optic neuritis: another look

1. Optic Neuritis?: Another look Storm Eye Institute December 3, 2010 K. Leanne Wickliffe-Keisler, MD Monet: Storm off the Belle Ile Coast , 1886 Monet: Storm off the Belle Ile Coast , 1886

2. Financial disclosure none

3. Case: PL

4. Visual acuity: OD 20/25, ph 20/20 OS Count fingers at 1 ft IOP: OD 18 OS 21 mmhg Pupil exam: EOM: full, ortho Color plates: OD: OS: 0/10 Anterior segment exam: Lids/Adnexa: normal OU Conj/Sclera: normal OU Corneas: clear OU Iris: round OU, normal Lens: clear OU Fundus Exam OD disc edema OS pallor with atrophy Need pupils, color plates, VF and fundus photos for initial visit Need pupils, color plates, VF and fundus photos for initial visit

5. Right optic nerve: disc edema A: Optic nerve photo, OD. Note grade 2 papilledema, characterized by 360 degree nerve elevation without obscuration of the vessels.A: Optic nerve photo, OD. Note grade 2 papilledema, characterized by 360 degree nerve elevation without obscuration of the vessels.

6. VF right eye at presentation

7. Management Presumed optic neuritis, with history concerning for MS Admitted to the hospital for IV steroids 500mg Q6 hours and MRI Consults: Rheumatology for autoimmune therapy Dermatology for skin biopsy ENT for sinus evaluation Neurology for exam and LP CSF studies negative: Lyme, ACE, MBP, VDRL, protein normal Elevated glucose (119)

8. Imaging MRI brain & orbits w&w/o contrast: Findings: enlargement and abnormal enhancement of right optic nerve throughout its course with loss of normal fluid signal intensity surrounding the optic nerve on T2-weighted imaging, likely representing underlying optic neuritis. The left optic nerve is normal in course

9. Work up CBC: anemic with H/H of 10.9/32 CMP: serum protein 8.5 (slightly elevated) ANA : 1:80 positive ESR: 34 CRP: 2.69 TSH: normal Lysozyme/ACE: normal Lupus anticoagulant: negative Anticardiolipin antibody: negative C3, C4: normal Anti-SCL 70, SSA/RO, SSB/LA, Smith, RNP/SM: negative Double stranded DNA: negative C-ANCA: negative UA: + for blood and amorphous crystals

10. Clinical Course… Discharged after 3 days of high dose IV steroids on moderate oral steroid taper 1 week later ESR 11, CRP 0.4, Vision unchanged Repeat MRI: decreased enhancement of right optic nerve 4 months later on methotrexate ANA negative MRI: optic nerves normal, without enhancement

11. Visual fields after recovery

12. Autoimmune optic neuropathy Originally described by Dutton in 1982 Relatively rare optic neuropathy, associated with recurrent episodes of progressive visual loss with serological or cutaneous evidence of autoimmune disease without a defined systemic autoimmune illness, requiring high dose steroids

13. AON Recent review by Frohman, et al 2009 describe it as at least 3 attacks of optic neuritis (bilateral counting as 2 attacks) with positive serology or skin biopsy but without definable collagen vascular disease H&E stain of non-lesional, non-sun exposed skin biopsy demonstrating vasculitis highly correlated AON Usually female patients

14. AON Left, Image from skin biopsy specimen shows dermoepidermal and perivascularIgM deposition; right, dermoepidermal and perivascular C3 deposition (immunofluorescent, magnification x100) Indicates immune complex deposition

15. AON 200x mag, H&E section of optic nerve Perivascular aggregates of chronic inflammatory cells No evidence of necrotizing vasculitis or optic nerve necrosis

16. AON Similar in course to chronic relapsing inflammatory optic neuropathy as described by Kidd, et al Steroid dependent & relapsing Pain with onset Usually bilateral Disc abnormalities Normal brain MRI, with 63% abnormal optic nerves

18. AON

19. Back to our case… Last exam Sept 2010: Vision stable : OD 20/25 OS HM @ face Ocular hypertension vs. glaucoma suspect – currently on XalatanQhs OU Followed by Rheumatology on maintenance dose of methotrexate 10mg Qweek

20. Most recent fundus photos showing pallor OUMost recent fundus photos showing pallor OU

21. Summary Autoimmune optic neuropathy is a relatively rare recurrent optic neuritis without an associated autoimmune disorder Responds to corticosteroids, but often results in permanent vision loss Skin biopsy can be beneficial in the diagnosis

22. Questions… Monet Rock Arch West of Etretat (The Manneport)1883 Monet Rock Arch West of Etretat (The Manneport)1883

23. References: Frohman LP, DellaTorre K, Turbin R, Bielory L. Clinical characteristics, diagnostic criteria and theraputic outcomes in autoimmune optic neuropathy. Br J Ophthalmol2009.93 1660-1666. Riedel P, Wall M, Grey A. Autoimmune optic neuropathy. Arch Ophthalmol. 1998;116:1121-1124. Dutton JJ, Burde RM, Klingele TG. Autoimmune retrobulbar optic neuritis. Am J Ophthalmol. 1982;94:11-17. Kupersmith MJ, Burde RM, Warren FA, Klingele TG, Frohman LP, Mitnick H. Autoimmune optic neuropathy: evaluation and treatment. J NeurolNeurosurg Psychiatry. 1988;51:1381-1386. Bielory L, Kupersmith M, Warren F, et al. Skin biopsies in the evaluation of atypical optic neuropathies. OculImmunolInflamm. 1993;1:231-241. Blenkinsopp WK, Clayton RJ, Haffenden GP. Immunoglobulin and complement in normal skin. J ClinPathol. 1978;31:1143-1146.