204-neuro basics.pdf

8/9/2019 204-Neuro Basics.pdf

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Neuro-ophthalmology:The Basics

MadhuMadhu R. Agarwal, M.D.R. Agarwal, M.D.California Orbital ConsultantsCalifornia Orbital Consultants

RedlandsRedlands, California, California

Who is Dr. Agarwal??? Neuro Neuro--ophthalmologyophthalmology

Cranial nerve palsiesCranial nerve palsiesOptic neuropathiesOptic neuropathiesBrain tumorsBrain tumors

Ocular plasticsOcular plasticsLid lesionsLid lesionsPtosisPtosisOrbital tumorsOrbital tumors

Adult StrabismusAdult Strabismus


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Skin Cancer

Right Sixth and Seventh Palsy


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Plain Old Droopy Eyelids

History of Present Illness

Time course:Time course:

progressive?? progressive??

Was there associated pain?Was there associated pain?

Are both eyes involved?Are both eyes involved?

Have ou ever had this before??Have ou ever had this before??

Are you having other neurologicalAre you having other neurological problems? problems?

Examples: headaches, paralysis, hearingExamples: headaches, paralysis, hearingloss.loss.


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Review of Systems

FatigueFatigueMalaiseMalaise

Weight lossWeight lossAnorexiaAnorexiaScalp tendersnessScalp tendersness

Past Medical History

Very importantVery important

DMDM

Heart DiseaseHeart Disease

Medications

Can be toxic to the optic nerve!Can be toxic to the optic nerve!

Viagra, Levitra, Cialis!!!Viagra, Levitra, Cialis!!!


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Exam

Vision: BEST CORRECTED!Vision: BEST CORRECTED!

PupilsPupilsAnisocoriaAnisocoriaAfferent Pupillary DefectAfferent Pupillary Defect

Pupils!

Beast of itself!Beast of itself!

Easy to create one!Easy to create one!

Careful in cases of strabismusCareful in cases of strabismus

Orbital Exam

Is there proptosis?Is there proptosis?

Is there a bruit?Is there a bruit?


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Motility Exam

Ductions?Ductions?

Comitant?Comitant?

Visual Field

ConfrontationConfrontation

--

Optic Nerve Exam

Is it normal in size or small??Is it normal in size or small??

Are the vessels normal in appearance??Are the vessels normal in appearance??


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TEMPORAL ARTERITIS

HEADACHE + VISUAL LOSSHEADACHE + VISUAL LOSSIS IT TEMPORAL ARTERITIS????IS IT TEMPORAL ARTERITIS????

VsVs ICP (no mass)ICP (no mass)Vs meningeal process (meningitis,Vs meningeal process (meningitis,infiltration)infiltration)Vs Corneal epithelial defectVs Corneal epithelial defect

TEMPORAL ARTERITIS

OPHTHALMIC MANIFESTATIONSOPHTHALMIC MANIFESTATIONSIschemic optic neuropathy (AION,Ischemic optic neuropathy (AION,PIONPIONChoroidal ischemiaChoroidal ischemiaCentral retinal artery occlusionCentral retinal artery occlusionStroke of CNS visual pathwaysStroke of CNS visual pathwaysIschemic cranial n/EOM dysfunctionIschemic cranial n/EOM dysfunction

ANTERIOR ISCHEMIC OPTIC NEUROPATHY

Is it temporal arteritis?Is it temporal arteritis?


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AION: IS IT TEMPORALARTERITIS?

AgeAgeAssociated symptomsAssociated symptomsAssociated signsAssociated signsSeverity of visual lossSeverity of visual lossBilateral simultaneous or rapid sequential onsetBilateral simultaneous or rapid sequential onsetDisc appearanceDisc appearanceChoroid appearanceChoroid appearance


ARTERITICARTERITICMean age 70Mean age 70S stemic s m toms: headache awS stemic s m toms: headache awclaudicationclaudication, temporal tenderness, weight loss, temporal tenderness, weight lossErythrocyte sedimentation rate mean 70 mm/hr Erythrocyte sedimentation rate mean 70 mm/hr Severe visual loss (< 20/200 in 65%)Severe visual loss (< 20/200 in 65%)Caucasians most commonly affected Caucasians most commonly affected

TEMPORAL ARTERITIS

HEADACHEHEADACHEMost common symptom (up to 90%)Most common symptom (up to 90%)

Often severe constantOften severe constantLocalized or generalized Localized or generalized Associated scalp/temporal tendernessAssociated scalp/temporal tenderness

Over temporal, occipital arteriesOver temporal, occipital arteries


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TEMPORAL ARTERITIS

CLAUDICATIONCLAUDICATIONJawJaw wellwell--known, but also neck, ear, pharynxknown, but also neck, ear, pharynx(facial artery occlusion)(facial artery occlusion)Differentiate from TMJ, other causes of painDifferentiate from TMJ, other causes of painHigh level of suspicionHigh level of suspicion


ARTERITICARTERITICHayreh et al (1998): 13/94 eyes (13.9%) with AAIONHayreh et al (1998): 13/94 eyes (13.9%) with AAION

presented with preceding amaurosis fugax presented with preceding amaurosis fugax- -like transientlike transientvisual lossvisual loss

TRANSIENT VISUAL LOSS PRECEDINGTRANSIENT VISUAL LOSS PRECEDING NONARTERITIC NONARTERITIC AION IS RAREAION IS RAREIf AION preceded by transient visual loss,If AION preceded by transient visual loss, suspectsuspecttemporal arteritistemporal arteritis

Hayreh SS, et al.Hayreh SS, et al. Am J Ophthalmol Am J Ophthalmol 1998;125:5091998;125:509- -2020


ARTERITICARTERITICDisc edema often paleDisc edema often palemay be chalk may be chalk- -whitewhite

rat orat o normanormaMay see peripapillaryMay see peripapillarychoroidal ischemiachoroidal ischemia(blur)(blur)


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ARTERITIS: LATEARTERITIS: LATECUPPING OPTICCUPPING OPTIC

NERVE NERVERare in NAIONRare in NAIONIf fellow eyeIf fellow eyeexcavated,excavated, considerconsiderAAIONAAION


ARTERITICARTERITICHayreh et al (1998): 21.2% of eyes withHayreh et al (1998): 21.2% of eyes withAAION had associated cilioretinal arteryAAION had associated cilioretinal arteryocclusionocclusionCILIORETINAL ARTERY OCCLUSION ISCILIORETINAL ARTERY OCCLUSION ISRARE IN NAIONRARE IN NAION

Hayreh SS, et al.Hayreh SS, et al. Am J Ophthalmol Am J Ophthalmol 1998;125:5091998;125:509- -2020


ARTERITICARTERITIC

If AION has associatedIf AION has associatedcilioretinal arterycilioretinal arteryocclusion,occlusion, suspectsuspecttemporal arteritistemporal arteritis


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ARTERITIC: PATHOGENESISARTERITIC: PATHOGENESISGranulomatous inflammation of SPCAs withGranulomatous inflammation of SPCAs withthrombotic occlusionthrombotic occlusionIschemia/infarct of optic nerve headIschemia/infarct of optic nerve head + + adjacentadjacentchoroid choroid


ARTERITIC FAARTERITIC FA


ARTERITIC FA:ARTERITIC FA:CHOROIDALCHOROIDALISCHEMIAISCHEMIA

Fundus may show noFundus may show novisible disc edemavisible disc edemaChoroid edematousChoroid edematous


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ARTERITIC FA:ARTERITIC FA:CHOROIDALCHOROIDALISCHEMIAISCHEMIA

Widespread choroidalWidespread choroidalnonfilling; disc fillsnonfilling; disc fillsnormally, peripapillarynormally, peripapillaryretinal leakageretinal leakage


Fluorescein angiographyFluorescein angiographyBlood testsBlood tests

Erythrocyte sedimentation rate (ESR)Erythrocyte sedimentation rate (ESR)CC--reactive protein (CRP)reactive protein (CRP)FibrinogenFibrinogenPlatelet countPlatelet count

Temporal artery biopsyTemporal artery biopsy


ARTERITIC: ERYTHROCYTEARTERITIC: ERYTHROCYTESEDIMENTATION RATE (ESR)SEDIMENTATION RATE (ESR)

Nonspecific inflammatory marker Nonspecific inflammatory marker Mean Westergren 70 mm/hr, often > 100 mm/hr Mean Westergren 70 mm/hr, often > 100 mm/hr

Normal max: age/2 (male); age + 10/2 (female) Normal max: age/2 (male); age + 10/2 (female)In elderly may be 50In elderly may be 50- -60 mm/hr 60 mm/hr

In GCA, 2% may beIn GCA, 2% may be < < 15 mm/hr 15 mm/hr


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ARTERITIC: CARTERITIC: C- -REACTIVE PROTEIN (CRP)REACTIVE PROTEIN (CRP)Acute phase plasma protein, not influenced by age orAcute phase plasma protein, not influenced by age orhematologic factorshematologic factors

ore spec c t anore spec c t anESR (>47 mm/hr) + CRP (>2.45 mg/dl) 97% specificESR (>47 mm/hr) + CRP (>2.45 mg/dl) 97% specific

for temporal arteritis in AIONfor temporal arteritis in AION

Hayreh SS:Hayreh SS: Am J Ophthalmol Am J Ophthalmol 1997;123:2851997;123:285- -296296


ARTERITIC:ARTERITIC:TEMPORALTEMPORALARTERY BIOPSYARTERY BIOPSY

Positive biopsy provesPositive biopsy provesdiagnosisdiagnosis


ARTERITIC: TEMPORAL ARTERY BIOPSYARTERITIC: TEMPORAL ARTERY BIOPSY Negative biopsy does Negative biopsy does notnot rule out diagnosisrule out diagnosis

False negatives:False negatives: Skip lesionsSkip lesions Insufficient sampleInsufficient sample Uni (contra) lateral lesionsUni (contra) lateral lesions Inadequate sectioningInadequate sectioning Inexperienced interpretationInexperienced interpretation


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ARTERITIC: TEMPORAL ARTERY BIOPSYARTERITIC: TEMPORAL ARTERY BIOPSYOptions:Options:

Frozen section 1Frozen section 1 stst side, biopsy 2side, biopsy 2 nd nd if negativeif negativePermanent section 1Permanent section 1 stst side, biopsy 2side, biopsy 2 nd nd if negativeif negativeand clinical suspicion highand clinical suspicion highBilateral biopsy initially all casesBilateral biopsy initially all cases


ARTERITIC: THERAPYARTERITIC: THERAPYSystemic steroidsSystemic steroids

IV methylprednisolone 1 gm/dayIV methylprednisolone 1 gm/dayOral prednisone > 1mg/kg/dayOral prednisone > 1mg/kg/day

May delay biopsy 7May delay biopsy 7- -10 after initiation10 after initiationIf GCA suspected,If GCA suspected, begin steroids immediately begin steroids immediately

Goal toGoal to prevent fellow eye visual loss prevent fellow eye visual lossFellow eye affected in up to 95% untreated Fellow eye affected in up to 95% untreated


ARTERITIC: THERAPYARTERITIC: THERAPYImprovement in affected eye not commonImprovement in affected eye not common

Breakthrou h on thera 10Breakthrou h on thera 10- -15%15%Risk of recurrence on steroid taper 7%Risk of recurrence on steroid taper 7%

Prime reason to biopsy: confidence in diagnosisPrime reason to biopsy: confidence in diagnosis Prevent too rapid taper, discontinuance ofPrevent too rapid taper, discontinuance of

steroids after initial therapysteroids after initial therapy


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AgeAgeAssociated symptomsAssociated symptomsAssociated signsAssociated signsSeverity of visual lossSeverity of visual lossBilateral simultaneous or rapid sequential onsetBilateral simultaneous or rapid sequential onsetDisc appearanceDisc appearanceChoroid appearanceChoroid appearance


Fluorescein angiographyFluorescein angiographyBlood testsBlood tests

Erythrocyte sedimentation rate (ERS)Erythrocyte sedimentation rate (ERS)CC--reactive protein (CRP)reactive protein (CRP)FibrinogenFibrinogenPlatelet countPlatelet count

Temporal artery biopsyTemporal artery biopsy

Case

25 year 25 year--old Caucasian male referred forold Caucasian male referred for worseningworsening33rd rd nerve palsy OS x 2 monthsnerve palsy OS x 2 months

Va 20/20 OUVa 20/20 OU

Pupils equal, round OUPupils equal, round OU

PF 10, 3: previously 6 mm OSPF 10, 3: previously 6 mm OS


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EOM: Worsening!EOM: Worsening!

MRI negativeMRI negative

MRA negativeMRA negative


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Here is a patient who is young with aHere is a patient who is young with aworsening 3worsening 3 rd rd nerve palsy, evolving over anerve palsy, evolving over afew MONTHS!few MONTHS!

If it were inflammatory or viral, he wouldIf it were inflammatory or viral, he would be better over months, not worse! be better over months, not worse!

Ischemic palsy in your 20s??Ischemic palsy in your 20s??

Any other thoughts?Any other thoughts?

Myasthenia gravisMyasthenia gravis

Graves diseaseGraves disease


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Myasthenia Gravis

Autoimmune disorder in which the bodyAutoimmune disorder in which the bodymakes autoantibodies for the acetylcholinemakes autoantibodies for the acetylcholinereceptor receptor

Characterized by severe fatigue of musclesCharacterized by severe fatigue of muscles

Women in 20s and 30s and men over 60Women in 20s and 30s and men over 60

Severe fatigue which improves with restSevere fatigue which improves with rest

Eye muscles: ptosis, diplopiaEye muscles: ptosis, diplopia

Facial muscles: transverse smile, difficultyFacial muscles: transverse smile, difficultychewing, swallowingchewing, swallowing

Trunk: limb weakness, respiratoryTrunk: limb weakness, respiratory

How to check

Cogans lid twitchCogans lid twitch

BienfangsBienfangs

Squeeze against your forceful openingSqueeze against your forceful opening


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Testing

Tensilon: acetylcholinestrase inhibitor toTensilon: acetylcholinestrase inhibitor tosee if diplopia or ptosissee if diplopia or ptosisimproves.improved improves.improved

Acetylcholine receptor antibody: 3.0 with aAcetylcholine receptor antibody: 3.0 with areference range of


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Pupils are equal without APD OUPupils are equal without APD OU

Palpebral fissure 10 mm OD, 4mm ODPalpebral fissure 10 mm OD, 4mm ODLevator Function, 18 mm OD, 7mm OSLevator Function, 18 mm OD, 7mm OS

No proptosis or orbital erythema No proptosis or orbital erythema

SLE: unremarkableSLE: unremarkable

,,

Photo


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MRI and MRA was brought in, negativeMRI and MRA was brought in, negative

1 week later, completely resolved 1 week later, completely resolved

rd rd

CN 3 palsy

Oculomotor NerveOculomotor NerveMedial RectusMedial Rectus

Superior RectusSuperior RectusInferior ObliqueInferior ObliqueLevator MuscleLevator Muscle


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Work-up

Rule out other neurological signsRule out other neurological signs

,,

If yes, emergent imagingIf yes, emergent imaging

Testing

Watch for pupil if partialWatch for pupil if partial

Viral common in childrenViral common in children

Treatment

In this case, observationIn this case, observation

If aneurysm, clip or coilIf aneurysm, clip or coil

If normal pupil and complete with ischemic riskIf normal pupil and complete with ischemic riskfactors, observefactors, observe

If anything funny, image!If anything funny, image!


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Case

65 year 65 year--old Caucasian male with binocularold Caucasian male with binocularhorizontal diplopia for 5 days.horizontal diplopia for 5 days.

DiabetesDiabetes

HypertensionHypertension

A bit obeseA bit obese

PupilsPupils

Anterior and Posterior segments are normalAnterior and Posterior segments are normal

No proptosis, tenderness No proptosis, tenderness

EOM: 10 PD ETEOM: 10 PD ET

18 PD ET in left gaze18 PD ET in left gaze

3 mm abduction deficit in left gaze3 mm abduction deficit in left gaze


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Photo

Slam Dunk or is it??

Of course, microvascular 6Of course, microvascular 6 thth nerve palsy!nerve palsy!

Patient was observed for one month andPatient was observed for one month andsent to Loma Linda for evaluation.sent to Loma Linda for evaluation.

MRI


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What lies beneath

Cavernous sinus usually involves multipleCavernous sinus usually involves multiplenervesnerves

MeningiomasMeningiomas

Vascular abnormalitiesVascular abnormalities

Case

45 year 45 year--old Chinese female referred forold Chinese female referred forbroken down phoriabroken down phoria

Pt states never had a problem with doublePt states never had a problem with doublevision until recently.vision until recently.

No personal or family history of strabismus No personal or family history of strabismus


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Past Medical History: HypertensionPast Medical History: Hypertension

done 3 months agodone 3 months ago

Exam

Vision 20/20 OUVision 20/20 OU

Orbital exam nlOrbital exam nl


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Case

55 year 55 year--old complaining of worseningold complaining of worseningvisual acuity OS x 2 yearsvisual acuity OS x 2 years

PMH: Hepatitis CPMH: Hepatitis CMeds: InterferonMeds: InterferonPSH: HEAVY drugs and alcohol x 25PSH: HEAVY drugs and alcohol x 25years, clean for the last 12 years.years, clean for the last 12 years.

Va cc 20/25 OD, 20/60 OSVa cc 20/25 OD, 20/60 OS

,,

1+ APD OS1+ APD OS


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Normal external exam Normal external exam

. .

Normal anterior segments. Normal anterior segments.

Optic Nerves

Is this interferon optic neuropathy?Is this interferon optic neuropathy?

--

Is this AION??Is this AION??


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Visual Fields

MRI

MRI


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Large Meningioma!

Symptoms of Sellar Masses

HeadacheHeadache

GalactorrheaGalactorrhea

GynecomastiaGynecomastia

Signs

Vision lossVision loss

Bitemporal hemianopsia!!Bitemporal hemianopsia!!


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Treatment

Hormone therapy to shrink Hormone therapy to shrink

Radiation for certain casesRadiation for certain cases

Case

44 year 44 year--old Chinese male reports slow,old Chinese male reports slow, progressive vision loss over 1 year in both progressive vision loss over 1 year in botheyeseyes

Extensive work Extensive work- -up done over 12 monthsup done over 12 monthsdocumenting severe vision and visual fielddocumenting severe vision and visual fieldlossloss

Neurology and Ophthalmology Neurology and OphthalmologyDifferentialDifferential

Optic neuritisOptic neuritisInfectious Optic neuropathyInfectious Optic neuropathyAutoimmune optic neuropathyAutoimmune optic neuropathy


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Meds: Biaxin, EthambutolMeds: Biaxin, Ethambutol

No PSH No PSH

Va CF OUVa CF OU

,,

Nl orbital exam Nl orbital exam

IOP, Anterior Segment Nl OUIOP, Anterior Segment Nl OU

Optic Nerves


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Ethambutol Optic Neuropathy

Recommended dosage 12.5mg/kg/day toRecommended dosage 12.5mg/kg/day torevent e e diseaserevent e e disease

Average dosage 15Average dosage 15- -20 mg/kg/day20 mg/kg/day

Our Patient

120 pound male120 pound male

Slow progressive vision loss.Slow progressive vision loss.

Ethambutol stopped p 14 months of useEthambutol stopped p 14 months of use

Treatment

Stop medication; often recovers within 1Stop medication; often recovers within 1year year

Work with pulmonologistWork with pulmonologist

Use multivitamins with heavy copper andUse multivitamins with heavy copper andzinczinc


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American Thoracic Society

Guidelines as of July, 2005Guidelines as of July, 2005

ethambutolethambutol

Watch color vision and visual acuityWatch color vision and visual acuity

Case

52 year 52 year--old Africanold African--American female hereAmerican female herefor routine diabetic eye exam.for routine diabetic eye exam.

PMH: Breast cancer s/p mastectomyPMH: Breast cancer s/p mastectomy

No medications No medications

Va 20/20 OUVa 20/20 OU

2mm pupil OD, 4mm OS2mm pupil OD, 4mm OSDark 3mm OD, 8mm OSDark 3mm OD, 8mm OS

No APD No APD

PF: 6 mm OD, 9mm OSPF: 6 mm OD, 9mm OS


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Lymphedema


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Differential Diagnoses of Horner'sDifferential Diagnoses of Horner'sSyndromeSyndrome1st order neuron:1st order neuron:

StrokeStrokeVertebrobasilar arter insufficiencVertebrobasilar arter insufficienc causing lateral medullary syndromecausing lateral medullary syndromeMultiple sclerosisMultiple sclerosisSyringomyeliaSyringomyelia

2nd order neuron (spinal cord,2nd order neuron (spinal cord,thoracic cavity, and low er neck)thoracic cavity, and low er neck)

Severe osteoarthritis of the neck withSevere osteoarthritis of the neck withbony spursbony spurs

, ,, ,neurofibroma, metastasis)neurofibroma, metastasis)

Aortic aneurysm Aortic aneurysmInferior neck trauma or postInferior neck trauma or post- -surgicalsurgicaldamagedamage

3rd order neuron (upper neck and carotid3rd order neuron (upper neck and carotidarterial pathway)arterial pathway)

Cluster headacheCluster headacheCarotid artery dissectionCarotid artery dissectionNasopharyngeal tumorsNasopharyngeal tumors

Cavernous sinus mass or inflammation, ie.Cavernous sinus mass or inflammation, ie.TolosaTolosa- -Hunt syndrome)Hunt syndrome)Herpes Zoster (HZO)Herpes Zoster (HZO)Otitis mediaOtitis mediaTrauma or postTrauma or post- -surgical damagesurgical damageRaeder paratrigeminal syndromeRaeder paratrigeminal syndrome


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204-neuro basics.pdf

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