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Introduction to Neuro-Ophthalmology Raed Behbehani , MD FRCSC
Neuro-ophthalmology Diseases of the eye and the neurological apparatus that serves it (optic nerve and chiasm, cranial nerves, visual pathways and cortex). 60% of our brain is linked to vision Afferent: Optic nerve, retina, chiasm, visual pathyways, cortx. Efferent: Cranial nerve III,IV,VI, ocular muscles, brain stem control centers.
Afferent System
Efferent System Cranial Nerves III, IV, VI Horizontal and Vertical Gaze Center Smooth Pursuit  and Saccade control
Symptoms Loss of vision (transient, constant, mono- or binocular).  Diplopia. Ptosis. Visual disturbances. Pupil irregularities. Eyelid or Facial spasms.
Clinical Approach History is the most important part or the assessment. “ Where” is the lesion ? “ What” can be the lesion ?  Is this an emergency ?
Diseases of the Afferent System Optic neuritis Ischemic optic neuropathy (Arteritic vs Non-Arteritic) Other optic neuropathies (compressive, papilledme, inflammatory, heriditary). Chiasmopathies. Strokes causing visual field defects.
Diseases of the Efferent System Cranial Neuropathies (III, IV, VI). Nystagmus. Ocular Myasthenia. Blepharospasm, Hemifacial Spasm. Pupillary Abnormalities.
What I can do for a patient with vision loss? Before you refer, you can do a lot ! History : Sudden or chronic (urgency) Check visual acuity (use near vision cards). Check for relative afferent pupillary defect. Do a visual field by confrontation. Ophthalmoscopy.
How to check for RAPD
Visual Field by Confrontation
Direct Ophthalmoscopy
Optic Neuritis Sudden loss of vision. Pain with eye movements. Females > Males. RAPD present. Optic disc normal. MRI is important for MS risk determination.
MRI in optic Neuritis White matter lesion predicts high risk for development of MS ( 70% over 15 years)
Ischemic Optic Neuropathy Age > 50. Acute ,  painless , loss of vision. Diabetes, hypertesnion, and hyperlipedemia. RAPD present. Ophthalmoscopy : disc edema +- hemorrhage.
Ischemic optic neuropathy
Arteritic Ischemic Optic Neuropathy Patient > 60. Headache, malaise, myalgia, weight loss fever, jaw claudications, and transient loss of vision. ESR, CRP are high.  Need to start systemic steroids  immediately  and do then do a TA biopsy.
Temporal Arteritis
Retinal Artery Occlusion Painless loss of vision. May be preceded by Amaurosis Fugax. Source of emboli usually carotid or cardiac. Less common causes: Vasuclitis (GCA,  Anti-phospholipid syndrome). Order Carotid Doppler Study and, Echocardiography.
Central Retinal Artery Occlusion
Branch Retinal Artery Occlusion
Compressive lesions Slowly progressive loss of vision. Can by uni-lateral or bilateral. Pituitary tumors, craniopahryngiomas, and meningiomas of the skull base. Require neuro-imaging (MRI) for diagnosis.
Visual field defects
Pituitary tumors
Pituitary Tumors
Homonymous Hemianopsia
Papilledema Disc edema due to raised intracranial pressure (mass, pseudotumor cerebri). Headache, transient visual obscurations, Diplopia, and tinnitus. Normal visual acuity and visual fields early. Ophthalmoscopy. Urgent CT scan of the head with contrast.
Papilledema
Idiopathic Intracranial Hypertension (pseudotumor cerebri) Women > Men (9:1) in childbearing age. 90% of affected women are obese. Normal CT/MRI/MRV and CSF analysis. Recent weight gain (last 6 months). Medications-linked : Tetracycline for acne , oral contraceptives, insulin-like growth factors in children. Aim of treatment is stop progressive loss of vision (Diuretics and Surgery).
Diplopia Key question “Is it only in one eye ?” , “ Does it go away when you close either eye ?” Monocular  diplopia is  always  refractive in origin (cataract, astigmatism). Examine lids and pupils in addition to eye movement. Examine all cranial nerves.
Oculomotor Nerve Palsy
Pupil-involving Third Nerve Palsy UrgentMRI/MRA or MRI/CTA
Abducens Nerve Palsy
Trochlear Neve Palsy Patients complain of vertical diplopia. Can present with abnormal head tilt. Can be congenital or acquired.
Trochlear Nerve Palsy - Head Tilt Test
Cranial Neuropathies (III,IV,VI) Ischemic (diabetes, hypertension and hyperlipidemia). Demyelinating. Compressive (tumor, aneurysm). Trauma. Raised ICP.
Multiple Cranial Neuropathies (III,IV,VI) Ischemic cranial neuropathies are almost always isolated. If multiple simultaneous CN, suspect lesion in the posterior orbit/cavernous sinus region. Usually due to mass lesion.
Cavernous Sinus
Ocular Myasthenia Myasthenic signs restricted to the ocular muscles. Fatiguable diplopia and ptosis. Ice test or rest test in the clinic demonstrate improvement. Acetylcholine receptor antibodies (positive in 50 % only). Single fiber EMG.
Ocular Myasthenia before ice test after ice test 2 minutes
Pupillary Abnormalities Anisocoria : Unequality of pupils size. It can be accidental discovery. Physiologic in 40% of patients It can be isolated or associated with lid or ocular motility abnormalities.  Can be iatrogenic or self-induced (pharamacologic). N
Pupil Examination Shine light directly at pupil (light response). Test near response (miosis with accomodation). Check pupil sizes and measure it in both light and dark. Parasympathetic (constrict) and Sympathetic (dilate) control.
Pupil Light Reflex
Diagnosis ?
Horner Syndrome A defect in oculosympathetic flow to the eye (pupil does not dilate in dark). Ptosis, miosis and pseudo-enophtalmos. Internal carotid artery dissection, neck trauma or surgery, brain stem strokes (Wallerburg Syndrome), Apical lung tumors. Urgent MRI/MRA of the head and neck for acute Horner’s Syndrome.
Oculosympathetic Pathway
Adies Pupil Pupil is larger with light/near dissociation (pupil does not constrict well to light but does for near). Can be associated with diminished deep tendon reflexes (Holmes-Adies Syndrome).
Benign Essential Blepharospasm
Hemifacial Spasm
Summary Neuro-ophthalmic problems of the afferent and efferent visual system are common. Afferent diseases include optic nerve, chisamopathies and visual pathway diseases. Efferent diseases include cranial neuropathies, pupillary abnormalities and facial spasms. There is no substitute for good medical history and examination.

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Introduction to Neuro-ophthalmology

  • 1. Introduction to Neuro-Ophthalmology Raed Behbehani , MD FRCSC
  • 2. Neuro-ophthalmology Diseases of the eye and the neurological apparatus that serves it (optic nerve and chiasm, cranial nerves, visual pathways and cortex). 60% of our brain is linked to vision Afferent: Optic nerve, retina, chiasm, visual pathyways, cortx. Efferent: Cranial nerve III,IV,VI, ocular muscles, brain stem control centers.
  • 4. Efferent System Cranial Nerves III, IV, VI Horizontal and Vertical Gaze Center Smooth Pursuit and Saccade control
  • 5. Symptoms Loss of vision (transient, constant, mono- or binocular). Diplopia. Ptosis. Visual disturbances. Pupil irregularities. Eyelid or Facial spasms.
  • 6. Clinical Approach History is the most important part or the assessment. “ Where” is the lesion ? “ What” can be the lesion ? Is this an emergency ?
  • 7. Diseases of the Afferent System Optic neuritis Ischemic optic neuropathy (Arteritic vs Non-Arteritic) Other optic neuropathies (compressive, papilledme, inflammatory, heriditary). Chiasmopathies. Strokes causing visual field defects.
  • 8. Diseases of the Efferent System Cranial Neuropathies (III, IV, VI). Nystagmus. Ocular Myasthenia. Blepharospasm, Hemifacial Spasm. Pupillary Abnormalities.
  • 9. What I can do for a patient with vision loss? Before you refer, you can do a lot ! History : Sudden or chronic (urgency) Check visual acuity (use near vision cards). Check for relative afferent pupillary defect. Do a visual field by confrontation. Ophthalmoscopy.
  • 10. How to check for RAPD
  • 11. Visual Field by Confrontation
  • 13. Optic Neuritis Sudden loss of vision. Pain with eye movements. Females > Males. RAPD present. Optic disc normal. MRI is important for MS risk determination.
  • 14. MRI in optic Neuritis White matter lesion predicts high risk for development of MS ( 70% over 15 years)
  • 15. Ischemic Optic Neuropathy Age > 50. Acute , painless , loss of vision. Diabetes, hypertesnion, and hyperlipedemia. RAPD present. Ophthalmoscopy : disc edema +- hemorrhage.
  • 17. Arteritic Ischemic Optic Neuropathy Patient > 60. Headache, malaise, myalgia, weight loss fever, jaw claudications, and transient loss of vision. ESR, CRP are high. Need to start systemic steroids immediately and do then do a TA biopsy.
  • 19. Retinal Artery Occlusion Painless loss of vision. May be preceded by Amaurosis Fugax. Source of emboli usually carotid or cardiac. Less common causes: Vasuclitis (GCA, Anti-phospholipid syndrome). Order Carotid Doppler Study and, Echocardiography.
  • 22. Compressive lesions Slowly progressive loss of vision. Can by uni-lateral or bilateral. Pituitary tumors, craniopahryngiomas, and meningiomas of the skull base. Require neuro-imaging (MRI) for diagnosis.
  • 27. Papilledema Disc edema due to raised intracranial pressure (mass, pseudotumor cerebri). Headache, transient visual obscurations, Diplopia, and tinnitus. Normal visual acuity and visual fields early. Ophthalmoscopy. Urgent CT scan of the head with contrast.
  • 29. Idiopathic Intracranial Hypertension (pseudotumor cerebri) Women > Men (9:1) in childbearing age. 90% of affected women are obese. Normal CT/MRI/MRV and CSF analysis. Recent weight gain (last 6 months). Medications-linked : Tetracycline for acne , oral contraceptives, insulin-like growth factors in children. Aim of treatment is stop progressive loss of vision (Diuretics and Surgery).
  • 30. Diplopia Key question “Is it only in one eye ?” , “ Does it go away when you close either eye ?” Monocular diplopia is always refractive in origin (cataract, astigmatism). Examine lids and pupils in addition to eye movement. Examine all cranial nerves.
  • 32. Pupil-involving Third Nerve Palsy UrgentMRI/MRA or MRI/CTA
  • 34. Trochlear Neve Palsy Patients complain of vertical diplopia. Can present with abnormal head tilt. Can be congenital or acquired.
  • 35. Trochlear Nerve Palsy - Head Tilt Test
  • 36. Cranial Neuropathies (III,IV,VI) Ischemic (diabetes, hypertension and hyperlipidemia). Demyelinating. Compressive (tumor, aneurysm). Trauma. Raised ICP.
  • 37. Multiple Cranial Neuropathies (III,IV,VI) Ischemic cranial neuropathies are almost always isolated. If multiple simultaneous CN, suspect lesion in the posterior orbit/cavernous sinus region. Usually due to mass lesion.
  • 39. Ocular Myasthenia Myasthenic signs restricted to the ocular muscles. Fatiguable diplopia and ptosis. Ice test or rest test in the clinic demonstrate improvement. Acetylcholine receptor antibodies (positive in 50 % only). Single fiber EMG.
  • 40. Ocular Myasthenia before ice test after ice test 2 minutes
  • 41. Pupillary Abnormalities Anisocoria : Unequality of pupils size. It can be accidental discovery. Physiologic in 40% of patients It can be isolated or associated with lid or ocular motility abnormalities. Can be iatrogenic or self-induced (pharamacologic). N
  • 42. Pupil Examination Shine light directly at pupil (light response). Test near response (miosis with accomodation). Check pupil sizes and measure it in both light and dark. Parasympathetic (constrict) and Sympathetic (dilate) control.
  • 45. Horner Syndrome A defect in oculosympathetic flow to the eye (pupil does not dilate in dark). Ptosis, miosis and pseudo-enophtalmos. Internal carotid artery dissection, neck trauma or surgery, brain stem strokes (Wallerburg Syndrome), Apical lung tumors. Urgent MRI/MRA of the head and neck for acute Horner’s Syndrome.
  • 47. Adies Pupil Pupil is larger with light/near dissociation (pupil does not constrict well to light but does for near). Can be associated with diminished deep tendon reflexes (Holmes-Adies Syndrome).
  • 50. Summary Neuro-ophthalmic problems of the afferent and efferent visual system are common. Afferent diseases include optic nerve, chisamopathies and visual pathway diseases. Efferent diseases include cranial neuropathies, pupillary abnormalities and facial spasms. There is no substitute for good medical history and examination.