REPORTOFCASESCASE1A31yearoldwomanwithnosignificantmedicalhistorywasadmittedtothehospitalforblurredvisioninthelefteyeandmildfrontalheadachefor2daysThepatientreportedhorizontaldiplopiaandpainonrigh ID: 187004
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SuspectingOpticNeuritis,DiagnosingCatScratchDiseaseJoannaJ.Gan,BS;AlanM.Mandell,MD;JamesA.Otis,MD;MadinaHolmuhamedova,MD;MichaelD.Perloff,MD,PhDcatscratchdiseaseisclassi-callyafebrileillness,inconjunctionwithlymphadenop-athyandcatexposure. REPORTOFCASESCASE1A31-year-oldwomanwithnosignificantmedicalhistorywasadmittedtothehos-pitalforblurredvisioninthelefteyeandmildfrontalheadachefor2days.Thepa-tientreportedhorizontaldiplopiaandpainonrightgaze.Visualacuityintherighteyewasbaseline(20/20);inthelefteye,itwas AuthorAffiliations:DepartmentsofNeurology(MsGanandDrsMandell,Otis, Downloaded From: http://archneur.jamanetwork.com/ by a Boston University User on 06/24/2014 (REPRINTED)ARCHNEUROL/VOL68(NO.1),JAN2011WWW.ARCHNEUROL.COM ©2011AmericanMedicalAssociation.Allrightsreserved. resonanceimaging,andbrainmagneticresonanceveno-gram)werenormal.Theresultsofroutinebloodtestswerenormal;thelevelsofglycatedhemoglobin,thyrotropin,andB12werenormalaswell.C-reactiveprotein(CRP)anderythrocytesedimentationrate(ESR)wereelevatedat3.9mg/dLand48mm/h,respectively(toconvertCRPtonanomolesperliter,multiplyby9.524).Lumbarpunc-tureopeningpressurewas175mmHO.Cerebralspi-nalfluid(CSF)hadanelevatedwhitebloodcellcount(10000/µL)(toconvertto/L,multiplyby0.001),withadifferentialcellcountof2%polymorphonuclearleukocytes,64%lymphocytes,0%eosinophils,0%ba-sophils,and34%macrophages(toconvertalltheper-centagestoaproportionof1.0,multiplyby0.01).Redbloodcellcount(9.0/L)(toconvertto/L,mul-tiplyby1.0)andprotein(29mg/dL)(toconverttogramsperliter,multiplyby10.0)andglucose(61mg/dL)(toconverttomillimolesperliter,multiplyby0.0555)lev-elswerewithinnormallimits.Achestradiographwasnormalandtuberculosisskintest(purifiedproteinde-rivative)wasnonreactive.Resultsofserumangiotensin-convertingenzyme,antinuclearantibody(ANA),antineutrophilcytoplasmicantibodies(pANCAorcANCA),andhumanimmunodeficiencyvirustestingwerenega-tive.Serologicresultsforserumrapidplasmareagin,Lyme,,andtoxoplasmosisantibodytiterswereallnega-tive,aswereCSFculture,VDRLtest,polymerasechainreactionforandLymeDNA,andmultiplescle-rosis(CSF)panel(myelinbasicprotein,oligoclonalbands,IgGindex).Onasecondmedicalhistoryintake,patient1re-vealedsporadiccontactwithacat,mostrecently2weekspriortohospitaladmission.Shedeniedhavingbeenscratchedorbitten.Noskinlesions,scratches,orbitemarkswerefound.Shedeniedrecentfeveroranysickcontacts.Patient1wastreatedempiricallywithacom-binationoforaldoxycyclinehyclate,100mg,andrif-ampin,300mg,twicedailyfor7weeks,onthebasisoffunduscopicexamination.Fivedaysafterhospitaldis-BhenselaeserologicresultsdemonstratedIgMandIgGdetectableatadilutionof1:512,indicatingcurrentinfection(indirectfluorescentantibodyenzyme-linkedimmunosorbentassay(ELISA);ARUPLaboratories,SaltLakeCity,Utah).Shereportedslightlyimprovedvisualacuityinthelefteye.After7weeks,headachehadre-solved,andvisualacuityreturnedtobaseline(20/20OS).Apartialmacularstarremained(Figure1D).CASE2A37-year-oldmanwithnosignificantmedicalhistorywasadmittedforblurredvisionintherighteyeandac-companyingretro-orbitalheadachefor2days.Second-arycomplaintsincludedrecurrentsinuspainandsub-jectivefever(intermittentself-perceptionofabnormalwarmthorcoldness)thatheattributedtohistypicalsi- LeftRightAdmissionFollow-up Figure1.Patient1.Colorfundusviewsoftheright(A)andleft(B)eyesathospitaladmissionand7weekslater,right(C)andleft(D)eyes.Noteopticdiscswellingthatispresentbilaterally,athospitaladmission,butresolvedat7weeks.Apartialmacularstarisseeninthelefteyeatadmissionandfollow-up(arrows). (REPRINTED)ARCHNEUROL/VOL68(NO.1),JAN2011WWW.ARCHNEUROL.COM ©2011AmericanMedicalAssociation.Allrightsreserved. nusproblems.Thepatientshospitaladmissiontempera-turewas37.4°C,andhehadasinglemaximumtempera-tureof37.6°Cthroughouthishospitalstay.Nolymphadenopathywasfound.Reducedrighteyevisualacuity(20/60OD),opticdiscswelling,retinalexudates,smallhemorrhages(Figure2A),andacentralscotomawerenoted.Visualacuitywasnormalinthelefteye(20/20),andfunduscopicexaminationdemonstratedalonecotton-woolspot(Figure2B).Hehadnormalcolorvi-sioninbotheyes.Resultsoftheneurologicalexamina-tionwereotherwiseunremarkable.Patient2hadscratchesonhisarmsfromdirectcontactwith2cats.Headcomputedtomographyshowedasmallmu-cousretentioncystintheleftmaxillarysinus.Brainmag-neticresonanceimagingdemonstratedmultiplesmallT2andfluid-attenuatedinversionrecoveryhyperintensi-tieswithinthebilateralsubcorticalwhitematter,aswellascentrumsemiovale,suggestingthepossibilityofde-myelinatingdisease.Resultsofthemagneticresonancevenogramwerenormal.Resultsofroutinebloodtestsandlevelwerenormal.Lumbarpunctureopeningpres-surewas200mmHO.Cerebrospinalfluidhadnormallevelsofwhitebloodcells(2000/µL),redbloodcells/L),protein(36mg/dL),andglucose(76mg/dL).Achestradiographwasnormal.Resultsofserumangiotensin-convertingenzyme,antinuclearantibody,an-tineutrophilcytoplasmicantibodies(pANCAandcANCA),rapidplasmareagin,Lyme,andhumanimmu-nodeficiencyvirustestingwerenegative,aswereCSFrou-tineculture,VDRLtest,angiotensin-convertingen-andLymepolymerasechainreaction,andamultiplesclerosispanel.Suspectingopticneuritis,a5-daycourseofintrave-nousmethylprednisolonewasinitiated,followingwhichthepatientsvisiongreatlyimproved.Erythrocytesedi-mentationratedecreasedfrom44mm/honday2ofmeth-ylprednisolonetreatmentto29mm/hondischargeday5.TheCRPlevelwas0.4mg/dL,testedonday3ofmeth-ylprednisolonetreatment.Onthedayofhospitaldis-charge,apreliminaryreportofBhenselaeIgMsug-gestedinfection(indirectfluorescentantibodyELISA;ARUPLaboratories),andacombinedcourseoforaldoxy-cyclinehyclate,100mg,andrifampin,300mg,twicedailyfor8weekswasstarted.Afterdischarge,BhenselaeIgMserologicresultsre-turnedpositiveat1:16,whereastheIgGtiterwasequivo-calat1:64.Threeweekslater,BhenselaeIgMwasnotdetected(1:16),butIgGwassuggestiveofrecentin-fection(1:256).Fourweeksafterhospitaldischarge,amacularstar(Figure2C)haddevelopedintherighteye,notprevi-ouslypresentduringthehospitaladmission(Figure2A).Visualacuityhadimprovedsignificantly.Thepatientwasexperiencingoccasionalsubjective LeftRightAdmissionFollow-up Figure2.Patient2.Colorfundusviewsoftheright(A)andleft(B)eyesathospitaladmissionand8weekslater,right(C)andleft(D)eyes.Noteopticdiscswellingandasmallhemorrhageneartheopticdiscarepresentintherighteyebutnotinthelefteye.Thereisacotton-woolspotinthelefteyeathospiadmission(),theareaisnotvisualizedat8weeks.Amacularstarisseenintherighteyeatfollow-up(arrow),whereasopticdiscswellinghasresolved. (REPRINTED)ARCHNEUROL/VOL68(NO.1),JAN2011WWW.ARCHNEUROL.COM ©2011AmericanMedicalAssociation.Allrightsreserved. feversbutwasafebrileatclinicvisits.At8-weekfollow-up,thesubjectivefeverhadfullyresolved,althoughhestillexperiencedoccasionalmildrightperiorbitalhead-ache.Visualacuityintherighteyehadimprovedbutnottobaseline. TheclassicpresentationofCSDisafebrileillness,inconjunctionwithahistoryoffelineexposure,lymph-adenopathy,andcutaneouslesions.Feverandlymph-adenopathyhavebeenconsidereddiagnosticcriteriaofCasesofCSDneuroretinitislackinglymphade-nopathyhavebeenreported.NocriterionstandardexistsforthedefinitivediagnosisofBhenselaeanddiagnostictestingreliesheavilyonapositivesero-logictestresult.Tissuebiopsy,culture,orpolymerasechainreactionhasvariablydemonstratedlowerspeci-ficityandsensitivity.Whilethereisvariabilityinmul-tiplestudiesofBhenselaeIgGandIgMindirectfluores-centantibodyandELISAserology,typicalsensitivityvaluesareapproximately50%to80%,whereasspecific-ityis90%to100%.SensitivityincreaseswhenthereisahigherclinicalindexofsuspicionforCSD,whenIgGandIgMaretestedincombination,andwhenserologictestingisrepeatedseveralweeksapart(asantibodypro-ductionvariesthroughthediseasecourse).andTsukaharafoundthatupto16%ofpatientssero-logicallydiagnosedashavingCSDpresentedwithnolymphadenopathy.Threeof30suchindividualswerealsoafebrile.However,unlikethecasesreportedherein,2ofthesepatientshadsymptomssuggestiveofsystemicAlargeretrospectivestudybyCarithersonstratedthatasmanyas41%ofpatientswithCSDmayhavenodocumentedfever.However,thecollectiveabsenceofsystemicsymptoms,fever,lymphadenopa-thy,andcatscratchmarksinCSDiseitherrareorunderreported.Presentingapproximately1yearapart,thesepatientsillustratethatCSDpresentingsolelywithvisualcomplaints(neuroretinitis)andheadacheisnotrare.Likely,CSDisunderdiagnosedwhensymptomsarelimitedtoblurredvisionandheadache.Onpoint,whenSuhleretalscreened14patientswithneuroretinitis,9of14hadelevatedtitersofBhenselaeIgMorIgG.Neuroretinitisishighlysuggestiveofinfection,whereasopticneuritisisanisolatedinflammatoryopticneuropathysecondarytodemyelination.funduscopicexaminationisimportantinraisingtheindexofsuspicionforCSDanddidsointheafore-mentionedpatients.Additionally,elevationininflam-matorymarkers(ESRandCRP)isatypicalforopticDifferentiationofneuroretinitisandopticneuritis()isnecessaryforinitiationofappropri-ateandprompttreatmentandimpactseventualprog-nosis.Themostworrisomeoutcomeistheriskofper-manentvisionloss.Typicalserologytimedelaycannecessitatetherapeuticdecisionspriortotheavailabilityofresults.Intravenousmethylprednisolonedoesnotchangethelong-termprognosisofopticneuritis,butitdoeshastenshort-termvisualrecovery.RecommendedtreatmentforCSDneuroretinitisisacombinationoforaldoxycyclineandrifampin.Therearenocontrolledclinicaltrialscom-paringanontreatmentgroupwiththosegivenantibiot-ics.However,retrospectivecaseseriesconsistentlyas-sociatedantibioticswithhastenedvisualrecoveryandimprovedvisualoutcome.CorticosteroiduseinCSDwitheyeinvolvementhashadmixedresults.Therewasgoodresponseinmultiplestudies,butconversely,re-centcasereportshavefoundnoimprovementinsymp-tomswithcorticosteroids.Inthecasesreportedherein,classicsignsofCSDwereeitherobscuredorabsent.Patientssoughtmedi-calattentionwithonlycomplaintsofblurredvisionandheadache.Similarabnormalitiesinrelativelyyoungpatients,statisticallyatriskformultiplesclero-sis,couldbemisinterpretedasdemyelinatingopticRelianceonserologictesting,whereasspe-candelaydiagnosisandtreatment.Classicfun-duscopicexaminationandelevatedinflammatorymarkerscansuggestBhenselaeasthecause,dictatingtheinitiationofantibiotics.ConsideringCSD,evenintheabsenceoffever,lymphadenopathy,orobviousscratches,willaidinmakingthediagnosis.AcceptedforPublication:April14,2010.MichaelD.Perloff,MD,PhD,NewYorkUniversityPainManagementCenter,LangoneMedicalCenter,317E34thSt,Ste902,NewYork,NY10016AuthorContributions:Studyconceptanddesign:Gan,Otis,andPerloff.Acquisitionofdata:Gan,Otis,Holmuham-edova,andPerloff.Analysisandinterpretationofdata:Mandell,andPerloff.Draftingofthemanuscript:Gan,Otis,Table.TypicalPresentationandManagementofNeuroretinitisvsOpticNeuritis VariableNeuroretinitisOpticNeuritisTypicalageChildren,youngadultsYoungadultsSex,M/F1:12:1VariablePaininOnsetHourstodaysHourstodaysVisualcomplaintUnilateralUnilateralMacularstar,discswelling,exudates,bilateralabnormalitiesNormalin2of3patients,rarelybilateralVisualfielddefectVariableCentralscotomaLymphadenopathyMorelikelyNotlikelyFeverMorelikelyNotlikelyInflammatoryElevatedESRandCRP,variableCSFNormalESRandCRP,1of3patientshaveCSFMRINormalorvariablyInflammationofopticnerve,otherlesionssuggestiveofMSTreatmentDoxycyclinehyclateandrifampinMethylprednisolone,IVAbbreviations:CRP,C-reactiveprotein;CSF,cerebrospinalfluid;ESR,erythrocytesedimentationrate;IV,intravenously;MRI,magneticresonanceimaging;MS,multiplesclerosis. (REPRINTED)ARCHNEUROL/VOL68(NO.1),JAN2011WWW.ARCHNEUROL.COM ©2011AmericanMedicalAssociation.Allrightsreserved. andPerloff.Criticalrevisionofthemanuscriptforimpor-tantintellectualcontent:Gan,Mandell,Otis,Holmuham-edova,andPerloff.Administrative,technical,andmate-rialsupport:GanandPerloff.Studysupervision:Otis,andPerloff.FinancialDisclosure:Nonereported. 1.CunninghamET,KoehlerJE.Ocularbartonellosis.AmJOphthalmol.2000;1302.FlorinTA,ZaoutisTE,ZaoutisLB.Beyondcatscratchdisease:wideningspec-trumofBartonellahenselae.2008;121(5):e1413-e1425.doi:3.CarithersHA.Cat-scratchdisease:anoverviewbasedonastudyof1,200patients.AmJDisChild.1985;139(11):1124-1133.4.SolleyWA,MartinDF,NewmanNJ,etal.Catscratchdisease:posteriorsegment.1999;106(8):1546-1553.5.ReedJB,ScalesDK,WongMT,LattuadaCPJr,DolanMJ,SchwabIR.ellahenselaeneuroretinitisincatscratchdisease:diagnosis,management,and.1998;105(3):459-466.6.TsuneokaH,TsukaharaM.Analysisofdatain30patientswithcatscratchdis-easewithoutlymphadenopathy.JInfectChemother.2006;12(4):224-226.7.BergmansAM,PeetersMF,SchellekensJF,etal.Pitfallsandfallaciesofcatscratchdiseaseserology:evaluationofBartonellahenselaebasedindirectfluorescenceassayandenzyme-linkedimmunoassay.JClinMicrobiol.1997;35(8):1931-8.SuhlerEB,LauerAK,RosenbaumJT.Prevalenceofserologicevidenceofcatscratchdiseaseinpatientswithneuroretinitis..2000;107(5):9.AtkinsEJ,BiousseV,NewmanNJ.Opticneuritis.SeminNeurol.2007;27(3):211-10.BrusaferriF,CandeliseL.Steroidsformultiplesclerosisandopticneuritis:ameta-analysisofrandomizedcontrolledclinicaltrials.JNeurol.2000;247(6):435-11.MatsuoT,YamaokaA,ShiragaF,etal.Clinicalandangiographiccharacteristicsofretinalmanifestationsincatscratchdisease.JpnJOphthalmol.2000;4412.LerdluedeepornP,KrogstadP,RobertsRL,StiehmER.Oralcorticosteroidsincat-scratchdisease.ClinPediatr(Phila).2003;42(1):71-73. CalendarofEvents:ANewWebFeature.OnthenewCalendarofEventssite,availableathttp://pubs.ama-assn.org/cgi/calendarcontentandlinkedoffthehomepageoftheArchivesofNeurology,individualscannowsub-mitmeetingstobelisted.Justgotohttp://pubs.ama-assn.org/cgi/cal-submit/(alsolinkedofftheCalendarofEventshomepage).Themeetingsarereviewedinternallyforsuit-abilitypriortoposting.Thisfeaturealsoincludesasearchfunctionthatallowssearchingbyjournalaswellasbydateand/orlocation.Meetingsthathavealreadytakenplaceareremovedautomatically. (REPRINTED)ARCHNEUROL/VOL68(NO.1),JAN2011WWW.ARCHNEUROL.COM ©2011AmericanMedicalAssociation.Allrightsreserved.