1. Neuro-Ophthalmic
Emergencies
Emergencies
Raed Behbehani , MD FRCSC

2. What is an emergency ?
• Vision threatening ?
• Life threatening ?
• Recognition.
• Proper investigations/imaging study.
• Appropriate referral.

3. Painful Diplopia

4. Case
• A 53 year old patient with acute diplopia.
• Previous episodes few years ago , which
lasted two months and recovered.
• Diabetes, and hyprlipedemia.
• Visual acuity : 20/20 OU.
• Pupils : Equally reactive , pupils equal in size.

5. Case

6. Pupil-Sparing Third
Nerve Palsy
• Diabetes, hypertension, hyperlipedemia, smoking, high
hematocrit.
• Pupils is spared.
• Pupil involvement reported only in 14%-32% , but
anisocoria (difference in pupil size) is less than 1 mm
(relative-sparing).
• Improve within 4-12 weeks (defer neuro-imaging).

7. Case
• 78 year old man with acute diplopia, and
headache.
• Diabetes, hypertension, atrial tachycardia.
• Prior history of tight feeling around the eye
with 20 seconds of diplopia.
• No history of jaw claudication or transient
visual loss.

14. Pupil-involving 3rd
Nerve Palsy
• Pupil involvement indicates compression of
the pupillary fibers.
• Posterior communicating artery
aneurysm, or mass.
• Appropriate neuro-imaging is (MRI/MRA,
MRI/CTA,Angiogram is the gold standard
for aneurysm detection).

16. 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

17. Painful Ptosis and
Anisocoria

18. Case
• A 67 year old man presents with pain in his right eye
for 5 days.
• Hypertension and ischemic heart disease on treatment.
• No double vision.
• VA : 20/30 OU.
• Mild nuclear sclerosis cataracts.
• Fundus: normal.

19. Case

20. Evaluation of Horner’s
• Misois, and ptosis (upper and lower lid).
• Dilatation lag, anisocoria worse in dark.
• Cocaine test.
• Hydroxyamphetamine (not used much).
• Iopidine.
• MRI/MRA of the head/neck/upper chest CT.

21. Oculo-sympathetic
Pathway

22. 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.

23. Horner’s Syndrome
(MRI)

24. ICA dissection
• Goal is to prevent secondary neurologic
deficit (stroke).
• Obsevation ,Anti-coagulation, or stent
implantation.
• Referral for a neurovasculr specialist.

25. Acute vision loss in an
elderly patient

26. Case
• A 68 year old patient with sudden loss of vision in
the right eye.
• History of episodes transient loss of vision.
• Diabetes for 30 years.
• Feeling unwell lately with, and loss of appetite,
malaise and myalgias.
• Visual acuity: Count finger right , 20/30 left.
• Right RAPD.

27. Case

28. Case
Cord-like STA

29. Case Investigations
• ESR = 86
• CRP positive.
• Platelets elevated ( 560).
• Mildly anemic.

30. Arteritic Ischemic
Optic Neuropathy
• 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) !
• Giant cell arteritis (systemic vasculitis, Aortitis in
20% consider PET/MRA).

31. GCA
Central retinal artery
occlusion Branch-retinal artery occlusion

32. Posterior Ischemic
Optic Neuropathy
(PION)
• Both the retina and optic nerve look normal.
• PION is relatively common in Giant Cell arteritis.
• Flourescin angiogram can show choroidal
hypoperfusion.
• Involvement of 2 circulations (systemic vasculitis),
retinal artery occlusion and AION indicate giant
cell arteritis.

33. GCA
Diplopia (transient,
constant)Ophthalmo
plegia

34. AAION Management
• Stat ESR , CRP and CBC (platelets).
• ESR can be normal in 15-20% of cases.
• CRP is more sensitive and specific.
• CRP and CBC have 97% sensitivity and specificity.
• Start high dose systemic steroids (IV or Oral)
immediately upon suspicioun ( AAION can develop in
fellow eye within days if untreated !)
• Arrange for temporal artery biopsy within 2 weeks ,
while patient is on steroids.

35. QuickTime™ and a
decompressor
are needed to see this picture.
Video

36. TAB

37. GCA Treatment
• Systemic steroids for a at least 1-2 years.
• Titrate dose according to laboratory
indices (CRP,ESR) and symptoms.
• Manage diabetes and osetoporosis.
• Collaboration with rheumatologist.

38. “Worst Headache”

39. Case
• 52-year-old, morbidly obese man presents with
severe headache (worst in his life).
• Ischemic cardiac disease and angioplasty, COPD,
hypertension, and NIDDM.
• On examination: complete right ptosiswith
unreactive mid-dilated right pupil، left partial
ophthalmoplegia with V1 hypesthesia.

40. Case

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 loss, and/or ophthalmoplegia ( uni- or bilateral).
• Patients usually present 2 weeks after ictus.
• > 80% did not have history of pituitary tumor
• Ophthalmoplegia (extension to cavernous sinus with
cranial nerve involvement).
• Life threatening (hypotension, shock) because of
hypo-pituitarism, and low cortisol levels, and diabetes
insipidus.

45. Case
• A 50 year old with blurred vision and
headache for the last 2 weeks.
• Medical History : Diabetes for 5 years.
• Smoker 15 years.
• No prior Surgeries

46. Case
• Conscious and oriented.
• Visual acuity : 20/20 OU
• Pupils : PERL no RAPD.
• Normal anterior Segment .
• Normal ocular motility.

47. Case

48. Case
• CT and MRI/MRV - normal.
• Blood pressure 220/150 !

49. 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.

50. Proptosis in
immunocompromised or
diabetic patient

51. Case
• 60 year old man with myelodysplastic
disorder on chemotherapy.
• Proptosis, fever, and dyspnea .
• Periorbital swelling and erythema, which
got worse over 3 days.
• Visual acuity : 20/20 Both eyes.
• Normal pupils, ocular motility and fundus
examination.

52. Case

53. CT

54. Mucormycosis
• Vascular thrombosis, tissue necrosis, and fungal
dissemination.
• The mortality rate is as high as 90%.
• Diabetic ketoacidosis , immunosuppressed, organ
transplant patients, steroid use, and
desferrioxamine.
• Other fungal organisms: Aspergillus.
• Pain and ophthalmoplegia.
• CT of the orbit/paranasal sinuses/cavernous sinus
or MRI of the orbit with fat suppression.

55. Mucor
• Immediate biopsy (ENT/Orbit) , with debridement.
• Orbital exenteration is not always needed.
• Correct any metabolic acidosis to reduce unbound
iron (critical for the proliferation of mucor)
• Local delivery of amphotericin B with indwelling
catheters.
• Systemic antifungal (IV liposomal encapsulated
Amph B less nephrotoxic +- posaconazole).
• Boost immunity (correct neutropenia).

56. Mucor
• Medical therapy and surgical debridement increase
the survival rate (78%) compared to medical
management alone (57.5%).

57. Mucormycosis

58. Mucor
Non-septate hyphae with branching
at 90 degrees.

59. Summary
• Pupil involvement in 3rd nerve palsy suggests compressive lesion
(aneuurysm), get and MRI/MRA or MRI/CTA.
• Always rule out ICA dissection in acute Horner’s syndrome.
• Always rule out GCA as the etiology for ophthalmoplegia or
visual loss in >60 year patients.
• In acute severe headache with ophthalmoplegia with multiple CN
involvement think of pituitary apoplexy.
• Proptosis and eye redness in diabetic/immunospressed patients
can be due to life-threatening fungal infection.