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So What’s the problem?
The problem is:
• There is a misdiagnosis in 25% of cases of
optic nerve disease on presentation with
young people being assumed to have Optic
Neuritis and older people AION
• There is no treatment for these conditions –
observation only. So there will be a delay in
initiating appropriate treatment if the
neuropathy is due to something else
Why does this happen?
Because we take a short
history, have a look then fit
what we have heard and seen
(with necessary perceptual
modification) to one of our
stock diagnoses.
Three common Optic
Neuropathies
1. Glaucoma
2. Optic neuritis
3. Ischaemic optic neuropathy
Glaucoma
• Typical History:
– None. Patients rarely present because of
symptoms of glaucoma
Glaucoma
• Typical Examination:
– Visual acuity is normal
– Colour testing is normal
– External examination is normal ie no proptosis,
ptosis etc
– Pupils are normal
– Disc changes are “glaucomatous” ie increased cd
ratio, not ISNT, notch, haem loss of nerve fibre
layer, and areas of preserved rim are not pale
Glaucoma
• Typical Perimetry
– Field loss crosses vertical midline and stops at
horizontal midline
– Defect matches disc changes
Ok So this is glaucoma
This probably is
This too even though IOP is low
• A 72 year old incidentally
noted to have a field
defect on routine testing
by his optometrist
• VA R&L 6/6
• Colour vision normal
• IOP 14mmHg R&L
• R disc cupped
• Glaucomatous field defect
LTG because
• Asymptomatic
• Colour vision normal
• Characteristic disc changes without pallor of
remaining nerve tissue
• Field defect matched disc changes
But this isn’t
• 63 year old investment
banker presents having
noticed slow reduction in
right vision
• VA 6/6 OU
• IOP R 29mmHg L
27mmHg
• Ishihara down on right
• Diffuse cupping of right
disc. Cd ratio 0.6 Pale rim
• Left disc looks normal CD
ratio 0.4
Not glaucoma because:
• Patient complained of progressive loss of vision
• Colour vision down
• Optic disc pallor
• Field did not match disc
• NB Diagnosed as glaucoma. After 2 years of
progressive vision loss a pituitary tumour was
found
Optic Neuritis
• Typical History:
– Age: 15 to 45 male or female
– Otherwise well
– Unilateral
– Acute onset worsening over hours to days
– Pain..worse on eye movement
– No diplopia or other neurological signs
Optic Neuritis
• Typical Examination
– No proptosis, ptosis, eye movements normal
– VA and colour vision down unilaterally
– RAPD
– Disc normal or mildly swollen…no disc pallor
– No signs of vasculitis or intraocular inflammation
– Other eye normal
Optic Neuritis
• Typical Perimetry
– Anything goes in either eye
• Symptoms stop getting worse within 2 weeks
and start to improve within 4 weeks
For example
• A 29 year old computer
programmer presents
with progressive loss of
right vision in her left eye.
Started 4 days ago and
has stabilised.
• Pain on eye movement
• VA R 6/6 L 6/18
• RAPD
• Colour vision down on
left
• Right disc normal Left disc
swollen
Probably optic neuritis because
• History
– Age 15 to 45
– Otherwise well
– Unilateral symptoms
– Rapid onset of symptoms
– Eye or orbital pain
– No other neurological symptoms
• Examination
– No orbital signs
– RAPD
– Disc normal or mildly swollen
– No disc pallor
– No signs of intraocular inflammation or vasculitis
– Other eye normal
This isn’t
• A 22yr old medical student
presents with a 6 day
history of painless subacute
loss of vision in her left eye.
• VA R 6/5 L 6/18
• Ishihara R 13/13 L 6/13
• Left RAPD
• Discs look normal
Advised that things should get better and routine
appointment made with a neurologist (for a few
months later)
6 weeks later represented with worse
vision and a swollen disc.
MRI showed a diffuse mass infiltrating the
optic nerve which turned out to be a
lymphoma
Not typical Optic neuritis because of:
• absence of pain
• slow onset of symptoms
• progression of symptoms beyond 2 weeks
Ischaemic optic neuropathy
• Typical History
– Age 40 or over male or female
– Vascular risk factors present OR symptoms of giant
cell arteritis
– One eye affected
– Acute onset and no improvement over time
– No pain
– No diplopia
Ischaemic optic neuropathy
• Typical Examination
– Markedly reduced VA and colour saturation
– No orbital or neurological signs (proptosis, ptosis,
abnormal eye movements)
– RAPD
– Mild to severe disc swelling: hyperaemic or pale
swelling, sectorial or diffuse
For example
• A 52 year old truck driver
presents with sudden
painless loss of right
vision while driving. No
change since onset of
symptoms
• VA R 6/36 L 6/6
• RAPD
• Disc swollen
• Left disc small, crowded
• No meds but smokes
Clinically AION Because:
• History
– Over 40
– Vascular risk factor present
– Unilateral
– Very sudden onset
– Painless
• Examination
– No orbital signs
– RAPD
– Diminished colour vision
– No other intraocular disease
– Swollen disc
– Other eye “Disc at RisK” but otherwise normal
But not this time!
• 46 year old accountant
presents with progressive
loss of right vision over 2
weeks
• VA R 6/18 L 6/6
• RAPD
• Colour vision down on the
right
• Disc is moderately swollen
• History of hypertension,
hypercholesterolaemia and
a family history of heart
disease
Not AION Because:
• Onset slow
• On further questioning there was an 8 week
history of night sweats and lethargy and on
further examination cervical
lymphadenopathy and a history of a cat
scratch
QUIZ TIME
Quiz 1
• A 20 year old newlywed is referred by ENT
colleague to have her pressures checked
because of headaches (having found no sinus
disease).
• Overweight but otherwise well with no visual
symptoms.
Visual acuity 6/6 OU
Pupils normal
IOP 16mmHg OU
Colour vision: some errors on Ishihara
Discs: Pharque! Look at that!
What is the diagnosis?
1. Optic neuritis
2. Ischaemic optic neuropathy
3. Acute domestic bliss
4. Other
Answer
• D. Other
Quiz 2
A 75 yr old man presents with acute
loss of vision in one eye.
Usually well but off colour lately and
not eating.
Bad headache for two weeks.
Had a fall a few months ago and
sustained fractured orbit
Visual acuity Right eye PL. Left eye 6/6.
IOP 16mmHg OU. Right APD.
Other signs
What is diagnosis?
1. Ischaemic optic neuropathy
2. Traumatic optic neuropathy
3. Optic neuritis
4. Pituitary tumour
Answer
• 1. ION
Quiz 3
A 70 yr old lady presents for glasses
check.
Incidentally found to have IOP of 48
mmHg right and 28 mmHg left.
VA 6/12 OU
Pupils reacting normally
Colour vision normal
Ant seg photo Right eye
Discs
Fields
What is the diagnosis?
1. CVA
2. Ischaemic optic neuropathy.
3. Open angle glaucoma.
4. Closed angle glaucoma
Answer
• 3 or 4

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Common Optic Neuropathies

  • 1. So What’s the problem?
  • 2. The problem is: • There is a misdiagnosis in 25% of cases of optic nerve disease on presentation with young people being assumed to have Optic Neuritis and older people AION • There is no treatment for these conditions – observation only. So there will be a delay in initiating appropriate treatment if the neuropathy is due to something else
  • 3. Why does this happen? Because we take a short history, have a look then fit what we have heard and seen (with necessary perceptual modification) to one of our stock diagnoses.
  • 4. Three common Optic Neuropathies 1. Glaucoma 2. Optic neuritis 3. Ischaemic optic neuropathy
  • 5. Glaucoma • Typical History: – None. Patients rarely present because of symptoms of glaucoma
  • 6. Glaucoma • Typical Examination: – Visual acuity is normal – Colour testing is normal – External examination is normal ie no proptosis, ptosis etc – Pupils are normal – Disc changes are “glaucomatous” ie increased cd ratio, not ISNT, notch, haem loss of nerve fibre layer, and areas of preserved rim are not pale
  • 7. Glaucoma • Typical Perimetry – Field loss crosses vertical midline and stops at horizontal midline – Defect matches disc changes
  • 8. Ok So this is glaucoma
  • 10. This too even though IOP is low • A 72 year old incidentally noted to have a field defect on routine testing by his optometrist • VA R&L 6/6 • Colour vision normal • IOP 14mmHg R&L • R disc cupped • Glaucomatous field defect
  • 11. LTG because • Asymptomatic • Colour vision normal • Characteristic disc changes without pallor of remaining nerve tissue • Field defect matched disc changes
  • 12. But this isn’t • 63 year old investment banker presents having noticed slow reduction in right vision • VA 6/6 OU • IOP R 29mmHg L 27mmHg • Ishihara down on right • Diffuse cupping of right disc. Cd ratio 0.6 Pale rim • Left disc looks normal CD ratio 0.4
  • 13. Not glaucoma because: • Patient complained of progressive loss of vision • Colour vision down • Optic disc pallor • Field did not match disc • NB Diagnosed as glaucoma. After 2 years of progressive vision loss a pituitary tumour was found
  • 14. Optic Neuritis • Typical History: – Age: 15 to 45 male or female – Otherwise well – Unilateral – Acute onset worsening over hours to days – Pain..worse on eye movement – No diplopia or other neurological signs
  • 15. Optic Neuritis • Typical Examination – No proptosis, ptosis, eye movements normal – VA and colour vision down unilaterally – RAPD – Disc normal or mildly swollen…no disc pallor – No signs of vasculitis or intraocular inflammation – Other eye normal
  • 16. Optic Neuritis • Typical Perimetry – Anything goes in either eye • Symptoms stop getting worse within 2 weeks and start to improve within 4 weeks
  • 17. For example • A 29 year old computer programmer presents with progressive loss of right vision in her left eye. Started 4 days ago and has stabilised. • Pain on eye movement • VA R 6/6 L 6/18 • RAPD • Colour vision down on left • Right disc normal Left disc swollen
  • 18. Probably optic neuritis because • History – Age 15 to 45 – Otherwise well – Unilateral symptoms – Rapid onset of symptoms – Eye or orbital pain – No other neurological symptoms
  • 19. • Examination – No orbital signs – RAPD – Disc normal or mildly swollen – No disc pallor – No signs of intraocular inflammation or vasculitis – Other eye normal
  • 20. This isn’t • A 22yr old medical student presents with a 6 day history of painless subacute loss of vision in her left eye. • VA R 6/5 L 6/18 • Ishihara R 13/13 L 6/13 • Left RAPD • Discs look normal
  • 21. Advised that things should get better and routine appointment made with a neurologist (for a few months later) 6 weeks later represented with worse vision and a swollen disc. MRI showed a diffuse mass infiltrating the optic nerve which turned out to be a lymphoma
  • 22. Not typical Optic neuritis because of: • absence of pain • slow onset of symptoms • progression of symptoms beyond 2 weeks
  • 23. Ischaemic optic neuropathy • Typical History – Age 40 or over male or female – Vascular risk factors present OR symptoms of giant cell arteritis – One eye affected – Acute onset and no improvement over time – No pain – No diplopia
  • 24. Ischaemic optic neuropathy • Typical Examination – Markedly reduced VA and colour saturation – No orbital or neurological signs (proptosis, ptosis, abnormal eye movements) – RAPD – Mild to severe disc swelling: hyperaemic or pale swelling, sectorial or diffuse
  • 25. For example • A 52 year old truck driver presents with sudden painless loss of right vision while driving. No change since onset of symptoms • VA R 6/36 L 6/6 • RAPD • Disc swollen • Left disc small, crowded • No meds but smokes
  • 26. Clinically AION Because: • History – Over 40 – Vascular risk factor present – Unilateral – Very sudden onset – Painless
  • 27. • Examination – No orbital signs – RAPD – Diminished colour vision – No other intraocular disease – Swollen disc – Other eye “Disc at RisK” but otherwise normal
  • 28. But not this time! • 46 year old accountant presents with progressive loss of right vision over 2 weeks • VA R 6/18 L 6/6 • RAPD • Colour vision down on the right • Disc is moderately swollen • History of hypertension, hypercholesterolaemia and a family history of heart disease
  • 29. Not AION Because: • Onset slow • On further questioning there was an 8 week history of night sweats and lethargy and on further examination cervical lymphadenopathy and a history of a cat scratch
  • 31. Quiz 1 • A 20 year old newlywed is referred by ENT colleague to have her pressures checked because of headaches (having found no sinus disease). • Overweight but otherwise well with no visual symptoms.
  • 32.
  • 33. Visual acuity 6/6 OU Pupils normal IOP 16mmHg OU Colour vision: some errors on Ishihara
  • 35. What is the diagnosis? 1. Optic neuritis 2. Ischaemic optic neuropathy 3. Acute domestic bliss 4. Other
  • 37. Quiz 2 A 75 yr old man presents with acute loss of vision in one eye. Usually well but off colour lately and not eating. Bad headache for two weeks. Had a fall a few months ago and sustained fractured orbit
  • 38. Visual acuity Right eye PL. Left eye 6/6. IOP 16mmHg OU. Right APD.
  • 40.
  • 41. What is diagnosis? 1. Ischaemic optic neuropathy 2. Traumatic optic neuropathy 3. Optic neuritis 4. Pituitary tumour
  • 43. Quiz 3 A 70 yr old lady presents for glasses check. Incidentally found to have IOP of 48 mmHg right and 28 mmHg left. VA 6/12 OU Pupils reacting normally Colour vision normal
  • 44. Ant seg photo Right eye
  • 45. Discs
  • 47. What is the diagnosis? 1. CVA 2. Ischaemic optic neuropathy. 3. Open angle glaucoma. 4. Closed angle glaucoma