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
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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
ANTERIOR ISCHEMIC OPTIC NEUROPATHY
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
ANTERIOR ISCHEMIC OPTIC NEUROPATHY
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
ANTERIOR ISCHEMIC OPTIC NEUROPATHY
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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ANTERIOR ISCHEMIC OPTIC NEUROPATHY
ARTERITIS: LATEARTERITIS: LATECUPPING OPTICCUPPING OPTIC
NERVE NERVERare in NAIONRare in NAIONIf fellow eyeIf fellow eyeexcavated,excavated, considerconsiderAAIONAAION
ANTERIOR ISCHEMIC OPTIC NEUROPATHY
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
ANTERIOR ISCHEMIC OPTIC NEUROPATHY
ARTERITICARTERITIC
If AION has associatedIf AION has associatedcilioretinal arterycilioretinal arteryocclusion,occlusion, suspectsuspecttemporal arteritistemporal arteritis
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ANTERIOR ISCHEMIC OPTIC NEUROPATHY
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
ANTERIOR ISCHEMIC OPTIC NEUROPATHY
ARTERITIC FAARTERITIC FA
ANTERIOR ISCHEMIC OPTIC NEUROPATHY
ARTERITIC FA:ARTERITIC FA:CHOROIDALCHOROIDALISCHEMIAISCHEMIA
Fundus may show noFundus may show novisible disc edemavisible disc edemaChoroid edematousChoroid edematous
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ANTERIOR ISCHEMIC OPTIC NEUROPATHY
ARTERITIC FA:ARTERITIC FA:CHOROIDALCHOROIDALISCHEMIAISCHEMIA
Widespread choroidalWidespread choroidalnonfilling; disc fillsnonfilling; disc fillsnormally, peripapillarynormally, peripapillaryretinal leakageretinal leakage
AION: IS IT TEMPORALARTERITIS?
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
ANTERIOR ISCHEMIC OPTIC NEUROPATHY
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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ANTERIOR ISCHEMIC OPTIC NEUROPATHY
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
ANTERIOR ISCHEMIC OPTIC NEUROPATHY
ARTERITIC:ARTERITIC:TEMPORALTEMPORALARTERY BIOPSYARTERY BIOPSY
Positive biopsy provesPositive biopsy provesdiagnosisdiagnosis
ANTERIOR ISCHEMIC OPTIC NEUROPATHY
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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ANTERIOR ISCHEMIC OPTIC NEUROPATHY
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
ANTERIOR ISCHEMIC OPTIC NEUROPATHY
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
ANTERIOR ISCHEMIC OPTIC NEUROPATHY
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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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
AION: IS IT TEMPORALARTERITIS?
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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