Associated
with other disorders
DDx:
• Inflammatory (optic neuritis)
• Vascular – Ischaemic optic neuropathy
• Compressive/Infiltrative
• Hereditary
o Isolated
o Syndromic
• Toxic/nutritional
• Traumatic
• Raised ICP
• Glaucoma
• Anomalous optic nerve
See Optic Neuritis
See GCA and PMR
• Occlusion of the small vessels supplying the anterior portion of the optic nerve
• Exact cause is still controversial
• Often likely to be caused by a compartment syndrome of the anterior disc
• Small crowded disk (Disk at risk)
• Optic nerve head drusen
• Anomalous disk
• Severe papilloedema
• Severe hypotension
• Severe anaemia
• Hypercoaguable disorders
• Radiation
• Acute intraocular hypertension
• Association with standard vascular risk factors
• No proven treatment
• Control vascular risk factors
• Avoid triggers that could affect the other eye – in particular avoid hypotension
• Aspirin trials equivicol – probably no harm
• Steroid trials negative (although controversial – some argue minor improvement)
• Operative decompression trialled – no benefit
• Typically altitudinal or arcuate defect inferiorly
• Rare (Arteritic PION is much more common))
• Presumed risk factors:
• Severe hypotension
• Severe anaemia
• Hypercoaguable disorders
• Radiation
• Painless, progressive visual loss
• Usually pale nerve (sometimes swollen)
Surgery is appropriate if there is ongoing compression (e.g. from bone fragment)
IONTS – steroids in traumatic optic neuropathy trial
No evidence of effectiveness
Particularly avoid if concurrent head injury (increased mortality in CRASH trial)
• Optic nerve sheath meningioma
• Sphenoid wing meningioma
• Pituitary tumours
• Craniopharyngioma
• Intraorbital tumour
Thyroid eye disease
Orbital pseudotumour
• Orbital haemorrhage
• Ophthalmic artery aneurysm
• Internal artery compression
• Fibrous dysplasia
• Paget disease
• Optic nerve glioma
• Metastasis
• Lymphoma
• Meningeal carcinomatosis
Sarcoidosis
• Progressive, symmetrical, central visual lsoss
• Disc often normal or slight swelling
• Progressive, bilateral, symmetica, central visual loss
• Dyschromatopsia
• Cecocentral scotomas
• Normal or pale nerve (often pale temporal first)
• B12 deficiency
• Tobacco use (Cigars >cigarettes)
• Methanol
• Ethylene glycol
• Medications
o Ethambutol (Dose related , early dyschromatopsia)
o Amiodarone (Disc oedema, mimics AION)
o Linezolid,
o Disulfiram
• Organic solvents
• Lead
•
• Bilateral, sequential, painless central visual loss
• Male >> female, age 15-35
• Hyperaemic optic nerve acutely
• Cecocentral scotomas
• Pale optic nerve late
• Poor visual prognosis
• ECG – cardiac abnormalities
• Mitochondiral DNA mutation, maternal inheritance
• Bilateral slowly progressive painless visual loss
• Usually presents in childhood
• Autosomal dominant, somatic mutation for mitochondrial protein
• DIDMOAD
• Friedreich Ataxia
• Spinocerebellar ataxia
• Charcot-Marie Tooth (HSMN type VI)