13.10  Neuro-Ophthalmology

ONTT [xcix,c]

18-46 years old
Acute unilateral ON
Visual symptoms ≤ 8/7
RAPD and VF defect
No previous ON
No previous steroid treatment
No systemic disease (other than MS) that could account for this presentation

IVMP – accelerates visual recovery, but no long-term VA benefits

po prednisone – may increase number of new attacks

MS risk – Caucasian, young adult, recurrent ON, female, prior history neurological symptoms, brain MRI lesions, spine MRI lesions, CSF oligoclonal bands

ON risk:

  • 50% risk of MS at 15yrs
  • Lesions on MRI = risk 75%
  • No lesions on MRI = risk 25%

1991. The Clinical Profile of Optic Neuritis: Experience of the Optic Neuritis Treatment Trial. Archives of Ophthalmology 109(12) 1673-1678.

Beck, R.W.M.D., Cleary, P.A.M.S., Anderson, M.M.J.P.A.C. et al. 1992. A Randomized, Controlled Trial of Corticosteroids in the Treatment of Acute Optic Neuritis. The New England Journal of Medicine 326(9) 581-588.


Controlled High Risk Avonex MS Prevention Study


10 year results

If 1st episode ON & ≥ 2 MRI lesions, give the ONTT regimen, then IM IFNβ-1a weekly to reduce risk of MS from 50% to 33% at 3 years. If low risk give ONTT regimen

10 year results: 40% reduction in CDMS if treated immediately with Avonex i.e. Early treatment of MS reduces long term morbidity

Galetta, S. 2002. The controlled high risk Avonex multiple sclerosis trial (CHAMPS Study). Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society 21 292-295.

ETOMS [ciii]

Early Treatment of MS

Beta interferon weekly 2 years decrease risk progression to CDMS if given for

  • 1 episode of neurological dysfunction +
  • 1MRI lesion

(beta interferon alternate days also decreased conversion to CDMS)

Comi, G., Filippi, M., Barkhof, F. et al. 2001. Effect of early interferon treatment on conversion to definite multiple sclerosis: a randomised study. Lancet 357(9268) 1576-1582.

IONTS [civ]

International Optic Nerve Trauma Study

IV steroids or surgical decompression does not improve outcome in traumatic optic neuropathy

  • Results limited by small sample size, delay in treatment and a tendency to operate and/or give high dose steroid to patients with very low vision (i.e. NPL) and direct optic nerve injury

Levin, L.A., Beck, R.W., Joseph, M.P. et al. 1999. The treatment of traumatic optic neuropathy: the International Optic Nerve Trauma Study. Ophthalmology 106(7) 1268-1277.

CRASH [cv]

Corticosteroid Randomisation

After Significant Head Injury

Increased all-cause mortality at 30 days with IV methylprednisolone if significant head injury (GCS < 14)

NASCIS [cvi]

National Acute Spinal Cord Injury Study

Spinal cord injury patients with high dose IV methylprednisolone (30mg/kg bolus then infusion) within 8 hours of injury had better motor/sensory outcomes

Young W. NASCIS. National Acute Spinal Cord Injury Study. J Neurotrauma. 1990 Fall;7(3):113-4.

NASCET [cvii]

North American Symptomatic Carotid Endarterectomy Trial

  • Stenosis 70 - 99% and symptomatic = surgery (reduced stroke risk at 2 years from 26% to 9% and major/fatal stroke from 13% to 2.5%)
  • Stenosis 50 - 69% and symptomatic = maybe surgery
  • 100% stenosis = no surgery
  • Antiplatelets and statins

Ferguson GG, Eliasziw M, Barr HW et al. The North American Symptomatic Carotid Endarterectomy Trial. Stroke. 1999 Sep;30(9):1751-8.

IIHTT [cviii]

Idiopathic Intracranial Hypertension Treatment Triald

  • Low sodium weight reduction plus maximally tolerated acetazolamide (up to 4g/day) or placebo for 6 months in IIH (and mild visual loss)
  • Acetazolamide gives:
    • Greater improvement in VF mean deviation
    • More weight loss

GiACTA [cix]

Design of the Tocilizumab in Giant Cell Arteritis Trial

  • Tocilizumab weekly or every other week combined with 26-week prednisone taper superior to 26-week or 52-week prednisone taper plus placebo with regard to sustained glucocorticoid-free remission in patients with GCA
  • At 52 weeks 56% with weekly tocilizumab sustained remission compared to 14% in placebo group

Stone, J.H., Tuckwell, K., Dimonaco, S. et al. 2017. Trial of Tocilizumab in Giant-Cell Arteritis. New England Journal of Medicine 377(4) 317-328.


Rescue of Hereditary Optic Disease Outpatient Study

  • Idebenone 900mg/day to minimise vision loss in LHON patients
  • Effect most pronounced in patients with discordant visual acuities
  • Minimal side effect profile
  • n = 85

Klopstock, T., Yu-Wai-Man, P., Dimitriadis, K. et al. 2011. A randomized placebo-controlled trial of idebenone in Leber's hereditary optic neuropathy. Brain : a journal of neurology 134(Pt 9) 2677-2686.

Steroids for NAAION [cxi]

Atropine for the Treatment of Myopia (ATOM) 1

  • Oral Prednisolone treatment initiated within 2-weeks of symptoms/onset
  • n = 696 eyes, 364 eyes opted to receive oral prednisolone, 332 opted for no treatment
  • At 6 months from onset of NAAION: visual acuity improvement in 69.8% in the treated group vs. 40.5% in the untreated group (p = 0.001)
  • Visual field defect at 6 months from onset of NAAION - improvement in 40.1% of the treated group and 24.5% of the untreated group (p = 0.005)
  • Both groups visual acuities and visual fields did not improve significantly after 6 months

Hayreh, S.S. and Zimmerman, M.B. 2008. Non-arteritic anterior ischemic optic neuropathy: role of systemic corticosteroid therapy. Graefes Arch Clin Exp Ophthalmol 246(7) 1029-1046.


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