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05年2月无明显诱因下以癫痫大发作一次起病，数十分钟后缓解无任何不适。查头颅MRI如下
以癫痫大发作一次起病，数十分钟后缓解无任何不适，神经系统体检无阳性发现
以癫痫大发作一次起病，数十分钟后缓解无任何不适，神经系统体检无阳性发现
抗囊虫治疗40天后
病例：
2006年3月病人处于去大脑状态并颅骨板开窗减压，CT：
本例病人最难的是影象学颅内多发病灶：从形态、好发部位、以及颅内继发改变上都很难。公布结果：Whippledisease:
http://www.grupoaran.com/mrmUpdate/lecturaPDFfromXML.asp?IdArt=457420TO=RVNEng=1
二、WD与中枢神经系统的关系：
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrievedb=pubmeddopt=Abstractlist_uids=16207456itool=iconfftquery_hl=1itool=pubmed_docsum
三、PCR检测Giardialamblia：
http://jcm.asm.org/cgi/content/full/43/7/3516?view=longpmid=16000494
四、非典型Whipple?sdisease：
http://www.indianjgastro.com/article.asp?issn=0254-8860;year=2005;volume=24;issue=1;spage=31;epage=31;aulast=Mehta
五、Whipple?sdisease：个案报道：
http://www.scielo.br/cgi-bin/fbpe/fbtext?pid=S0004-282X2004000200028lng=ennrm=isotlng=es
http://www.revneurol.com/LinkOut/formMedLine.asp?Refer=2003335Revista=Revneurol
六、园子里也有，zhaochob站友的文章：
http://www.dxy.cn/bbs/post/view?bid=46id=413563sty=3
NeurologicPresentationofWhippleDisease:Reportof12CasesandReviewoftheLiterature
WhipplediseaseisararemultisystemicinfectiousdiseasecausedbyTropherymawhippelii.Thisbacteriaisprobablyacommensalorganismofthegastrointestinaltractbutmaycauseinfectionifunderlyingimmunologicabnormalitiesarepresent，especiallyofmacrophages.Thediseaseismultifocalbutusuallyaffectsthegastrointestinaltract，andduodenalbiopsyremainsthestandarddiagnosticprocedure.T.whippeliicannotbeculturedbytraditionalmethods，sothediagnosisisbasedontypicalhistologiclesionsandidentificationofthecausativeorganismbypolymerasechainreaction.Thediseasecanbecuredwithprolongedantibiotictreatment.InvolvementofthecentralnervoussystemisaclassicalfeatureofWhipplediseasebutclinicalsymptomsusuallydevelopinthelaterstageofthedisease.Neurologicinvolvementhasbeenreportedparticularlyinpatientswhopreviouslyreceivedantibiotictreatmentsthatcouldnotcrosstheblood-brainbarrier.WeconductedthepresentstudytodescribeneurologicaspectsofWhippledisease，basedontheanalysisof12originalcasesandareviewofthepublishedliterature.Case1
Mrs.M.wasbornin1955.Hermedicalhistorybeganin1975，withthesuddenonsetoflefthemiparesissparingtheface.NeithersensitivitydisordersnorBabinskisignwasnoticed.Examinationoftheocularfunduswasnormal.Theerythrocytesedimentationratewas11mm/h.Cerebrospinalfluidwasnormal.Computedtomographyofthebrainshowedanexpansivelesionintheposteriorpartoftherightfrontallobe.Aneurosurgicalresectionofthetumorwasperformed.Thehistologicanalysisshowedanecroticlesionwithnoevidenceofmalignancybutwithinflammatorygranulomatoustissueinfiltratedbynumerouscells.ThemacrophagespresentedonlyafewperiodicacidSchiff-positivegranules.InAugust1998，thepatientpresentedwithagitation，behaviordisorders，andconfusionassociatedwithclonicmovementsontherightsideanddecreasedbilateralvisualacuity.Physicalexaminationrevealedbilateraluppermotorneurondisorder.Biologicinvestigationsshowedahemoglobinof107g/LandanESRof25mm/h.ThebrainCTscanrevealedprominentedemaofthelefthemisphereassociatedwithahypodenselesionintheleftoccipitallobewithamasseffect.ThepatientwastransferredtotheneurosurgerydepartmentinSeptember1998withtotaldementiawithmutism.Stereotacticbiopsyoftheleftoccipitallobewasperformed.Thehistologywassimilartothatobservedin1975.Thelesionsinvolvedbothwhiteandgraymatter，mimickingencephalitis.TherewerealsoassociatedperivascularandintraparenchymatousmacrophagesexhibitingPAS-positiveinclusionssuggestiveofWhippledisease.AnalysisoftheCSFdidnotshowanycellwithPAS-positivecytoplasm.ThespecificsearchforT.whippeliiwithDNAextractionandspecificPCRcontrolledwithhybridizationintheCSFwasnegative.AttheendofOctober1998，becauseofdelirium，agastroscopywasperformed.Thegastroscopydidnotrevealanymacroscopicabnormality.Duodenalandjejunalbiopsieswerenormal.Treatmentwithtrimethoprim-sulfamethoxazole320+1，600mg/dwasintroducedandsignificantneurologicimprovementwasnoticed.Thedeficitintherightpartofthebodydecreased，deliriumdisappeared，andthepatientwasabletotalkandperformdailyactivitiesagain.ACTscanofthebrainwasperformedforfollow-upinDecember1998:thehypodenseedematouslesionshadclearlyshrunk，especiallyintheleftpartofthebrainFig.1.CerebralCTscanwithcontrastmedium.ProminentedemainthelefthemispherewithoutdeepSylviantumorouslesion;leftSylvianfissureheterogeneousinappearance，associatedwithhypodenselesionintheleftoccipitallobewithamasseffectbutwithoutspecificcontrastenhancement.
Fig.2.CerebralCTscanwithcontrastmedium.Clearregressionofhypodenseedematouslesions，notablyontheleft.
网友[drzhenghb]：http://www.google.com/search?hl=zh-CNq=Whipple+disease+btnG=Google+%E6%90%9C%E7%B4%A2lr=
Whipple?sdiseasecausesweightloss，incompletebreakdownofcarbohydratesorfats，andmalfunctionsoftheimmunesystem.Whenrecognizedandtreated，Whipple?sdiseasecanusuallybecured.Untreated，thediseasemaybefatal....A68-year-oldmanwasadmittedforwalkingdifficulties，memoryloss，andincontinencethathadbeenprogressingfor5months.For12years，hehadcomplainedofarthralgiasofthehandsandwrists.Thediagnosisofrheumatoidpolyarthritishadbeenmade.Aneurologicexaminationrevealedatetrapyramidalsyndromeassociatedwithapathyandmemoryloss.Bloodtestsindicatedaninflammatorysyndromewithanelevationoftheerythrocytesedimentationrateandanincreaseofproteins.Theresultsofantinuclearantibody，HIV，BandChepatitis，latex，andWalerRosetestswerenegative.TheCSFtestshowedanincreaseintheproteinratewithanabsoluteincreaseofimmunoglobulinG，butthecellcountandglucoselevelwerenormal.PolymerasechainreactionforTropherymawhippeliiwasnegative.MRimagesofthebrainhadareasofT2hyperintensityintherightandleftmediobasaltemporallobe，anteriorcommissure，rightandleftmamillarybodies，leftcerebralpeduncle，rightmiddlecerebellarpeduncle，bulb，rightlobeofthecerebellum，andopticchiasm.Theselesionsshowednomasseffect.AfterIVinfusionofacontrastagent，mildenhancementoftherighttemporallesioncouldbeseen.MRimagingofthespinalcordwasalsoperformed，showingT2panmedullarycentralhyperintensity.FIG1.Imagesfromthecaseofa68-year-oldmanwhowasadmittedforwalkingdifficulties，memoryloss，andincontinencethathadbeenprogressingfor5months.
A，AxialT2-weightedMRimageofthebrainshowshyperintensityinthemediobasalpartoftherightandlefttemporallobes，inthemamillarybodies，andintheopticchiasm.B，AxialT2-weightedfluid-attenuatedinversionrecoveryMRimageofthebrainshowsdiffusehyperintensityinvolvingtherightmediobasalandleftmediotemporallobes，aswellasinvolvementoftheleftcerebralpeduncle.Notethelackofmasseffectdespitethesizeofthelesion.C，Coronalfluid-attenuatedinversionrecoveryMRimageofthebrainshowshyperintensityinthemediobasalpartofrightandlefttemporallobes，brainstem，andrightmiddlecerebellarpeduncle.D，CoronalT1-weightedMRimageofthebrainshowsmildenhancementoftherighttemporallesionsaftertheIVadministrationofacontrastagent.E，SagittalT2-weightedMRimageofthespineshowshyperintensityinthecentralpartofthespinalcord.F，CoronalT2-weightedfluid-attenuatedinversionrecoveryMRimageofthebrain，obtainedafter1yearoftreatment，showsnearlycompleteresolutionoftherighttemporallesion.Thepatientunderwentaduodenalbiopsy，whichshowedmacrophagesthatstainedpositivelywithperiodicacid-Schiffstain.PolymerasechainreactionforTropherymawhippeliiwaspositiveontheduodenalbiopsy.
FIG2.Duodenalbiopsywasperformed.Notethecharacteristicfoamymacrophagescontainingperiodicacid-Schiffstain-positiverod-shapedstructuresinthesubmucosalregion
reviewof84casesdescribedintheliteraturerevealedthatclinicallydetectableinvolvementoftheCNSrangesfrom6%to43%ofpatientssufferingfromWhippledisease.In43ofthe84reportedcases，CTorMRimagingoftheheadwasperformed，andin23，focalabnormalitieswerefound.However，DobbinspostulatesthatallpatientssufferingfromWhipplediseasehaveanatomic-pathologicCNSinvolvement.Itconsistsofmultiplesmallcircularorovallesions，measuringanaverageof2mmindiameter，disseminatedthroughoutthegraymatterandcharacterizedbytheaccumulationofmacrophages，stainingveryintenselywithperiodicacid-Schiffstain，asshowninpostmortemstudies.Electronmicroscopyshowsthatthemacrophagescontainabacilliformorganism，namedTropherymawhippeliibyRelmanetal，revealingthebacterialcauseofthedisease.Thesamelesionsarefoundinotherorganswhentheyareaffectedbythedisease.Recently，molecularbiologyallowedthedetectionofthepresenceofTropherymawhippeliiintheaffectedorgansbyusingpolymerasechainreaction.Previousstudieshaveshownagoodcorrelationbetweenpreferentialsitesofanatomic-pathologiclesionsandthelesionsrevealedbyMRimagingandCT.ThefirstneuroradiologicdescriptionsofCNSinvolvementwerereportedasseenonCTscans，andtheypresentednospecificcharacteristics.TheCTscancanbenormal，regardlessoftheclinicalfeatures，orcanrevealfocalizedlesions，whichcanbehypo-orhyperdense，contrastenhancedornot，andwithorwithoutmasseffect.However，MRimaginghasproveditssuperiorityforthedetectionofsmalllesions.InareviewoftheliteraturepresentedbyLouisetal，43patientshadundergoneCTorMRimagingandthreepatientswithnormalCTfindingshadfocalabnormalitiesontheirMRimages.Theselesions，whichoccurin53%ofthecases，oftenconsistofT1hypointensityandT2hyperintensity，shownomasseffect，andarelocatedinthemedialpartofthetemporallobes，inthehypothalamicregion，orinthepons.Theselesionsaresometimesenhancedafterinfusionofcontrastmedia.Moreover，associatedmoderateatrophyoccursin42%ofthecases.Multiplemasslesionshaverarelybeendescribed.TheyusuallyappearhypointenseonT1-weightedimagesandhyperintenseonT2-weightedimagesandenhanceafterinfusionofcontrastmedia.However，asmallnumberofpatientshavenormal-appearingMRimages.Theinvolvementofthespinalcordhasrarelybeenreported.WefoundonlyonereportofmyelopathysecondarytoWhippledisease，diagnosedbyusingMRimaging.Itwaslocatedinthecervicalregion，anditappearedhyperintenseonT2-weightedimagesafterIVinjectionofgadolinium.Involvementoftheopticchiasmhasalsorarelybeenrevealedbyneuroradiologicexamination，althoughitismoreoftendiscoveredduringpostmortemstudies.WefoundonlyonereportwithanincreasedT2signalintensitylocatedintheoptictracts.However，clinicalopticalmanifestationsarecommonincasesofWhippledisease.EarlytreatmentofWhipplediseaseleadstoimprovementofthelesionsrevealedbyneuroradiologicexamination，asshowninourcase，butdiagnosisisoftendifficult.Diagnosisisoftendifficulttodetermineonthebasisofpolymerasechainreactionorhistologicfindings;moreprecisely，63%ofpatientsunderwentbiopsy.Morerarely，thediagnosisismadebasedonlymphnodebiopsyorcerebralbiopsy.However，polymerasechainreactionhasshownsensitivityandspecificitytoconfirmadiagnosisofWhippledisease，andrecentstudieshaverevealedgeneticmaterialofTropherymawhippeliiinperipheralbloodmononuclearcellsandincellsofpleuraleffusion，suggestingthatthediagnosiscansometimesbemadeonthebasisofpolymerasechainreactionofperipheralblood.ThediagnosiscanalsobeobtainedbyconductingananalysisoftheCSFforperiodicacid-Schiffstain-positivecells，butthevalueofpolymerasechainreactioninCSFhasnotyetbeenevaluatedbecauseoftherarityofthedisease.Treatmentisoftendelayed，however，resultingindeathorirreversiblecerebrallesions，suchasatrophy.Moreover，relapseofthediseaseisfrequent，leadingtothereappearanceoftheCNSlesionsonMRimages.Inconclusion，thiscasereportshowstheimportanceofneuroradiologicexaminations，particularlyMRimaging，forthedetectionofCNSlesionsintheinitialevaluationofpatientssuspectedofhavingWhipplediseaseandforthelong-termfollow-upofthesepatients.ItisnecessarytoperformMRimagingofthebrainandspinalcordtoascertainthedifferentlocationsoftheselesionsInAugust1994，a65-year-oldwomanpresentedwitha2-monthhistoryofupperandlowerlimbparesthesiasand1-weekparaparesisandsphincterdysfunction.Onclinicalexamination，sensorymotormyelopathywithanuppercervicallevelwasconfirmed.
FIG1.August1994.Sagittalfastspin-echoT2-weightedimageshowsenlargedandinhomogeneouslyhyperintensespinalcordfromthecervico-occipitaljunctiontotheupperdorsalregion.Sagittalspin-echoT1-weightedimageswithgadopentatedimeglumineshowperiphericalenhancement.Eightmonthslater，thecordisnormal
FIG2.November1995.SagittalT2-weightedimageshowsenlargedandinhomogeneouslyhyperintensespinalcordfromdistalcervicaltoupperthoraciccord.Onemonthlater，thecordisnormal
FIG3.May1997.Coronalfast-FLAIRimageshowsnodularsignalhyperintensitywithslightmasseffectinleftmiddlecerebellarpeduncle.
January2000.Coronalfast-FLAIRimagesshowregression:onlyslightsignalabnormalitiesinthestructurespreviouslyinvolved，andnonewlesions
March1998.Coronalfast-FLAIRimagesshowpersistenceofthelesionsobservedpreviously，increasedinvolvementofmiddleandnewinvolvementofsuperiorrightcerebellarpeduncles
