North American Neuro-Ophthalmology Society 38th Annual Meeting
Neuro-Ophthalmology and Multiple Sclerosis
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Transcript of Neuro-Ophthalmology and Multiple Sclerosis
The Neuro-Ophthalmology The Neuro-Ophthalmology of Multiple Sclerosisof Multiple Sclerosis
Charles Maxner MD, FRCPCCharles Maxner MD, FRCPCProfessor, Departments of Medicine (Neurology) and OphthalmologyProfessor, Departments of Medicine (Neurology) and Ophthalmology
Dalhousie UniversityDalhousie UniversityConsultant, Dalhousie MS Research Unit Consultant, Dalhousie MS Research Unit
Halifax , N.S.Halifax , N.S.
Dr. C.E. Maxner: DisclosureDr. C.E. Maxner: DisclosureDr. Maxner has attended and conductedDr. Maxner has attended and conductededucational events and participated in MSeducational events and participated in MSresearch studies affiliated with the followingresearch studies affiliated with the followingfirms:firms:
Berlex Berlex Biogen IdecBiogen IdecSerono Serono TevaTeva
The Visual SystemThe Visual Systemand MSand MS
Objectives:Objectives:
Briefly review MS as a disorder Briefly review MS as a disorder Review how it affects:Review how it affects: The Afferent Visual SystemThe Afferent Visual System The Efferent Visual SystemThe Efferent Visual System
MS: Historical PerspectiveMS: Historical Perspective
Augustus d’Esté (1794-1848)
Grandson King George III
Carswell ~1836
J.M. Charcot (1825-1893)(1868 leçons: ”sclérose en plaques disseminées” from Vulpian)
Multiple SclerosisMultiple Sclerosis
Disorder of Central Myelin (Oligodendroglia)Disorder of Central Myelin (Oligodendroglia)Brain and Spinal CordBrain and Spinal CordImmune BasedImmune BasedInflammatory demyelinating disorderInflammatory demyelinating disorderAxonal injury (Disability)Axonal injury (Disability)
Demyelination Axonal Loss
Inflammation
Multiple Sclerosis:Multiple Sclerosis:3 Components3 Components
Courtesy Dr. G. Rice
Multiple Sclerosis Multiple Sclerosis PathologyPathology
Gross Pathology
Luxol Fast Blue
Multiple Sclerosis Multiple Sclerosis PathologyPathology
Optic Nerves
Chiasm
Optic Tract
Anterior Visual Pathway
Luxol Fast Blue
Action Potential Transit in MS
1. Delayed Conduction
2. Conduction Block
Concepts
Courtesy Dr. A.Bar-Or
Natural Progression of MSNatural Progression of MS
Level of disability
Accumulated MRI lesion burden
Gadolinium enhancement Cognitive dysfunction
Brain atrophyRelapses
SubclinicalMono-
symptomatic Relapsing-Remitting Secondary Progressive
Relapsing Forms
Time
Clin
ical
Wor
seni
ng
Initial demyelinating
eventClinically
definite MS Relapse
Courtesy Dr. G. Rice
MRI Dissemination in MRI Dissemination in Space and TimeSpace and Time
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Presenting Symptoms of MSPresenting Symptoms of MS
SymptomSymptom Approximate PrevalenceApproximate Prevalence
Weakness in one or more Weakness in one or more limbslimbs 40-50%40-50%
Sensory loss/paresthesiasSensory loss/paresthesias 40-45%40-45%Visual lossVisual loss 16-36%16-36%Gait disturbance/ataxiaGait disturbance/ataxia 5-15%5-15%DiplopiaDiplopia 7-15%7-15%Dizziness/vertigoDizziness/vertigo 5%5%PainPain 3%3%Sensory in faceSensory in face 3%3%
Neuro-ophthalmological Neuro-ophthalmological IssuesIssues
Loss of Vision Loss of Vision (Monocular and Binocular)(Monocular and Binocular)
DiplopiaDiplopiaOscillopsiaOscillopsia
MS and the Visual SystemMS and the Visual System
Afferent Visual SystemAfferent Visual SystemVision loss and distortionVision loss and distortion
Efferent Visual SystemEfferent Visual SystemDiplopia and OscillopsiaDiplopia and Oscillopsia
MS and The Afferent MS and The Afferent Visual SystemVisual SystemPre-chiasmalPre-chiasmalOptic NerveOptic Nerve
ChiasmalChiasmalBitemporal VF defect rareBitemporal VF defect rareJunctional Scotoma defect not uncommonJunctional Scotoma defect not uncommon
Post-ChiasmalPost-ChiasmalOptic tractOptic tractGeniculocalcarine pathwayGeniculocalcarine pathway
Case: Ms. H.B. 35 YOWFCase: Ms. H.B. 35 YOWF
MS diagnosed 12 years priorMS diagnosed 12 years priorCopaxone TherapyCopaxone TherapyDecreased vision left eyeDecreased vision left eyeProgressed over 48 hoursProgressed over 48 hoursPain on eye movementPain on eye movementImpaired depth perceptionImpaired depth perception““Can’t drive”Can’t drive”
Case: Ms. H.B. 35 YOWFCase: Ms. H.B. 35 YOWF ExaminationExamination
Va 6/6 Right, HM LeftVa 6/6 Right, HM LeftCentral scotoma left eyeCentral scotoma left eyeRAPD 1.5 log units left eyeRAPD 1.5 log units left eyeImpaired colour perception leftImpaired colour perception leftOcular motility normalOcular motility normalLeft disc slightly swollen and hyperemicLeft disc slightly swollen and hyperemic
Pupil Testing
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Case: Ms. H.B. 35 YOWFCase: Ms. H.B. 35 YOWF
Goldmann Visual FieldsGoldmann Visual Fields
Case: Ms. H.B. 35 YOWFCase: Ms. H.B. 35 YOWF
Va 6/6 Right, 6/9 LeftVa 6/6 Right, 6/9 LeftCentral blur left eyeCentral blur left eyeRAPD 0.6 left eyeRAPD 0.6 left eyeColour improvedColour improvedTemporal pallor left discTemporal pallor left disc
Follow Up: 3 months laterFollow Up: 3 months later
Optic NeuritisOptic NeuritisCommon SymptomsCommon Symptoms
MonocularMonocularCentral Vision lossCentral Vision lossPain (eye movement)Pain (eye movement)Altered colour visionAltered colour vision
Recovery commonRecovery commonUhthoff’s symptomUhthoff’s symptomFlashesFlashesPulfrich phenomenonPulfrich phenomenon
Uhthoff’s SymptomUhthoff’s Symptom
Uhthoff described 3 patients in whom Uhthoff described 3 patients in whom exertion and fatigue caused a desaturation exertion and fatigue caused a desaturation in colour visionin colour vision
Patient XVIII had decreased acuity after Patient XVIII had decreased acuity after walking around the roomwalking around the room
What did he describe?
Who was Uhthoff?
Uhthoff’s SymptomUhthoff’s SymptomWilhelm Uhthoff (1853-1927)Wilhelm Uhthoff (1853-1927)Born Warin , GermanyBorn Warin , GermanyStudied in Tübingen, Göttingen, BerlinStudied in Tübingen, Göttingen, BerlinConsultant at Westphal’s Clinic (With Oppenheim, Consultant at Westphal’s Clinic (With Oppenheim,
Wallenberg, Thomsen, Möbius)Wallenberg, Thomsen, Möbius)Named Professor of Ophthalmology at Breslau 1896Named Professor of Ophthalmology at Breslau 1896Eye Symptoms in Diseases of the Nervous System Eye Symptoms in Diseases of the Nervous System
(Published 1915)(Published 1915)Described by Bielschowsky as the “true originator” Described by Bielschowsky as the “true originator”
of clinical neuro-ophthalmologyof clinical neuro-ophthalmology
Wilhelm UhthoffWilhelm Uhthoff
Uhthoff’s SymptomUhthoff’s Symptom
Uhthoff’s symptom in optic Uhthoff’s symptom in optic neuritis:relationship to MRI and neuritis:relationship to MRI and development of MS.development of MS. (Scholl GB, Song HS, (Scholl GB, Song HS, Wray SH) Ann Neurol 1991; 30(2):180-4Wray SH) Ann Neurol 1991; 30(2):180-4
Uhthoff and his Symptom Uhthoff and his Symptom (Selhorst JB, (Selhorst JB, Saul RF) Journal of Neuro-ophthalmology Saul RF) Journal of Neuro-ophthalmology 1995; 15(2):63-91995; 15(2):63-9
FlashesFlashesMovement phosphenes in optic Movement phosphenes in optic neuritis: A new clinical signneuritis: A new clinical sign (Davis F, (Davis F, Bergen D, Schauf C, McDonald I, Deutsch W) Bergen D, Schauf C, McDonald I, Deutsch W) Neurology 1976; 26: 1100-1104.Neurology 1976; 26: 1100-1104.
Bright flashes in darkBright flashes in darkEye movementEye movementDifferentiate from Lightning Streaks of MooreDifferentiate from Lightning Streaks of MooreEye equivalent of Lhermittes symptomEye equivalent of Lhermittes symptom
Pulfrich PhenomenonPulfrich Phenomenon
Optic Neuritis: Physical Optic Neuritis: Physical FindingsFindings
Decreased visual acuityDecreased visual acuityVF defect VF defect (Central/Altitudinal 29% )(Central/Altitudinal 29% )
DyschromatopsiaDyschromatopsiaAfferent Pupil Defect Afferent Pupil Defect (RAPD)(RAPD)Optic disc swelling 35%Optic disc swelling 35%
Abnormal Contrast Abnormal Contrast SensitivitySensitivityAbnormal VEPAbnormal VEPAltered Flicker Altered Flicker PerceptionPerceptionAltered depth perceptionAltered depth perceptionOptic disc pallor Optic disc pallor
Optic Neuritis: Optic DiscOptic Neuritis: Optic Disc
Case: Ms. A.B. 23 YOWFCase: Ms. A.B. 23 YOWF
Two months impaired vision both eyesTwo months impaired vision both eyesProgressive courseProgressive courseBlurred centrally right eyeBlurred centrally right eyeHazy to left of fixation both eyesHazy to left of fixation both eyesOccasional migraineOccasional migraine
Case: Ms. A.B. 23 YOWFCase: Ms. A.B. 23 YOWF
Va 6/15 Right, 6/7.5 LeftVa 6/15 Right, 6/7.5 LeftConfrontation VF: Left Central HHConfrontation VF: Left Central HHNo RAPDNo RAPDAO Plates: 7/14 Rt 10/14 LtAO Plates: 7/14 Rt 10/14 LtOcular motility normalOcular motility normalAnomalous discs both eyesAnomalous discs both eyes
Case: Ms. A.B. 23 YOWFCase: Ms. A.B. 23 YOWFAutomated PerimetryAutomated Perimetry
Case: Ms. A.B. 23 YOWFCase: Ms. A.B. 23 YOWF MRI ImagingMRI Imaging
Case:Case: Ms. C.S. 41 YOWFMs. C.S. 41 YOWF
2 week hx of L sided visual blurring2 week hx of L sided visual blurringBoth eyes involvedBoth eyes involved15 years ago “poor balance”15 years ago “poor balance”MigrainesMigrainesSister with MSSister with MS
Case: Ms. C.S.Case: Ms. C.S.ExaminationExamination
Va: 6/6 Both EyesVa: 6/6 Both EyesAO Plates: 13/14 Rt, 11/14 LtAO Plates: 13/14 Rt, 11/14 LtPupils normalPupils normalOcular motility normalOcular motility normalFundi normalFundi normalDTR’s brisk, Unsteady RombergDTR’s brisk, Unsteady RombergVF’s abnormalVF’s abnormal
Ms. C.S. Visual FieldsMs. C.S. Visual Fields
Ms. C.S. MRIMs. C.S. MRI
Ms. C.S. MRI (2 mos later)Ms. C.S. MRI (2 mos later)
Optic Neuritis: Optic Neuritis: The DifferentialThe Differential
AION (Ischemic Optic Neuropathy)AION (Ischemic Optic Neuropathy) Vasculitic Disorders (i.e. SLE)Vasculitic Disorders (i.e. SLE) Hereditary (i.e. Leber’s)Hereditary (i.e. Leber’s) Toxic/Nutritional (ETOH)Toxic/Nutritional (ETOH) Infectious (i.e.Bartonella, Lyme)Infectious (i.e.Bartonella, Lyme) Inflammatory (i.e. Sarcoid)Inflammatory (i.e. Sarcoid) Neoplastic/Paraneoplastic (i.e. lymphoma)Neoplastic/Paraneoplastic (i.e. lymphoma) Compressive (i.e.Tumours, Grave’s orbitopathy)Compressive (i.e.Tumours, Grave’s orbitopathy) AmblyopiaAmblyopia
Neuro-ophthalmological Neuro-ophthalmological IssuesIssues
DiplopiaDiplopiaHorizontal, Vertical, MixedHorizontal, Vertical, MixedFluctuatingFluctuating
OscillopsiaOscillopsia
Ocular Motility DisordersOcular Motility DisordersInfranuclear or NerveInfranuclear or NerveSaccadic systemSaccadic systemPursuit systemPursuit systemInternuclear abnormalitiesInternuclear abnormalitiesVestibulo-ocular dysfunctionVestibulo-ocular dysfunctionNystagmusNystagmus
Ocular Motility DisordersOcular Motility Disorders
Nuclear Palsies: RareNuclear Palsies: RareInfranuclear or NerveInfranuclear or Nerve
VI: Most commonVI: Most commonIII: Partial or CompleteIII: Partial or CompleteIV: RareIV: Rare
Ms. H.M. 34 YOWFMs. H.M. 34 YOWF
CC: DiplopiaCC: DiplopiaHx: 6 months progressing diplopiaHx: 6 months progressing diplopiaInitially intermittent, now persistentInitially intermittent, now persistentOtherwise asymptomaticOtherwise asymptomaticSister has MSSister has MSO/E: Incomitant esotropiaO/E: Incomitant esotropiaLeft abduction deficitLeft abduction deficit
Ms. H.M. 34 YOWFMs. H.M. 34 YOWF
Ms. H.M. 34 YOWFMs. H.M. 34 YOWF6 Months Later6 Months Later
Ocular Motility DisordersOcular Motility DisordersSaccadic abnormalitiesSaccadic abnormalitiesHypometricHypometricHypermetricHypermetricDysmetriaDysmetriaSaccadic IntrusionsSaccadic Intrusions
Square wave jerksSquare wave jerksSaccadic pulsesSaccadic pulsesOcular flutterOcular flutter
Saccadic AbnormalitiesSaccadic Abnormalities
From: Leigh & Zee. The Neurology of Eye Movements, F.A. Davis Company
Saccadic OscillationsSaccadic OscillationsSaccadic Dysmetria
Macrosaccadic Oscillations
Square Wave Jerks
Macro Square Wave JerksOcular Flutter
From: Leigh & Zee. The Neurology of Eye Movements, F.A. Davis Company
Ocular Motility DisordersOcular Motility Disorders
Square Wave Jerks
Ocular Flutter
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Ocular Motility DisordersOcular Motility DisordersPursuit DysfunctionPursuit Dysfunction
Saccadic IntrusionsSaccadic Intrusions
Internuclear OphthalmoplegiaInternuclear OphthalmoplegiaMLF LesionMLF Lesion
Skew DeviationSkew DeviationVertical diplopiaVertical diplopia
Gaze PalsiesGaze PalsiesDorsal Midbrain SyndromeDorsal Midbrain Syndrome
Ocular Motility DisordersOcular Motility DisordersPursuit DysfunctionPursuit DysfunctionSaccadic IntrusionsSaccadic Intrusions
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Ocular Motility DisordersOcular Motility DisordersInternuclear Ophthalmoplegia:MLF LesionInternuclear Ophthalmoplegia:MLF Lesion
From: Kline & Bajandas. Neuro-ophthalmology Board Review Manual; Slack Inc
Ms.C.P. 24 YOWFMs.C.P. 24 YOWF
CC: Blurred VisionCC: Blurred VisionHx: Hx: • 2 week history of “dizzy” feeling and 2 week history of “dizzy” feeling and
disorientation with looking downdisorientation with looking down• Difficulty focussing on rapid EOM’sDifficulty focussing on rapid EOM’s• 2003 sensory symptoms in legs and Lhermittes 2003 sensory symptoms in legs and Lhermittes
symptomsymptom
O/E: Abnormal EOM’sO/E: Abnormal EOM’s
Ms.C.P. 24 YOWFMs.C.P. 24 YOWF
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Ms.C.P. 24 YOWFMs.C.P. 24 YOWF
T 2 Flair
Internuclear OphthalmoplegiaInternuclear OphthalmoplegiaMRI Detection of MLF LesionsMRI Detection of MLF Lesions Proton density>T2>FlairProton density>T2>Flair Frohman et al Neurology 2001; 57:762-768Frohman et al Neurology 2001; 57:762-768
Proton Density
T2 Flair
Internuclear OphthalmoplegiaInternuclear Ophthalmoplegia
Versional Disconjugacy IndexVersional Disconjugacy Index: Assess adduction : Assess adduction vs abduction saccade peak velocityvs abduction saccade peak velocity
Most accurate method for identification of INO is Most accurate method for identification of INO is quantitative EOM recordingquantitative EOM recording
Clinical detection accuracy vs RecordingClinical detection accuracy vs Recording93% severe INO93% severe INO75% moderate INO75% moderate INO29% mild INO29% mild INOFrohman et al. Neurology 2003;61:848-850Frohman et al. Neurology 2003;61:848-850
Ocular Motility DisordersOcular Motility Disorders
Vestibulo-ocular DysfunctionVestibulo-ocular Dysfunction VOR MismatchVOR Mismatch
Failure of VOR SuppressionFailure of VOR Suppression
Vestibulo-Ocular reflex
From: Leigh & Zee. The Neurology of Eye Movements, F.A. Davis Company
Vestibulo-Ocular reflex
From: Kline & Bajandas. Neuro-ophthalmology Board Review Manual; Slack Inc
Head Thrust TestHead Thrust TestHalmagyi ManeuverHalmagyi Maneuver
Thrust head 20-30 degrees while fixating targetThrust head 20-30 degrees while fixating targetAbnormal:Abnormal:
Refixation saccadeRefixation saccade
Headshake TestHeadshake Test
Shake head for 20 seconds at 2 hz (horizontal Shake head for 20 seconds at 2 hz (horizontal and vertical) with eyes closed, then open and vertical) with eyes closed, then open and observe for nystagmus (Frenzel lenses)and observe for nystagmus (Frenzel lenses)
Abnormal:Abnormal:Unidirectional nystagmus in plane of Unidirectional nystagmus in plane of headshake (peripheral)headshake (peripheral)Vertical nystagmus after horizontal Vertical nystagmus after horizontal headshake (central)headshake (central)
Dynamic Visual Acuity Test*Dynamic Visual Acuity Test*
Read eye chart with eyes open and with slow Read eye chart with eyes open and with slow head shakehead shake
Abnormal:Abnormal:>3 line drop in acuity>3 line drop in acuity
* VOR test
Fixation Suppression Test*Fixation Suppression Test*
Fixate own thumb while chair rotatesFixate own thumb while chair rotatesAbnormal:Abnormal:
Nystagmus in direction of rotationNystagmus in direction of rotation
* VOR suppression test
Failure of VOR SuppressionFailure of VOR Suppression
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Ophthalmoscopic TestingOphthalmoscopic Testing
Spontaneous nystagmusSpontaneous nystagmusRetinal slip: Observe fundus while patient fixates Retinal slip: Observe fundus while patient fixates
target and oscillates head at frequency greater than target and oscillates head at frequency greater than 1 cps1 cps
Abnormal: If the VOR gain is too high the disc Abnormal: If the VOR gain is too high the disc appears to move with the head , if too low, appears to move with the head , if too low, opposite the headopposite the head
Provocative TestingProvocative Testing
Caloric stimuliCaloric stimuliHyperventilationHyperventilationPressure stimulusPressure stimulusSound stimulus (Tullio’s Phenomenon )Sound stimulus (Tullio’s Phenomenon )
NystagmusNystagmus
Gaze evokedGaze evokedDirection changing Direction changing
cerebellarcerebellarDirection selectiveDirection selective
vestibularvestibularAtaxic of INOAtaxic of INOVertical (Upbeat or Vertical (Upbeat or
downbeat)downbeat)
ReboundReboundTorsionalTorsionalAcquired pendularAcquired pendularPeriodic alternatingPeriodic alternatingLid nystagmusLid nystagmusSuperior oblique Superior oblique
myokymiamyokymia**
Interesting but rarely localizing
*Not really a “nystagmus”
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Nystagmus(es) in MS Patient
Ocular Motility DisordersOcular Motility Disorders
Congenital strabismusCongenital strabismusLatent nystagmusLatent nystagmusDVD (Dissociated Vertical Divergence)DVD (Dissociated Vertical Divergence)Convergence spasmConvergence spasmVoluntary nystagmusVoluntary nystagmusCongenital or chronic IVth (FAT scan)Congenital or chronic IVth (FAT scan)Duane’s Retraction SyndromeDuane’s Retraction Syndrome
There are some ocular motility disturbances that have nothing to do with MS.
What is this?What is this?
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Jelly nystagmus: Constant Ocular Oscillation seen in association
with poor vision
Thank You !
Time for Questions