This lecture was given to 6th year medical students on October 16 , 2018 at the Faculty of Medicine of Kuwait University

1. Neuro-ophthalmic
Emergencies
Raed Behbehani , MD FRCSC

2. What is an emergency ?
• Vision threatening ?
• Life threatening ?
• Recognition.

3. Painful Diplopia

4. 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.

8. 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 .

9. 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, nausea) “rule

11. Acute Painful loss of
Vision
• A 30 year old lady presents with acute
“grey” vision in the left eye .
• Dull pain with eye movements .
• Visual acuity : 20/20 OD Count fingers OS
• Color vision : 13/13 OD 0/13 OS
• Pupils : Left RAPD
• Fundus : Normal

12. “Typical” Optic
Neuritis
• Women (77%)
• 20-50Year Age Group
• Pain with eye movements .
• Normal optic disc appearance (2/3 cases)
• Improvement over several weeks.

13. “Atypical” Optic Neuritis
• Bilateral onset in an adult.
• No pain.
• Ocular findings : uveitis, exudate, retinitis.
• Severe disc swelling and Hemorrhages
• No improvement after 6 weeks.
• Age > 50 years.
• Pre-existing diagnosis of a systemic disease.

14. Atypical Optic Neuritis

15. Ischemic Optic
Neuropathy

16. Sarcoid Optic
Neuropathy

17. MRI in Optic Neuritis
T1 fat suppressed views with Gd Enhancement

18. MRI in MS

19. MS Risk ONTT

20. OCT in Optic Neuritis

21. OCT Ganglion Cell
Analysis in ON

22. AcuteVision Loss in An
Elderly Patient

23. 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.

24. Case

25. Case

26. Laboratory Investigations
• ESR = 86
• CRP positive.
• Platelets elevated ( 560).
• Mildly anemic.

27. 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) !

28. AAION in Temporal
Arteritis

29. GCAVisual 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.

30. TAB for GCA

31. Acute Anisocoria and
Neck Pain

32. • 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

33. Case

34. 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.

35. 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.

36. Oculo-sympathetic
Pathway

37. Horner’s Syndrome
(MRI)

38. ICA dissection
• Goal is to prevent secondary neurologic
deficit (stroke).
• Anti-coagulation.

39. Acute Bitemporal
Hemianopsia

40. 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.

41. Visual Fields

42. Visual Field Defects in
Chiasmal Syndrome

43. MRI
Pituitary mass with high signal on T1

44. 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.

45. Headache and Bilateral
Disc Edema

46. Case
• A 24 year old woman with blurred vision
and mild headache for the last 6 weeks.
• Headaches are severe 10/10 scale , worse in
the morning and leaning forward.
• Weight gain of 15 kilos over the last 3
months
• Visual acuity : 20/20 OU

47. Fundus Examination

48. OCT

49. Visual Felds

50. Papilledema
• Bilateral disc edema due to raised ICP.
• Normal visual acuity.
• Visual fields : enlarged blind spots (early)

51. Case
• CT with contrast and MRI/MRV - normal.
• Lumbar puncture – Opening CSF pressure
of 500 mm/Hg.
• Normal CSF analysis.

52. Idiopathic Intracranial
Hypertension
1.1. Signs and symptoms of increased ICP.Signs and symptoms of increased ICP.
2.2. No localizing neurological signs (except uni/bilateral VINo localizing neurological signs (except uni/bilateral VI
nerve palsy)nerve palsy)
3.3. No evidence of an intracranial mass lesionNo evidence of an intracranial mass lesion
4.4. Normal CSF compositionNormal CSF composition

53. Treatment of IIH
• Diuretics (Acetazolamide , Freusoamide)
• Weight loss (Bariatric Surgery)
• Optic Nerve Sheath Fenestration
(progressive visual loss).
• Neurosurgical shunts (LP orVP shunt)

54. Optic Nerve Sheath
Fenestration

55. 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.

56. Acute Proptosis and Red
Eye

57. • 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

58. Case 1

59. Differential Diagnosis
• Graves disease .
• Idiopathic Orbital inflammatory Disease
• Orbital Cellulitis
• Carotid Cavernous Fistula
• Infiltrative , Neiplastic

60. 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 .

61. Graves Disease

62. Treatment
• Medical : tears and cold compressors , IV
Steroids, Rituximab.
• Surgical (inactive phase) : Orbital
decompression , strabismus surgery , eyelid
repositioning , Blepharoplasty .
• Orbital radiation

63. 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).

64. Idiopathic Orbital
Inflammatory Disease

65. Treatment of IOID
• Steroids
• Immunosuppressive agents (Azathioprine ,
Methotrexate , Mycophenolate Mofetil )
• Biologic agents : anti-TNF

66. Orbital Cellulitis
•Fever and leukocytosis , patient is ill.
•Sinusitis , dacryocystitis, dycryoadenitis.
•Less common is trauma or endogenous
speread.
•Beware in diabetes mellitus and
immunocompromised patients (mucormycosis)
!

67. CT Orbital Cellulitis

68. Mucormycosis

69. Periorbital Necrotizing
Fasciitis

70. Periorbital Necrotizing Fasciitis
• Severe, potentially vision-threatening or life-
threatening bacterial infection involving the
subcutaneous soft tissues, and superficial and deep
fasciae.
• Group A beta-hemolytic Streptococcus , other
gram positive and gram negative organisms.
• Immunocompromised (diabetes) and
immunocompetent patients.
• Initial presentation (pre-septal cellulitis , shock like
syndrome) hypotension, renal failure, and adult
respiratory distress syndrome.

71. 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 .