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Neuro-ophthalmic
Emergencies
Raed Behbehani , MD FRCSC
History
• Loss of vision
• Diplopia
• Eye pain
• Eyelid drooping (ptosis)
Key Aspects of the
Examination
• Visual acuity
• Color vision Pupils : size and reaction in light
and dark
• Extraocular motility
• Lids
• Visual fields
Ishihara color plates
Pupils
Relative afferent pupillary defect
Direct pupil reflex
Anisocoria
Extra-ocular Motility
Testing
Eyelids
MRD1 and MRD2 Levator function assessment
Visual Field Testing
Vision Loss
• Monoculr or binocular
• Key is localization (refractive
, retina or optic nerve)
• Visual acuity assessment
with pinhole. (refractive)
• Look for RAPD .
• Metamorphopsia .
• Visual fields.
Case
• A 70 year old woman with sudden loss of
vision in the right eye.
• Transient loss of vision and jaw pain.
• Feeling unwell lately with, and loss of
appetite ( 10 Kg) , malaise and myalgias.
• Hypertension on Metoprolol.
• Visual acuity: Count finger right , 20/30 left.
• Pupils : Right RAPD.
Case
Laboratory Investigations
• ESR = 86
• CRP positive.
• Platelets elevated ( 560).
• Mildly anemic.
Temporal Arteritis
• Systemic vasculitis (Aortitis in 20% consider
PET/MRA).
• New onset of headache (temporal) , acute or
transient loss of vision, jaw claudication, weight loss,
fever, and myalgias.
• Age usually over 60.
• Occult GCA ( No systemic symptoms, transient
diplopia or transient visual loss).
• A true neuro-ophthalmic emergency (54-95%
second eye involvement if untreated) !
AAION in Temporal
Arteritis
GCA Visual loss
Management
• Stat ESR , CRP and CBC (platelets).
• CRP and CBC have 97% sensitivity and specificity.
• Start high dose systemic steroids (IV or Oral)
immediately upon suspicioun ( AAION or CRAO
can develop in fellow eye within days if untreated !)
• Arrange for temporal artery biopsy within 2 weeks ,
while patient is on steroids.
Case
• 52-year-old previously healthy presents
with severe headache and blurred vision in
both eyes.
• Visual acuity 20/80 OD and 20/60 OS.
• Confrontation visual fields : Bitemporal
Hemianopia.
Visual Fields
Visual Field Defects in
Chiasmal Syndrome
MRI
Pituitary mass with high signal on T1
Pituitary Apoplexy
• “Worst headache in my life”.
• Visual field loss, and/or ophthalmoplegia ( uni- or
bilateral).
• Patients usually present 2 weeks after ictus.
• > 80% did not have history of pituitary tumor
• Life threatening (hypotension, shock) because of
hypo-pituitarism, and low cortisol levels, and
diabetes insipidus.
Case
• A 52 year old man with blurred vision
and mild headache for the last 6 weeks.
• Headaches are severe 10/10 scale ,
worse in the morning and leaning
forward.
• Visual acuity : 20/20 OU
Fundus Examination
OCT
Visual Felds
Papilledema
• Bilateral disc edema due to raised ICP.
• Normal visual acuity.
• Visual fields : enlarged blind spots
(early)
Malignant
Hypertesnion• Accelerated hypertension with target
organ damage.
• Papilledema must be present for
diagnosis !
• Dysfunction of cerebral blood flow
autoregultaion causing cerebral edema.
• Pre-eclampsia .
• Encephalopathy can be present.
Evaluation of Diplopia
• Mono-ocualr or bin-ocular .
• Orientation of images (vertical , horizontal , oblique)
• Associated signs and symptoms (pain , loss of vision)
• Follow-my-finger exam may not be enough !
• Maddox-rod or cover-uncover test .
• Always check the eyelid and pupil !
Case
• 78 year old man with acute diplopia,
and headache.
• Headache and nausea .
• Diabetes, hypertension, atrial
tachycardia.
• Limitation in adduction , elevation and
depression in the right eye.
Pupil-involving 3rd Nerve
Palsy
• Posterior communicating artery
aneurysm, or mass.
• Appropriate neuro-imaging is
(MRI/MRA, MRI/CTA, Angiogram is the
gold standard for aneurysm detection).
• CTA is better for detecting aneurysms.
• MRI is better to rule out masses .
Risk of Aneurysm and
“Rule of Pupil”
Ophthalmoplegia Pupil Aneurysm Risk
Complete/Partial Complete 86%-100%
Partial Spared 30%
Complete Spared very low
If signs of sub-arachnoid hemorrhage present (headache, photophobia,
Anisocoria
• Acute onset vs acute awareness .
• Associated symptoms (headache , neck pain , eye
pain)
• Is the anisocoria worse in the dark or light ?
• Pharmacologic testing and neuro-imaging aid in
localization.
• A 67 year old man presents with pain in his right eye
for 5 days associated with neck pain after
chiropractic treatment.
• Hypertension and ischemic heart disease on
treatment.
• No double vision.
• VA : 20/30 OU.
• Right partial ptosis (1 mm with right pupil smaller
then left more in dark than light)
Case
Case
Evaluation of
Horner’s• Misois, and ptosis (upper and lower lid).
• Dilatation lag, anisocoria worse in dark.
• Topical Cocaine test-> Horner’s pupil
will not dilate (Greater Anisocoria)
• Hydroxyamphetamine test – distinguish
pre- from post-ganglionic
• Apraclonidine Reversal of
Anisocoria.
Acute Horner’s
Syndrome
• Painful Horner’s syndrome is a
neurologic emergency.
• Although can be seen in many types of
headaches (Cluster, Migraine etc).
• Rule out ICA dissection.
• MRI/MRA of the head/neck/upper
mediastinum is indicated.
Oculo-sympathetic
Pathway
Horner’s Syndrome (MRI)
ICA dissection
• Goal is to prevent secondary neurologic
deficit (stroke).
• Anti-coagulation.
Acute Proptosis
• Associated ocular symptoms (pain , diplopia , loss of
vision).
• Associated systemic symptoms (fever , malaise , loss
of weight , excessive sweating , tremor or palpitations
)
• Past Medical History : Thyroid eye disease , diabetes
, immunocompromised , recurrent sinusitis , recent
tooth extraction )
• A 55 year old woman with with painful proptosis in
the left eye .
• Medical History : Rheumatoid Arthritis treated by
NSAID.
• Visual acuity : 20/20 Both eyes.
• Anterior Segment : Conujnctival hyperemia
• Exophthalmometry : 24 mm and 20 mm OS
• Normal pupils, ocular motility and fundus
examination.
Case
Case
Differential Diagnosis
• Graves disease .
• Idiopathic Orbital inflammatory Disease
• Orbital Cellulitis
• Carotid Cavernous Fistula
• Infiltrative , Neoplastic
Graves Disease
• Female with underlying thyroid disease .
• Typically bilateral but can be unilateral.
• Lid retraction , lid lag , and chemosis .
• CT : extraocular muscle enlargement ,
fat expansion .
Graves Disease
Treatment
• Medical : tears and cold compressors ,
IV Steroids, Rituximab.
• Surgical (inactive phase) : Orbital
decompression , strabismus surgery ,
eyelid repositioning , Blepharoplasty .
• Orbital radiation
Orbital Inflammatory
Disease
• Males = Females
• Acute onset , no eyelid
lag or retraction .
• CT : enlarged and
irregular muscles , often
unilateral.
• Can be associated with
systemic disease (SLE ,
Crohn’s , GPA ,
Rheumatoid Arthritis).
Idiopathic Orbital
Inflammatory Disease
Treatment of IOID
• Steroids
• Immunosuppressive agents
(Azathioprine , Methotrexate ,
Mycophenolate Mofetil )
• Biologic agents : anti-TNF
Orbital Cellulitis
•Fever and leukocytosis ,
patient is ill.
•Sinusitis , dacryocystitis,
dycryoadenitis.
•Less common is trauma or
endogenous spread.
•Beware in diabetes mellitus
and immunocompromised
patients (mucormycosis) !
CT Orbital Cellulitis
Mucormycosis
Orbital Cellulitis
• Treatment : IV antibiotics , anti-fungal
agents.
• Close monitoring for complications
(intracranial extension , or cavernous
sinus involvement)
• Additional debridement : Mucormycosis,
Necrotizing Fasciitis.
• ENT consultation for drainage of sinuses
(FESS) or abscess drainage .
Conclusion
• Appropriate history and systemic examination is key
in diagnosing neuro-ophthalmic emergencies .
• Acute loss of vision in an elderly patient should raise
the suspicion of Giant-Cell Arteritis .
• Pupil-involving third nerve palsy can be the
presenting sign of an intracranial aneurysm.
• Acute painful anisocoira can be due to internal-carotid
artery dissection .
• Acute proptosis is in diabetic or immunocompromised
patients can be due to life-threatening fungal orbital
cellulitis .

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Neuroophth Emergencies MDS - 2019

  • 2. History • Loss of vision • Diplopia • Eye pain • Eyelid drooping (ptosis)
  • 3. Key Aspects of the Examination • Visual acuity • Color vision Pupils : size and reaction in light and dark • Extraocular motility • Lids • Visual fields
  • 5. Pupils Relative afferent pupillary defect Direct pupil reflex Anisocoria
  • 7. Eyelids MRD1 and MRD2 Levator function assessment
  • 9. Vision Loss • Monoculr or binocular • Key is localization (refractive , retina or optic nerve) • Visual acuity assessment with pinhole. (refractive) • Look for RAPD . • Metamorphopsia . • Visual fields.
  • 10. Case • A 70 year old woman with sudden loss of vision in the right eye. • Transient loss of vision and jaw pain. • Feeling unwell lately with, and loss of appetite ( 10 Kg) , malaise and myalgias. • Hypertension on Metoprolol. • Visual acuity: Count finger right , 20/30 left. • Pupils : Right RAPD.
  • 11. Case
  • 12. Laboratory Investigations • ESR = 86 • CRP positive. • Platelets elevated ( 560). • Mildly anemic.
  • 13. Temporal Arteritis • Systemic vasculitis (Aortitis in 20% consider PET/MRA). • New onset of headache (temporal) , acute or transient loss of vision, jaw claudication, weight loss, fever, and myalgias. • Age usually over 60. • Occult GCA ( No systemic symptoms, transient diplopia or transient visual loss). • A true neuro-ophthalmic emergency (54-95% second eye involvement if untreated) !
  • 15. GCA Visual loss Management • Stat ESR , CRP and CBC (platelets). • CRP and CBC have 97% sensitivity and specificity. • Start high dose systemic steroids (IV or Oral) immediately upon suspicioun ( AAION or CRAO can develop in fellow eye within days if untreated !) • Arrange for temporal artery biopsy within 2 weeks , while patient is on steroids.
  • 16. Case • 52-year-old previously healthy presents with severe headache and blurred vision in both eyes. • Visual acuity 20/80 OD and 20/60 OS. • Confrontation visual fields : Bitemporal Hemianopia.
  • 18. Visual Field Defects in Chiasmal Syndrome
  • 19. MRI Pituitary mass with high signal on T1
  • 20. Pituitary Apoplexy • “Worst headache in my life”. • Visual field loss, and/or ophthalmoplegia ( uni- or bilateral). • Patients usually present 2 weeks after ictus. • > 80% did not have history of pituitary tumor • Life threatening (hypotension, shock) because of hypo-pituitarism, and low cortisol levels, and diabetes insipidus.
  • 21. Case • A 52 year old man with blurred vision and mild headache for the last 6 weeks. • Headaches are severe 10/10 scale , worse in the morning and leaning forward. • Visual acuity : 20/20 OU
  • 23. OCT
  • 25. Papilledema • Bilateral disc edema due to raised ICP. • Normal visual acuity. • Visual fields : enlarged blind spots (early)
  • 26.
  • 27. Malignant Hypertesnion• Accelerated hypertension with target organ damage. • Papilledema must be present for diagnosis ! • Dysfunction of cerebral blood flow autoregultaion causing cerebral edema. • Pre-eclampsia . • Encephalopathy can be present.
  • 28. Evaluation of Diplopia • Mono-ocualr or bin-ocular . • Orientation of images (vertical , horizontal , oblique) • Associated signs and symptoms (pain , loss of vision) • Follow-my-finger exam may not be enough ! • Maddox-rod or cover-uncover test . • Always check the eyelid and pupil !
  • 29. Case • 78 year old man with acute diplopia, and headache. • Headache and nausea . • Diabetes, hypertension, atrial tachycardia. • Limitation in adduction , elevation and depression in the right eye.
  • 30.
  • 31.
  • 32.
  • 33. Pupil-involving 3rd Nerve Palsy • Posterior communicating artery aneurysm, or mass. • Appropriate neuro-imaging is (MRI/MRA, MRI/CTA, Angiogram is the gold standard for aneurysm detection). • CTA is better for detecting aneurysms. • MRI is better to rule out masses .
  • 34. Risk of Aneurysm and “Rule of Pupil” Ophthalmoplegia Pupil Aneurysm Risk Complete/Partial Complete 86%-100% Partial Spared 30% Complete Spared very low If signs of sub-arachnoid hemorrhage present (headache, photophobia,
  • 35.
  • 36. Anisocoria • Acute onset vs acute awareness . • Associated symptoms (headache , neck pain , eye pain) • Is the anisocoria worse in the dark or light ? • Pharmacologic testing and neuro-imaging aid in localization.
  • 37. • A 67 year old man presents with pain in his right eye for 5 days associated with neck pain after chiropractic treatment. • Hypertension and ischemic heart disease on treatment. • No double vision. • VA : 20/30 OU. • Right partial ptosis (1 mm with right pupil smaller then left more in dark than light) Case
  • 38. Case
  • 39. Evaluation of Horner’s• Misois, and ptosis (upper and lower lid). • Dilatation lag, anisocoria worse in dark. • Topical Cocaine test-> Horner’s pupil will not dilate (Greater Anisocoria) • Hydroxyamphetamine test – distinguish pre- from post-ganglionic • Apraclonidine Reversal of Anisocoria.
  • 40. Acute Horner’s Syndrome • Painful Horner’s syndrome is a neurologic emergency. • Although can be seen in many types of headaches (Cluster, Migraine etc). • Rule out ICA dissection. • MRI/MRA of the head/neck/upper mediastinum is indicated.
  • 43. ICA dissection • Goal is to prevent secondary neurologic deficit (stroke). • Anti-coagulation.
  • 44. Acute Proptosis • Associated ocular symptoms (pain , diplopia , loss of vision). • Associated systemic symptoms (fever , malaise , loss of weight , excessive sweating , tremor or palpitations ) • Past Medical History : Thyroid eye disease , diabetes , immunocompromised , recurrent sinusitis , recent tooth extraction )
  • 45. • A 55 year old woman with with painful proptosis in the left eye . • Medical History : Rheumatoid Arthritis treated by NSAID. • Visual acuity : 20/20 Both eyes. • Anterior Segment : Conujnctival hyperemia • Exophthalmometry : 24 mm and 20 mm OS • Normal pupils, ocular motility and fundus examination. Case
  • 46. Case
  • 47. Differential Diagnosis • Graves disease . • Idiopathic Orbital inflammatory Disease • Orbital Cellulitis • Carotid Cavernous Fistula • Infiltrative , Neoplastic
  • 48. Graves Disease • Female with underlying thyroid disease . • Typically bilateral but can be unilateral. • Lid retraction , lid lag , and chemosis . • CT : extraocular muscle enlargement , fat expansion .
  • 50. Treatment • Medical : tears and cold compressors , IV Steroids, Rituximab. • Surgical (inactive phase) : Orbital decompression , strabismus surgery , eyelid repositioning , Blepharoplasty . • Orbital radiation
  • 51. Orbital Inflammatory Disease • Males = Females • Acute onset , no eyelid lag or retraction . • CT : enlarged and irregular muscles , often unilateral. • Can be associated with systemic disease (SLE , Crohn’s , GPA , Rheumatoid Arthritis).
  • 53. Treatment of IOID • Steroids • Immunosuppressive agents (Azathioprine , Methotrexate , Mycophenolate Mofetil ) • Biologic agents : anti-TNF
  • 54. Orbital Cellulitis •Fever and leukocytosis , patient is ill. •Sinusitis , dacryocystitis, dycryoadenitis. •Less common is trauma or endogenous spread. •Beware in diabetes mellitus and immunocompromised patients (mucormycosis) !
  • 57. Orbital Cellulitis • Treatment : IV antibiotics , anti-fungal agents. • Close monitoring for complications (intracranial extension , or cavernous sinus involvement) • Additional debridement : Mucormycosis, Necrotizing Fasciitis. • ENT consultation for drainage of sinuses (FESS) or abscess drainage .
  • 58. Conclusion • Appropriate history and systemic examination is key in diagnosing neuro-ophthalmic emergencies . • Acute loss of vision in an elderly patient should raise the suspicion of Giant-Cell Arteritis . • Pupil-involving third nerve palsy can be the presenting sign of an intracranial aneurysm. • Acute painful anisocoira can be due to internal-carotid artery dissection . • Acute proptosis is in diabetic or immunocompromised patients can be due to life-threatening fungal orbital cellulitis .