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Michael Burdon
Queen Elizabeth Hospital Birmingham
Neuro-Ophthalmology:
First Contact
Declarations of Interest
• Financial: none
• Professional:
– President, Royal College of Ophthalmologists
– Consultant Ophthalmologist
– Chairman, NICE Cataract Guidelines
Outline
• Key features of the neuro-ophthalmic history
and examination
• Conditions that require immediate
investigation and treatment
Afferent Pathway: loss of vision
• Papilloedema
• Stroke
Afferent Visual Pathway
Afferent Pathway: History
• One or both eyes
• Nature of visual loss
– dimming
– field loss
– blurring
• Onset
– sudden
– gradual
– transient
• Associated symptoms
– pain
– double vision
– weakness/loss of sensation
• Past medical history
– neurological disease
– tumours
• FH/SH
• Medications
Afferent Pathway: History
• One or both eyes
• Nature of visual loss
– dimming
– field loss
– blurring
• Onset
– sudden
– gradual
– transient
• Associated symptoms
– pain
– double vision
– weakness/loss of sensation
• Past medical history
– neurological disease
– tumours
• FH/SH
• Medications
Afferent Pathway: Examination
• Function
– Visual acuity
– Pupils (RAPD)
– Colour vision
– Visual fields
• Structure
– Tear film
↓
– Optic disc
Pupil Reflex Pathway
Normal Pupil Response to Light
dark
light in L eye
light in R eye
R L
Left Optic Nerve Damage
Pupil Response to Light with Left Optic
Nerve Damage
dark
light in L eye
light in R eye
R L
Relative Afferent Pupil Defect
• Caused by optic nerve damage
• Not caused by:
– Cataracts
– Macular disease
– Neurological disease beyond the optic nerve
• Test everyone
The Visual Field
“All the space that one eye can see
at any given instant” (Tate and Lynn 1977)
Confrontation Visual Field Testing
• Gross screening test
• Able to detect
– Hemianopia
– Quadrantinopia
– Altitudinal field defect
Confrontation Visual Field Testing
X
X X
X
Confrontation Visual Field Testing
X
X X
X
Confrontation Visual Field Testing
X
X X
X
• Papilloedema
Papilloedema
• Optic disc swelling due to raised intracranial
pressure
• Brain abscess
• Meningitis
• Tumours
• Hydrocephalus
• Venous sinus thrombosis
• Subarachnoid haemorrhage
• ...
• IIH
Papilloedema - Aetiology
Papilloedema - Presentation
± Headache
± Diplopia
± Obscurations
± Photopsia
± Nausea / vomiting
± Pulsatile tinnitus
± Localising symptoms
Papilloedema Pathway
Casualty
Patient
Optom GP
Neurology
- Imaging
- Lumbar puncture
Papilloedema: First Contact
• Priority in A&E
• Doctor to confirm if disc swelling consistent with
papilloedema
• If possible arrange
– Perimetry
– OCT of optic discs (including peri-papillary RNFL)
• Refer to on call neurology team
• Stroke
NICE - Stroke Guidelines
• Admission to a specialist acute stroke unit
• Immediate imaging
• Treatment
– thrombolysis /clot retrieval + antiplatelet & anticoag (6 hours)
– decompressive hemicraniectomy + antiplatelet & anticoag
– intracerebral haemorrhage management
• Secondary stroke prevention & rehabilitation
NICE CG68 last revision May 2019
NHS “FAST” Campaign
Visual Loss?
Hemianopia - Thrombolysis
• 6 patients with posterior cerebral artery stroke
– 4 with hemianopia
• Intravenous thrombolysis (0.9mg/kg rtPA)
• Symptom onset to treatment 185 to 390 minutes
• Resolution of heminanopia in 3 out of 4 patients
Förster A et al. AJNR 2011 32(2):419-421
Hemianopia - Thrombolysis
• Three patients with homonymous hemianopia due to
posterior circulation stroke
• Intravenous thrombolysis following CT scan
• At six months:
– Two: normal Goldmann fields
– One: “modest” visual field defect
Strbian D, et al. Acta Neurol Scand 2012 126 e17-19
Stroke: First Contact
• Good history and confrontation field at A&E triage
• If stroke suspected:
– Immediate confirmation by A&E doctor
– Immediate transfer to stroke team
Efferent Pathway: double vision
• Third Cranial Nerve Palsy
Causes of Double Vision
Monocular
Diplopia
Binocular
Loss of fusionMotility Disorder
Vergence
Brainstem
Peripheral
N-M junction
Muscle/orbit
Optical
Macula
Cortical
Psychogenic
Sensory
Motor
Optical Diplopia
“Does the double vision persist if one or other eye is covered?”
Optical Diplopia
“Does the double vision persist if one or other eye is covered?”
Optical Diplopia: Effect of Pinhole
Diplopia: History
• Orientation (horizontal, vertical, torsional)
• Variation with eye position
• Onset
• Progression
• Constant/Intermittent/Variable
Diplopia: History
• Past medical history
– Ophthalmic
– Neurological
– Tumours
– Diabetes
– ….
• Medications
– Epileptics
– Statins
– Penicillamine
– ….
• Systems review
• Neurologic
– Headache
– Weakness
– ¯ Sensation
– …
• Ophthalmic
– Head position
– Proptosis
– Ptosis
– Anisocoria
– Pain / pain on eye movement
– Visual loss
Motility Disorders
Eye Movements
• Fixation
• Saccades
• Pursuit
• Vergence
• VOR/OKN
Localisation
• Vergence
• Brainstem
• Peripheral
• N-M junction
• Muscle/orbit
Fixation
• Examine patient’s ability to maintain fixation on
a target in both the primary position and
eccentric gaze
Fixation
• Examine patient’s ability to maintain fixation on
a target in both the primary position and
eccentric gaze
? Steady
? Nystagmus
Alignment
• Cover test (manifest deviation: tropia)
• Alternate cover test (latent deviation: phoria)
• Light or accommodative target?
• Near, distance, eccentric gaze?
Alignment
• Cover test (manifest deviation: tropia)
• Alternate cover test (latent deviation: phoria)
• Light or accommodative target?
• Near, distance, eccentric gaze?
Versions
MRLR
IOSR
SOIR
LRMR
SRIO
IRSO
Ductions
MRLR
IOSR
SOIR
LRMR
SRIO
IRSO
Ductions and Versions
• Torch (observe corneal reflexes)
• If limitation observed compare duction with
version
– Restrictive: duction = version
– Paralytic: duction > version
Saccades
• Two targets
– Horizontal
– vertical
? Slow - brain, nerve, muscle
? Hypo- or hypermetric – brainstem or
cerebellar disease
? Fatigue
Convergence
• Test with accommodative target or patient’s
finger
? Convergence > adduction – INO
Eye Movement Examination
• Visual function
• Pupils
• Cranial nerves II to VIII
• Fundoscopy
• +/- PNS examination
(additional neurology usually localises the
palsy)
Questions
• What is the eye movement abnormality?
– Unilateral imitation of abduction
– Bilateral failure of up-gaze
– …
• What could cause the observed eye movement
abnormality?
– Vergence
– Brainstem
– Cranial nerve
– Neuromuscular junction
– Muscle/orbit
Cranial Nerve
• III (SR, MR, IR, IO, LPS, Sphincter pupillae, ciliary body)
• IV (SO)
• VI (LR)
• Isolated?
• Localisable?
– V, VI, VII, & VIII – CPA angle
– II, III, IV, Va,b, VI, Horner's – Cavernous sinus / orbital apex
• Third Cranial Nerve Palsy
Causes of a Third Cranial Nerve Palsy
• Aneurysm
- Posterior communicating
- Cavernous carotid
- Basilar
• Microvascular
• Parasellar neoplasm
• …..
Third Cranial Nerve Palsy
• 10-56% of isolated non-traumatic III CN palsies
are due to Pcom or basilar artery aneurysms
• Issues:
– Aneurysm rupture - risk of death or severe
disability
– Recovery of palsy - early treatment is associated
with better chance of recovery
Aneurysm Rupture
• 40% die within the first 24 hours
• Further 25% die from complications within 6
months
• Remainder often have permanent neurological
disability
• III cranial palsy indicates that the aneurysm has
expanded and may rupture within hours
III CN Palsy due to Aneurysm
• Age range - children to elderly
• Any combination of external and internal
paresis except isolated ptosis and isolated
dilated pupil
• Pain does not discriminate between III CN
palsies due to aneurysms from those due to
ischaemia
Complete Third Cranial Nerve Palsy
Partial Third Cranial Nerve Palsy
Third Cranial Nerve Palsy: Management
• Immediate imaging
– CTA
– MRA
– Reported by an experienced radiologist
Third Cranial Nerve Palsy: First Contact
• There are many serious causes of double vision
• The first priority is to diagnose/rule out a third cranial
nerve palsy because of the risk of death due to an
underlying aneurysm
• Therefore, in A&E, prioritise all cases of binocular
double vision
Pupil Pathway: unequal pupils
• Horner’s syndrome
Iris Muscles
Sphincter Pupillae
Dilator Pupillae
Causes of Anisocoria
• Physiological
• Sympathetic denervation
– Horner’s syndrome
• Parasympathetic denervation
– III cranial nerve palsy
– Adie’s pupil
• Local ocular disease
• Pharmacological
Anisocoria - History
• How was the anisocoria first noticed?
– Associated with ophthalmological or neurological symptoms
– Incidental finding
• Duration
• Associated symptoms
– Ptosis
– Double vision
– Loss of vision
– Headache
– Numbness
– Weakness
Anisocoria - History
• Past ophthalmic history
– Trauma
– Surgery
• Past medical history
– Neck trauma (surgery)
– Cancer
• Medications
• Occupation
• Systems review
Anisocoria - Examination
1. Measure anisocoria in bright and dim light
2. Assess pupil light and near responses
3. Examine the iris and pupil on a slit lamp
4. Look for associated signs
– Ptosis
– Eye movements
Anisocoria
Abnormal
Reaction to light
Normal
Physiological
Horner’s
Ocular examination
Normal Abnormal
III CN palsy
Drugs
Adie’s
Ocular
Physiological Anisocoria
• 20% of population
• <0.5 mm
• Same in all illumination
• Varies / reverses with time
• Horner’s Syndrome
Horner’s Syndrome
• Miosis
• Ansiocoria increases in dark
• Pupil redilation lag
• 1 to 2 mm ptosis
• Elevation of lower lid
• Heterochromia if congenital / long duration
Horner’s Syndrome - Aetiology
• First neurone
- CVA
- Demyelinating disease
• Second neurone
- Neoplasia
- Trauma
• Third neurone
- Internal carotid artery disease*
- Basal skull tumours
Horner’s Syndrome: Apraclonidine
• Apraclonidine 0.5 -1%
– Alpha-2 Adrenergic agonist with weak alpha-1 agonist
activity
– Dilates Horner’s pupil and reduces ptosis – attributed to
denervation hypersensitivity (pre- & post-ganglionic)
– May not dilate recent onset Horner’s
– (not to be used in young children – risk of extreme
drowsiness/unresponsiveness)
Horner’s Syndrome - Investigation
• MRI head to upper thorax with contrast
• (urinary catecholamine – neuroblastoma in children)
Horner’s Syndrome: First Contact
• Patients with recent onset (days) of anisocoria, with
an ipsilateral ptosis (+/- ipsilateral facial pain) should
be assessed urgently for possible carotid artery
dissection.

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Neuro-ophthalmology – First Contact

  • 1. Michael Burdon Queen Elizabeth Hospital Birmingham Neuro-Ophthalmology: First Contact
  • 2. Declarations of Interest • Financial: none • Professional: – President, Royal College of Ophthalmologists – Consultant Ophthalmologist – Chairman, NICE Cataract Guidelines
  • 3. Outline • Key features of the neuro-ophthalmic history and examination • Conditions that require immediate investigation and treatment
  • 4. Afferent Pathway: loss of vision • Papilloedema • Stroke
  • 6. Afferent Pathway: History • One or both eyes • Nature of visual loss – dimming – field loss – blurring • Onset – sudden – gradual – transient • Associated symptoms – pain – double vision – weakness/loss of sensation • Past medical history – neurological disease – tumours • FH/SH • Medications
  • 7. Afferent Pathway: History • One or both eyes • Nature of visual loss – dimming – field loss – blurring • Onset – sudden – gradual – transient • Associated symptoms – pain – double vision – weakness/loss of sensation • Past medical history – neurological disease – tumours • FH/SH • Medications
  • 8. Afferent Pathway: Examination • Function – Visual acuity – Pupils (RAPD) – Colour vision – Visual fields • Structure – Tear film ↓ – Optic disc
  • 10. Normal Pupil Response to Light dark light in L eye light in R eye R L
  • 12. Pupil Response to Light with Left Optic Nerve Damage dark light in L eye light in R eye R L
  • 13. Relative Afferent Pupil Defect • Caused by optic nerve damage • Not caused by: – Cataracts – Macular disease – Neurological disease beyond the optic nerve • Test everyone
  • 14. The Visual Field “All the space that one eye can see at any given instant” (Tate and Lynn 1977)
  • 15. Confrontation Visual Field Testing • Gross screening test • Able to detect – Hemianopia – Quadrantinopia – Altitudinal field defect
  • 16. Confrontation Visual Field Testing X X X X
  • 17. Confrontation Visual Field Testing X X X X
  • 18. Confrontation Visual Field Testing X X X X
  • 20. Papilloedema • Optic disc swelling due to raised intracranial pressure
  • 21. • Brain abscess • Meningitis • Tumours • Hydrocephalus • Venous sinus thrombosis • Subarachnoid haemorrhage • ... • IIH Papilloedema - Aetiology
  • 22. Papilloedema - Presentation ± Headache ± Diplopia ± Obscurations ± Photopsia ± Nausea / vomiting ± Pulsatile tinnitus ± Localising symptoms
  • 24. Papilloedema: First Contact • Priority in A&E • Doctor to confirm if disc swelling consistent with papilloedema • If possible arrange – Perimetry – OCT of optic discs (including peri-papillary RNFL) • Refer to on call neurology team
  • 26. NICE - Stroke Guidelines • Admission to a specialist acute stroke unit • Immediate imaging • Treatment – thrombolysis /clot retrieval + antiplatelet & anticoag (6 hours) – decompressive hemicraniectomy + antiplatelet & anticoag – intracerebral haemorrhage management • Secondary stroke prevention & rehabilitation NICE CG68 last revision May 2019
  • 28. Hemianopia - Thrombolysis • 6 patients with posterior cerebral artery stroke – 4 with hemianopia • Intravenous thrombolysis (0.9mg/kg rtPA) • Symptom onset to treatment 185 to 390 minutes • Resolution of heminanopia in 3 out of 4 patients Förster A et al. AJNR 2011 32(2):419-421
  • 29. Hemianopia - Thrombolysis • Three patients with homonymous hemianopia due to posterior circulation stroke • Intravenous thrombolysis following CT scan • At six months: – Two: normal Goldmann fields – One: “modest” visual field defect Strbian D, et al. Acta Neurol Scand 2012 126 e17-19
  • 30. Stroke: First Contact • Good history and confrontation field at A&E triage • If stroke suspected: – Immediate confirmation by A&E doctor – Immediate transfer to stroke team
  • 31. Efferent Pathway: double vision • Third Cranial Nerve Palsy
  • 32. Causes of Double Vision Monocular Diplopia Binocular Loss of fusionMotility Disorder Vergence Brainstem Peripheral N-M junction Muscle/orbit Optical Macula Cortical Psychogenic Sensory Motor
  • 33. Optical Diplopia “Does the double vision persist if one or other eye is covered?”
  • 34. Optical Diplopia “Does the double vision persist if one or other eye is covered?”
  • 36. Diplopia: History • Orientation (horizontal, vertical, torsional) • Variation with eye position • Onset • Progression • Constant/Intermittent/Variable
  • 37. Diplopia: History • Past medical history – Ophthalmic – Neurological – Tumours – Diabetes – …. • Medications – Epileptics – Statins – Penicillamine – …. • Systems review • Neurologic – Headache – Weakness – ¯ Sensation – … • Ophthalmic – Head position – Proptosis – Ptosis – Anisocoria – Pain / pain on eye movement – Visual loss
  • 38. Motility Disorders Eye Movements • Fixation • Saccades • Pursuit • Vergence • VOR/OKN Localisation • Vergence • Brainstem • Peripheral • N-M junction • Muscle/orbit
  • 39. Fixation • Examine patient’s ability to maintain fixation on a target in both the primary position and eccentric gaze
  • 40. Fixation • Examine patient’s ability to maintain fixation on a target in both the primary position and eccentric gaze ? Steady ? Nystagmus
  • 41. Alignment • Cover test (manifest deviation: tropia) • Alternate cover test (latent deviation: phoria) • Light or accommodative target? • Near, distance, eccentric gaze?
  • 42. Alignment • Cover test (manifest deviation: tropia) • Alternate cover test (latent deviation: phoria) • Light or accommodative target? • Near, distance, eccentric gaze?
  • 45. Ductions and Versions • Torch (observe corneal reflexes) • If limitation observed compare duction with version – Restrictive: duction = version – Paralytic: duction > version
  • 46. Saccades • Two targets – Horizontal – vertical ? Slow - brain, nerve, muscle ? Hypo- or hypermetric – brainstem or cerebellar disease ? Fatigue
  • 47. Convergence • Test with accommodative target or patient’s finger ? Convergence > adduction – INO
  • 48. Eye Movement Examination • Visual function • Pupils • Cranial nerves II to VIII • Fundoscopy • +/- PNS examination (additional neurology usually localises the palsy)
  • 49. Questions • What is the eye movement abnormality? – Unilateral imitation of abduction – Bilateral failure of up-gaze – … • What could cause the observed eye movement abnormality? – Vergence – Brainstem – Cranial nerve – Neuromuscular junction – Muscle/orbit
  • 50. Cranial Nerve • III (SR, MR, IR, IO, LPS, Sphincter pupillae, ciliary body) • IV (SO) • VI (LR) • Isolated? • Localisable? – V, VI, VII, & VIII – CPA angle – II, III, IV, Va,b, VI, Horner's – Cavernous sinus / orbital apex
  • 51. • Third Cranial Nerve Palsy
  • 52. Causes of a Third Cranial Nerve Palsy • Aneurysm - Posterior communicating - Cavernous carotid - Basilar • Microvascular • Parasellar neoplasm • …..
  • 53. Third Cranial Nerve Palsy • 10-56% of isolated non-traumatic III CN palsies are due to Pcom or basilar artery aneurysms • Issues: – Aneurysm rupture - risk of death or severe disability – Recovery of palsy - early treatment is associated with better chance of recovery
  • 54. Aneurysm Rupture • 40% die within the first 24 hours • Further 25% die from complications within 6 months • Remainder often have permanent neurological disability • III cranial palsy indicates that the aneurysm has expanded and may rupture within hours
  • 55. III CN Palsy due to Aneurysm • Age range - children to elderly • Any combination of external and internal paresis except isolated ptosis and isolated dilated pupil • Pain does not discriminate between III CN palsies due to aneurysms from those due to ischaemia
  • 56. Complete Third Cranial Nerve Palsy
  • 57. Partial Third Cranial Nerve Palsy
  • 58. Third Cranial Nerve Palsy: Management • Immediate imaging – CTA – MRA – Reported by an experienced radiologist
  • 59. Third Cranial Nerve Palsy: First Contact • There are many serious causes of double vision • The first priority is to diagnose/rule out a third cranial nerve palsy because of the risk of death due to an underlying aneurysm • Therefore, in A&E, prioritise all cases of binocular double vision
  • 60. Pupil Pathway: unequal pupils • Horner’s syndrome
  • 62. Causes of Anisocoria • Physiological • Sympathetic denervation – Horner’s syndrome • Parasympathetic denervation – III cranial nerve palsy – Adie’s pupil • Local ocular disease • Pharmacological
  • 63. Anisocoria - History • How was the anisocoria first noticed? – Associated with ophthalmological or neurological symptoms – Incidental finding • Duration • Associated symptoms – Ptosis – Double vision – Loss of vision – Headache – Numbness – Weakness
  • 64. Anisocoria - History • Past ophthalmic history – Trauma – Surgery • Past medical history – Neck trauma (surgery) – Cancer • Medications • Occupation • Systems review
  • 65. Anisocoria - Examination 1. Measure anisocoria in bright and dim light 2. Assess pupil light and near responses 3. Examine the iris and pupil on a slit lamp 4. Look for associated signs – Ptosis – Eye movements
  • 66. Anisocoria Abnormal Reaction to light Normal Physiological Horner’s Ocular examination Normal Abnormal III CN palsy Drugs Adie’s Ocular
  • 67. Physiological Anisocoria • 20% of population • <0.5 mm • Same in all illumination • Varies / reverses with time
  • 69. Horner’s Syndrome • Miosis • Ansiocoria increases in dark • Pupil redilation lag • 1 to 2 mm ptosis • Elevation of lower lid • Heterochromia if congenital / long duration
  • 70. Horner’s Syndrome - Aetiology • First neurone - CVA - Demyelinating disease • Second neurone - Neoplasia - Trauma • Third neurone - Internal carotid artery disease* - Basal skull tumours
  • 71. Horner’s Syndrome: Apraclonidine • Apraclonidine 0.5 -1% – Alpha-2 Adrenergic agonist with weak alpha-1 agonist activity – Dilates Horner’s pupil and reduces ptosis – attributed to denervation hypersensitivity (pre- & post-ganglionic) – May not dilate recent onset Horner’s – (not to be used in young children – risk of extreme drowsiness/unresponsiveness)
  • 72. Horner’s Syndrome - Investigation • MRI head to upper thorax with contrast • (urinary catecholamine – neuroblastoma in children)
  • 73. Horner’s Syndrome: First Contact • Patients with recent onset (days) of anisocoria, with an ipsilateral ptosis (+/- ipsilateral facial pain) should be assessed urgently for possible carotid artery dissection.