Neuro-ophthalmic Diagnoses You Don't Want To Miss !

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The presentation will discuss common life-threatening of vision-threatening neuro-ophthalmic emergencies.
Target: Ophthalmologists/Neurologists/Family Physicians/Internists/Emergency Physicians.

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Neuro-ophthalmic Diagnoses You Don't Want To Miss !

  1. 1. Neuro-ophthalmicEmergencies YouShould Not Miss ! Raed Behbehani , MD FRCSC
  2. 2. What is an emergency ?• Vision threatening ?• Life threatening ?• Recognition.• Proper investigations/imaging study.• Appropriate referral.
  3. 3. Painful Diplopia
  4. 4. Case 1• 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. 5. Case 1
  6. 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. 7. Case 2• 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.
  8. 8. 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).
  9. 9. Risk of Aneurysm and “Rule of Pupil”Ophthalmoplegia Pupil Aneurysm RiskComplete/Partial Complete 86%-100% Partial Spared 30% Complete Spared very low If signs of sub-arachnoid hemorrhage present (headache, photophobia, nausea) “rule
  10. 10. MRA or CTA ? CTA MRA Scanning time short LongerPatients with implanted Can be used Contraindiacted metal Not needed in 2D and Contrast Iodinated contrast 3D TOF Felxibility of display SuperiorNeck vessel evaluation Problematic Superior Aneurysm Detection 2-3 mm 3-5 mm
  11. 11. Case 3• BREAST CANCER S/P LUMPECTOMY, CHEMO AND XRT 10 YEARS AGO.• HYPERTENSION.• DEPRESSION.
  12. 12. Case 3
  13. 13. MRI : T2W
  14. 14. MRI : T1W + Gad contrastSTEREOTACTIC BIOPSY:
  15. 15. Case 4• 50 year old man with intermittent diplopia for 8 months.• No headache or vision loss.• No diabetes, hypertension or hyper- lipedemia.
  16. 16. Case 4
  17. 17. Aberrant regeneration of the 3rd nerve• “Miswiring” of the re-generating nerves resulting in dykinetic movements (lid, pupil, ocular motility).• Indicates long-standing compression (axonal injury).
  18. 18. MRA
  19. 19. Painful Ptosis and Anisocoria
  20. 20. Case 1• 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.
  21. 21. Case 1
  22. 22. 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.
  23. 23. Oculo-sympathetic Pathway
  24. 24. 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.
  25. 25. Horner’s Syndrome (MRI)
  26. 26. ICA dissection• Goal is to prevent secondary neurologic deficit (stroke).• Obsevation , Anti-coagulation, or stent implantation.• Referral for a neurovasculr specialist.
  27. 27. Acute vision loss in an elderly patient
  28. 28. Case 1• 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.
  29. 29. Case 1
  30. 30. Case 1Cord-like STA
  31. 31. Case 1 Investigations• ESR = 86• CRP positive.• Platelets elevated ( 560).• Mildly anemic.
  32. 32. 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).
  33. 33. GCACentral retinal artery occlusion Branch-retinal artery occlusion
  34. 34. 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.
  35. 35. GCA Diplopia (transient, constant)Ophthalmo plegia
  36. 36. 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.
  37. 37. TAB for GCA
  38. 38. TAB
  39. 39. 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.
  40. 40. “Worst headache in my life” and Diplopia
  41. 41. Case 1• 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 ptosis, and left partial ophthalmoplegia with unreactive mid- dilated right pupil with V1 hypesthesia.
  42. 42. Case 1
  43. 43. Visual Fields
  44. 44. Visual Field Defects in Chiasmal Syndrome
  45. 45. MRIPituitary mass with high signal on T1
  46. 46. 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.
  47. 47. Proptosis and Chemosis in A Diabetic Patient
  48. 48. Case 1• 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.
  49. 49. Case 1
  50. 50. Case 1- CT
  51. 51. Mucormycosis fungal• Vascular thrombosis, tissue necrosis, and dissemination.• The mortality rate is as high as 90%.• Diabetic ketoacidosis , immunosuppressed, organ transplant patients, steroid use, and desferrioxamine.• Other fugnal organisms: Aspergillus (more common).• Pain and ophthalmoplegia (orbital apex syndrome).• CT of the orbit/paranasal sinuses/cavernous sinus or MRI of the orbit with fat suppression.
  52. 52. 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).
  53. 53. Mucor• Medical therapy and surgical debridement increase the survival rate (78%) compared to medical management alone (57.5%).
  54. 54. Mucot
  55. 55. MucorNon-septate hyphae with branchingat 90 degrees.
  56. 56. Aspergillus• 3 year old child previously healthy.• Being treated for “orbital cellulitis” for more than three weeks with antibiotics.• Proptosis and chemosis left eye.• Child is otherwise in good health.• Neurosurgery : scalp tissue and bone debridement : “granulamtous inflammation”
  57. 57. Aspergillus
  58. 58. Asperrgillus
  59. 59. Histopathology and Treatment• Histpathology : Invasive Aspergillosis.• Started on IV Amphoterecin B.• Further work by pediatric immunologist : Chronic granulmatous disease.
  60. 60. Diplopia in A Patient with a “Shunt”
  61. 61. Case• A 38 year old woman with history of Hydrocephalus for 20 years ( etiology unknown)• Multiple V-P shunts ( 4 times ).• Had a VP shunt 2 weeks ago .• Few days after surgery, the neurosurgeon noted that “ she cannot look up “.• MRI : Small ventricles , otherwise normal.• BCVA 20/30 OU.• Pupils small and not reacting well to light.• Fundus : No papilledema or optic atrophy.
  62. 62. Case QuickTimeª and aMotion JPEG OpenDML decompressor are needed to see this picture.
  63. 63. Dorsal Midbrain Syndrome• Convergence retraction nystagmus and upgaze palsy.• Light near dissociation, Lid retraction (Collier’s sign).• Ischemia , tumors (pineal gland), Hydrocephalus, and Shunt malfunction.
  64. 64. VP Shunt Malfunction• Suspect even in the absence of ventriculomegaly or papilledema• Immediate recognition is important in preventing further permanant visual loss.• Shuntogrm (Shunt series) is indicated to assess shunt function.
  65. 65. 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.• Do visual fields by confrontation to detect large visual field defects (bitmporal, homonymous) in acute severe headache.• Proptosis and eye redness in diabetic/immunospressed patients can be due to life-threatening fungal infection.• Shunt malfunction should be suspected even in the absence of papilledema/ventriculomegaly.

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