Case#
February,
Presentedby:
MichellePeng,MD
andMatthewDenny,MD
A39-year-oldobesewomanwithacutebilateralpainlessvisionloss
39岁肥胖女性，双眼急性无痛性视力下降
Figure1A:Fundusphotographoftherighteye.Notediscedemaandthesubtleretinalwhiteningintheinferiormacula(arrows)
Figure1B:Fundusphotographofthelefteye.Notetiltedmyopicdiscthatisalsoswollen.
1
CaseHistory
A39-year-oldmorbidlyobesewomanpresentedwithacuteonsetpainlessbilateralvisionlossfor2weeks.Inparticular,shenotedadiscreterightsuperiorvisualfielddefect.Herlefteyewasamblyopic.
一名39岁病态性肥胖女性，出现急性无痛性双侧视力丧失2周。她特别注意到右眼上方视野缺损。左眼是弱视眼。
Onexamination,visualacuitywas20/intherighteyeandlightperceptioninthelefteye.Pupilswereequalroundandreactivewithnoafferentpupillarydefect.
检查：右眼视力20/，左眼光感。PERRL，RAPD（-）。
Theintraocularpressuresandanteriorsegmentwereunremarkable.Fundusexaminationdemonstratedbilateraldiscedemawithasubtleretinalwhiteningintherightinferiormacula(Figure2A).
眼压和眼前节正常。眼底检查双眼视盘水肿，右眼黄斑右下方细微的变白样改变。
Fluoresceinangiographyrevealednormalperfusion,apatentcilioretinalarteryintherighteye,andlateleakageoftheopticnerve(Figure2B).Ocularcoherencetomography(OCT)showedelevationoftheopticnerveaswellasahyperreflectivebandintheinnernuclearlayer(Figure2)whichcorrespondedtoanareaofdecreasedperfusiononOCTangiographywithinthedeepcapillaryplexus(Figure3).
FFA提示灌注正常，右眼睫状动脉，晚期视神经渗漏（图2B）。OCT提示视神经抬高，内核层高反光带。
Figure2A:Fluoresceinangiogramoftherighteye.Fullvascularfillingispresent.
Figure2A:Fluoresceinangiogramoftherighteye.Thismid-phaseangiogramshowsdiscleakagewhichprogressedfurtherinthelaterphases(notshown).
Figure3:SD-OCTscanthroughtherightmaculaandnerve.Notethehyperreflectivebandinthemiddleretinallayers.
Figure4:En-faceOCToftherightmaculashowingtheextentofthehyperreflectivechangesintheinnernuclearlayersoftherightmacula.TheOCT-Angiogramoftherightmaculashowsreducedretinalperfusioninthedeepretinalcapillaryplexus.
2
DifferentialDiagnosis
Acutemacularneuroretinopathy,
急性黄斑区神经视网膜病变
Retinalvascularocclusion,
视网膜血管阻塞
Idiopathicintracranialhypertension,
特发性颅内压增高症
Vasoconstriction(Migraine,Medicationinduced)
血管收缩性疾病（偏头痛、药物源性）
3
AdditionalHistoryandDiagnosis
Thepatienthadahistoryofmorbidobesityandwastakingamitriptylineforchronicheadaches.Sheunderwentmagneticresonanceimaging,lumbarpuncture,aswellasanextensivelaboratoryworkupwithaneurologistwhichonlyrevealedanelevatedopeningpressure.
该患者有病态肥胖病史，并且服用阿米替林治疗慢性头痛。她接受了磁共振成像，腰椎穿刺，很多的实验室检查，显示较高的开放压力。
Shewasdiagnosedwithparacentralacutemiddlemaculopathy(PAMM)inthesettingofidiopathicintracranialhypertension(IIH)andstartedonacetazolamideinadditiontoaweightlossregimen.Threemonthslaterthediscedemawasseentoresolvewithimprovementinthevisionto20/32and20/intherightandlefteyesrespectively.Focalatrophyoftheinnernuclearlayerintheregionofpreviousischemiawasobserved(Figure5).
她被诊断患有旁中心急性中间黄斑病变（PAMM），并且除了减肥方案外，还开始服用乙酰唑胺。三个月后，视盘水肿逐渐消退，右眼和左眼的视力分别改善至20/32和20/。先前观察到的局部缺血区域内核层的局灶性萎缩（图5）。
4
Discussion
ParacentralacutemiddlemaculopathyisaclinicalentityfirstdescribedbyDavidSarrafasavariantofacutemacularneuroretinopathy(AMN).Wenowunderstandthattheyrepresentdistinctentities.Acutely,PAMMlesionsarecharacterizedbyhyperreflectivebandsattheleveloftheinnernuclearlayeronspectraldomainOCTwhichsparestheouterretina.Typicallytheselesionsthenprogresstoatrophyoftheaffectedinnerretinaleavingpersistentvisualdeficitsasinourpatient.InAMN,thehyperreflectiveband-likelesionsoccuratthejunctionoftheouterplexiformandouternuclearlayers.
PAMM是临床一类疾病，首先由DavidSarraf描述为急性黄斑神经视网膜病变（AMN）的变异。我们现在明白它们代表了不同的实体。急性期，PAMM病变在OCT上特征性表现为内核层高反光，保留外层视网膜。这些受影响的内层视网膜继续发展为萎缩病灶留下永久性视力缺失。在AMN中，高反射性带状损伤发生在外丛状和外核层的交界处。
UnderstandingthemicrovascularanatomyofthecentralretinalarteryandveinaidsinexplainingthemechanismofPAMM.Thecentralmaculacontainscapillaryplexusnetworkswhichsproutintoasuperficial,intermediate,anddeepsystems.Thesuperficialsystemlieswithintheganglioncellandnervefiberlayers.Theintermediateplexusissuppliestheinnerportionoftheinnernuclearlayer(INL)whilethedeepplexussuppliestheouterpartoftheINL.Parafoveally,limitedcapillarydensityandoxygendiffusionintheselayersformawatershedzone.IschemiaofthesesusceptibleregionsresultsinPAMMwhentheintermediateanddeepcapillarysystemsarespecificallyaffected.IncontrastAMNisthoughttobeduetoischemiaspecificallyofthedeepcapillaryplexus,thoughsomestudiesnowalsosuggestapossiblecontributingrolefromthechoriocapillaris.
PAMMmaybeidiopathicorduetosecondarycauses.Potentialetiologiesmaybeseparatedintolocalretinalvascularetiologies(ie.retinalvascularocclusions,diabeticretinopathy,hypertensiveretinopathy,vasculitis)andextrinsicvascularcauses(ie.migraines,medications,hypovolemia,orbital哈尔滨最好的白癜风医院白癜风治疗的较有效医院