXR and ACE are positive in 70 %. MRI and Galium scan are positive in MRI and Galium scan are positive in

80%-90%.80%-90%. Evolving role of PET scan.Evolving role of PET scan.

SarcoidosisSarcoidosis

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

Neuro-sarcoidosisNeuro-sarcoidosis

Neuro-sarcoidosisNeuro-sarcoidosis

Auto-immune Optic Auto-immune Optic NueropathyNueropathy

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

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

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

Multiple recurrences.Multiple recurrences. Treatment : Gamma globulins.Treatment : Gamma globulins.

SummarySummary

RAPD and dyschromatopsia are the hallmarks RAPD and dyschromatopsia are the hallmarks of optic neuropathy.of 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, Suspect atypical optic neuritis (bilateral,

painless, uveitis, no improvement after 6/52, painless, uveitis, no improvement after 6/52, severe disc edema and hemorrhages).severe disc 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).