Discussion of clinical approach to typical (demyelnating) and atypical optic neuritis (immune/inflammatory/infectious) optic neuritis with evidence-based review. Target: Ophthalmologists/Neurologists

1. Clinical Approach to Optic
Neuritis
Raed Bbehbehani , MD, ABO

2. Pearls
 History : Pain ( 92 % mild) and vision
loss.
 Diagnosis : Pupil + Color vision.
 MS Prognosis
 Neuro-imaging : MRI brain/orbit ( T1 fat
suppressed views of the orbit with Gad).

3. Optic neuritis
 Young, female
 Pain
 Visual acuity can be normal.
 RAPD
 Dyschromatopsia
 Visual field defect.
 MRI

4. MRI in optic neuritis

6. 3-year ONTT
Beck et al. NEJM 1993
 IV + oral steroids speed visual recovery
but does not provide long-term benefit.
 Oral steroids alone are associated with
increased risk of recurrence of ON.
 IV+ oral steroids reduced the rate of
CDMS in the first 2 years, but not after.
 MR findings is the best predictor for
development of CDMS.

7. MRI brain

8. Should I order Blood work up ?
 Optic neuritis treatment trial (ONTT).
 ANA < 1:320 in 13% , >1:320 in 3 %.
 Only 1 out of 457 was eventually
diagnosed with collagen vascular disease !
 FTA-ABS positive in 6 patients but none
had syphilis.
 CXR normal in all patients.
 No lab studies required for typical optic
neuritis.

9. Should I do LP ?
 ONTT : LP abnormalities did not add any
additional unsuspected diagnosis in 141
patients.
 Normal CSF does not preclude future
development of MS.
 Consider in atypical optic neuritis or if the
MRI is normal.

10. Should I do VEP ?
 Not necessary in classic optic neuritis.
 Does no alter diagnosis or treatment in
classic optic neuritis.
 May be useful to identify a second site of
involvement in a patient with MS to
strengthen clinical diagnosis.

11. The predictive value of MRI to
develop MS (10 year ONTT Data)
 22% if normal initial MRI.
 56% if >= 1 baseline lesion (3mm
diameter).
 Risk does not increase appreciably with
increasing lesions.

12. ONTT 10 year Data
 No cases of CDMS if normal MRI and any of :
 Severe disc edema (n=22)
 Painless (n=18)
 Disc hemorrhages (n= 16)
 Retinal exudates (n=8)
 NLP visual acuity (n=6)
 Low risk for CDMS (5%) if :
- Male
- Disc edema
- normal MRI

13. Course of optic neuritis
 Vision recovery starts within 2 weeks.
 ONTT : at 3 months, visual acuity was
>=20/40 in 93 %.
 35 % recurrence in the affected or fellow
eye ( 10 year ONTT)
 Recurrence twice more common in MS
patients than non-MS patients.

14. What should I tell the patient ?
Neurologic impairment 10 years after optic neuritis
ONTT Study group. Arch Neurol 1994
 The degree of disability was unrelated to
whether the initial MRI was lesion-free or
showed lesions and to the number of lesions.
 65% of patients having an Expanded Disability
Status Scale score lower than 3.0.
 Most patients who develop CMD following an
initial episode of optic neuritis will have a
relatively benign course for at least 10 years.

15. Mimickers of Typical Optic Neuritis
 Ischemic (AION, PION).
 Compressive.
 Infectious/ para-infectious.
 Inflammatory and infiltrative.
 Leber’s optic neuropathy.
 Auto-immune.
 Paraneoplastic.

16. Atypical optic neuritis
 Bilateral onset in an adult.
 No pain.
 Ocular findings : uveitis, exudate, retinitis.
 Severe disc swelling.
 Marked hemorrhages.
 No improvement after 6 weeks.
 Recurrences within a short interval or during
steroid taper.
 Age > 50 years.
 Pre-existing diagnosis of a systemic disease.

17. Non-Arteritic Anterior Ischemic
Optic Neuropathy (NAION)
http://medstat.med.utah.edu/NOVEL/

18. Non-Arteritic Anterior Ischemic
Optic Nuropathy (NAION)
 Age> 40.
 Unilateral visual acuity/field loss.
 Disc edema ( initially pallid ) , can be sectoral or
diffuse.
 Small cup/disc ratio (anamolous disc).
 Vascular risk factors (diabetes,hypertension,
smoking, hypercholesterolemia).
 Usually remains static but can improve in 42.7
% or progress over several weeks in 25 %.

19. NAION
Hemorrhage
Anamolous
disc

20. NAION
Segmental pallor
Narrowed arterioles

21. Case
 66 year female with painless, decreased
vision over 1 month.
 PMH : Hypertension, and hyperlipedemia.
 Meds : Lotrel, protonix, avocar, aspirin
and biotin.
 No h/o fever, weight loss,rashes or joint
pain.

22. Case - Exam
 VA : 20/30 both eyes.
 Color : full both eyes.
 Pupils : small right RAPD.
 Inferior visual field defect right eye.

23. Case

24. Case
 ANA, C-ANCA, P-ANCA, Lyme titers, FTA-ABS
and ACE levels normal.
 Chest x-ray normal.
 VA decreased to 20/80 right eye and color vision
to 2/6 over 3 months despite oral steroids.
 LP: normal CSF.
 Vitreous cells seen.
 CT of chest and abdomen: normal.
 PET scan: normal.

25. MRI
12/03
3/04

26. Optic Nerve
Sheath Biopsy
Positive CD20 and CD45
Behbehani et al.
Am J Ophthalmol.
2005;139:1128-30.

27. Neuro-retinitis
http://medstat.med.utah.edu/NOVEL

28. Neuro-retinitis
 Cat scratch (Bartonella henselae).
 TB
 Syphilis
 Sarcoidosis
 EBV / CMV / HSV / HZV / Mumps
 Lyme
 Toxoplasmosis

29. Sarcoid optic neuopathy
 CXR and ACE are positive in 70 %.
 MRI and Galium scan are positive in
80%-90%.
 Evolving role of PET scan.

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

31. Neuro-sarcoidosis

32. Neuro-sarcoidosis

33. Auto-immune Optic Nueropathy
 ANA + , anticardiolipin antibody + (89
% IgM).
 Does not meet criteria of collagen vascular
disease.
 Skin biopsy 92% abnormal ( 67%
immunofloresence)
 Multiple recurrences.
 Treatment : Gamma globulins.

34. Summary
 RAPD and dyschromatopsia are the hallmarks of
optic neuropathy.
 Typical optic neuritis is a clinical diagnosis.
 Low risk of MS if normal MRI, disc edema and
male.
 Suspect atypical optic neuritis (bilateral,
painless, uveitis, no improvement after 6/52,
severe disc edema and hemorrhages).
 Atypical optic neuritis should be investigated
more intensively (serology, LP, biopsy).