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GRAND ROUNDS

GRAND ROUNDS. Denise A. John VEI July 28th, 2006. Case. HPI: 37 y/o ♂ presents with 6-day history of blurry vision OS. (+) redness, tearing, pruritis; Ø floaters/photopsias/diplopia ROS: Unremarkable, except upper face pressure POHX: ø corrective wear/trauma/surgeries/laser

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GRAND ROUNDS

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  1. GRAND ROUNDS Denise A. John VEI July 28th, 2006

  2. Case • HPI: 37 y/o ♂ presents with 6-day history of blurry vision OS. • (+) redness, tearing, pruritis; Ø floaters/photopsias/diplopia • ROS: Unremarkable, except upper face pressure • POHX: ø corrective wear/trauma/surgeries/laser • PMHX: Hyperlipidemia, obesity, sleep apnea s/p bilateral inf. turbinate reduction, sinusitis, eustachian tube dysfunction • FHX: DM; cancer; Paget’s dz • SHX: Occ. ETOH; ø tobacco/IVDA • ALL: Codeine • Meds: ø

  3. Case 20/30 -1.00+0.25x160  20/20 • VA sc 20/70 -0.50+0.25x056  NI • Motility: Full OU 21 • IOPT 21 • External/SLE: Unremarkable, except mild papillary reaction OU

  4. Differential Diagnosis • Anterior ischemic optic neuropathy • Optic neuritis (idiopathic, demyelinating, infectious) • Infectious optic neuropathy (sinusitis, syphilis, lyme disease) • Inflammatory optic neuropathy (sarcoidosis, SLE & other vasculitides) • Infiltrative optic neuropathy (leukemia, lymphoma) • Posterior scleritis • Compressive optic neuropathy • Optic disc drusen

  5. More Information… • History & ocular exam • Humphrey visual field • Imaging

  6. Scheduled for HVF; MRI + contrast/FLAIR sequence History: Next day: VA sc OS 20/400 Pain with upgaze Further probing…past episodes of diplopia & muscle weakness Ocular exam: 14/14 Color vision 1/14 75% red de-saturation OS Full CVF Constricted Pupils: 1.8 log APD OS More History & Exam…

  7. More Information… • History & ocular exam • Humphrey visual field • Imaging

  8. More Information… • History & ocular exam • Humphrey visual field • Imaging

  9. Imaging • MRI • Head  discontinued/limited study • 1.2cm hyperintense FLAIR signal in the corpus callosum • Non-specific finding: inflammatory, infectious, demyelinating plaque, or neoplastic lesion

  10. More Information… • History & ocular exam • Humphrey visual field • Imaging

  11. Optic Neuritis

  12. Definitions • Papillitis • More common in children • Post - or para -infectious; post -immunizations • Retrobulbar neuritis • More common in adults • Multiple sclerosis (MS) • Neuroretinitis • Least common • Viral infections; cat-scratch fever, syphilis, lyme dz

  13. Acute Demyelinating Optic Neuritis

  14. Epidemiology • Annual incidence: 5/100,000 • Prevalence: 115/100,000 • Age: 20-50 yrs • ♀ mean age: 30.2 (9-55yrs) • ♂ mean age: 31.1 (16-60yrs) • More common in ♀ • ♀:♂ 1.8:1 • Caucasians of northern European descent • Rare in Asians & Africans

  15. Demyelinating Diseases • Isolated optic neuritis • Multiple sclerosis (MS) • Devic dz (neuromyelitis optica) • Schilder dz

  16. Multiple Sclerosis • 70% of MS pts  evidence of optic neuritis (ON) • 1st manifestation in 20% • 1st episode of ON & nrl brain MRI  16% develop MS within 5 yrs • 1st episode of ON & ø signs of MS  50% with demyelinating lesions on brain MRI •  risk of developing clinical definite MS (CDMS) within 5-10 yrs • CDMS: 2 attacks > 24hrs, separated > 1 month, separate parts of the CNS + abnormal neurologic exam

  17. Pathophysiology • Autoreactive abs & T-Cells cross blood-brain barrier & damage myelin  demyelination • Genetic & environmental factors predispose to an autoimmune response • Genetic: HLA-Dw2; HLA-DR2 • Environmental: Infection, stress, systemic antigens & metabolites

  18. Pathophysiology • Early: • Myelin sheath loss • Preservation of axons • macrophages, lymphocytes & plasma cells • Late: • Loss of axons • Astrocytic proliferation  glial scar (plaque)

  19. Anatomy of Optic Neuritis • Optic nerve head: 45% • Retrobulbar: 61% • Intracanalicular: 34% • Intracranially (prechiasmatic): 5% • Chiasmatic: 2%

  20. Clinical Symptoms • 70% unilateral • Retrobulbar pain (53-88%) • Dull ache/sinus pain +/- globe tenderness • Esp. with EOM • Precedes visual symptoms • Subacute visual loss • Haze, cloud or dimness • Progresses over 2-7 days • < 20/60: 52% • 20/70-20/100: 48% • < 20/200: 38%

  21. Clinical Symptoms • Obscuration of vision in bright light • Dyschromatopsia • ALWAYS present •  vividness of saturated colors • Photopsias/phosphenes • Induced with horizontal EOM/loud noise

  22. Uthoff’s Phenomenon • 50% of cases of ON • Active or recovered • Transient obscuration of vision with body temp • Exercise • Hot bath/shower • Hot weather • Bad prognostic sign: •  presence of multifocal white matter lesions on brain MRI •  conversion to CDMS within 3.5 yrs •  recurrent ON

  23. Optic nerve dysfunction: Visual acuity Contrast sensitivity Stereo–acuity Visual field defects: Central 30° > altitudinal/arcuate > focal central/cecocentral scotomas Mild defects in fellow eye Dyschromatopsia Esp. for red APD Optic disc 64.7% nrl appearance +/- temporal disc pallor in fellow eye Other Findings: Peripheral retinal venous sheathing Uveitis Clinical Signs

  24. Optic Neuritis Treatment Trial • 15 centers in the U.S. (1988-92) • 457 pts: acute unilateral ON & ø MS • 18-46 yrs of age; 77% ♀; 85% caucasian • 3 treatment groups: • (1) IV methylprednisolone 250mg Q6hrs x 3 days  11 days PO prednisone (1mg/kg) • (2) PO prednisone (1mg/kg) x 14 days • (3) Placebo • Baseline gadolinium-enhanced MRI of brain/orbits • 1° visual outcome measures: visual acuity, color vision, contrast sensitivity & visual field; 2° outcome measure: development of CDMS

  25. ONTT • Effect of corticosteroids on speed & degree of visual recovery • PO steroids VS placebo: ø statistically significant difference in speed of visual recovery/degree of visual recovery at 6 months • IV steroids VS placebo: Faster visual recovery within first 2 wks; after 6 months ø difference in visual acuity between 3 treatment groups • Effect of corticosteroids on visual recovery • All pts showed improvement in vision within 1 month • Identification of factor(s) which may affect visual recovery • Degree of initial loss of vision best predictor of 6 month visual acuity outcome

  26. ONTT • Identification of side-effects of short-term use of corticosteroids • All pts reported sleep disturbances, mood changes, stomach upset, skin flushing & weight gain • IV steroid group: 1 case each of psychotic depression & acute pancreatitis • Visual Field Profile of pts with ON • Variable patterns • Chiasmal/retrochiasmal defects  76% with abnormal baseline MRI • 68.8% of fellow eyes with mild, but abnormal VF

  27. ONTT • Gadolinium - enhanced, T2-weighted brain/orbit MRI  likelihood of developing CDMS • 5-yr data, MS risk: • Ø lesions = 16% • 1-2 lesions = 37% • > 3 lesions = 51% • Effects of corticosteroids on development of MS • IV steroids:  risk of CDMS in pts with an abnormal MRI (> 2 white matter lesions) during first 2 yrs • Effect of corticosteroids on recurrent ON • PO steroids  rate of recurrent ON • 30% of pts: > 1 new episode of ON in either eye by 2nd yr; IV steroid group: 13%; placebo group: 16% •  recurrence in pts subsequently diagnosed with MS

  28. CHAMPS/ETOMS • CHAMPS: Controlled High-risk Subjects Avonex Multiple Sclerosis Prevention Study; ETOMS: Early Treatment of Multiple Sclerosis • Pts with 1st episode of clinical demyelinating syndrome + lesions on brain MRI associated with  risk for CDMS • CHAMPS: placebo VS IFN ß-1a (Avonex) 30mcg IM weekly x 18 months • ETOMS: placebo VS IFN ß-1a (Rebif) 22mcg SC weekly x 24 months • CHAMPS & ETOMS:  conversion (44% & 24%, respectively) to CDMS within 18 to 24 months in IFN ß-treated groups. •  # of new/enlarging MRI lesions •  time to occurrence of second relapse

  29. Recommendations • Typical acute monosymptomatic demyelinating ON • Gadolinium - enhanced MRI of brain/orbits to determine risk for CDMS • > 2 white matter lesions (> 3mm in diameter, >1 lesion periventricular/ovoid:) risk for CDMS • IV methylprednisolone 1gm/day x 3 days  oral prednisone (1mg/kg/day) x 11 days  4-day taper (20mg, then 10mg, then 0mg, then 10mg) • Avonex 30mcg IM weekly or Rebif 22mcg SC weekly • < 2 white matter lesions or pt with prior ON/known MS: use of IV methylprednisolone considered on an individual basis • Oral prednisone ALONE should be avoided

  30. THE IMPACT OF THE ONTT ON PRACTICES OF OPHTHALMOLOGISTS & NEUROLOGISTS • Trobe et al. The impact of the ONTT on the practices of ophthalmologists & neurologists. Ophthal. 1999; 106:2047-53

  31. Prognosis • Maximal visual recovery usually reached by 6 months • ONTT: + treatment • 1-yr VA: • > 20/40: 90% • 5-yr VA: • > 20/25: 87%; 20/25-20/40: 7%; 20/50-20/190: 3%; < 20/200: 3% • Abnormalities may be seen/perceived in other visual parameters despite return to normal acuity • ONTT • 63% of pts reported vision not recovered by 6 months • 80% -> 1-4 abnormal visual parameters • 20% -> all 4 visual parameters normal • A mild APD may remain

  32. Back to our patient… • Assessment: Acute optic neuritis • Plan: • 1gm IV solumedrol x 3 days  60mg PO prednisione daily x 11 days  PO taper • F/U 1 month  Neuro-ophthalmology: VA OS 20/3020/20; color vision 10/14; VF • Referred to Neurology (2 months later) • VA OS 20/20 • (+) paresthesias right torso; binocular diplopia; bilateral INO • LFT’s, ANA, ANCA, ESR, RF, Anti-DNA, Anti-SSA/SSB  negative • MRI (cervical & thoracic spine): Herniated disc; several T2 hyperintense signals throughout cervical spine consistent with MS • Diagnosis: Relapsing/remitting MS • Started on Rebif 44mcg SC 3x/wk

  33. HVF

  34. Take Home Points… • Classic triad: (1) loss of vision (2) eye pain (3) dyschromatopsia • Atypical ON ( visual loss progressing > 1 wk, vitritis, > 45 yrs of age, ø pain): work-up for another etiology • Typical cases & ø history of ON/MS: IV + PO steroids +/- IFN ß-1a • Anti-ulcer medication • Steroid-dependent optic neuropathies (neoplastic, paraneoplastic & inflammatory) worsen when off steroids; ø typical of ON

  35. Bibliography • BCSC. Neuro-ophthalmology. AAO. 2004-05 • BCSC. Pathology. AAO. 2004-05 • Yanoff. Ophthalmology, 2nd Ed. Mosby. 1263-66 • Kanski. Clinical Ophthalmology, 5th Ed. Butterworth Heinemann. 601-03. 2003 • E-medicine: Optic Neuritis • Beck RW, Cleary PA, Anderson MA, et al. A randomized, controlled trial of corticosteroids in the treatment of acute optic neuritis. N Engl J Med. 1992; 326:581–8. • Beck RW, Cleary PA, Backlund JC, et al. The course of visual recovery after optic neuritis: experience of the Optic Neuritis Treatment Trial. Ophthalmology. 1994; 101:1771–8. • Arnold AC. Visual field defects in the Optic Neuritis Treatment Trial: central vs. peripheral, focal vs. global. Am J Ophthalmol. 1999;128:632–4 • Beck RW, Kupersmith MJ, Cleary PA, et al. Fellow eye abnormalities in acute unilateral optic neuritis: experience of the Optic Neuritis Treatment Trial. Ophthalmology. 1993;100:691–8. • Beck RW, Cleary PA, Trobe JD, et al. The effect of corticosteroids for acute optic neuritis on the subsequent development of multiple sclerosis. N Engl J Med. 1993;329:1764–9. • Cleary PA, Beck RW, Bourque LB, et al. Visual symptoms after optic neuritis: results from the Optic Neuritis Treatment Trial. J Neuroophthalmol. 1997; 17:18–28. • Trobe JD, Sieving PC, Guire KE, et al. The impact of the Optic Neuritis Treatment Trial on the practices of ophthalmologists and neurologists. Ophthalmology. 1999;106:2047–53. • Jacobs LD, Beck RW, Simon JH, et al. Intramuscular interferon β-1a therapy initiated during a first demyelinating event in multiple sclerosis. N Engl J Med. 2000;343:898–904. • CHAMPS Study Group. Interferon β-1a for optic neuritis patients at high risk for multiple sclerosis. Am J Ophthalmol. 2001;132:463–71 • Comi G, Filippi M, Barkhof F, et al. Effect of early interferon treatment on conversion to definite multiple sclerosis: a randomized study. Lancet. 2001;357: 1576–82.

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