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The Neuro-Ophthalmology of Multiple Sclerosis Charles Maxner MD, FRCPC

The Neuro-Ophthalmology of Multiple Sclerosis Charles Maxner MD, FRCPC Professor, Departments of Medicine (Neurology) and Ophthalmology Dalhousie University Consultant, Dalhousie MS Research Unit Halifax , N.S. Dr. C.E. Maxner: Disclosure. Dr. Maxner has attended and conducted

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The Neuro-Ophthalmology of Multiple Sclerosis Charles Maxner MD, FRCPC

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  1. The Neuro-Ophthalmology of Multiple Sclerosis Charles Maxner MD, FRCPC Professor, Departments of Medicine (Neurology) and Ophthalmology Dalhousie University Consultant, Dalhousie MS Research Unit Halifax , N.S.

  2. Dr. C.E. Maxner: Disclosure Dr. Maxner has attended and conducted educational events and participated in MS research studies affiliated with the following firms: Berlex Biogen Idec Serono Teva

  3. Objectives: Briefly review MS as a disorder Review how it affects: The Afferent Visual System The Efferent Visual System The Visual Systemand MS

  4. MS: Historical Perspective Augustus d’Esté (1794-1848) Grandson King George III J.M. Charcot (1825-1893) (1868 leçons: ”sclérose en plaques disseminées” from Vulpian) Carswell ~1836

  5. Multiple Sclerosis Disorder of Central Myelin (Oligodendroglia) Brain and Spinal Cord Immune Based Inflammatory demyelinating disorder Axonal injury (Disability)

  6. Multiple Sclerosis:3 Components Inflammation Demyelination Axonal Loss Courtesy Dr. G. Rice

  7. Multiple Sclerosis Pathology Gross Pathology Luxol Fast Blue

  8. Multiple Sclerosis Pathology Optic Nerves Chiasm Optic Tract Anterior Visual Pathway Luxol Fast Blue

  9. Action Potential Transit in MS Concepts 1. Delayed Conduction 2. Conduction Block

  10. Courtesy Dr. A.Bar-Or

  11. Relapsing Forms Natural Progression of MS Mono-symptomatic Subclinical Relapsing-Remitting Secondary Progressive Initial demyelinating event Clinically definite MS Relapse Clinical Worsening Time Level of disability Gadolinium enhancement Cognitive dysfunction Relapses Accumulated MRI lesion burden Brain atrophy Courtesy Dr. G. Rice

  12. MRI Dissemination in Space and Time

  13. Presenting Symptoms of MS

  14. Loss of Vision (Monocular and Binocular) Diplopia Oscillopsia Neuro-ophthalmological Issues

  15. Afferent Visual System Vision loss and distortion Efferent Visual System Diplopia and Oscillopsia MS and the Visual System

  16. Pre-chiasmal Optic Nerve Chiasmal Bitemporal VF defect rare Junctional Scotoma defect not uncommon Post-Chiasmal Optic tract Geniculocalcarine pathway MS and The Afferent Visual System

  17. MS diagnosed 12 years prior Copaxone Therapy Decreased vision left eye Progressed over 48 hours Pain on eye movement Impaired depth perception “Can’t drive” Case: Ms. H.B. 35 YOWF

  18. Examination Va 6/6 Right, HM Left Central scotoma left eye RAPD 1.5 log units left eye Impaired colour perception left Ocular motility normal Left disc slightly swollen and hyperemic Case: Ms. H.B. 35 YOWF

  19. Pupil Testing

  20. Goldmann Visual Fields Case: Ms. H.B. 35 YOWF

  21. Va 6/6 Right, 6/9 Left Central blur left eye RAPD 0.6 left eye Colour improved Temporal pallor left disc Case: Ms. H.B. 35 YOWF Follow Up: 3 months later

  22. Monocular Central Vision loss Pain (eye movement) Altered colour vision Recovery common Uhthoff’s symptom Flashes Pulfrich phenomenon Optic NeuritisCommon Symptoms

  23. Uhthoff described 3 patients in whom exertion and fatigue caused a desaturation in colour vision Patient XVIII had decreased acuity after walking around the room Uhthoff’s Symptom What did he describe? Who was Uhthoff?

  24. Wilhelm Uhthoff (1853-1927) Born Warin , Germany Studied in Tübingen, Göttingen, Berlin Consultant at Westphal’s Clinic (With Oppenheim, Wallenberg, Thomsen, Möbius) Named Professor of Ophthalmology at Breslau 1896 Eye Symptoms in Diseases of the Nervous System (Published 1915) Described by Bielschowsky as the “true originator” of clinical neuro-ophthalmology Uhthoff’s Symptom

  25. Wilhelm Uhthoff

  26. Uhthoff’s symptom in optic neuritis:relationship to MRI and development of MS. (Scholl GB, Song HS, Wray SH) Ann Neurol 1991; 30(2):180-4 Uhthoff and his Symptom (Selhorst JB, Saul RF) Journal of Neuro-ophthalmology 1995; 15(2):63-9 Uhthoff’s Symptom

  27. Movement phosphenes in optic neuritis: A new clinical sign(Davis F, Bergen D, Schauf C, McDonald I, Deutsch W) Neurology 1976; 26: 1100-1104. Bright flashes in dark Eye movement Differentiate from Lightning Streaks of Moore Eye equivalent of Lhermittes symptom Flashes

  28. Pulfrich Phenomenon

  29. Decreased visual acuity VF defect (Central/Altitudinal 29% ) Dyschromatopsia Afferent Pupil Defect (RAPD) Optic disc swelling 35% Abnormal Contrast Sensitivity Abnormal VEP Altered Flicker Perception Altered depth perception Optic disc pallor Optic Neuritis: Physical Findings

  30. Optic Neuritis: Optic Disc

  31. Two months impaired vision both eyes Progressive course Blurred centrally right eye Hazy to left of fixation both eyes Occasional migraine Case: Ms. A.B. 23 YOWF

  32. Va 6/15 Right, 6/7.5 Left Confrontation VF: Left Central HH No RAPD AO Plates: 7/14 Rt 10/14 Lt Ocular motility normal Anomalous discs both eyes Case: Ms. A.B. 23 YOWF

  33. Automated Perimetry Case: Ms. A.B. 23 YOWF

  34. MRI Imaging Case: Ms. A.B. 23 YOWF

  35. 2 week hx of L sided visual blurring Both eyes involved 15 years ago “poor balance” Migraines Sister with MS Case:Ms. C.S. 41 YOWF

  36. Examination Va: 6/6 Both Eyes AO Plates: 13/14 Rt, 11/14 Lt Pupils normal Ocular motility normal Fundi normal DTR’s brisk, Unsteady Romberg VF’s abnormal Case: Ms. C.S.

  37. Ms. C.S. Visual Fields

  38. Ms. C.S. MRI

  39. Ms. C.S. MRI (2 mos later)

  40. AION (Ischemic Optic Neuropathy) Vasculitic Disorders (i.e. SLE) Hereditary (i.e. Leber’s) Toxic/Nutritional (ETOH) Infectious (i.e.Bartonella, Lyme) Inflammatory (i.e. Sarcoid) Neoplastic/Paraneoplastic (i.e. lymphoma) Compressive (i.e.Tumours, Grave’s orbitopathy) Amblyopia Optic Neuritis: The Differential

  41. Diplopia Horizontal, Vertical, Mixed Fluctuating Oscillopsia Neuro-ophthalmological Issues

  42. Infranuclear or Nerve Saccadic system Pursuit system Internuclear abnormalities Vestibulo-ocular dysfunction Nystagmus Ocular Motility Disorders

  43. Ocular Motility Disorders Nuclear Palsies: Rare Infranuclear or Nerve VI: Most common III: Partial or Complete IV: Rare

  44. Ms. H.M. 34 YOWF CC: Diplopia Hx: 6 months progressing diplopia Initially intermittent, now persistent Otherwise asymptomatic Sister has MS O/E: Incomitant esotropia Left abduction deficit

  45. Ms. H.M. 34 YOWF

  46. Ms. H.M. 34 YOWF6 Months Later

  47. Saccadic abnormalities Hypometric Hypermetric Dysmetria Saccadic Intrusions Square wave jerks Saccadic pulses Ocular flutter Ocular Motility Disorders

  48. Saccadic Abnormalities From: Leigh & Zee. The Neurology of Eye Movements, F.A. Davis Company

  49. Saccadic Oscillations Saccadic Dysmetria Macrosaccadic Oscillations Square Wave Jerks Macro Square Wave Jerks Ocular Flutter From: Leigh & Zee. The Neurology of Eye Movements, F.A. Davis Company

  50. Ocular Motility Disorders Square Wave Jerks Ocular Flutter

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