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Neuro-ophthalmology神经眼科学荷露斯-古埃及最重要的神之一是太阳神Osiris与保护女神Isis的孩子通常被描述为戴着埃及皇冠的神鹰古埃及的法老们都视自己为荷露斯的化身鹰头人身的天神荷露斯(Horus)Visual

PathwayPage77opticchiasmoptictractopticradiationOpticcortexTheopticnerveIntraocularportion1millionaxons—RGC--nervefiberlayerTheorbitalsegment

25–30mmlongBecomemyelinatedincreasingthediameterfromØ1.5mm->3mmTheintracanalicularportionmeasures4–9mm.thebonyopticcanalIntracranialcourse10mmjoinstheoppositeopticnerveformtheopticchiasmSchematicrepresentationofthecourseofganglioncellaxonsintheretinaWingedVictoryofSamothrace(NikeofSamothrake)Relativeafferentpupillarydefect(RAPD)Visualfielddefects

inopticnervediseasea.centralscotoma b.centrocaecalscotomac.nervefibrebundle d.altitudinalTypesofvisualfieldsdefectsandlesionsofvisualpathwayVisualEvokedPotential(VEP)Congenitalabnormalities

ofopticnerveOpticdischypoplasiaCongenitalopticpitsTiltedopticdiscMorninggloryopticdiscanomalyMedullatedretinalnerveHypoplasticopticdiscouterring-thejunctionofthescleraandlaminacribrosa,innerring-theterminationoftheretinaandretinalpigmentepithelium,bothabnormallyextendcentrallyoverthelaminacribrosaCongenitalpit

oftheopticnerveheadA.GrayandinferotemporallylocatedpitMildperipapillarychorioretinalchanges.B.SecondarymacularretinaldetachmentTiltedopticdiscMorninggloryopticdiscanomalyTheretinalvesselsenterandleavethenerveheadatitsmarginsassociatedrhegmatogenousretinaldetachmentMedullatednervefiberINFLAMMATORYOPTICNEUROPATHIES:OPTICNEURITISThemedicalrubric“opticneuritis”isperhapsmoreaclinicalsyndromethanatopicaldiseaseBythesuffix“-itis”isunderstoodinflammationofthenervebutthismodestdefinitionfailstoconveythecomplexnosologicspectrumthatembracesthefollowingconditions:demyelinative,otherimmune-mediated,infective,andidiopathicopticneuritides;inflammatorydiseasesoftheadjacentparanasalsinuses,brainandmeninges,cranialbase,andorbit,whichcontiguouslyinvolvetheopticnerves;granulomatousinfiltrationssuchassarcoidosis;infectionssharedwiththeretinaOpticneuritisOphthalmoscopicclassifications:PapillitisRetrobulbarneuritisAxialretrobulbarneuritisRetrobulbarperineuritisTransverseopticneuritisNeuroretinitisOpticneuritisPapillitisPapillitisneuroretinitismacularstar8-year-oldboywhocomplainedofvisualloss2weeksafterhis4-year-oldsisterdevelopedmumpsmilddiscswellinganddeepretinalexudatesVisionof20/80(6/24)ultimatelyclearedto20/15(6/5)withnotherapy.macularhemistarAcutelossofvisionina27-year-oldhealthywomanPrepapillaryhazeisduetocellsinthevitreousArteriesarenarrowed,andtheperipapillaryretinaisthickandelevatedRetinalexudatessurroundthediscandformCausesofOpticNeuritisUnknownoriginMultiplesclerosisViralinfectionsofchildhood(measles,mumps,chickenpox)withorwithoutencephalitisPostviral,paraviralinfectionsInfectiousmononucleosisHerpeszosterContiguousinflammationofmeninges,orbit,sinusesGranulomatousinflammations(syphilis,tuberculosis,cryptococcosis,sarcoidosis)

IntraocularinflammationsThegeneralclinicalcharacteristicsofnoninfectiveopticneuritisRelativelyacuteimpairmentofvisionprogressingrapidlyforhoursordaysvisualfunctionusuallyreachesitslowestlevelby1to2weeksafteronsetbutitmayactuallybeimprovingbythattime.Thetypicalepisodeinvolvesoneeyeonlyalthoughinchildrenespeciallyitisnotunusualforbilateralneuritis(withdiscswelling)tofollowviralillnesses,includingmeasles,mumps,andchickenpox.Tendernessoftheglobeanddeeporbitalorbrowpain,especiallywitheyemovements,mayprecedeorcoincidewithvisualimpairmentThegeneralclinicalcharacteristicsofnoninfectiveopticneuritisVisualfunctionisdepressedovertheentirefieldmostmarkedlyinvolvesthecentral20°variablediminutionofacuity,colorsense,andcontrastsensitivitynormalornear-normalacuitymaybepreservedPerimetricfindings:admixturesoffollowingscentralandcecocentralscotomasnervefiberbundleandaltitudinaldefectsgeneralconstrictionsPerimetricfindingsA.Centralscotomapattern finger-countingvisionB.Inferioraltitudinal(nervefiberbundle)pattern sparingthefixationalarea acuityof20/20C.Completelossoftemporalfield 4/200acuity Peripheralfieldreturnedcompletelywithin2months centralfunctionremained20/40.Visualfielddefects:OpticNeuritisTreatmentTrial(ONTT)diffusedepressionabout48%especiallyverticalaltitudinalhalfandquadrantlocalizeddefectsstrictlycentralorcecocentralscotomaslessthan10%varioussingleordoublearcuatedefectsunilateralnasalortemporalhemianopiasaschiasmalandretro-chiasmalpatternsfielddefectswerefoundintwo-thirdsoffellow(non-acute)eyesThegeneralclinicalcharacteristicsofnoninfectiveopticneuritisvisualfunctionbeginstoimproveinthesecondorthirdweekInthemajorityofcases,especiallyindemyelinatingdiseasemanypatientsenjoynormalornear-normalvisionbythefourthtofifthweekinothers,followingafairlyrapidimprovementtomodestlevelsofacuity(20/60to20/40),visionslowlybutsteadilyimprovesoverseveralmonthsInasmallpercentageofcases,visiondoesnotimprovetofunctionallevelsevenmorerarely,visiondoesnotimproveatallaftertheinitialprecipitouslossVisualsymptomatology

inopticneuritisSubjectivelyapproximatedbyviewingthroughaneutraldensityfilterordarklensDiminishedcentralacuityandfieldlossDrabness(desaturation)ofcoloredobjectsmixedtypesofdyschromatopsiawithoutcorrelationwithacuityfluctuationsovertimeapparentdimnessoflightintensities(e.g.,roomlightingappearsreducedwhenviewedwithaffectedeye)Visualsymptomatology

inopticneuritisImpairmentofbinoculardepthperception(stereo-illusion)especiallywithmovingobjects(Pulfrich'sphenomenon)inter-eyedisparityoflightsenseorretinalilluminationUhthoff'ssymptomincreaseinvisualdeficitwithexerciseorotherelevationsofbodytemperatureinthechronicorrecoveredphasePERSISTafterreturnofreadingacuitytonormallevelspatientscontinuetobevisuallysymptomaticinspiteofgoodacuityandfieldPrognosisTreatmentsofopticneuritisPapilloedemaEtiology:

ConditionsAssociatedwithPapilledemaandIncreasedIntracranialPressure(ExcludingSpace-OccupyingLesions)

SubarachnoidhemorrhageStatusepilepticusPaget'sdiseaseOpticochiasmaticarachnoiditisNeoplasticdiseasesCarcinomatous“meningitis”LeukemiaSpinal-cordtumorsHematologicdiseasesInfectiousmononucleosisIdiopathicthrombocytopenicpurpuraPerniciousanemiaPolycythemiaIron-deficiencyanemiaHemophiliaCirculatorydiseasesCongestiveheartfailureMediastinalneoplasmCongenitalcardiaccyanosisHypertensiveencephalopathyPulmonaryemphysemaDural-sinusthrombosisChronicpulmonaryhypoventilationSleepapneaRenaldiseasesChronicuremiaDevelopmentaldiseasesSyringomyeliaCraniostenosisAquaductalstenosis(adulttype)ToxicconditionsHeavy-metalpoisoning:Lead,arsenicHypervitaminosisATetracyclinetherapyNalidixicacidtherapyProlongedsteroidtherapySteroidwithdrawalLithiumAllergicdiseasesSerumsicknessAllergiesInfectiousdiseasesBacterialSubacutebacterialendocarditisMeningitisChronicmastoiditis(lateral-sinusthrombosis)BrucellosisRadicalnecksurgeryViraldiseasesPoliomyelitisAcutelymphocytemeningitisCoxsackieBvirusencephalitisInclusion-bodyencephalitisRecurrentpolyneuritisGuillain-BarrésyndromeParasiticdiseasesSandflyfeverTrypanosomiasisTorulosisNeurocysticercosisMetabolicendocrineconditionsEclampsiaHypoparathyroidismAddison'sdiseaseScurvyOralprogestationalagentsDiabeticketoacidosisMenarcheObesityMenstrualabnormalitiesPregnancyThyrotoxicosisDegenerativediseasesSchilder'sdiseaseMusculardystrophyHeadtraumaMiscellaneousdiseasesGastrointestinalhemorrhageLupuserythematosusSarcoidosisSyphilis(AdaptedfromBuchheitWA,BurtonC,HaagBetal:Papilledemaandidiopathicintracranialhypertension:reportofafamilialoccurrence.NEnglJMed280:938,1969)VisualdefectswithpapilledemaA.Long-standingpapilledemainpseudotumorcerebri Blindspotsmaybeofsufficientsizetomimicbitemporalhemianopia FieldisgenerallyconstrictedwithpreferentialinvolvementofinferiornasalareasB.Extremelossofperipheralfieldwithlong-standingfrontalglioma NoteretentionofthecentralfieldinthelefteyeCoursesofpapilledemaA.Papilledemaofraisedintracranialpressure --frontalastrocytoma rightdiskshowsearlyedemaofsuperiorpoleB.Moreadvancededema --Leftdiskofsamepatient absenceofhemorrahges/exudates/engorgment.C.Fullydevelopedpapilledema --pseudotumorcerebri MultiplesuperficialinfarctsNFL(CWS) Veinsaredilatedandtortuous. diameterappearsenlarged -spreadslaterallyinto,andelevatesNFL CenterofdiskrelativelysparedD.Severepapilledema --duralvenoussinusthrombosis Noteexudativepartial“star”figureatfoveaE.Chronicpapilledemaofmanymonthsduration “Champagnecork”--resolutionofhemorrhagesF.Chronicpapilledemaafterdetumescenceofedema pallor formationofretinochoroidalvenousshuntsIschemicopticneuropathyAION(anterior)infarctionoftheopticnerveheadcompromiseoftheposteriorciliaryarterycirculationTwogeneralvarietiesofAION:nonarteriticoridiopathicformarteriticform,secondarytogiantcellarteritisPION(posterior)BloodsupplyofopticnerveAION:clinicalfeathersthevasculopathicagegroupdecreasedacuityvisualfielddefectsswollen,variablypaleopticdiscNonarteriticanteriorischemicopticneuropathy.

A:Acuteonsetofsuperiorsegmentalswellingoftherightopticdisc,with20/20vision.

B:Twoweekslater,visiondiminishedtohandmovements,

theinferiordiscwasswollen,andthesuperiorportionwasbecomingpaleandatrophic.

Thistypeofsecondinfarctioninthesameeyeisrare,occurringin4%ofpatientsarcuatescotomaattitudinalscotomaTreatmentsandPrognosisNodefinitivetreatmentTreatunderlyingsystemicpredispositionsSmokingdiscourageed30%-50%involementoffelloweyewithinmonthsoryearsmaybereducedwithaspirinPseudo-FosterKennedysyndromeOpticatrophy+discedemacontralateralNeverrecurinsameeyeArteriticanteriorischemicopticneuropathy.GiantcellarteritisDiagnosticcriteriaa.jawclaudicationb.neckpainc.C-reactiveprotein>2.45mg/dlandESR>47mm/hTreatmentI.V.methyoprednisolonetaperedbyoralsteriodsLong-term:governedbysymptoms&CRP/ESRTemporalarterybiopsy

OpticNeuritisPapilledemaIschemicNeuropathySymptoms

VisualRapidlyprogressivelossofcentralvision;acuityrarelysparedNovisualloss;±transientobscurationsAcutefielddefect,commonlyaltitudinal;acuityvariableOtherTenderglobe,painonmotion;orbitorbrowacheHeadache,nausea,vomiting;otherfocalneurologicsignsUsuallynone;cranialarteritistoberuledoutBilateralRarelyinadults;mayalternateinMS;frequentinchildren,especiallypapillitisAlwaysbilateral,withextremelyrareexceptions;maybeasymmetricTypicallyunilateralinacutestage;secondeyeinvolvedsubsequentlywithpictureof“Foster-Kennedy”syndromeSignsPupilNoanisocoria;diminishedlightreactiononsideofneuritisNoanisocoria;normalreactionsunlessasymmetricatrophyNoanisocoria;diminishedlightreactiononsideofdiscinfarctAcuityUsuallydiminishedNormalacuityAcuityvariable;severeloss(includingNLP)commoninarteritisFundusRetrobulbar:normal;Papillitis:variabledegreeofdiscswelli

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