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Neuro-ophthlamology Cases Syndee J Givre, MD, PhD Raleigh Neurology Associates Adjunct Professor Ophthalmology, UNC Chapel Hill

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Neuro-ophthlamologyCasesSyndeeJGivre,MD,PhD

RaleighNeurologyAssociates

AdjunctProfessorOphthalmology,UNCChapelHill

Case1• 34yearoldmansentfor

consultationforchangeinvisionOS

• awokewith"glare"inthetemporalfieldOSabout1.5weekspriorandithadn'tchangedsince

• noeyepainorheadache

• nootherchangeinhealth

• PMH:eczema

• SH:none

• POH:none

• FH/FOH:none

• Medications:none

• Allergies:none

• Tobacco:none

• EtOH:3drinksperweek

Case1

EXAM

• BCVA:20/20OD&20/30OS

• CVF:OD=full,OS=temporaldepression

Case1

Case1

• Pupils:noanisocoria,noRAPD

• Motility:fullOU

• SLE:normalODandOS

• IOP(mmHg):17OD,18OS

• Fundus:normaldisc,vessels,maculaandperiphery

ODandOS

Case1“glare”temporalfield MRI

Case1Case1 Normal

Case1

Case2

• 37yearoldwomansentforconsultationforchangeinvisionOD

– for9daysnoticedaspotofglare,liketheafterimageofabrightlightOD,inthelowernasalcornerofvision

– spotisintheshapeofadiagonalline

– noeyepain/headache

– visionOSfine

• PMH:migraine,sinusinfections,eczema

• POH:none

• SH:orthopedic

• FH:DM,HTN

• FOH:none

• Medications:ExcedrinMigraineprn

• Allergies:PCN

• Tobacco:10cigarettesperdayX25years

• EtOH:onedrinkperday

Case2

EXAM

• BCVA:20/20OD&OS

• CVF:fullODandOS

Case2

Case2

• SLE:normalOD&OS

• IOP:12OD,11OS

• Pupils:noanisocoria,noRAPD

• Motility:fullOU

• IOP(mmHg):18OD&18OS

• Fundus

– Normaldisc,vessels,macula,peripheryOD

– OS

Case2

Case2glareinferiornasalfield cottonwoolspot

glarepituitarytumor cottonwoolspot

Case3

• 17yearoldyoungmansentforconsultationforpapilledema

• neckpainX2months-(evalby

orthonegative)

• doubleandblurredvisionX1month

• infrequentheadaches

• noiseof“drumbeat”inears

• PMH:ADD

• POH:none

• FH:DM,HTN

• FOH:none

• SH:neckcystremovedage2

• 11thgrade,notobacco

• Medications:Adderall

• Allergies:none

Case3

EXAM

• Weight:250lbs

• Bloodpressure:109/91mmHg

• BCVA:20/20OD&20/20OS

• CVF:fullOD&OS

Case3

Case3

• Pupils:noanisocoria,noRAPD

• Motility:fullOU

• SLE:normalODandOS

• IOP(mmHg):9OD,12OS

• Fundus

Case3

Case3

• bilateralopticdiscedemawithgoodvisual

functionandsymptomsofhighICPin250lb

youngman(whosnoresheavilypermom)

Case3

Case3

Case3

• hadpartialresectionwithpathology“inconclusive”

• presumedtobebenigndermoidorepidermoid

cyst

• symptoms,hydrocephalusresolvedwithbiopsy

Case4

• 66yearoldmansentforconsultationforpapilledema

• incidentalfindingonroutineeyeexam(wantednewglasses)

• PMH:prostatecancer(surgery),acidreflux,HTN

• POH:none

• SH:prostate

• FH:none

• FOH:none

• Medications:sildenafil,loperamide

• Allergies:Septra

• Tobacco:none

• EtOH:2drinkspermonth

Case4

EXAM

• Height:70inches

• Weight:184lbs

• Bloodpressure:150/89mmHg

• BCVA:20/20OD&OS

• CVF:fullOD&OS

Case4

Case4

• Pupils:noanisocoria,noRAPD

• Motility:fullOU

• SLE:normalOD&OS

• IOP(mmHg):16OD&12OS

• Fundus

Case4

Case4

• incidentallyfoundoptic

discedema,bilateralwith

goodopticnervefunction

• onquestioning,no

headaches,neckpainor

pulsatiletinnitus(no

symptomshighICP)

• MRIbrainnormal

• MRVbrainnormal

Case4

• Nextstep?

LP

• OP=15(normal)

• RBC=3(normal)

• WBC=38(0-5)

• Glucose=normal

• Protein=47.4mg/dL(0-44)

• Gramstainandculturenegative

• VDRL:1:2(<1:1)

• Subsequent

– RPR:reactive,1:256

– TPPA:positive

Bilateraldiscedemapapilledemafromtumorin250lbchild syphilisin66yearoldman

Case5

• 86yearoldsentforconsultationforpainlessvisionlossOS

• visiongettingworseforacoupleofweeksandthenabout5daysagoarapiddecline

• noeyepain,neckpain,scalptendernessortroublechewing

• occasionalheadaches(norecentchangepatternofthis)

• visionODisfine

• alreadysawretina-someAMD,nothingacute

Case5

• PMH:breastcancer(lumpectomy,XRT,tamoxifen),thyroidproblems,DM,HTN,dyslipidemia,onefunctioningkidney

• POH:cataractoperationOU

• SH:R&Lcarotidendarterectomy,thyroid,lumpectomy

• FH:DM,HTN,stroke

• FOH:none

• Tobacco:none/EtOH:none

• Medications:zocor,ASA,metformin,norvasc,metoprolol,benazipril,synthroid

• Allergies:sulfa

Case5

• Exam:

• BCVA:20/40OD&CFOS

• CVF:fullOD&canseeHMinallquadrantsOS

• Pupils:noanisocoria,+RAPDOS

• Motility:tracelimitationleftabductionotherwise

fullOU

Case5

• SLE:PCIOLOU

• IOP(mmHg):19OD&19OS

• Fundus:normaldisc,vessels,peripheryOU;

changesofAMDinmaculaOU,noDR

Case5

• Comeswithnormal

ESR,CRP,PLT • Imaging

Case5

Case5

Case5

• immediatelystartedPrednisoneand

underwentendoscopicorbitaldecompression

withinaweek

• within2weeksaftersurgeryvisionOS

improvedto20/40

Case5

• 8monthslatercomesinforregularfollowup

sayingvisionODhasbeengettingworsefora

fewweeks

• OSisstable

Case5

EXAM

• BCVA20/200OD&20/50OS

• CVF:ODconstricted,OSfulltoHM

• remainderexamunchanged

Case5

Case5initialCT 8monthslater

Case5

• shehadendoscopicrightorbital

decompressionandvisionreturnedto20/40

OD

Case6

• 56yearoldmansentforpossibleNA-AIONOS

• noticedsuddenvisionlossabout3weeksprior

• nochangesince

• somepaininandbehindtheeyewhenthevisionlossfirstoccurredbutthat

wentawaywhenheresumedtakingBPandcholesterolmedication.

• ODisfine

• wifesayshesnoresheavily

• PMH:HTN,dyslipidemia

• POH:none

• SH:none

• FOH:none

• FH:DM

• Tobacco:none/EtOH:none

• Medications:HCTZ,amlodipine,crestor

• Allergies:none

Case6

• EXAM

• BCVA:20/15OD,HMOS

• CVF:fullOD

• Pupils:noanisocoria,+RAPDOS

• Motility:fullOU

• SLE:normalOU

• IOP(mmHg):12OD,13OS

Case6

Case6

• suddenonsetvision

loss,notprogressive,+/-

painwithswollendisc

onexam

Case6

• ForAION

– suddenvisionloss,not

progressive

– riskfactors-HTN,

dyslipidemia,heavysnoring

(probablesleepapnea)

• AgainstAION

– ?painatonset

– other(right)discnotcrowded

Case6

– other(right)discnot

crowded

Case6

Case6

Case7

• 47yearoldmanforconsultationforincidentallyfoundleftopticdiscedema.

• inforaroutinecheckfornewglasseswhenthiswasnoted

• PMH:DM,HTN,OSAdiagnosedbutnevertreated

• POH:none

• SH:appendectomy

• FH:DM

• FOH:glaucoma

• Tobacco:none/EtOH:2drinksperday

• Medications:insulin,losartan

• Allergies:none

Case7

EXAM

• BCVA20/20OD&20/20OS

• CVF:fullODandOS

Case7

Case7

• SLE:normalOD&OS

• Pupils:noanisocoria,noRAPD

• Motility:fullOU

• IOP(mmHg):18OD&17OS

• Fundus

Case7

Case7

Case7

• unilateral,

asymptomaticopticdisc

edema,noRAPD,good

acuityandVF

opticdiscedema

• inflammation/neuritis/infectious

• ischemia

• retrobulbarcompression

• asymmetricpapilledema

• trauma

• toxic

• hereditary/LHON

• diabeticpapillitis

• papillophlebitis

Case7

• retrobulbarcompression

• asymmetricpapilledema

• diabeticpapillitis

• papillophlebitis

Case7

• normalMRIandMRV

• LP

– OP17

– CSFcompositionnormal

• retrobulbar

compression

• asymmetric

papilledema

• diabeticpapillitis

• papillophlebitis

Case7

• patientseenmultipletimeswithnochangeinexam

• onscheduledf/uabout4monthsafterinitialexam

complainsof4daysofblurrinessOD,painless

• repeatsleepstudy

confirmedOSAandpatientusingCPAPregularly

• bloodsugarsunderbestcontrolsincediagnosis

• otherwisenochangein

medicalhistory

Case7

EXAM

• BCVA20/20OD&20/20OS

• CVF:fullODandOS

Case7

Case7

Case7

Case7

• bilateralsequentialdiscedema

– initialeyeimprovingwithoutvisuallos

– secondeyepresentingwithinferiorfieldloss

• ?sequentialAIONwithfirsteyeaborting

withoutinfarct

Help!• ClinicaltrialofQPI-1007inimprovingvisualoutcome

inAION

• QPI-1007isacustom-designed,small-interfering

RNAthatbindstothemessengerRNAofcaspase2

andpreventsthemessengerRNAfrombeing

translatedintoaprotein

ThankYou

Help!

• apoptosisisonecellularprocessbelievedtounderlieretinalganglioncelldeath(opticnervedeath)inAION

• caspase2isaninitiatorofapoptosis

• byinterferingwiththeproductionofcaspase2,QPI-1007mayinhibitapoptosisanddecreasecelldeath(=preservevision)inAION

Help!

• multicenter,double-blind,placebocontrolledstudy

• patientswithin14daysofonsetofsymptomsofAIONwillreceiveintravitrealinjectionofeitherQPI-1007orashaminjectionandwillbefollowed

• measuredvisualoutcomeswillbeBCVAandautomatedperimetry(SITA)

Help!

• 14daysofonsetofsymptomsofAION

Case8• 28yearoldwomansentbyneurologyforconsultationforbilateral

opticneuritisseenonMRI12/2015

• senttoneurologyforfurtherworkupandmanagementin3/2015

• bythetimeIsawhershehadbeentreatedwithsteroidsandpain

medicationandsaidvisionwasbacktobaselinebutshewasstillhavingsevereheadaches3timesperweek.

• shehadalreadyhadMRIbrainX2,MRIspine(cervicalandthoracic)andalumbarpuncture-allnegativeexcepttheopticnervefindings

onMRI

Case8• PASTMEDICALHISTORY:headaches,h/ochildhoodleukemia

successfullytreated10yearsprior

• SURGICALHISTORY:cholecystectomy,myringotomytubes,benigngrowthleftear

• PASTOCULARHISTORY:congenitalptosisOD,h/ograduallyworseningvisionODoverpastfewyearstoldduetoastigmatism

• SOCIALHISTORY:neversmoker,1drinkpermonh

• FAMILYMEDICALHISTORY:heartdisease

• FAMILYOCULARHISTORY:glaucoma,AMD

• Medications:birthcontrol

• Allergies:none

Case8

EXAM

• BCVA:20/30OD&20/20OS

• CVF:fullOD&OS

Case8

Case8

• SLE:mildRULptosis(unchangedperpatient)

otherwisenormalOD&OS

• Pupils:noanisocoria,0.6logunitRAPDOD

• Motility:fullOU

• IOP(mmHg):9OD,11OS

Case8

• DILATEDFUNDUSEXAM

• OpticDiscs

• OD:pale,noedema,c/d0.1

• OS:nopallororedema,c/d0.1

• Vitreous:clearOU

• Retina

• Macula:normalOU

• Vessels:normalOU

• Periphery:normalOU

Case8

• DATA

• MRIbrainandorbits-

• MRIthoracicandcervicalspine(lookingforevidenceMS)-negative

• Labs

– blood:ACE,ANA,NMO,CBC,anticardiolipinantibodiesallnegativeornormal

– CSF:cellcount,protein,glucose,cytology,MSmarkersallnegativeornormal

Case8

Case8

• patienthadbeensenttouniversityeyeclinicJanuary2013forabnormaldiscandVFdefectOD

• discwasthoughttobeeitherpaleoranomalousanditwasrecommendedtothePCPtoorderan

MRIbutthisnevergotdone(until12/2015-patientwentintoPCPwithcomplaintofheadaches)

Case8

• bythetimeIsawherMRIhadbeenrepeated

(monthslater,aftersteroids)andwas

unchanged

Case8

Timeline

• 1/2013:abnormalVFanddiscOD,MRIrecommended

• 12/2014:presentstoPCPwithheadaches,MRIshows

bilateralopticnerveenhancement(“opticneuritis”)

• 3/2015:lab,CSF,MRIspineworkupforcausesoptic

neuritisnegative

• 6/2015repeatorbitMRIunchanged

Case8

• Whyisn’tthis(bilateral)opticneuritis?

– Progressionveryslow-20/30ODafter>2years

– Painless(presumingtheintermittentheadaches

areunrelated)

– VisualfunctionOSisnormaldespitethatnerve

lookingjustasbadasODontheMRI

Case8

Case9

• 69yearoldwomansentneurologistfortroublewithperipheralvision

• milddementia,accompaniedbysonwhohelpswiththehistory.

• sonsaysthepatient’seyedoctordidaperipheralvisiontestandmom“failed”

• Whenaskedwhathenoticessayshas

difficultyseeingifthelightisdimordarkX1yearorso

• PMH:COPD,depression,GERD,RA,neuropathy,seizures,OSA,dementia,

hypothyroid

• POH:cataractoperations

• SX:hysterectomy,orthopedic

• FH/FOH:negative

• Tobacco:neversmoked

• EtOH:onedrinkperweek

• Medications:aricept,gabapentin,

synthroid,fluoxetine,pantoprazole,requip,celexa,lamictal,trazedone

• Allergies:sulfa

Case9

EXAM

• BCVA:20/40OD&20/40OS

• CVF:couldseefingerwigglinginall4quadrantsOD&OS

• Pupils:noanisocoria,noRAPD

• Motility:fullOD&OS

• SLE:PCIOLOD&OSotherwisenormal

• IOP(mmHg):14ODand17OS

• Fundus:

Case9

Case9

Case10

• 59yearoldwomansentforseeingflashesoflight

• noticedabout14-16monthsagowhileonHerceptintreatmentforbreastcancer

• initiallynoticedasquare"windowoflight"thatmovedaroundinthe

peripheryoflefteyeforafewsecondsthendisappears

• afterafewmonthsshebegantonoticethesymptomODbut“notasbad”

• PMH:breastcancer(sx,xrt,chemo),

HTN,IBS

• POH:refractivesurgeryOU,CEOU

• SX:carpaltunnel,hysterectomy,mastectomy,orthopedic

• FH:DM,thyroiddisease

• FOH:retinaldetachment

• Tobacco/EtOH:never

• Meds:quinapril,tamoxifen

• Allergies:PCN

Case10

EXAM

• BCVA:20/20OD&20/20OS

• CVF:fullODandOS

Case10

Case10

• Pupils:noanisocoria,noRAPD

• Motility:fullOU

• SLE:normalODandOS

• IOP(mmHg):14OD&14OS

• Fundus:

Case10

Case10

ThankYou