CanadMAJMay201961vol84GENERALPrAcmcEPOSITIVESIGNSOFNEUROSIS1133marvelledattheirsurvivalThispatientsattitudeisthatgoodhealthisavulgarityofwhichshemustnotbesuccessfullyaccusedSimilarlyingoing ID: 294375
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1132GENERALPRACTICE:POSITIVESIGNSOFNEUROSISTHEPOSITIVESIGNSOFNEUROSIST.F.ROSE,M.D.,*Victoria,B.C.INTHEmedicalcommunitythereisstillfartoogreatatendencytodiagnoseaneurosisonlyafterinvestigationhasruledouteveryorganicconditionwhichthepatient'scomplaints,plausiblyorim-plausibly,mightrepresent.Thisprocessisdescribedponderouslyas"givingthepatientthebenefitofthedoubt".Itmaybedescribedasgivingthepatientthebenefitofbadmedicine.Toomanyhealthypeoplearesolemnlystuffedwithbarium,coupledtospirometers,partiallyexsanguinated,gastricallydredgedandspiedonthrougheverybodyorificebeforethephysicianhasruledoutalldisease,in-cludinghistoplasmosis,andisfinallywillingtorecognizehispatientasanineffectual,marriedtoaboor.Thepatienthasbeenputtoneedless,andoftenserious,expense;thecrowdedfacilitiesofourlabor-atoriesandx-raydepartmentshavebeenfrivolouslyabused;butworstofall,theverytypeofpersonalreadydisposedtothinkthatherachesandpainsbulklargeinthecosmicscheme,isnowsurethatherconditionmustbesomethingspecialtobemetwithsuchmightyshenanigans,andherneurosisisgreatlyreinforced.Inthedoctor'smentalpictureofhimself,heisdrapedinthetogaofscience,butinrealityheismistreatingninety-ninepatientsinordertobesurethathedoesjusticetothehun-dredth.Everyoneishappyexceptthepatient'shusbandandSirWilliamOsler,turninginhisgrave.Theneurotic,onceidentifiedassuch,shouldhavethebriefestrelevantinvestigationforpossibleconcomitantorganicdisease.Thesoonersheisidentified,thesoonershecanbeproperlytreated.ThefollowingaretheidentifyingcharacteristicswhichIhaveobserved.Quiteoftenapresumptivediagnosiscanbemadebeforewehaveanycontactwiththepatient.Inphoningforanappointmentshemayinsistongivingoursecretaryadetailedaccountofhersymp-toms,andwithpeoplewhodothis,thesumofthesymptomsequalsoneanxietystate.Or,attheotherextreme,theprospectivepatientmaymysteriouslyrefusetogiveanyinformationatall,whichsimplyisnotdonebypeoplewithhonestcomplaints.Sometimesthepatientdemandstoseethedoctoratonce;ifthereisnoappointmentavailable,shewouldbequitehappytograbthetimewhichsomeoneelsehaswaitedfor.Thissortofurgencyusuallyattendsapurelyemotionalincontinence.Oursecretariesalsohearfromthosewhoprotestthattheycanbefittedinbetweenappointments,becausetheywon'tkeepthedoctortwominutes.*209RoyalTrustBldg.,612ViewStreet,Victoria,B.C.Canad.M.A.J.May20,1961,vol.84Ithinkwehaveallwalkedintothisboobytrapaboutonce.Thispatienthasonlytwocomplaints:shehasachesandpainsalloverandsheistiredallthetime.Ifallowed,shewillstayfortwohours.OnceinourwaitingroomstheonlywayIknowinwhichtheneuroticlabelsherselfisbyswooning,andhavingtoberestoredwithlittlesipsofwaterandconsiderableattention.Isupposeweallusherpatientsintoourconsult-ingroomswithasmileandsomeminorpleasantry,almostinvariablyreturned.Butsometimesthepa-tientwillstalkbyus,stoney-faced.Thisisthemartyredexpressionofonewhoknowsgreatsuffer-ing,anditalsowarnsusthatweareembarkingonamattersogravethatlevityisoutofplace.Itisabsolutelypathognomonicofneurosis,oneofthebestdiagnosticsignswehave.Anotherreliableoneismanifestamomentafterthepatiententerstheconsultingroom.Shesitsdown,andrepliestoone'sinitialquestionbymakingbravelittlefacesandburstingintotears.Sometimes,askedwhatthetroubleis,there-sponseis,"Well,doctor,I'mprobablyjustanotherofthoseneurotics",oralternatively,"Well,doctor,Ihopeyouwon'tthinkI'moneofthoseneurotics."Ineithercase,sheis.Avariantofthisself-consciousapproachistheonewhovolunteersalmostatonce"Thereisnothingpsychologicalaboutme."She'salwayswrong.Andtheonewhoopensupobse-quiouslywith"Isupposeyou'reterriblybusy.Youpoordoctors-nolifeofyourown,"etc.,willuseupasmuchofyourlifeasshecan,andtoverylittlepurpose.Stillotherpatientsobliginglysupplyalmostinstantdiagnosisbycominginwithalittlebagcontainingtheireightortencurrentmedications,andlinethebottlesupbeforeusonourdesks.Ofcourse,itisintheprocessofhistory-takingthatmostofourneuroticsrevealthemselves.Eventhefamilyhistorycanbemorethansuggestive.Howoftenarewetold"Mymotherwasaninvalid."Well,that'stoobad;whatwasthematterwithher?"Oh,alotofthings."Thispatientisgenerallyanexampleofthefactthatdaughtersgrowupliketheirmothers.Manyneuroticsdatealltheirwoesfromsomeemotionallysignificantillnessorexperience,andrepeatedlyinsist,"Iwassostrongandwelluptothen."Yetfactualquestioningabouthospitalad-missions,illnessesandoperationsshowsthesameasthenicpatternoneithersideofthefanciedmile-stone.Othersrefusetoadmitthattheywereeverwell.Unabletorecallspecificdisease,theyquotetheirmothersasauthoritythatevenaschildrentheywerealwayssickly.Oroftentheyhadscarletfeverthreetimesanddiphtheriatwice.Sometimestheoldfamilydoctorgavethemup,sometimeshegavethemsixmonths.Generallyheabsolutely Canad.M.A.J.May20,1961,vol.84GENERALPrAcmcE:POSITIVESIGNSOFNEUROSIS1133marvelledattheirsurvival.Thispatient'sattitudeisthatgoodhealthisavulgarityofwhichshemustnotbesuccessfullyaccused.Similarlyingoingthroughafunctionalenquiry,wearefamiliarwiththosepeoplewhofeelthatdenyinganysymptomatallislikebeingtouchedinafencingmatch.Iftheycannotpleadguiltytothespecificsymptomputtothem,theyreplyobli-quelywithanother.Question:"Doyourankleseverswellup?"Answer:"Well,myfeethurtalot."Theneuroticpatientregardsheroperationsasclinicaltriumphs,andifmultipleviolationsofherabdomenhavebeencommittedbyonesurgeon,sheisthespeciallyprivilegeddevoteeofacultofwhichheisthehighpriest.Shegetsparticularsatisfactionfrommulti-purposeoperations."Hedidthreeoperationsinone,"shereportsproudly.IfhetookouthergallbladderorheruterusorthestandardcysticrightovaryANDherappendix,andyouorIinterjectthattheappendectomywasaroutineby-blow,shehurriespastthesubjectinthemannerofoneoftheFaithfulavoidingcontro-versywithanUnbeliever.Anotherfavouriteattitudeisforthepatienttoheapextravagantpraise,onthedoctorwhousedtolookafterher.Itissoonclearthathismostpraise-worthycharacteristicswerehissolicitude,hispromptandfrequentattendance,andthegraveviewwhichhetookofhercondition.Thisisaninvitationforthenewdoctortomeetthestandardofattentionwhichshewishestheoldonehadset.Thisinvokingofotheropinionsisafavouritegambitofthewomanwhosepostureisindomit-ablepluckinthefaceofoverwhelmingdisability."I'mnotonetogiveup,doctor.IgountilIdrop.That'sjustme."Youaskanyofherfriends.Theyallmarvelatthesheergritwithwhichshekeepsgoing.Similarlywearetoldhowdeeplyherfamilyareimpressedwithherpain,ortheviolenceofhercough,orherpallor;inthiswaycomplaintsaresupposedtoachievevalidity.Sometimesthead-miringspouseisactuallybroughttoouroffices,toplayoneoftworoles.Eitherheexpressesamaze-mentthatfleshandbloodcanenduresomuch,whilethepatientsitsback,lookingnoble;orthepatienttellsthestory,whilethespousefunctionsasasortofGreekchorus,supplyingthesepulchralamens.Anothervariantofthestoicalpitchis"Iheldoutforalongtime,butDr.So-and-sofinallyorderedmetohospital.""Doctor,Iamsosensitive."Howoftendoesonehearthatstatementfromanyonebutanotveryintelligent,coarse-grained,unhappyneurotic?Andhowlongarewegoingtosufferfromthelayman'shappyconfusionbetweensensitivitytoallergensand-thesensitivityoftheartistorthearistocrat?Thisisresponsibleforthebattleofsensitivityovertheteacups.IfMrs.Jonesissensitivetosomething,thenMrs.Smithbecomessensitivetotwothings,andisthusonlyhalfasmuchapeasantasMrs.Jones.Sometimesanexceptionallygiftedpatientisabletoreportthatsheis"sensitivetoalldrugs"andthisvirtuallymakesheraduchess.Inmuchthesameveinasthesatisfactionovermultipleallergiesistheproudboastofmultiplefoodin-tolerances.IfIcan'teatthisorthat,itdistinguishesmefromthecommonherdassomeonespecial.Similarlywehear:"Ican'ttakepills"or"Ican'ttakeliquidmedicine."Somuchneurosisbeingbasedonunhappiness,itisnotsurprisingthatsomanyneuroticsvolunteerrepeatedlyhowhappytheyare.Happypeopledonotdothis.Nordohappilymarriedpeoplegooffintounsolicitedaccountsofmaritalbliss.WhenweareofferedadescriptionofDarbyandJoaninarose-coveredcottage,weknowthatwearegoingtofindtroubleinthatcottage.Andthereisthepatientwhointerjectsintoawelterofdiffusecomplaintsthatshe"onlywantstobewelr.Thispuzzlingstatement,infreetranslation,meansthatshewantssomeonetomakeherhappy.Asamatteroffact,usuallyshedoesnotwanttobewell.Ifsheacknowledgedhealth,shewouldhavetostandonherownfeet,andshewantstobedependent.Ifherenvironmentdoesnotprovideherwithafatuoushusbandorspinsterdaughter,itmaybethewretcheddoctor'slapintowhichshewill-throwallherinadequacies,andelecthimresponsibleforthem.Sickpeople,whowanttobewell,aredisappointedifmedicationdoesnotim-provethem.Butnotthispatient.Hervoiceonthetelephone,"Doctor,IhatetobotheryouonaSunday,but..."isrichwithsatisfactionthatyourlastpillsdidhernogoodatall.It'syourplayagain.Finally,ifshecanclaimthatyourmedicinemadehermuchworse,thenyoureallyareholdingthebaby.Asfunctionalsymptomsareusuallyvague,theirdescriptionisequallyso.Thelocationofpainisindicatedbywidegestures,andthepatientmayevengetabithuffyatrepeatedinvitationstobemorespecific.Enquiriesastothecharacterofpainareparriedwithsuperlativessuchasintolerableandunbearable.Question:"Nowwhatkindofpainisthis?"Answer:"IYsmurder."Weseldomhearthesymptomsoforganicdiseasedescribedas"torture'.Everymedicalstudentknowsthatthewrittenlistofcomplaintsidentifiesaneurotic,butthereisawideareaofexceptiontothis.Elderlyarterio-scleroticpersonsoftenhavetopreparelistsoftheirentirelygenuinecomplaints,becauseofdefectivememory.Butconversely,exceptintheseoldpeople,thepresentingcomplaintsoffailingmemoryandinabilitytoconcentrategenerallycomefrompeoplewhoareconcentratingsosuccessfullyontheirneurosesthatthereisnoremainingscopeforpay-ingattentiontobusiness.Itisinterestingthatpeoplewhoaretryingtoimpressonushowsicktheyare,shouldsooftentellabouttheirsicknesswithsuchnaivegusto.Theysimplydon'tknowhowsickpeopleact.Utterexhaustionisdescribedwithvivaciousgesturesandsparklingeyes.Exquisitepainisheldouttouswith 1134GENERALPRACrICE:POSITIVESIGNSOFNEuiRosIstheairofachildanxioustoshareitstoys.Themosthorrendousmiseriesarerecitedwiththetriumphofonewhohasjustlearnedaclevernewtrick,andexpectspraise.Andweoccasionallyseethatyoungwomanwhotellsushowshepassedoutcold,andthenpauses,smilinghappilyandbattinghereyelidsasthoughshewerewaitingtobepattedonthehead.Ihavebeenusingtheword"she"ratheroften,perhapsmorethanisjustified.Buttherearesomeneuroticattitudespeculiartomen.Thereistheonewholeadsoffinavirile,condescendingmanner,sayingthathehopes,Doctor,youdon'tmindhissayingthis,buthehasneverthoughtverymuchofthemedicalprofession,andhasalwaysavoideddoctors.Bythetimewehavemethodicallyques-tionedthisfellow,itisevidentthatovertheyearshehasseenalotofdoctorsforalotofcomplaints.Thereistheamusedandsupercilioussmirkinre-sponsetoseriousquestions,whichisintendedtosay"Thisisallalotofnonsensetoahe-manlikeme",butactuallyconveysquiteanotherimpression.Afterexaminingthemanwhotalksabout"theoldticker",wecanusuallyreassurehimabouthisheart.Weallknowthemanwhoproteststoomuchaboutwantingtogetpatcheduponlysothathecangetonwiththejob.Thereisthemiddle-agedbusiness-manwhosaysthathedoesn'tminddying,butonlywantstoknowthetruth;intruthheverymuchmindsdying,butgenerallythetruthaswecanrevealittohimisgoodnews.OccasionallyweencountertheheartyEnglishmanwhosumsuphisabilitytobitethebulletbysaying,"Ha,ha,thebulldogbreed,youknow".Abouttheonlythinghehasincommonwithabulldogisastrongjaw.Hereareafewunrelatedobservations,whichIsubmitarenotlikelytobeassociatedwithanorganicdiagnosis:Thepatientwhoreferstohis"casehistory".Idonotrecalleverfindingseriousdiseaseinapa-tientusingthisphrase.Theonewhostatesdynamicallythatnowthetimehascome"toreallygettothebottomofthisthing"-thereisnobottom.Thewomanwhotalkswithhereyesclosed.Themanwho,duringhalfanhour'svariedcon-versation,ceaselesslymassagesorkneadsthesiteofhiscomplaint.Thepatientwhostaresfixedlyattheoppositewallandreciteshiscomplaintsinamonotone.Thepatientwhocomplainsresentfullythatovertheyearshehasspentumpteendollarsondoctors,alltonopurpose.Thepatientwhotalksfasterthanonecantakenotes,orkeepsontalkingwhilewearereadinghisdocuments.Theattitudethatthedoctorisluckytobegiventhecareofthisfascinatingcase.Thestatement,attheendofanhour'sdiffusecomplaints,allofthemhorriblyfamiliar:"I'llbetyouneverhadapatientlikemebefore."Canad.M.A.J.May20,1961,vol.84Thestatement:"It'sfunnyaboutme;Inevergetanythingsimple;Ialwaysgetthecomplicatedthings."Thecompulsivelydetaileddiscussionoftriviainresponsetoquestions.Thepatientwhoknowsallabouthistemperature.Thealmostlovinglyintimatedescriptionofstools.Thereportoftheself-administeredenema.Thepresentingcomplaintofbreathlessness,de-scribedas"Ican'tgetmybreath-thenIhavetotakeabigdeepone."Theabdominalorjointswellingwhichperverselyisnotpresent,justtoday.Babytalkinthefemaleofmatureyears.Afterourpatienthastoldusmoreabouthimselfthanherealizesintheconsultingroom,heisputontheexaminingtablewheretheroutineofphysi-calexaminationmayelicitmanypositivesignswhicharenotofphysicalsignificance.Tomanyoftheitemsinthefollowinglist,itmaybefairlyobjectedthattheyarenotsignsofoutrightneurosis,butmerelyofatensepersonality.Myreplyisthatthemajorityoftensepeopleattendingadoctoraresufferingonlyfromtension,andthesesignsareworthnoting:Thelividflushovertheneckandupperchest.Thisisusuallyinthefemale.Theacrobaticco-operation,sometimessoviolentastomakeexaminationdifficult.Thehandsputbackupbehindtheheadasoftenastheyareputdownatthesides.Theclosedeyesduringexamination.Belchingintheexaminer'sfacetodemonstratetheimpressivevolumeofgas.Thespartanhissingundertheophthalmoscopebeam.Thespartanwrithingunderthebloodpressurecuffatnormalpressures.Ingeneral,theover-reactiontoalltheminorstimuliofphysicalexamination,andoftenamajorover-reactiontorectalorpelvicexamination.ThereactiontoelicitingtheplantarresponsewhichIcalltheBabinskiShriek.Thespasticthoraxoninvitationtodemonstrateexpansion.Thesmart-aleckmalepatientwhogoeson"1-2-3-4-5-6...Thewetpatchesonthesheetfromthesweatingaxillae.Thecold,clammy,cyanosedhands.Themoistlittlehandkerchief,alwaysinthatmoistlittlepalmwhichtheexaminerwantstouse-hastilytransferredtotheother.Thelegheldthemorerigidlyabovethetablethemorethepatientisaskedtorelaxit.Whilethelowerextremitiesarebeingexamined,thebird-likeheadwhichwillnotremainbackonthepillow,evenifthisisalmostcontinuouslyre-quested.Massagingtheareaofabdominalpain,sothattheexaminercannotgethishandsthere,orgrab-binghishandandkneadingthesite. Mayn0.1961,M.ol.8.CASEREPORTS:ALBRIGHT'SSYNDRONIEANDMIONGOLIS-M113;5Morethantwoabdominalscars.Therequestforperiodicbulletinsduringex-aminationastowhetheranythinghasbeenfoundyet.Inthefemale,inabilitytopassaurinespecimen.Havingrecognizedourneurotic,hehasafewmorewaysofidentifyinghimself,whenwearesumminguphisconditionattheendofourinter-view.Thecommonestisincredulityandobviousdisappointmentwhenweinformhimthathehasnoseriousdisease.Themostexasperatingiswlhenwvetrytogivrethepatientanorderlvexplanationforhisconditionandhekeepsbreakinginirrelevantlytorepeatrandomcomplaints.Themostspecificisindignationatthesuggestionthatheseeapsy-chiatrist.Intheunhappyeventthathospitalinvestigationiswarranted,thehappypatientmaybeinanim-portunatehurrytorushtoherbed-butsheiswillingtowaitaslongasshemust,soasnottobeputonacertainfloor."Thatnurse!"shesays.Thatnturseisalvaysagoodnurse.Oncein,thepatientwillexpressunlimitedco-operation;shewilldoanything,anythingatalltogettotllebottomofhercondition-exceptperhapsgastricanalysis,orlumbarpuncture."Icouldn'tstandthat."Myfavouritehospitalsignisnotmyowniobser-vation.ItisthesignoftheLittlePillow.Itmavbeelicitedonlyfromthefemalepatientwithaspecialnurse,whoinhercapacityaslady'smaidiskeptchangingthepositionoftheLittlePillow.Tomakethepatientcomfortableitmayhavetobetuckedunderoneshoulder,undertheelbow,orundertheflank.TheimportantthingisthattheLittlePillowmustbeplacedjustso.Thisisaspathognomonicasthefrozenfaceenteringthecon-sultingroom.CONCLUSIONItisdoubtfulifanvofthesignlsofneturosispresentedinthispaperwillsupplementtheper-sonalexperienceofanvcolleague,buthavingsufferedlonghoursinelicitingthem,thereisatleastsomefuninrecordingtheimi..H.CO-EXISTENCEOFALBRIGHT'SSYNDROMEANDMONGOLISM*1).L.A.BASTED)O,MI.D.,Kitclwe,wetr,Onit.THISISareportofachildwh1opreseintedthefeaturesoftwopediatricdisorders,oneofwhichiscommonandprobablytheoldestdescribedinrecordedmedicine;theotherisuncommonandbutrecentlydescribed.Bothareequallydisappointingfromthetherapeuticpointofview.Thejustifica-tionforsuchapresentationliesintheneveraofmolectlargeneticsintowhichouirmedicalunder-standingisbeingdrawn.Changingmethodsofbio-logicalinvestigationandthoughtarerevolution-izingacceptedconcepts,oldandnew.In1937Albrightetal.1firstdrewattentiontothebizarresyndromewhichnowbearshisname.Al-thoughitwvasdescribedbrieflythey'earbeforebyMeCtune,Albright,withfournevreportedcasesand14reviewedcases,wasthefirsttoreviewthissyndromewhichhecharacterizedbythefollowingfeatures:(1)Multiplebonecysts,whiclhhaveadistributionsuggestingarelationtonerveroots,orsuggestinganembryonicdefectinthemyo-tomes.(2)Areasofpigmentation,whiclhhaveadistributionsuggestingsomeconnection\viththebonecysts.(3)Precociouspubertyinfemal-es.*PresentedattheSouthwesternOntarioRegionalMeetingoftheRoyalCollegeofPhysiciansandSutrgeonsofCanada,london,Ont.,Novemiibei15,1960.Inasubsequentpaperin1947,Albright2effec-tivelydemonstratedthatthisuniqueentitywasneitheraformofvonRecklinghausen'sneurofibro-matosisnoraformoflipoidxanthomatosis.Herecommendedthatitbetermed"polyostoticfibrousdysplasia".Althoughtheetiologyremainedobscure,Albrightfeltthatthesymptomsofearlypubertywererelatedtotheearlyproductionoffollicle-stimtulatinghormone(F.S.H.).Thepatientwasa6-year-oldwhitegirl,nowaliveandwellwithmongolismandAlbright'ssyndrome.Theparentsandtwooldersiblingswereallaliveanidwell.Therewasnofamilyhistoryofskinpigmen-tationi,bonedisease,orcongenitaldeformities.Themotherwas35yearsofagewhenthischildwasde-livered.Theprenatalandnatalhistorywereunevent-ful.Therewasnobirthanoxiaordifficulty.Themotherhashadnosubsequentpregnancies.Thisgirlwasfirstseenatonedayofagebecauseofthetypicalstigmataofmongolism,allofwhichthischildpossessed.Hernewbornandinfantiledevelop-mentwastypicallymongoloid,withtheusualretarda-tioInofmilestones.Therewerenonewbornorchild-hoodillnessesorproblems.Itwasnoticedduringherinfancyexaminationsthattherewereseveralareasofbrownpigmentationoverthebackandtherightbuttocks(Fig.1).Themarginsoftheseareaswerejaggedandirregular,orasdescribedbyAlbright,"likethecoastofMaine".(ThesmoothcontouroftheskinlesioIsofvonRecklinghausen'sdiseaseis"likethecoastofCaliforniia".)