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Dementia

EDITEDBY

BernardCoope,MBChB,MMedSci,MRCPsych

AssociateMedicalDirector,ConsultantOlderAdultPsychiatrist

WorcestershireHealthandCareNHSTrust

FelicityA.Richards,MBChB,MRCPsych,DGM

ConsultantOlderAdultPsychiatrist

WorcestershireHealthandCareNHSTrust

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ABCofdementia/editedbyBernieCoopeandFelicityA.Richards. p.;cm.

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ListofContributors,vii

Introduction–ACalltoAction,ix

1 DementiaintheUK,1 BernardCoope

2 CausesofDementia,5 GeorgiosTheodoulou

3 Assessment,10 BernardCoopeandFelicityA.Richards

4 EarlyInterventionforDementia,14 BernardCoopeandTanyaJacobs

5 DrugTreatment,18 SolmazSadaghiani

6 DementiaandFamilies,22 JennyLaFontaine

7 Person-CentredCare,26 DawnBrooker

8 BehaviouralandPsychologicalSymptomsofDementia(BPSD),31 DhanjeevMarrieandSallyWilliams

9 DementiainYoungerPeople,37 PeterBentham

10 DementiainPrimaryCare,42 SimonRumley

11 DementiaintheAcuteHospital,47 ElizabethWard,DarylL.LeungandGeorgiosTheodoulou

12 DementiaandtheLaw,51 FelicityA.RichardsandJelenaJankovic

13 End-of-LifeCareinDementia,56 KaydeVries Index,61

ListofContributors

PeterBentham

BirminghamMemoryAssessmentandRareDementiaServices, Birmingham,UK

DawnBrooker

AssociationforDementiaStudies,UniversityofWorcester,Worcester,UK

BernardCoope

WorcestershireHealthandCareNHSTrust,Worcestershire,UK

TanyaJacobs

WorcestershireHealthandCareNHSTrust,Worcestershire,UK

JelenaJankovic

DudleyandWalsallMentalHealthPartnershipTrust,Dudley,UK

JennyLaFontaine

AssociationforDementiaStudies,UniversityofWorcester,HenwickGrove, Worcester,UK

DarylL.Leung

ElderlyCareandDementiaServiceRoyalWolverhamptonNHSTrust, Wolverhampton,UK

DhanjeevMarrie

WorcestershireHealthandCareNHSTrust,Worcestershire,UK

FelicityA.Richards

WorcestershireHealthandCareNHSTrust,Worcestershire,UK

SimonRumley

AylmerLodgeCookleyPartnership,Kidderminster,Worcestershire,UK

SolmazSadaghiani

WorcestershireHealthandCareNHSTrust,Worcestershire,UK

GeorgiosTheodoulou

WorcestershireHealthandCareNHSTrust,Worcestershire,UK

KaydeVries

GraduateSchoolofNursing,Midwifery&Health(GSNMH), VictoriaUniversityofWellington,NZ;AssociationforDementiaStudies, UniversityofWorcester,UK;UniversityofWashington,Seattle,USA

ElizabethWard

RoyalWolverhamptonNHSTrust,Wolverhampton,UK

SallyWilliams

WorcestershireHealthandCareNHSTrust,Worcestershire,UK

Introduction–ACalltoAction

BernardCoopeandFelicityA.Richards

WorcestershireHealthandCareNHSTrust,Worcestershire,UK

Historically,dementiahasnotfeaturedhighlyinthetrainingof eithermedicalstudentsorjuniordoctors.Dementiaisnowgaining increasingrecognitionasahealthcareissueinit’sownright.The fieldofdementiacareistransformingrapidly(Figure1),and whatevertheareaofhealthcareyouareinvolvedin,youwillat somepointbetouchedbydementia.Withthiscomestheneedfor allclinicianstolearnaboutdementia.Itishopedthatthisbookwill playitspartinhelpingcliniciansunderstandtheevolvingnatureof theevidencebase,changesinsocietalexpectationsandtheeffects dementiahasontheperson,theirfamilyandthehealthservice.

Whyistheneedtolearnaboutdementia changing?

Theevidencebase

Thepasttwodecadeshaveseenarapidgrowthinthescientific understandingofdementiaandthebraindiseasesthatcauseit, includingdevelopmentsinneuropathology,aetiology,imagingand theroleofgenetics.Inturn,thisknowledgeledtotheintroduction ofpharmacologicaltreatmentsand,consequently,anevidencebase fortheirlimitedeffectiveness.Asyet,noavailabletreatmentsmodifythebraindiseasescausingdementiaandthereisnoevidenceto supportscreeningprogrammes.

Continuedresearchhasalsoledtothediscoverythatmanyofthe treatmentsthathavebeenusedfordecadeswiththeclinicalaimof helpingpeoplewithdementiadomoreharmthangood.Sedating treatmentssuchasantipsychoticsareoflittleclinicaleffectiveness andareknowntoincreasemortalityinthosepatientsprescribed them.Fortunately,wearemovingawayfrompharmacological treatmentstowardsarangeofnon-medicalinterventionstohelp thosemostdistressedormostchallengingtocarefor.Centralto thishasbeentheflourishingofresearchandtheoryinthefieldof person-centredcare.

Afinalillustrationofthechangingevidencebaseisthedemonstrationoftheeffectivenessofserviceinterventions.Supporting carersbyprovidinginformationandemotionalsupportcan prolongacarer’sabilitytoprovidecareathome,reducingthe

needforamovetolongertermplacement.Awell-madeand well-communicatedearlydiagnosis,backedupbypost-diagnostic support,canimprovethequalityofcareforthepersonwithdementiaandtheirfamilies,aswellasreducingtheriskofcrisesinthe future.

Changesinsociety

Dementiaisaconditionthatgetscommonerwithage.Morethan halfofthosewithdementiaintheUKareover80.Itisthisagegroup thatisgrowingincountriesacrosstheworldandwiththisgrowth, thenumberofpeoplewithdementiaisclimbing.

Asthenumberofpeoplewithdementiahasbeenrising,thestructureoffamilieshasbeenchanging.Closemembersofthefamily havetraditionallysteppedintoprovidecareforpeoplewithdementia,withthemajorityofcarersbeingeitherspousesordaughters. Thereisanincreasingtrendforpeopletoenteroldageseparated andforchildrentobelivingatagreaterdistancefromtheirparentsandtobeinfulltimework.Thecapacityoffamilymembersto takeonacaringrolewillhaveanimpactonwhatisexpectedfrom professionalsinhealthandsocialcare.

Evidence Base

Changing Role for Dementia Care Policy

Changes in Society

Figure1 Thechangingﬁeldofdementiacare.

Box1 DementiaActionAlliance:CalltoAction

1.Ihavepersonalchoiceandcontrolorinﬂuenceover decisionsaboutme

• Ihavecontrolovermylifeandsupporttodothethingsthat mattertome.

• Ihavereceivedanearlydiagnosis,whichwassensitively communicated.

• Ihaveaccesstoadequateresources(privateandpublic)that enablemetochoosewhereandhowIlive.

• IcanmakedecisionsnowaboutthecareIwantinmylaterlife.

• Iwilldiefreefrompain,fearandwithdignity,caredforbypeople whoaretrainedandsupportedinhigh-qualitypalliativecare.

2.Iknowthatservicesaredesignedaroundmeand myneeds

Ifeelsupportedandunderstoodbymygeneralpractitioner(GP)and getaphysicalcheck-upregularlywithoutaskingforit.

Therearearangeofservicesthatsupportmewithanyaspectofdaily livingandenablemetostayathomeandinmycommunity,enjoyingthebestqualityoflifeforaslongaspossible.

IamtreatedwithdignityandrespectwheneverIneedsupportfrom services.

IonlygointohospitalwhenIneedtoandwhenIgettherestaff understandhowIcanreceivethebesttreatmentsothatIcanleave assoonaspossible.

Carehomestaffunderstandalotaboutmeandmydisabilityand knowwhathelpsmecopeandenjoythebestqualityoflifeevery day.

Mycarercanaccessrespitecareifandwhenneeded,alongwithother servicesthatcanhelpsupportthemintheirrole.

3.Ihavesupportthathelpsmelivemylife

• Icanchoosewhatsupportsuitsmebest,sothatIdon’tfeela burden.

• Icanaccessawiderangeofoptionsandopportunitiesforsupport thatsuitsmeandmyneeds.

• IknowhowtogetthissupportandIamconﬁdentitwillhelpme.

• IhaveinformationandsupportandIcanhavefunwithanetwork ofothers,includingpeopleinapositionsimilartomine.

• Mycareralsohasasupportnetworkthatsuitstheirownneeds.

4.Ihavetheknowledgeandknow-howtogetwhatIneed

• It’snotaproblemgettinginformationandadvice,including informationabouttherangeofbeneﬁtsIcanaccesstohelpme affordandcopewithlivingathome.

• IknowwhereIcangettheinformationIneedwhenIneedit,and Icandigestandre-digestitinawaythatsuitsme.

• Ihaveenoughinformationandadvicetomakedecisionsabout managing,nowandinthefuture,asmydementiaprogresses.

• Mycarerhasaccesstofurtherrelevantinformation,and understandswhichbeneﬁtstheyarealsoentitledto.

5.IliveinanenablingandsupportiveenvironmentwhereI feelvaluedandunderstood

• Ihadadiagnosisveryearlyonand,ifIwork,anunderstanding employer,whichmeansIcanstillworkandstayconnectedto peopleinmylife.

• Iammakingacontribution,whichmakesmefeelvaluedand valuable.

• Myneighbours,friends,familyandGPkeepintouchandare pleasedtoseeme.

• Iamlistenedtoandhavemyviewsconsidered,fromthepointI wasﬁrstworriedaboutmymemory.

• Theimportanceofhelpingmetosustainrelationshipswithothers iswellrecognised.

• IfIdevelopbehaviourthatchallengesothers,peoplewilltaketime tounderstandwhyIamactinginthiswayandhelpmetotryto avoidit.

• Mycarer’sroleisrespectedandsupported.Theyalsofeelvalued andvaluable,andneitherofusfeelsalone.

6.Ihaveasenseofbelongingandofbeingavaluedpartof family,communityandciviclife

• Ifeelsafeandsupportedinmyhomeandinmycommunity,which includesshopsandpubs,sportingandculturalopportunities.

• NeithermyfamilynorIfeelashamedordiscriminatedagainst becauseIhavedementia.Peoplewithwhomwecomeinto contactarehelpfulandsupportive.

• MycarerandIcontinuetohavetheopportunitytodevelopnew interestsandnewsocialnetworks.

• Itiseasyformetocontinuetoliveinmyownhome,andmycarer andIwillbothhavethesupportneededformetodothis.

7.Iknowthereisresearchgoingonthatdeliversabetterlife formenowandhopeforthefuture

• Iregularlyreadandhearaboutnewdevelopmentsinresearch.

• Iamconﬁdentthatthereisanincreasinginvestmentindementia researchintheUK.

• Iunderstandthegrowingevidenceaboutpreventionandrisk reductionofdementia.

• Asapersonlivingwithdementia,IamaskedifIwanttotakepart insuitableclinicaltrialsorparticipateinresearchinotherways.

• IbelievethatresearchiskeytoimprovingthecareI’mreceiving now.

• IbelievethatmoreresearchwillmeanthatmychildrenandIcan lookforwardtoarangeoftreatmentswhenIneeditandthere willbemoretreatmentsavailablefortheirgeneration.

• Iknowthatwithadiagnosisofdementiacomessupporttolive wellthroughassistivetechnologiesaswellasmoretraditional treatmenttypes

Atleastasimportantasdemographicgrowthisthechanging expectationsofanewgenerationofpeoplewithdementia,illustratedbytherelativelymoderndemandforearlydiagnosis.The pastdecadehasseenadramaticshiftfromindividuals‘turning ablindeye’tochangesintheirmentalperformance,toconcern aboutwhetherthesechangescouldrepresenttheonsetofdementia. Crucially,thisconcernisoftenaccompaniedbyawishtoknowif thisisthecaseandaskingforassessment.Thisnewopennesshas revealedanotherfact;peopleover50nowfeargettingdementia morethantheyfearcancer.

Policyandpolitics

Dementiabecameapoliticalissuein2007followingpublication ofthereportbytheNationalAuditOffice‘Improvingservicesand

supportforpeoplewithdementia’.Includedinthereportwasan economicanalysiswhichshowedthatdementiacoststheUKmore thanheartdisease,strokeandcancercombined.Mostofthiscost wasforcare,eitherthroughSocialServicesordirectlybypatients andtheirfamilies,ratherthanNHScosts.Nolongerwasdementia aminormatter;itbecamethemostexpensivehealthconditionthe countryfaces.

OneconsequencewastheNationalDementiaStrategy,whichhas guidedchangesinNHScaresince,furtherclarifiedbytheintroductionoftheNationalInstituteforHealthandClinicalExcellence (NICE)DementiaStandards.Currently,dementiahasahighpoliticalprofile,withtheprimeministerlaunchinghisownDementia Challengein2012.

Thisbookiswrittenasapracticalguidetotheclinicianwho wishestohelpthelivesofthosetouchedbydementia.Perhaps thenweshallbeguidedbythevoiceofpatientsandthefamilies whosupportthem.TheDementiaActionAllianceismadeup ofover100organisations.Throughworkingwithpeoplewith dementiaandtheirfamilies,theDementiaActionAlliancedrewup theDementiaDeclaration:ACalltoAction,sevenambitiousbut achievablestatementsabouthowpeoplecanlivewellandwhatthey wouldwishforfromprofessionals–statementsweshouldconsider inoureverydayclinicalpractice(Box1).

OVERVIEW

DementiaintheUK

BernardCoope

WorcestershireHealthandCareNHSTrust,Worcestershire,UK

• Therearecurrently800000peoplewithdementiaintheUK, andthisnumberwillrisetooveramillionby2021.

• Dementiaisthemostexpensivehealthcareissuethecountry faces.Theeconomicimpactfor2012wasover£23billion,more thanheartdisease,strokeandcancercombined.

• Thesyndromeofdementiaconsistsofimpairmentofcognitive skills,resultingfromdiseaseofthebrain,whichissevereenough toimpairdailyfunctioning.

• Thereismoretodementiathanmemoryimpairment.

• Notalloldpeoplehavedementiaandnotallpeoplewith dementiaareold.

Introduction

Dementiaisaclinicalsyndrome.ThischapterexaminesthedefinitionofdementiaandexploresanumberofissuesrelatedtodementiaasitisexperiencedintheUK.

Definition:Thesyndromeofdementia

Thesyndromeofdementiaconsistsofthreecomponents:

1 impairmentofcognitiveskills, 2 resultingfromdiseaseofthebrain, 3 whicharesevereenoughtoimpairdailyfunctioning.

Itisworthtakingtimetolookattheimplicationsofthisdefinition.

• Dementiaasasyndrome.Centraltothedefinitionisachange inmentalskills.Todiagnosedementia,thesechangesneedtobe confidentlyidentified,whichusuallymeansobtainingacareful historysupportedbyanassessmentofmentalskills.Dementia relatestohowwellaperson’sbrainisworkingratherthanthepresenceofpathology,andcan’tbediagnosedfromabrainscanany morethanaplainX-rayofanarthriticjointcanshowpain.The diseasesthatcausedementiaarecoveredinChapter2.

• Cognitivefunctionsareadiverseassortmentofbrainactivity.Theterm‘cognitivefunctions’coversmemoryencoding, long-termstoresofknowledge,wordfinding,languagecomprehension,faceorobjectrecognition,planningandorganisingof activityandjudgement.Differentbraindiseasesleadtodifferent patternsofcognitivechange.Thereismoretodementiathan memoryimpairment.

• Todiagnosedementia,theremustbegoodreasontosuspect diseaseofthebrain.Brainimagingmayhelp,butinpractice, braindiseaseisusuallyinferredfromchangeinmentalskill.

• Itisquitepossibletohavebrainpathologywithoutdementia Asimagingtechniquesdevelop,itmaysoonbecommontodiagnoseAlzheimer’sdiseaseyearsbeforeanysymptomshavedeveloped.Vascularchangesonimagingareverycommonandontheir owndon’tmeanvasculardementia.

• Impairmentofdailyfunctioningisanimportantbutimprecise term.Veryminorchangesinmentalskillsarenotusuallyreferred toasdementia,althoughtheremaybeadifferenceofopinion betweenpatient,carerandclinicianaboutwhenthechangeof functioninghasoccurred.Forthosewithsomedetectablechanges inmentalfunctionthatarenotimpactingondailyfunctioning, theterm mildcognitiveimpairment (MCI)issometimesused.

• Whendiscussingdementia,weshouldbecarefulnottousesyndromeandpathologyasinterchangeableconcepts.

Terminology

Inthe1970s,thelateGeriatricianBernardIsaacsencouragedthe useoftheterm‘chronicbrainfailure’asanalternativetodementia. Theaimwastoemphasiseorganfailureandtobringthedefinitioninlinewithothercommonlyusedterms,suchasheartfailure. ‘Acutebrainfailure’representeddelirium.Ultimately,thetermwas droppedasithadtoomanynegativeconnotations,buttheprincipleisworthreflectingon.Dementiaiswhatweexperiencewhen changesinbrainfunctionimpactondaytodaylife.

TheNationalAuditOfficeinitsgroundbreakingreport‘ImprovingServicesandSupportforPeoplewithDementia’tookamore bluntapproachandchosethesimpleterm‘progressiveandterminal braindisease’.Whilstthismaynotrespectthesyndrome/pathology distinction,ithadthemeritofcommunicatingtheseriousnessof dementiatoawiderangeofopinionformersandpoliticians.

Prevalenceanddemography

TheAlzheimer’sSocietycollatedthefollowingfactsaboutdementia in2012.

• Therearecurrently800000peoplewithdementiaintheUK.

• Thereareover17000youngerpeoplewithdementiaintheUK.

• Thereareover11500peoplewithdementiafromblackand minorityethnicgroupsintheUK.

• Therewillbeoveramillionpeoplewithdementiaby2021.

• Two-thirdsofpeoplewithdementiaarewomen.

• Thereare670000carersofpeoplewithdementiaintheUK.

• FamilycarersofpeoplewithdementiasavetheUKover£8billion ayear.

• Eightypercentofpeoplelivingincarehomeshaveaformof dementiaorseverememoryproblems.

• Two-thirdsofpeoplewithdementialiveinthecommunitywhile one-thirdliveinacarehome.

• Only44%ofpeoplewithdementiainEngland,Walesand NorthernIrelandreceiveadiagnosis.

Economicimpact

UntiltheNationalAuditOfficepublisheditsreportondementia in2007,dementiahadneverbeenconsideredapriority.Itwas theimpactofdementiaontheUKhighlightedinthisreportthat broughtaboutanewpoliticalwilltoaddressthecondition.The reportdetailedtherealsituationaboutdementia–notonlyhow muchtheNHSandSocialServiceswerespendingondementia butalsowhatindividualsspentontheirowncareandhowmuch lostearningscouldbeattributedtocarerstakingtimeoffworkto providecare.Theanswerwasalittleover£17billion.

Thesignificanceofthissumwasthatifotherconditionsunderwentthesameanalysis,dementiacosttheUKmorethanheart disease,strokeandcancercombined.Dementiaisthemostexpensivehealthcareissuethecountryfaces,anditwillcontinueon anupwardtrajectory.Theeconomicimpactfor2012wasover £23billion.

Whohasdementia?

Thediseasesthatcausedementiagetcommonerasageadvances, sothemajorityofthoselivingwithdementiaareover80.Butnot alloldpeoplehavedementiaandnotallpeoplewithdementiaare old.Dementiacanoccurinpeopleunder65,whenspecialistskills areneededtoaddressthecomplexitiesofdiagnosticdiversity,complexpersonalandfamilyresponsesandage-relatedissuessuchas employment.Theneedsofyoungerpeopleandtheirfamiliesare examinedinChapter9.

Asmentendtodieyoungerthanwomen,two-thirdsofthosewith dementiaarewomen.Agediscriminationisgenderdiscrimination! Thiscanmeanthatcaresettingsforpeoplewithdementiamayhave agreaterproportionofwoman–bothresidentsandstaff.Maleresidentsmayfeellesscomfortableintheseenvironments,andaremore likelytobeperceivedaschallenging.

Dementiainlearningdisabilityisanotherareathatrequires specialistskills–fromdiagnosis,tohelpingthatpersonlivewell. Dementiaismorelikelytodevelopatayoungageinthosewith learningdisability,especiallyDown’ssyndrome.Theobservation thatpeoplewithDown’ssyndromecommonlydevelopeddementia intheir40sindicatedalinktochromosome21andultimatelyto thediscoveryoftheamyloidprecursorgene.

Dementiaismorelikelytohaveayoungeronsetinblackorethnicminoritygroups,butthesegroupsareunderrepresentedinservicesfordementia.Servicesneedtotakeactivestepstomakesure thattheyreachouttominoritycommunitiesandtacklebarriersin assessmentandsupport.Thetechnicalaspectsofmakingadiagnosishavetotakeintoaccountlanguage,withtestingbeingcarriedout intheperson’spreferredlanguagewherepossible.Consideration needstobegiventoeducationalbackgroundandalsothecultural preconceptionsembeddedinmanycognitivetests.Evenasimple questionlike‘Whatistheseason?’maybeinfluencedbywherean individualisfrom.

TheDiversityofdementia

Itiscommonforprofessionalstoclassifydementiaintothreestages ofseverity;mild,moderateandsevere.However,itmaybebetterto thinkofdementiaasajourneyapersonismovingalong,ratherthan aspectrumofdiseaseseverity–fromwhatthatindividualpreviouslyconsideredas‘normal’,throughnoticeablechangesinmental skillsthatbecomeconsistentandthenclearenoughtowarrantthe useofthetermdementia.Asthepersonmovesthroughthedifferent stagesofthecondition,thereislossofmentalskills.Otherfeatures maycomeandgo,andqualityoflifeisnotcloselylinkedtodementiaseverity.Asanincurablecondition,theperson’sjourneywillend indeathresultingfromdementiaorothercauses.

Thejourneyofdementiaisrarelymadealone.Usually,close familysupportthepersonwithdementiaandinevitablytheirlives arealsoalteredbyit.Relativesstarttobereferredtoas‘carers’, althoughmanyareunhappywiththatdescription.Carers’research commonlystatesthenegativesofthisrole,suchasburden,strain andstress,buttherearealsothepositives.Itismoreusefulto thinkabouthowthepresenceofdementiainafamilychanges relationshipsandhowdementiaisexperiencedinitsentirety.This intricateinterplayisaddressedindetailinChapter6.

Thechangingjourney

Lossofcognitiveskillsisthecorefeatureofthedementiasyndrome, butthereismuchmoretoit.Arangeofnon-cognitivefeaturescan beexperienced.

Psychosis

Thepresenceofdelusions(fixed,falsebeliefs)andhallucinations (perceptionswithoutacorrespondingobject)arecommonatsome pointindementia,althoughtheseexperiencesmaybetransient. Complexvisualhallucinationsareacorefeatureofdementiawith Lewybodydisease.Suchfeaturessometimeshavelittleimpact

onthepersonwithdementia,andhaveevenbeenknowntobe enjoyable;however,theycanalsobeextremelydistressingandthe causeofriskyoraggressivebehaviour.Apersonwithdementiawho believesthatthespouseisanimpostermaybecomeunderstandably angryorviolenttowardsthem.Seeingdismemberedbodiesor snakesinthehousecanbefrighteningandleadapersontorunout ofthehome.Nolongerbelievingyourhomeisyourowncanbe upsetting.

Affectivedisturbance

Depressioncommonlyaccompaniesdementia,andagainmaybe transient.Thecausemaybemultifactorial,fromthesubjective experienceoffindingtheworldabewilderingplace,havinginsight intotheirloss,thebehaviourofothersordirectlyduetobrain disease.Depressionisespeciallycommonincarehomeresidents. Itisusualtouseantidepressanttherapy,althoughthereislittle supportiveevidenceforthis.Strategiesaimedatimprovingquality oflifethroughperson-centredcareandmeaningfulactivitymaybe moreproductive.

Behaviouralchange

Behaviourthatisoutofcharacterorchallengingtoothersisdistressingtolovedonesandcanbeamajorissueforthoseprovidingcare.Itisimportantnottoseeitasasymptomofdementia, althoughclearlythedementiaisinfluencingit.Angerorshouting mayarisefromboredom,orpain.Behaviourisacommunication andweshouldaskourselveswhatisbeingcommunicated.Theagitatedwomanlookingforhermothercaneasilybeseenasjustforgetfulandmistaken,orinsteadcanbethoughtofasexpressingthe needforcomfortandfamiliarattachment,leadingtoattemptsto meetthatneed.Theissueofbehaviourthatchallengesisexploredin Chapter8.

Attachment

Theword attachment appearsrepeatedlyinthisbook.Theconcept wasdevelopedbyJohnBowblyanddescribesthenatureofimportantandstrongrelationships.Wehaveanevolvedpredispositionto formstrongsocialbondsandthenatureofourpatternofattachmentformingisshapedbyexperiencesearlyinlife.Theneedfor attachmentnevergoesandisahealthypartofadultlife.Eventhose withseveredementiawillhaveaneedforattachment,althoughthe expressionofthisneedmaynotbeobvious,forexample,searching foralong-lostmotherwhenanxious.Understandingthepersonand theindividualpatternofrelationships,inthepastandpresent,isa crucialcomponentofperson-centredcare.

Physicaldisabilityanddeath

Thebraindiseasesthatcausedementiasarecommonerinolder people,andsofrequentlyaccompanyotherhealthconcerns thatmaybelife-limiting.Ifthisisnotthecase,dementiaswill

becomelife-limitingintheirownright.Lossofmobilityandpoor swallowingresultinahigherriskofinfections.Itisprobablybetter toviewthisasthelatestagesofseverebraindisease,ratherthan simplyanacuteillness.Goodpalliativecareattheendoflifestarts withrecognisingtheimpactofseveredementia.Earlydiagnosis offersachanceforpeopletomaketheirfuturewishesknownand planappropriatecareandplaceofdeath.

Butthejourneyforthefamilygoesonafterdeath.Research showsthatbereavementexperiencesmaybelongeranddeeper afteradeathfromdementia.Indeed,griefreactionsoccurin relativeslongbeforethepersonwithdementiahasdied.Good careattheendoflife,workingwithfamiliesandbereavement supportcanhelpthis.Chapter13addressesthisimportantand oftenneglectedarea.

Whocanhelp?

Thepersonwithdementia

Familyandfriends

Primarycare

Socialcareandhousing

Acutehospitals

Specialistmentalhealthteams

Voluntarysector

Thepersonwithdementia.Notthemostobviousstart,aspeople withdementiaareoftenthoughtofaspassiverecipientsofcare. Anearlydiagnosisgivesthepersonwithearlydementiatimeto planlifeandexpresswishesforthefuture.

Familyandfriends.Peoplewhoprovidethebulkofdementiacare intheUK.Professionalscouldseetheirroleashelpingfamilies tounderstanddementiaandhowtoprovidecare.

Primarycare.Generalpractitioners(GPs)andotherprimary carestaffarewellplacedtohelpthepersonwithdementialive well.Theremayalreadybeanestablishedrelationshipwitha GP,whomayalsoknowothermembersofthefamilyandwill haveamuchbroaderviewoftheentiretyoftheperson’shealth thanspecialists.Dementiainprimarycareisthesubjectof Chapter10.

Socialcare.Peoplewithdementiaandtheircarersareentitledtoa benefitscheck–forattendanceallowanceandcarers’allowance. Acarers’assessmentshouldbeofferedtoallinformalcarers. Formalcarearrangementsmayenableapersontoremainat homewithadditionalsupportformanyyears,forexample, offeringhelpwithpersonalcare,nutritionandmedication compliance.Communitysocialworkerscanhelpguidepeople withdementiaandtheirfamiliesinthisarea.Serviceslikedirect paymentsareparticularlyusefulinearlydementia,toallowan individualwithdementiaplantherequiredsupport.Respite placementsarealsoanoptiontogiveinformalcarersabreak fromthisrole.

Assistivetechnology

Recentyearshaveseenagrowthintechnologicalwaystosupporta personwithdementiamaintainindependenceandstaysafe.AGlobal PositioningSystem(GPS)locatormayhelpgivegreaterconﬁdence tosomeonewhoenjoysadailywalkbutmightgetlost.Automated tabletdispenserswithalarmscanhelpincompliance.Itisarapidly changingﬁeldthatcansupportbutnotreplacethepresenceofa carer.

Carehomes.Itmaywellbethattheneedsofapersonwithdementia changetoalevelwherelong-termplacementhastobearranged. Athirdofpeoplewithdementialiveinaresidentialornursing home.Developingskillsindementiacaretakestimeandeffort butitisimportantforstaffandresidentsalike.

Acutehospitals.Peoplewithdementiafindacutehospitalschallengingplacestobein,andhospitalscanfindpeoplewithdementiachallenging,too.Theseissuesandsolutionsarediscussedin Chapter11.

Specialistmentalhealthteams.OlderadultCommunityMental HealthTeams(CMHTs)aremultidisciplinaryteamsthatwork withpeoplewithdementiaandotherswithpoormentalhealth. Theywillusuallyincludepsychiatrists,communitypsychiatric nurses(CPNs),occupationaltherapistsandsocialworkers.They willusuallyworkwithpeopleatgreatestneed(risk,challengingbehaviour,carerbreakdown)andarenotinapositiontofollowuppeopleindefinitely.Theyoffer‘steppingstones’ofspecialistcarealongthejourneyofdementia.MemoryClinicsorEarly InterventionDementiaServicescandealwithdiagnosticassessmentsanddrugtreatments.Admiralnurses,specialistsinsupportingfamiliesaffectedbydementia,sometimesworkalongside CMHTs.

Voluntarysector.OrganisationsliketheAlzheimer’sSocietyand AgeUKcanprovideawealthofinformation,throughexcellent Internet-basedsitestomoredirectandpersonalisedprovision. Insomepartsofthecountry,theseservicesarecommissionedto providedementiaadvisors,peoplewhocanbeapproachedfor adviceandsignposting.DementiaCafésareregulargatherings forpeoplewithdementiaandtheirfamiliesforpeersupportand theymakeaverypositivecontributiontohelpingthosewith dementialivewell.

Conclusion

Forthefirsttime,dementiaisbeingrecognisedasanimportant issuefacedbythecountryandonethatchallengestheNHSin manyways.Betweentheold,futilepessimismof‘There’sno pointinthinkingaboutdementiaasnothingcanbedone’and thenewoversimplifiedoptimismof‘Wecanpreventdementia orstopitgettingworsesoeveryoneshouldhaveanearlydiagnosis’,thereliesawealthofrealopportunitiestohelpthelives ofthoselivingwithdementia.TheNationalInstituteofClinical Excellencehaspublished10qualitystandardsfortheNHSthat encapsulatethis.

NICE10qualitystandards

Statement1Peoplewithdementiareceivecarefromstaff appropriatelytrainedindementiacare.

Statement2Peoplewithsuspecteddementiaarereferredtoa memoryassessmentservicespecialisinginthe diagnosisandinitialmanagementofdementia.

Statement3Peoplenewlydiagnosedwithdementiaand/ortheir carersreceivewrittenandverbalinformation abouttheircondition,treatmentandthesupport optionsintheirlocalarea.

Statement4Peoplewithdementiahaveanassessmentandan ongoingpersonalisedcareplan,agreedacross healthandsocialcare,thatidentifiesanamedcare coordinatorandaddressestheirindividualneeds.

Statement5Peoplewithdementia,whiletheyhavecapacity,have theopportunitytodiscussandmakedecisions, togetherwiththeircarer/s,abouttheuseof advancestatementsoradvancedecisionstorefuse treatment,lastingpowerofattorneyandpreferred prioritiesofcare.

Statement6Carersofpeoplewithdementiaareofferedan assessmentofemotional,psychologicalandsocial needsand,ifaccepted,receivetailored interventionsidentifiedbyacareplantoaddress thoseneeds.

Statement7Peoplewithdementiawhodevelopnon-cognitive symptomsthatcausethemsignificantdistress,or whodevelopbehaviourthatchallenges,are offeredanassessmentatanearlyopportunityto establishgeneratingandaggravatingfactors. Interventionstoimprovesuchbehaviouror distressshouldberecordedintheircareplan.

Statement8Peoplewithsuspectedorknowndementiausing acuteandgeneralhospitalinpatientservicesor emergencydepartmentshaveaccesstoaliaison servicethatspecialisesinthediagnosisand managementofdementiaandolderpeople’s mentalhealth.

Statement9Peopleinthelaterstagesofdementiaareassessedby primarycareteamstoidentifyandplantheir palliativecareneeds.

Statement10Carersofpeoplewithdementiahaveaccesstoa comprehensiverangeofrespite/short-break servicesthatmeettheneedsofboththecarerand thepersonwithdementia.

CausesofDementia

GeorgiosTheodoulou

WorcestershireCareandHealthNHSTrust,Worcestershire

• Differentdiseasesofthebraincausedifferentchangesinmental function.

• Alzheimer’sdisease(AD)isstilltheleadingcauseofdementiain theUK.

• Thereisaninterplaybetweentheenvironmentandgenesinthe developmentofdementia.

• Vascularriskfactorsareassociatedwithahigherriskfor dementiasincludingAD.

Introduction

Thevariedphenotypesofdementiareflectamultitudeofcauses (seeTable2.1).Abetterunderstandingoftheseaidsanaccurate diagnosis,which,inturn,shouldhelpthepersonwithdementia andtheircarer(s)accesstheappropriatesupportandavailablepharmacologicaltreatmentswhereindicated.Moreover,abetterunderstandingofthepathologyofthedifferentphenotypeswillhopefully leadtonovelandmoresuccessfulpsychosocialandpharmacologicaltreatments.ThemaincausesofdementiaarelistedinTable2.2 andarediscussedindetailinthischapter.Thelesscommoncauses ofdementiasyndromesarealsolisted(seeTable2.3).

Alzheimer’sdisease(AD)

TherearethreemainADphenotypes:

1 TypicalAD

2 PosteriorCorticalAtrophy(PCA)

3 LogopenicAphasia(LA)

Clinicalfeatures

Ineachcase,thecellularpathologyissimilarbutthedistributionof thepathologyleadstothecharacteristicclinicalpresentations.

TypicalAD

TypicalADmostlyaffectsolderpeople,especiallythoseintheirlate 70s–80s.Itpresentswithinsidiouslyworseningmemory,mostobviouslyinnewlearningbutalsoinrecallofpreviouslylearntmemories.Attentionandconcentrationarerelativelywellpreserved.

Thisamnesicpresentationrelatestothepredilectionof Alzheimer’spathologyformedialstructuresofthehippocampus andcingulategyrus.

Overanumberofyears,thediseaseprocessspreadsoutacrossthe cortextoaffectthetemporal,parietalandfrontallobes,withrelative sparingoftheoccipitallobes.Subtlelanguageimpairmentscommonlyoccurwithamnesia,andfrontalinvolvementleadstopoor planningandorganisingskills.

Table2.1 Classiﬁcationofprimaryneurodegenerativepathologiescausing dementias.

TauopathiesProgressiveSupranuclearPalsy,Corticobasilar Degeneration,PiDandFTDP17(?AD) �� -synucleinopathiesParkinson’sdiseasedementia/dementiawith Lewybodies

AmyloidopathiesAlzheimer’sdisease

PriondiseaseCreutzfeldt–Jakobdisease(CJD) PolyglutaminediseaseHuntington’sdisease

Table2.2 Maincausesofdementia(relativeproportioninpercentage).

Alzheimer’sdisease(62%)

Cerebrovasculardisease(17%)

MixedAlzheimerandcerebrovasculardisease(10%)

DementiawithLewybodies/Parkinson’sdisease(6%)

Frontotemporaldementiasyndromes(2%)

Lesscommoncausesofdementiasyndromes

Alcohol

Multiplesclerosis

Normalpressurehydrocephalus

Paraneoplastic/autoimmune

Huntington’sdisease

Wilson’sdisease

CJD

HIV/AIDSdementiacomplex

Metachromicleucodystrophy

Space-occupyinglesions

Withdiseaseprogression,languageimpairmentsbecomemore obvious,withanomiaandgrammaticalchanges.Inseveredisease, anindividual’sbasicunderstandingandcommunicationbecomes severelyaffectedandgreaterphysicaldisabilityoccurs,withdeterioratingmobility,swallowingdifficultiesandincontinence.Theonset ofthesefeaturesmakestheindividualvulnerabletolife-limiting infectionsandpoornutrition(seeChapter13).

Posteriorcorticalatrophy

InPCA,theADpathologymostlyaffectsoccipitalareas,leadingto earlychangeinvisuospatialskills.Visualrecognitionofobjectsis affectedasisthelocationofobjectsinspace.Thecanbethought ofasthe‘what’andthe‘where’ofspatialthinking.Itisnotjust visualskillsbuttheperson’sabilitytothinkinthreedimensionsthat changes;thiscanleadtoearlylossofpracticalskillssuchasdressing.

ThetriadofBalint’ssyndromeofoculomotorapraxia(difficulty fixingthegazeonanobject),opticataxia(difficultyguidingthe handtoanobjectbyvision)andsimultanagnosia(difficultyperceivingmultipleobjectsinthevisualfield)issometimeselicited.

Inearlystages,otherareasofthebrainarespared,somemory, languageandfrontalfunctionsarelessaffected.Patientsoftenhave clearrecalloftheirdifficultiesandcanarticulatethemclearly.Paradoxically,thismeansthattheymaynotbetakenseriously,aproblemcompoundedbyoftengoodperformancesonsimplecognitive assessments.PCAisfoundinayoungeragegroupthantypicalAD.

Logopenicaphasia

LAisaprimaryprogressiveaphasia,manifestingasprominent earlychangesinfluentspeechproduction.Itresemblesprogressive non-fluentaphasia(PNFA,atypeoffrontotemporaldementia)but isusuallynotapurelanguagedisorderasitisoftenaccompanied withdeficitsinvisualmemoryandvisuospatialskills.Theabsence ofspeechapraxia(problemsrepeatingpolysyllabicwords)helps distinguishitfromPNFA.Astheconditionprogresses,itstartsto resembletypicalAD.

PathologyofAlzheimer’sdisease

Allthreesubtypeshavethepathologicalhallmarksofbetaamyloid(A�� )-containingsenileplaquesandhighlyphosphorylated tau-protein-containingneurofibrillarytangles(NFTs),which, tosomeextent,alsooccurinhealthybrains–the‘amyloidcascadehypothesis’(seeBox2.1).Thepathologyleadstodisruption inacetylcholineneuronalsystemsprojectingfromthenucleus basalisofMeynert,the‘cholinergichypothesis’.Serotenergicand noradrenergicprojectionsarealsoaffectedtoalesserdegree.

Box2.1 Breakdownoftheamyloidcascadehypothesis

–Amyloidprecursorprotein(APP)iscodedonchromosome21and issequentiallycleavedbyalpha,betaandgammasecretase enzymesintoA��

–A�� variesinlengthbetween37and42aminoacids.Senile plaquesinADarepredominantlycomposedofA�� of42amino acidlength(A�� 42).Wherethereisadisproportionateamountof gammasecretaseactivity,agreaterproportionofA�� 42is produced(thepredominantcleavagepathwayisthatofthealpha secretase(95%)leadingtonon-amyloidgenicpathways).

–Gammasectretasehastwoidentiﬁedmutations–Presenilin1and Presenilin2andaccountsfor5%offamilialAlzheimer’sdisease (FAD).

–Themodiﬁedamyloidcascadehypothesisstatesthatabnormal amyloiddepositionprecedestheformationofNFTs,althoughhow oneleadsontoanotherisnotclear.Tauproteinisimportantfor microtubulefunctionandsodysfunctionhereasinNFTformation willdisruptcellstructureleadingtocelldeath.

Vasculardementia(VaD)

Clinicalfeatures

Thefunctioningofthebraincanbeimpairedbyischaemia.The clinicalpresentationofvasculardementiaisdiverse,withvariationsintimecourseandchangesinmentalskills.Thestepwise deteriorationwithplateausclassicallydescribedinVaDisperhaps overstated,reflectingtheuncommonpresentationofpuremultiple strategicinfarcts.

Areasofpoorbrainfunctionduetocorticalischaemiacanproducefocalcognitivechange(e.g.dysphasiaorimpairedfacialrecognition),yetthesameindividualmayhavesomebrainfunctionsthat areunaffected.

Aninsidiousonsetofpatchyepisodicmemoryimpairment similartoanADpresentationiscommoninVaD.Executiveand visuospatialdifficulties,inattention,mentalslowingandapathy tendtobemorecommonearlierinitscoursecomparedtoADas areemotionalchangesofdepressionorlabileemotions.Dailyfluctuationsandnocturnalworseningarecommon(cf.AD).Psychotic phenomenaoccurinVaDandmaymimicsymptomsassociated withLewybodydisease.

Sub-corticalimpairmentmaycauselessobviouschangesinconcentrationormotivation,whichcanbebothdisablingandchallengingtocarers.

PathologyofVaD

Ischaemicdamagetothebraincanoccurthroughmicroangiopathy (diffusesmallvesseldisease),strategicinfarctionormultiplelacunar infarction.Vasculitismayalsocauseischaemicdamage.Thegreater thecellvolumeloss,thegreatertheimpairment,althoughthesite ofdamageisalsorelevant,forexample,bilateralthalamicinfarcts mayproduceprofoundamnesia.Adominantlyinheritedformof diffusewhitematterdiseaseisknownascerebralautosomaldominantarteriopathy,withsubcorticalinfarctsandleukoencephalopathy(CADASIL).

Therewillusuallybeevidenceofvascularriskfactorsandvasculardiseaseelsewhere.

MixedADandVaD

ClinicalfeaturesandpathologyasforVaDandAD

ThecoexistenceofbothAlzheimer’spathologyandvasculardiseasemayproducedementiaevenwherethepresenceofeither pathologyalonewouldnotbesufficienttoproduceadementiasyndrome.Bothconditionsarecommonbutoccurtogether

morecommonlybychancealone.Thisisprobablybecausethey sharesomeriskfactors,forexample,diabetesandtheApoE genotype.

DementiawithLewybodies (DLB)/Parkinsondiseasedementia(PDD)

Clinicalfeatures

DementiawithLewybodiesandParkinson’sdiseasedementia areclinicalpresentationsofLewybodypathologyinthebrain. Bothconditionsareconsideredtobeonthesamespectrum ofdisorder.

InDLB,theinitialpresentationisofcognitivechange,commonlyinvolvingvisuospatialskillswithrelativelyintactmemory. Otherfeaturesaccompanythedeficitsinmentalskills,includingvisualhallucinations,markedfluctuationsinattentionand consciousness,extrapyramidalsigns,falls,rapideyemovement (REM)sleepbehaviourdisorderandneurolepeticsensitivity.The sensitivitytoneurolepticorantipsychoticdrugsissevereandcan belife-threatening.Thesechangesmaymimicacutedelirium,and thediagnosiscandependonadetailedlongitudinalhistoryfrom aninformant.

InDLB,theparkinsonianfeaturesmaybesubtle,alackoffacial expression,bradykinesiaandrigidity,ormonotonousvoicethatcan easilybemistakenfordepression.Thegaitmaybeshufflingwithloss ofarmswing,buttremorislesscommon.

Upto80%ofthosewithestablishedParkinson’sdiseasemaygo ontodevelopdementiaandthisisusuallyreferredtoasParkinson’s diseasedementia,withapresentationverysimilartoDLB.

PathologyofDLB/PDD

Lewybodiesareinclusionswithinthecytoplasmofneuronscomposedof �� -synucleinaswellasotherproteinsincludingubiquitin andneurofilamentprotein.Alzheimer’spathologyalsocoexistsin DLB.ThedeficitofacetylcholinefunctionisgreaterthaninADand patientsmaygainsymptomaticbenefitfromtreatmentwithacetycholinesteraseinhibitors.

Frontotemporaldementias(FTDs)

Thefrontotemporaldementias(FTDs)areaheterogeneousgroup ofdementiascausedbyanincreasingnumberofindividuallyidentifiedpathologies.Broadly,threephenotypeshavebeendescribed todate:

1 Behaviouralvariant(bv-FTD)

2 Semanicdementia(SD)

3 Progressivenon-fluentaphasia(PNFA)

Clinicalfeatures

Behaviouralvariant

Bv-FTDpresentswithchangesinpersonalityandbehaviour.Loss ofsocialawareness,disinhibition,impulsiveness,apathy,mental rigidity,newobsessionsandchangesineatinghabits,oftenwith

apredilectionforsweetfoodsandpersonalneglect,arecommon features.

Semanticdementia

SDpresentswithlossofmemoryforthemeaningofwords,thatis, semanticknowledge.Speechproductionreducesandissimplified, forexample,usingcategoricalwordslike‘animal’or‘thing’rather thanmorespecificterms.Thereisanassociatedlossofknowledge ofobjectsthatgoesbeyondworduse.Memoryandorientationare notusuallyaffectedearlyon.Moretypicalfeaturesofthebv-FTD phenotypewilldeveloplaterinthecourse.

ProgressiveNon-FluentAphasia

PNFApresentswithaseveredisruptionofspeechoutput,with grammaticalandphonologicalerrors.Thespeechishesitant,and producedwithgreateffort.Mildexecutivedysfunctionisalso common,butmemoryandattentionremainrelativelyintactearly on.Symptomscanoftencausegreatdistress.Theconditioncan seemrelativelystableandunchangingformanyyears.

PathologyofFTD

AnumberofdifferentpathologiesareassociatedwithFTD. Approximately50%ofFTDisassociatedwithtaupathology. Frontotemporaldementiasyndromescanbeassociatedwithother conditions.Progressivesupranuclearpalsy(PSP),corticobasal degeneration(CBD),FTDwithparkinsonismlinkedtochromosome17(FTDP-17)andmotorneurondisease(MND)canall independentlybeassociatedwithFTD.

Normalpressurehydrocephalus

Clinicalfeatures

Normalpressurehydrocephalususuallypresentswithatriadofmild cognitivedifficulties,awide-basedgaitandurinaryincontinence. Manyofthesesymptomsarecommonintheelderly,andthelattertwoareusuallyfeaturesofthemiddletolaterstagesofother dementias.

PathologyofNPH

NPHissomewhatofamisnomer,asonaveragetheintracranial pressureisalittleabovenormallimitswithfrequentadditional pulsesknownas‘B’waves.Theunderlyingmechanismofhow thistranslatesintocerebraldysfunctionanddamageisnotyet elucidatedbutisthoughtto,atleastinpart,leadtochronicmild periventricularwhitematterischaemia.

Risksandprotectivefactorsinthe aetiologyofdementia

Aetiologicalstudiesindementiahavetodatemainlyfocusedon thedementiasyndromeingeneralorspecificallyonAlzheimer’s dementia.Studiesontheaetiologyofotherdementiasareless common.Byidentifyingindividualfactorsthatprotectagainstor increasetheriskofdementiawemaybeabletodevelopprimary preventioninterventions.

Table2.3 Othertypesofdementia.

Huntington’sdisease

Autosomal-dominantdegenerativediseasecharacterisedbyprogressivepsychiatricandmovementdisorderfollowedbydementia.Atripletrepeatdisorder withamutationintheHuntingtongeneonchromosome4,whichcauses anenlargedpolyglutamineportiontobeaddedtotheHuntingtonprotein. Mutatedformsaggregatewithinneurons,causingcelldeath.

Creutzfelt–Jacobdisease(CJD)/priondisease

Rapidlyprogressivedementiaassociatedwithepilepsy.Pathogenicprocess involvesconversionofanormalcellsurfaceproteintermed cellularprionprotein intoanabnormallyfoldedandprotease-resistantisoform.Asmallminority (15%)arefoundtobecausedbyamutationintheprionproteingenebutthe majorityaresporadic,classicallycausedbyCJD.

Multiplesclerosis

Chronicdiseaseofthecentralnervoussystem(CNS).Involvesinﬂammatory,demyelinatingandneurodegenerativeprocesses.Primary,secondaryand relapsing/remittingforms.Dementiamayformpartoftheneuropsychiatric presentationofmultiplesclerosis(MS),andonrareoccasionsbetheonlymanifestationofMS.Patternandseverityofcognitivedeﬁcitsnotcorrelatedwith eitherdiseasedurationorphysicaldisability.

Limbicencephalitis

Encompassesarangeofinﬂammatoryconditionsselectivelyaffectingthelimbicsystem(amygdala,hippocampus,hypothalamus,insularcortexandcingulatecortex).Thesecanpresentwitharelativelyrapid-onsetdementiaand/or otherneuropsychiatricsignsandsymptoms.Originallythoughttoonlybeassociatedwithanautoimmuneresponsetocancerorinfectionelsewhereinthe body,itisnowrecognisedtoberelatedtoautoantibodiestovoltage-gated potassiumchannels(VGKCs)andN-methyld-aspartate(NMDA)receptors. Potentiallytreatablewithplasmaexchange.

HIV/AIDSdementiacomplex

FeatureofadvancedHIV1diseaseprogression,rarewithearlyantiretroviral use.HIV1isneurotropicbutnecessarilypathogenicintheCNS;nevertheless, itistheorisedthatinsomecasesinfectionleadstoaninﬂammatorycascade, leadinginturntocelldeaththroughcytokine.

Metachromicleucodystrophy

Oneofagroupofgeneticlipidstoragedisorders.Thoughttobecausedby arylsulfataseAenzymedeﬁciency.Thisinturnimpairsgrowthordevelopment ofthemyelinsheath.Adultformpresentswithneurologicalsignsaswellas dementia.

Space-occupyinglesions(SOLs)–tumours/subduralhaematomas

Accountfor3%ofallcasesofdementia.SOLsincertainpartsofthebrain mayincreaseintracranialpressure,causingdementia.Aclassictumourlesion producingsignsofdementiaisfrontallobemeningioma.

Alcohol

Alcoholhasdirectneurotoxiceffectsonbraincellswithchronicexcessive exposure.

(i) Age

Dementiaincreasesinprevalenceandincidencewith increasingage.

(ii) Gender

Approximatelytwo-thirdsofpeoplewithdementiaare women.

(iii) Education

Educationmayenhanceneurologicalreserve,sogreater pathologyisrequiredtocausecognitivedeficitsaswell astherebeingagreatercapacitytocompensateforthe pathology.

(iv) Genetics

TheAPOE �� 4alleleisanestablishedriskfactorfordevelopingdementia.Itispresentinabout25–30%ofthepopulation andinabout40%ofallpeoplewithlate-onsetAlzheimer’s. AlthoughtheAPOE �� 4alleleisasusceptibilitygene,homozygoticcarriersdonotnecessarilydevelopdementia.Thereis tentativeevidenceofaninteractionbetweentheAPOE �� 4 allelewithstroke,hypertensionandalcoholinspecifically increasingtheriskofdementia.TheAPOE �� 2alleleappears toprotectagainstthedevelopmentofdementia.TheAPOE geneonchromosome19playsapartinlipidmetabolismin neuronalhealth.

Therearerarefamilieswhereyoung-onsetADhasafamilialdominantlyinheritedpatterninvolvingthepresenilin1 (Ch14)and2(Ch1)genesencodingforthesplicingofAPP. InDown’ssyndrome(trisomy21),thereisanassociation withexcessAPPproductionandabnormalsecretaseactivity bothcodedforonCh21leadingtoAD. ItisincreasinglyrecognisedthatoftheheritableFTDs,alarge minorityareassociatedwithabnormalitiesonCh17where tauproteinmetabolismiscodedfor(FTDP-17).

(v) Smoking

Morerecentstudieshaveshownthatsmokingincreasesthe riskofdevelopingdementia,althoughitisdebatableifnicotinealonemayhaveaprotectiveeffectforsomedementias.

(vi) Alcohol

Thereisaclearrelationshipwithheavyalcoholuseleadingto anincreasedriskofdementia.Thereareseveralmechanisms includingthedirecttoxiceffectsofalcoholonthebrainas wellastheincreasedprevalenceofcerebrovasculardisease inthosewhodrinkexcessivealcohol.Indirecteffectsinclude thegreaterprevalenceofheadinjuryandthiaminedeficiency,whicharebothriskfactorsforcognitiveimpairment inheavyalcoholusers.

(vii) Cholesterol

Theevidenceatpresentisequivocalastotheinfluence cholesterolhasonthedevelopmentofdementia.Theuse ofstatinshasnotbeenfoundtoreducetheincidenceof dementia.

(viii) Bloodpressure

Mid-lifehypertensionisassociatedwithbothVaDandAD laterinlife.Inthoseover75yearsofage,itishypotensionthat appearstobethemainriskfactor,andindeedbyraisingthe bloodpressurethebrainappearstobenefitfortheincreased perfusion.

(ix) Diabetesmellitus

DMincreasestheriskofmostdegenerativedementias.Inthe veryold,thereisanincreasedriskofdevelopingdementia withimpairedglucosetoleranceevenintheabsenceoffrank DM.Putativetheoriesincludetheeffectsoflong-termhyperglycaemiacausingneurodegenerationaswellastheeffectsof raisedinsulinlevelsinthebrainforTypeIIDM.

(x) Heartdisease

HeartdiseaseisassociatedwithanincreasedriskofbothVaD andAlzheimer’sespeciallyinthosewithcombinedperipheralvasculardisease.Atrialfibrillationandcongestivecardiac

failurealsocontributetotheriskofdevelopingdementiaand maybeindependentriskfactors.

(xi) Bodymassindex

Duringmid-life,ahighBMIisassociatedwithanincreased riskofdementia.Inlaterlife,arelativelysteadydrop inBMIoverafewyearsmayheraldthebeginningsof dementia.

(xii) Diet

Evidencethatspecificcomponentsofahealthydietaffectingtheriskofdevelopingdementiaislacking.Antioxidant vitaminsA,EandCareunlikelytohavelong-termneuroprotectiveeffects.Thereisarelationshipbetweenvitamin B12,folicacidandhomocysteineinthatthefirsttworeduce theserumlevelsofthelatter.Araisedserumhomocysteine levelisaknownriskfactorforheartandcerebrovascular disease,which,inturn,increasestheriskofdementia.The OPTIMA(OxfordProjecttoInvestigateMemoryandAging) studyfoundthatbloodhomocysteinelevelsweresignificantlyhigher,andbloodfolicacidandvitaminB12levels significantlylower,inpatientswithconfirmedAlzheimer’s disease.

Saturatedfats,ingeneral,areassociatedwithanincreased riskofvasculardisease,raisingthepossibilitythatthey increasetheriskofdementia.Fishpolyunsaturatedfatsmay haveamoredirectrelationshipwithreducingdementiarisk, althoughthestudiestodateareequivocal.

(xiii) Social,leisureandphysicalactivity

Maintaininganactive,enjoyableandmentallystimulating lifestyle,particularlyinmidtolaterlifeisassociatedwith reducingtheriskofdevelopingdementia.

Table2.4 Possibleprimaryprevention.

Controlforvascularriskfactors

Eatahealthyandbalanceddiet

Drinkalcoholinsmallquantities

Keepphysicallyactive

Maintainagoodsocialnetwork

Continuehobbiesandinterestsintolaterlife

Keepenjoyinglife

(xiv) Otherfactors

Hormonereplacementtherapy(HRT)andnon-steroidal anti-inflammatorydrugs(NSAIDs)arenotevidencedas reducingdementiarisk.Traumaticbraininjury,depression andlaterlifedeliriummaybeassociatedwithanincreased riskofdementia.

Primaryprevention

Todate,thereisnoevidencetoshowthatdementiacanbeprevented ordelayed,eveninthebrainsofthoseathigherrisk.Nevertheless, takingintoaccounttheavailableevidenceonriskfactorsfordevelopingdementia,sensibleadviceishighlightedinTable2.4.

CHAPTER3 Assessment

BernardCoopeandFelicityA.Richards

WorcestershireHealthandCareNHSTrust,Worcestershire,UK

• Makingadiagnosisofdementiaisessentiallyaclinicaldecision reachedbyassimilatingthepatient’shistory,cognitive assessmentandimaging.

• Thereismoretoassessmentthanmakingadiagnosis.Whatelse needstobeaddressedtohelpthepersonwithdementia?

• Alwaysconsiderapatient’swishesaroundassessment,including theirwishtoknow,ornotknowthepotentialdiagnosis.

• Cognitivetestingvariesgreatly,andtestingshouldbeguidedby theclinicalhistoryofchange.

• Adiagnosisisnevermorethanthebestexplanationforthe ﬁndings,notabsolutefact.

Introduction

Inthischapter,theassessmentofdementiaisaddressedwiththeuse ofthreequestions:

1 Why?Whatisthepurposeoftheassessment?

2 What exactlyareyouassessing?

3 How doyougoaboutgatheringtheinformationyouneedin ordertomakeadiagnosis?

Why?

Beforecommencinganassessmentwithapersonwithpossible dementia,itisimportanttoconsiderthequestionofwhyyouare doingit.Itisworthstatingtheobvioushere;thereasonweassess anindividualisinthehopethatitwillbehelpfultothatpersonin someway.Thismaybedirectlyhelpful,thatis,clarifyingthediagnosistoapersonwithconcernsaboutmemory.Orbenefitsofan assessmentmaybemoreindirect;itcanbevaluabletoprofessionals orfamilymemberstoknowaboutthediagnosisinordertobetter understandtheconditionormakethebestplansforcare.

Inthepastdecade,societyhasmovedawayfromthebeliefthat adiagnosisofdementiaisunimportant,toonewheremakinga diagnosisisviewedunquestioninglyasagoodthing.Thiscomplete

aboutturnhasledtoconsiderablepressuretoincreasediagnosis ratesasthoughthisisanendinitself.Itisnot.

Weshouldalsobemindfulthatlikeanyhealthcareintervention, dementiaassessmenthasthepotentialtodoharm.Properconsiderationshouldalsobegiventoinformedconsent.Inthecaseofan individuallackingthementalcapacitytoconsent,theassessment mustbeinthatperson’sbestinterests.

What?

Ahealthassessmentmayhavemanydifferentcomponents(see Box3.1).Wemaybeinterestedinfindingoutifthepersonhas aclinicalsyndromeofdementia.Ifso,wemaywantmoredetail, forexample,howsevereisit,orwhatisthelikelydiseaseprocess thatiscausingthedementia?Fromapracticalpointofview,it maybevaluabletounderstandthepatternofcognitivelossesand preservedskills,toaidunderstandingandcareplanning.

Ifwearetohelpapersonlivewellwithdementia,wemaybe moreinterestedinthesourcesofsupport,whothecarersareand howthecarers/familymaybefeeling.Isthereanyriskarisingfrom thedementia?Ifthereareimportantdecisionstobemade,doesthe personhavethecapacitytomakethesedecisions?

Box3.1 Differentreasonstoassessforpresenceofa dementia

Isdementiapresent?

Whatbraindiseaseispresent?

Whatcareneedsarethere? Isthererisk?

Doesthepersonhavethementalcapacitytomakeadecisionatthis time?

Howisthecarerandwhatwouldhelpthem?

How?

Theexactnatureoftheassessmentwillbedeterminedbyitspurpose.Iftheaimistomakeadiagnosisofdementiaandalsoto clarifytheunderlyingbraindisease,theassessmentisessentially athree-piecejigsawpuzzleconsistingofaclinicalhistory,cognitivetestingandimaging.Ofthese,thehistoryisbyfarthemost

important,withcognitivetestingandimagingprovidinguseful supportiveinformation(Box3.2).

Box3.2 Theassessmentofdementia–Athree-piecejigsaw

1 Clinicalhistory

2 Cognitivetesting

3 Imaging

Thestoryofchange

Agooddescriptionofchangeinmentalskillsiswhereassessment starts(seeBox3.3).Theextenttowhichthepersoncancontribute totheassessmentprocesswilldependontheseverityortypeof dementia.Mostpeoplewithmildormoderatedementiawillbeable togivesomesubjectivedescriptionofhowtheyare.

Contrarytoconventionalteaching,mostpeoplewithmild dementiaareveryawareofchangeinmentalskills.Itisalso extremelyimportanttogatherthisdescriptionofchangefrom atleastoneotherpersonwhoknowsthemwell.Thiscanbean uncomfortablesituation,butitisusuallybesttoholdtheinterview togetherratherthantotalksecretively.Quitecommonly,aninformantwillbemoreconcernedaboutmentalskillsthanthepatient. Sometimesapersonwillseekhelpalone,sayingthattheydon’t wanttoworrytheirfamily,andinthesecasesdementiaislesslikely tobepresent.

Box3.3 Pointsofdiagnosticvalue

Durationandchangeovertimethatmaysuggestlikely pathologicalprocess

Suddenonset:suggestsavascularaetiology

Gradualchangeoverafewyears:suggestsdegenerativedisease

Progressivechangeoverdays,weeksormonths:veryconcerning,may indicatespace-occupyinglesion,subdural,delirium,andsoon Past20years/allmylife:unlikelytobedegenerativeconditionsuchas dementia

Whathaschanged?

Memory

Language

Visuospatialskills

Judgementandpersonality

Whathaschanged?

Memory

‘Mymemoryisdreadful,doctor’isnotenough.Thisisacommon subjectiveconcernandcanaccompanydepression,lowself-esteem orafearofgettingdementia.Mostchangesinmemoryleadto impairmentinstoringnewmemories,sometimesinaccurately describedas‘short-termmemory’.Poorrecallofoldmemoriescan alsooccur,ascanalossofgeneralknowledge.

Askforspecificexamplessuchasforgettingconversationsor recentevents.Repetitivenessusuallyaccompaniesforgetting, althoughitmustbedistinguishedfromtherepetitivenessthatmay accompanydepression,anxietyorobsessionalthinking.Memory deficitswillnearlyalwaysbemoreobvioustotheinformant.

Language

Changesinlanguageskillsarecommonandimportanttodetect. Insomedementiasyndromes,theymaybethemajorpresenting featureandcancausegreatdistress.Disturbanceinlanguageskills indicatesdysfunctionofthedominant(usuallyleft)hemisphere. Changesinlanguagemaybesubtle.Problemswithfindingwords (anomia)maybemistakenlydescribedas‘forgetting’words.Itmay leadtobreakswhenspeakingortheincreasinguseofparaphasias (‘thingummy’)orcircumlocutions(‘thingthattellsyouthetime’for clock).Sometimestheremaybeworderrors,thewrongwordslippingout,eitherasimilarsoundingword‘parcark’for‘carpark’ora wordfromasimilarsemanticcategory.Otherimportantlanguage skillchangesincludechangesinspelling,grammarorarticulation ofpolysyllabicwords(speechapraxia).

Visuospatialskills

Changesinthebrain’sabilitytoprocessvisualinformationmayhave abigimpactondailylivingskills,forexample,puttingonclothes, navigatinginfamiliarorunfamiliarplaces(topographicalmemory), puttingthingsintherightplaceorrecognisingorusingfamiliar objects.

Theseskillscaninvolvethe‘what’ofvisualinformation,leadingtorecognitionerrors,orthe‘where’,leadingtodifficultiesin locatingthings,forexample,reachingpastobjects.Thesechanges suggestdysfunctionofparietaloroccipitallobes.Problemsrecognisingfaces(prosopagnosia)isassociatedwiththeinferioroccipital lobeandfusiformgyrusandcanoccurindependentlyofvisuospatialdeficitsforotherobjects.

Inthehistory,visuospatialdifficultieswillsometimesbetalked aboutasamemoryproblem,thatis,‘myfather’sforgottenhowto usethecooker’‘mysistercan’trememberwherethebathroomis’, soitisimportanttoelucidatetheexactnatureofthesecomplaints.

Judgementandpersonality

Behaviourthatappearsoutofcharactercansometimesbeassociatedwithorganicbraindisease,althoughitcanalsooccurformany otherreasons.Itisassociatedwithpathologyinthefrontallobes. Lossofempathyisacorefeature,withfamilymembersdescribing alossofregardforthefeelingsofothers.

Behaviourmaybecomemoresociallyinappropriate,sometimes includingoffensiveorillegalactivitiessuchasshopliftingorsexualassault.Importantly,suchactsareimpulsiveanddon’tshowany advancedplanning.Despitethesechanges,apathyanddiminished motivationcommonlyaccompanytheclinicalpicture,whichcan helpdifferentiatethestatefromhypomania.

Testingcognitiveability

Measurementofcognitiveskillsisanessentialcomplementtothe history.Formaltestingmustbeguidedbytheinformationgathered

inthehistory:bothchangesinmentalskillsandanyotherfactor(s) thatcouldconfoundthetestscore(seeBox3.4).

Box3.4 Factorsthataltercognitivetestscores

Highpremorbidintellect

Loweducationalattainment,truanting,poorliteracy

LowIQ,learningdisability

Poorvisionorhearing

Notcarryingouttestinﬁrstlanguage

Motivation/cooperation/mood

Culturaldifferences

Cognitivetestsvarydependingonthepurposeandalsoonthe severityofdementia.Pickinguppossiblesignsofdementiato guidereferralorhospitalcarecanbeachievedwithashorttest suchasAbbreviatedMentalTestScore(AMTS),GeneralPractitionerAssessmentofCognition(GPCOG),or6-ItemCognitive ImpairmentTest(6CIT).Themoreadvancedthedementia,the moreobviousthechangesincognitiveskillstendtobe;therefore, confirmingthepresenceofseveredementiamayonlyrequiresome brieftestsofmemoryandorientation.

Toconfidentlydiagnoseearlydementiawillrequiremore detailedtesting.Sometimesitmaybeimportanttomeasurethe severityofdementiaforstaging,measuringtreatmentresponseor research.Forspecialistmemoryassessmentservices,theAddenbrookesCognitiveExamination(ACE)iscommonlyused(see Box3.5).Further,moredetailedneuropsychometrictestingmay berequired.

Cognitivetestingneedstobecarriedoutwithskillandcare.The sameconsiderationneedstobegivenaswouldbegivenforaphysicalexamination,inotherwords,explanationofthetestandwhyit isbeingdone,privacyandconsent.Thepatientwillusuallyneedto beseatedatatable,withnobackgroundnoiseandreadingglasses orhearingaidsavailable.Thepresenceoffamilymaybereassuring ordistractingandembarrassing.Askpatientshowtheyfeelabout othersbeingpresent.

Box3.5 Cognitivetests

Shorttestsforbriefscreeningintheacutesettingorprimary care

AMTscore.A10-pointtestthatcanidentifysevereimpairmentof cognitiveskillsasfoundinamoderate/severedementiaordelirium. MiniMentalStateExamination(MMSE).Widelyusedtest,30points. Uselimitedbyauthorsretainingcopyright.

TestYourMemory(TYM).Ashort,self-administeredtestdeveloped forscreening.

TheMontrealCognitiveAssessment(MOCA).A30-pointtestsensitivetomildcognitivechangesandcoveringarangeofcognitive functions.

GeneralPractitionertestofcognitivefunction(GPCOG).Abrief testdevelopedforuseinprimarycare.Relativelyunaffectedby educationalandculturalbackground.Includesatestfortheperson withconcernsandacarerquestionnaire.

6CIT.Abrieftestthattakes5mintoadminister;validatedinprimary care.

Longertestformemoryclinic/diagnosis

AddenbrookesCognitiveAssessment(ACE).A100-pointtest,takingapproximately20mintocomplete.Providesanoverallscoreand subscoresofdifferentcognitivedomains.Includessectionstodetect non-Alzheimer’sdementia.

Examplesofdetailedcognitivetestsforspecialistuseor research.

Intelligence((VerbalandPerformance):WAIS-RWechslerAdultIntelligenceScale

RepeatableBatteryfortheAssessmentofNeuropsychologicalStatus (RBANS)

Imaging

Wherecognitivetestinggivesanindicationofbrainfunction,brain imaginggivesanindicationofitsappearance.Imagingiscurrently recommendedasonecomponentofdiagnosticassessment.Itis importanttorecognisethelimitationofimaging,andforboth cliniciansandpatientsnottooveremphasisetheimportanceof imaginginmakingadementiadiagnosis.Rememberthatwith advancingage,brainscansappearincreasinglyabnormal.Atrophy andsignsofvasculardiseasearealmostinvariableinapersonover 70,regardlessofcognitivefunction.

–Istheresomethingthatshouldn’tbethere?

Subduralhaematomas,tumoursornormalpressurehydrocephaluscanbeidentifiedonstructuralimaging. –Isthebraindifferentfromexpected?

Localisedatrophymaysupportadiagnosis,especiallyifit matchesfindingsfromhistoryandtesting.Thiscanbe valuableinAlzheimer’sdisease,frontotemporaldementia syndromesorposteriorcorticalatrophy,especiallywhen investigatingyoungerpeoplewhereatrophywouldbeunexpected.Vascularchangescanalsobevisualised,althoughthe presenceofvasculardiseasedoesnotmeanthatthisisthe onlypathology,anddoesnotinitselfdiagnosedementia. Likewise,ascancanbenormalinapatientwithAlzheimer’s disease.

Box3.6 Listofcommonlyusedimagingtechniques

Computerisedtomography(CT)

Quick,availableandwelltoleratedbythemajorityofpatients.CTis suitableforexcludingspace-occupyinglesions.

Magneticresonanceimaging(MRI)

Thistestisnoisy,slowandconﬁned,andthereforecanbeanuncomfortableforsomeolderorimpairedpatients.Imagesshowstructures

inhigherresolution,detectvascularchangeswellandallowsvisualisationfromdifferentviews.Coronalimagesmayallowhippocampal atrophyinAlzheimer’sdiseasetobeseen.

Functionalimaging

Thisisnotcurrentlyrecommendedforroutineclinicaluse,althoughit hasvalueinresearchandmaybecomemorecommonlyusedaspart ofdiagnosticinvestigation.

Thediagnosis

Makingadiagnosisisessentiallyaclinicaldecisionreachedby assimilatingthepatient’shistory,cognitiveassessmentandimaging.Themorethesecomponentsmarryup,themoreconfidence thecliniciancanfeelaboutthediagnosis.

Itcanbehelpfultothinkofthediagnosisintwostages:

1 Isthereevidenceofthedementiasyndrome?

2 Whatisthelikelydiseaseprocesscausingthedementia?

Itisalsoimportanttorememberthatadiagnosisisnevermore thanthebestexplanationforthefindings,notabsolutefact.

Finally,aswithmostareasofmedicine,itiseasiertosaythat enoughevidencehasbeenfoundtomakeadiagnosisthanitistosay thatthereisnothingwrongwithapatient.Patientslookingforreassurancemayfindthisinabilitytoconfirmtheabsenceofdementia challenging.

Box3.7 Examplesofcommonpresentations

ExampleOne

A2-yearhistoryofprogressivechangeinmemory,anACEscoreof 70withpoormemorysubscoresandimpairedverbalﬂuency,together withascanthatshowsonlytemporalatrophystronglysuggestsamild dementiacausedbyAlzheimer’sdisease

ExampleTwo

A5-yearhistoryofsubtlechangesinpersonality,withlabilityof mood,emerginglackofempathy,lapsesinjudgementandrelatively well-preservedmemorywithsomesemanticloss.AnACEscoreof 60withimpairedﬂuency,lossofsemanticmemory,perseveration notedontasksthroughout,lackinginsightintolossofskillsstrongly suggestsafrontotemporaldementia(FTD).

ExampleThree

An18-monthhistoryofprogressivecognitivedecline,ﬂuctuantin nature,accompaniedbyvividvisualhallucinations,andagnosias.A 10-yearhistoryofdisruptioninsleeparchitectureandrapideyemovement(REM)sleepdisturbance,recentchangesingait,andfalls.CT headscanshowsnormalage-relatedchange.ACEscore78,losing pointsonmemorytasksandvisuospatialtasks,withpreservationof orientationintimeandplace,ﬂuencyandlanguage.SuggestsadiagnosisofLewybodydementia.

EarlyInterventionforDementia

BernardCoopeandTanyaJacobs

WorcestershireHealthandCareNHSTrust,Worcestershire,UK

OVERVIEW

• TheaimofanEarlyInterventionDementiaServiceistohelp thosewithdementialivewellnow,andintothefuture.

• Thefocusofthisserviceishelpinganindividualadapttothe diagnosisandtoworkwithfamiliestobuildconﬁdence, resilienceandskillstofacethefuture.

• ‘Intervention’isthewholeprocessofprofessionalengagement, notjustsomethingthathappensafteradiagnosis.

• Makingadiagnosisisonesmallpart.Sharingitwellneedsgood communicationskillsandtherightsetting.

• Specialistsmustworkwithpartnerswhowillprovidesupport andadvice,andcommunicatewellwithprimarycare.

Introduction

Society’sviewsofdementiaarechangingandwiththistheexpectationsandwishesofourpatientsarechangingtoo,challengingcliniciansandservicestoadapt.Alargeproportionofpeopleexperiencingtheearlysignsofdementiaarenowwishingtoknowiftheyare developingthecondition.Previously,theprevailingviewinsociety wastorefrainfromdiscussingdementia–aviewsharedbymost clinicians.Aself-perpetuatingcycleemerged.Lackoftrainingon dementiaforhealthprofessionalsledtothesensethatdementiawas notahealthcarepriority,resultinginlittlerecognitionoftheclinicalneedsofpatients.Twocommonbeliefshaveledtotheillusion thatdoingnothingisbest.

• Peopledevelopingdementiaareunawareoftheirimpairments andarehappyintheirignorance.

• Thereisnothingthatcanbedoneifadiagnosisismadeearly,as dementiaisanincurablecondition.

Anyonewhohasspenttimewiththosedevelopingdementiawill knowthatthefirstoftheseissimplyuntrue.Themajorityofthose withearlydementiaareonlytooawareofthechangesthataretaking place,asaretheirfamilies.Thispainfulrealisationleadstothepsychologicaldefenseofdenial,ratherthananorganiclackofawareness.

Thereisnowasteadydemandfromthosewithearlychangesin memoryandothermentalskillsforanearlyassessmentanddiagnosis.Thisleadstothesecondpoint;whatcanbedonetohelpthose diagnosedearly?

Exploringtheconceptofearly interventionindementia

Whatis‘earlyintervention’?

Theconceptofearlyinterventionstartedinservicesforyoung people,whereprovidingacomprehensiveinterventionearlyin developmentaimedtosupportamoreadaptivefuture.Inmental health,thisconcepthasdevelopedmostlyasearlyinterventionfor psychosis.Here,supportinganearlydiagnosiswithappropriate treatment,adviceandfamilysupportcanhelpapersonwhois developingmentalillnessmaintainthedirectionoftheirlife,stay ineducationoremploymentandmaintainimportantrelationships withfamilyandfriends.Animportantpartoftheconceptisthat althoughearlyinterventionmayincludemedicaltreatment,it ismuchmorethanthisandconsistsofusingabroadrangeof psychosocialinterventionstohelpsupportthatpersontolivewell.

The‘MemoryClinic’

Asdemandforearlydiagnosisfordementiastartedtogrow,partly drivenbythelaunchofacetylcholinesteraseinhibitortherapy,the commonserviceresponsewasthe‘MemoryClinic’.Therearemany differentmodels,allwiththesamefocus–toprovideatechnically expertdiagnosis,sometimesbyamultidisciplinaryteam,thenmedicaltreatmentifappropriate.

Whilethismodelmayprovideanearlydiagnosis,patientssometimesreportfeelingunpreparedfortheassessmentprocess,with consentsometimesassumed,andalackofsupportaftertheassessmenthighlighted–afterthediagnosiswasmade,whatthen?Should thepatientbetold?Shouldtherelativesbetoldthisconfidential information?AndinthewordsofTerryPratchett,‘Afteradiagnosis peopleneedtobeshownthepath,notshownthedoor’.

Earlyinterventionindementia

Theprincipalaimofearlyinterventionindementiaistohelpthose affectedbyearlydementialiveaswellaspossible,bothnowandinto

thefuture.Animportantconsequenceofthisbroaddefinitionisthat ‘intervention’isnotsomethingdonetopeopleafterthediagnosis. Ratheritisthecombinedimpactofcontactwithservices,before, duringandaftertheassessmentprocess.

Anotherconsequenceisthat‘thoseaffectedbyearlydementia’ mayincludethoseexperiencingtheconditionandthoseclosestto them.Experiencehasshownthatseparatingtheneedsof‘patients’ and‘carers’atthispointisunhelpfulandadistinctionthatahusband andwife,ormothersupportedbyherchildren,wouldnotrecognise.

TheEarlyinterventionServiceforDementiainCroydonbrokenew groundindevelopingtheservicemodelandprovidingevidencefor itseffectiveness.

Here,theEarlyinterventionDementiaServiceinWorcestershire isdescribedasanexample.

Thenameoftheservice:mentioningthe‘D’word

TheEarlyInterventionDementiaServiceinWorcestershirewas developedfollowingwideconsultationwithpeoplewithdementia, theirfamilies,andpartnerorganisationssuchastheAlzheimer’s

Society.Animportantprinciplewastohelppeopleusetheword ‘dementia’withoutbeingoverwhelmedbyit.Thecomparisonwith thechanginguseoftheword‘cancer’wasafrequentlyquoted example.Thisviewcouldbesummarisedinthecommentmadeby onepersonlivingwithdementia;

Howdoyouexpectustofeelcomfortabletalkingaboutdementiaifyoucan’t?

Thechoicetoopenlytitletheservicea‘dementia’servicecame fromthis,andconsequentlystartsthepatientonthelongpathof adjustment,startingfromthepointofreferral.

ThepathwaythroughtheEarlyInterventionDementiaServiceis summarisedinBox4.1:

Awordonconsent

Investigatingpossibledementiaissimilartootherareasofmedicine. Ithasthepotentialtobeofgreatvalue,butneedstobeundertaken eitherwithapatient’sconsent,orifunabletoconsent,carriedoutin thatperson’sbestinterests–withskill,withproperaftercareand, aboveall,withaclearintenttobeofhelptothepatient.

Box4.1 ThejourneythroughtheEarlyInterventionDementiaService–TheWorcestershireModel Intervention

Beforethereferral

–Raisingpublicawarenessoftheservicebyarticlesinthepress,localradio,postersandﬂiers.

–Liaisingwiththird-sectororganisations,e.g.leavingleafletsinAgeUKandAlzheimer’sSocietyoffices,meetingwith WellCheckOfficers(AgeUK)encouragingthemtopromptpeopletovisittheirGPwithconcernsaboutcognition.

–Focusonhard-to-reachgroups.Awarenessraisingwithreferrersincludingface-to-facemeetingsandsendingout postcard-sizedreferralcriteriacards.

Afterreferral-TheInitial Appointment.

–Allreferralsscreened.Ifaccepted,thepersonisofferedaninitialappointmentathome.Appointmentisgenerally conductedawayfromaclinicalsetting,withthefocusonpromotingchoiceandcontrol.Anindividualmayfeelmore empoweredtohavethisappointmentinanenvironmentthatisfamiliarandcomfortable.Theallocatedpractitioner (normallyanurse)undertakesthisvisitandremainsaconstantthroughouttheperson’sengagementwiththeservice.

–Thenatureofassessmentisdiscussedandthepossibilityofadementiadiagnosisisopenlyexplored.Itcanbeuseful totalkaboutthemeaningoftheterm‘dementia’,itssymptomsandcausesatthispoint,especiallyifpeoplehave preconceivedideasorfearsaboutthecondition.Itisalsoanopportunitytopointoutthepossibleadvantagesofan earlydiagnosisandpotentialimplications.

–Choiceandconsentgathered;tohavetheassessment,whethertoheartheoutcome,orconsenttoshareinformation withothers.ExperienceinWorcestershireisthat97%expressthewishtobetoldthediagnosisinthepresenceof theirfamily.

–Forthosewhochoosenottoproceed,theyareinformedverballyabouttheprocessforre-referralandaletterissent remindingthemofthisandtheissuesdiscussedduringtheinitialappointment.

Assessment

–SeeChapter3.Usuallyinaclinicsetting.

–Patientsandfamiliesknowwhattoexpect.Ahistoryandexaminationofcognitiveskillscarriedoutbyadoctoranda nurse.Brainimagingusuallyarranged.Furtherdetailedneuropsychologicalassessment,ifindicated.Opportunityfor families/carerstospeakseparatelyifnecessary.

Discussionofresults

Postdiagnostic intervention

–‘Honestandopen’discussionofresults,usuallywithfamilyinaccordancewithexpressedconsentofpatient.Inan outpatientsetting,orathomeifthepatientwishes.Enoughdetailtoclarifythereasonandnatureofthediagnosis, forexample,showingapersontheirbrainimaging.Medicationinitiatedifappropriate.Conversationsummarisedin personallettertothepatient.

–Coordinatedandmostlyprovidedbythesamenursewhoconductedtheinitialassessment.Theaimistoenhance personaladaptationandfamilyresilience.

LiasingwithPrimary Care

Intervention

–Emotionalsupportforpersondiagnosedandfamily.Explorationofthediagnosisbothverballyandwithwritten information.Supportalongwithadjustingtothediagnosiscanvaryanddifferconsiderablybetweenpatientsand relatives.ConsiderintroductionofAdmiralNurses(Box4.2)and/orteampsychologistformoreintensesupport.

–Adviceandinformation,supportedbydementiaadvisorandpartnerssuchasAlzheimer’sSociety.Individualised, especiallyinnon-Alzheimer’sdiseasedementiaandyoungerpeople.

–Supportforworkingagedementia.Addressingsocialissuesincludingemploymentorapplicationsforrelevant beneﬁts.Addressingtheneedsofchildren–liaisingwithschoolsandchildren’sservices.Providinginformationabout theworkingageDementiaCafé.Linkstoongoingsourcesofpeersupport,forexample,DementiaCafé(Box4.2)and dementiaInternetforums.

–Carers–referralstoSocialServicesforacarers’assessment.Thiscanincludecontingencyplanningintheeventof carerabsenceandexplorationofrespiteopportunities.Linkingcarerswithvoluntarysupportagencieswhocan providefurtheremotionalandpracticalsupportandaccesstoeducationalsessions.Makinglinkswithdementia supportgroups.

–Identifysocialneeds–referralstosocialservicesforcarepackages.

–Financial,legalandbenefitadvice–forexample,discussionaboutsimplifyingfinancessettingupdirectdebits. InformationaboutLastingPowerofAttorney(LPA)andhowtoapplyforthis.SupportwithapplyingforAttendance Allowance–requestingbenefitscheckfromAgeUK.

–Developmentofskillsforcontinuingtolivewell,supportedbyOccupationalTherapistandsupportworker.Functional assessmenttoestablishlevelofabilityanddevelopmentofstrategiestoaiddailyfunctionandindependencee.g. assistivetechnology.Providedindividuallyorat‘LivingwellwithDementia’days(seeBox4.2).

–Thinkingaboutthefuture.Encouragingconsiderationofwishesforthefuture-viainformaldiscussionsorformalised withLPAsandAdvanceStatements(SeeChapter12)

Drivingadvice.MostpeoplewithearlydementiastillabletodrivesafelybutdiagnosismustbecommunicatedtotheDrivingandVehicleLicensingAgencyDVLA.SeeChapter12.Arrangeanincardrivingassessmentifconcernsareidentiﬁed. Explorealternativemodesoftransportandsupportwithconﬁdencetoaccesspublictransport.

–Promotionofhealthlifestyle–identifypossibleopportunitiestoimprovephysical,mentalandsocialactivity.Liaising withprimarycareregardingpossibleimpactofimpairedcognitiononphysicalhealthtocoordinatejointmanagement plans,forexample,diabeticservices,dieticiansandpharmacies.

–Dentaladvice.Dentalcarecanbehardtoprovideinmoreseveredementiaanddentalpaincanbehardtodetectand treat.Gooddentalhealthatthispointmaypreventfutureproblems.

–Clearcommunicationregardingdiagnosisandinterventionwithprimarycare.

–Reviewofeffectivenessofacetylcholinesteraseinhibitorsafterthreemonths.

DischargefromEarly InterventionTeam

Mostpeopledischargedafterapproximatelythreemonths.Personallettersenttothepatient.LettertoGPsuggesting follow-upplanandreferralrouteifspecialistcareisneededinthefuture.

Ifthepersoncangiveconsent,thenitisimportantnotonlytodiscusstheprocessofassessmentbutalsowhatthepossibleoutcome mightbe.Thisalsogivesanopportunitytofindoutifthepatient wishestobeinformedoftheassessment’soutcome,thatis,thepossibilityofreceivingadiagnosisofdementia.Ifapersonlacksthe capacity,thenassessmentmustbylawbeintheirbestinterestsand thishastobedemonstrated.Theassumptionmustbethataperson iscapabletogiveinformedconsent,unlessthereisevidencetothe contrary.

TheEvolvingServiceModel

Ifthequestionis‘Whatcanbedonetohelpthispersonwithdementiaandthosearoundliveaswellaspossible?’thentheremaybe morethanoneanswer.TheEarlyInterventionforDementiamodel willcontinuetoimproveandchange.Itwillneedtoadapttogrowingdemandwithinthecontextoffinancialconstraint,shapedby

theneedtobebothofhighqualityandcost-effective.Somelikely themesforfuturedebatesarelistedbelow.

(i) Preventionanddiseasemodification

Thereisepidemiologicalandpathologicalevidenceofpotentiallymodifiableriskfactorsfordementia.Theseincludevascularriskfactors,diet,physicalactivityanddepression.Some cliniciansarealreadyproposingthatinterventionstargetedat earlydiagnosis(e.g.cognitivebehaviouralstrategiesaimedat preventingfuturedepression)willalterthefuturecourseof dementiainacost-effectiveway,althoughthereislittlecurrentevidencetosupportthis.Inasimilarway,medications areoftenreportedasbeingdiseasemodifying,‘stavingoffthe conditionforyears’,ratherthancorrectlyoutliningthelimited efficacyandonlymodestsymptomaticbenefitastheevidence suggests.Futuretechnologydevelopmentsandagrowingevidencebasewillhopefullyleadtochangesinthisarea.

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