derek ott, m.d., m.s. associate clinical professor ucla ... behavior help 2… · long...
TRANSCRIPT
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DerekOtt,M.D.,M.S.AssociateClinicalProfessor
UCLADavidGeffenSchoolofMedicineDivisionofChild&AdolescentPsychiatryDirector,PediatricNeuropsychiatryClinic
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Whatisbehavior?� Chronic/learned/conditionedresponse� Adaptationtonewenvironmentorcircumstance� Symptomsofapsychiatricissue� Medicationsideeffects� Symptomsofmedicalissues
� UltimatelyBehaviorIScommunication
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Whatisbehavior?� Chronic/learned/conditionedresponse
� Chronicself-injuriousbehavior(SIB)assensoryseeking/soothing� Adaptationtonewenvironmentorcircumstance
� NewonsetSIBasacopingmechanism� Symptomsofapsychiatricissue
� NewonsetSIBasanxietydisorder/OCD� Medicationsideeffects
� NewonsetSIBrelatedtoconfusion,cognitiveissues,etc.� Symptomsofmedicalissues
� NewonsetSIBrelatedtogastrointestinal/painissues
� UltimatelyBehavioriscommunication
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Maladap2vebehaviorinau2sm� Asmanyas40%ofindividualswithdevelopmentaldisabilitymayexperienceaperiodofdisturbedbehavior/functionatsomepointintheirlives
� Suddenchangeinbehavior/functioningshouldpromptmedicalorclinicalevaluationtoidentifiedanytreatablemedicalcauses
� Needtounderstandpriorbaselinebehavior� Needtocoordinatewithotherproviderssuchasneurologist,internist,pediatrician,behaviorist,therapist,etc.
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Maladap2vebehaviors-common(orReasonforPsychiatricReferral)
� Aggression� Moodlability/irritability� Lowfrustrationtolerance/tantrums/propertydestruction� Noncompliance/oppositionalbehavior� Impulsive/Hyperactivity� Sleepdisturbance� Regressioninfunctioning� Elopement
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Maladap2vebehaviors-lessfrequent� Primarilypresentinindividualswhoarenonverbalorhavemoresevereintellectualdisability
� Stereotypies� Repetitiveorritualisticmovements,postureorutterance
� Selfinjuriousbehavior(SIB)� Veryintense/repetitive
� Fecalsmearing� Pica
� Swallowingofnonfooditems� Rumination
� Regurgitationoffoodfollowingconsumption.
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E2ologyofbehaviorinau2sm� Chronicbehaviors
� Stereotypies/SIB� Adaptivedysfunction� Psychiatricdisorders
� Medicationissues� Medicalissues
� Pain� G.I.� Neurologic� Sleep
✳ Oftenmultiplecauses/triggers
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Stereotypicalbehavior� Primarilypresentinindividualswhoarenonverbalorhavemoresevereintellectualdisability
� Stereotypies� Repetitiveorritualisticmovements,postureorutterance� Typicallychronicbutcanchangeovertime� Frequency,intensity,circumstances,etc.
� Selfinjuriousbehavior(SIB)� Consideredstereotypicalbehavioraswell� Canvaryfrommild/lowfrequency(“habitual”)toveryintense/repetitive
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Stereotypicalbehavior/SIB� Needtodeterminewhatisbaselinestereotypicalbehavior� Chronicvsnew/acute� Distinguishfromtics,dyskinesia(abnormalmovements),dystonia(musclestiffness),otherabnormalmovements
� Someofthesecouldbemedicationsideeffects� Antipsychotics-risperidone,Abilify,etc.� Antinausea/G.I.drugs-Reglan
� Indicativeofmedicalissues� Gastrointestinal-reflux,constipation,gastritis,etc.� Pain-dental,ear,etc.� PANDAS/PANS
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Adap2vedysfunc2on� Mismatchbetweenneeds,abilities,goalsofindividualandenvironment/circumstances/expectations� Home� Residence� School� Dayprogram
� Changeinparents/careproviders� Changeinhealth-illness/pregnancy/death� Changeinenvironment� Changeinpeople-staff,students,residents� Changeinschedule-home,school,dayprogram,residence
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“Diagnos2covershadowing”� =tendencytoassesscomorbidpsychopathologyinpersonswithintellectualdisabilitylessaccuratelythanpersonswithout(Rice,Leviton+Szyszko(1982))
� Assumethatcognitivedeficitsnegativelyimpactclinicianjudgmentsaboutpsychopathology
� Mayimpact� Severity-howseverethesymptomsare?� Category/diagnosis-whatdiagnosisthepersonhas� Treatment-howthedisordershouldbetreated
� Jopp,Keys,diagnosticovershadowingreviewedandreconsidered,AmJMR,2001
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DevelopmentalDisability+Psychopathology
� PsychopathologyinpersonswithIDis3-4xhighervsgenpop
� 35-40%ofchildren/adolescentswithID-diagnosablepsychiatricdisorder
� 30-42%vs7%-IsleofWightstudyRutter,etal.,IsleofWightstudies1964-1974,PsychMed,1976
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Medicalcausesofchallengingbehaviors� Medicationissues
� Druginteractions� Sideeffects� GenericsvsBrand
� Medicalissues� Cardiac-bradycardia� GIrefluxpicaconstipation� Pain-dentalear� Hormonal� Sleep-apnea� Neurologic-HA,seizures
� Oftenmultiplecauses
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Medica2onEffects� Druginteractions� SideEffects� GenericsvsBranded
� Multiplemedications� Confoundedby
� Multipleproviders� Current+historicalinformationoftenlimited� Medicationnoncompliance
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Medica2on–Druginterac2ons� Anticonvulsants
� Somecaninducemetabolismviaimpactonliverenzymefunction� Carbamazepine/Tegretol,valproicacid/Depakote,phenobarbital
� Asaconsequence,theeffectivedoseofotherdrugisdecreased� Thusmayrequirehigherdosesinthepresenceoftheseanticonvulsants
� Antidepressants� Inhibitmetabolismviaimpactonliverenzymes(cytochromes)
� Fluoxetine/Prozac,paroxetine/Paxil� Asaconsequencetheeffectivedoseofanotherdrugcanbeincreased
� Risperidoneinthepresenceofeitherdrugcouldbeeffectivelyincreasedtwofold
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Medica2onSideEffects-an2convulsants� Phenobarbital
� Notcommonlyused� Attention,otheraspectsofcognition,hyperactivity,depression
� Topiramate/Topomax� Memoryissues,wordfindingdifficulties
� Gabapentin/Neurontin� Typicallyverywelltoleratedespeciallyinadults� Psychosisespeciallyinyoungerindividuals
� Leviteracetam/Keppra� Widelyusedbecauseoflackofdruginteractions� Well-establishedmoodsymptoms-20%� Irritability,agitation,aggressionanddepression� MaybenefitfromtreatmentwithvitaminB12(50-100mg)
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Medica2onSideEffects-benzodiazepines� Drowsiness� Confusion/mentalclouding� Memoryproblems
� especiallyifolder+long-termuse� Disinhibition?
� Appearasifintoxicated� Morelikelyinindividualswithdevelopmentalissues?
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Medica2onSideEffects-benzodiazepines� Longacting/half-life-Clonazepam(Klonopin)
� Accumulate>drowsiness&mentalclouding+confusion
� Short-acting/halflife-Alprazolam(Xanax)� Interdosereboundsymptoms
� Worseningofanxietypriortoscheduleddoses>higherdoses>dependence/tolerance
� Riskofseizuresinthepresenceofabruptdiscontinuation
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Medica2onSideEffects-an2psycho2cs� Risperidone/Risperdal,aripiprazole/Abilify
� Parkinsonism/akathisia(restlessness)� Confusedwithworseningagitation� Canleadtocounterproductiveincreaseindose
� Alertness/mentalperformance� Somehavemorenegativecognitiveimpact-haloperidol
� Precipitousreductionindosage� >agitation,behavioraldeterioration� >worseningabnormalinvoluntarymovements(transientwithdrawaldyskinesias)
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AtypicalAn2psycho2c-SideEffects� Weightgain
� Canbesubstantial20-40pounds� Createsnewissues
� ↑Glucoselevels� Newonsetdiabetes
� ↑Lipidlevels� ↑Prolactinlevels
� Gynecomastia(breastgrowth)
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GenericvsBrandedMedica2ons� Branded+genericmedicationsdoindeedcontaintheexactsameactiveingredientorDRUG� GenericsareBIOEQUILIVENT
� Branded+genericmedicationsmaydifferin2significantways� AsestablishedbytheFDA,genericproductsareconsideredbioequivalentif
theycontain80-125%doseoftheactivecompound� Genericmedicationsusuallycontainlessratherthanmore
� Brandstypicallyvarybyonly3-5%ofthedose� Packing,fillers,colorsmaydiffersignificantly
� Thesedifferencesmayimpactefficacyandsideeffects� Canimpactdrugbreakdown,absorption,bloodlevelrise,etc.� Therefore,notallgenericsareconsideredtohavethesame
BIOAVAILABILITY
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GenericvsBrandedMedica2onsConcertaProblem
� BRAND� OriginallymanufacturedbyOrthoMcNeilJanssen� Veryspecificreleasemechanism(OROS)responsiblefortheextendedrelease
� Specificallydesignedandpatentedbarrelshapedcapsule� GENERICS
� WhenConcertawentgenericin2014,severalgenericalternativesemergedincludingWatson(purchasedbyActavis)andseveralothers
� Theseareverydifferentintermsofsize,shape,designandultimatelyrelease.
� ManyoftheseareNOTOROSdesign
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GenericvsBrandedMedica2onsConcertaProblem� Concertaproblems� OriginallymanufacturedbyOrthoMcNeilJanssen+subsequentlybyWatson/Actavis
� VersionsbyMalinckrodt+Kudco
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GenericvsBrandedMedica2onsConcertaProblem
� Concerta/methylphenidateERwasdesignedtoreleasethedrugoveraperiodof10-12hours
� AnalysisbytheFDArevealedthatMallinckrodtandKudcoproductstheydeliveredthedrugataslowerrateof7-12hours
� Asaresult,theFDAchangethetherapeuticequivalenceratingfortheseproductsfromABtoBX� TheseproductsarestillapprovedandcanbeprescribedthatarenolongerrecommendedasautomaticsubstitutionthatthepharmacyforConcerta
� FDArequestedthatthesemanufacturersconfirmthebioequivalenceoftheirproductsorvoluntarilywithdrawthemfromthemarket
� http://www.fda.gov/Drugs/DrugSafety/ucm422568.htm
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Medicalcausesofchallengingbehaviors� Medication
� Druginteractions� Sideeffects� GenericsvsBrand
� Medicalissues� General� Gastrointestinal� Pain� Hormonal� Sleep� Neurological
� Oftenmultiplecauses
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MedicalEvalua2onofPersonswithMRreferredforPsychiatricAssessment(Ryan,Sunada,1997)
� InterdisciplinaryteamevaluationclinicspecificallyforthosewithIDandbehavioralchallenges
� Consecutivesampleof1135adults� aveage32.9yrs� ID(moderatetosevere)� 50%nonverbal� 46%nonpsychiatricmeds
� Evaluatedby2-stepprocess(physicalexam+screeninglabsfollowedbyselectivetesting)
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MedicalEvalua2onofPersonswithMRreferredforPsychiatricAssessment(Ryan,Sunada,1997)
Condition % of cases Epilepsy (untreated or undiagnosed) 45.8 Hypothyroidism 12.7 Tourette’s syndrome 11.5 Gastroesophageal reflux 9.7 Severe closed head trauma 8.8 Chronic pain 8.7 Cerebral palsy (complicated) 6.5 Open brain injury 6.3 Abnormal (spike-wave) EEG 5.4 Arthritis (autoimmune) 5.0 Hypertension, symptomatic 4.7 Scoliosis (untreated) 4.1 Peptic ulcer disease 4.0
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MedicalEvalua2onofPersonswithMRreferredforPsychiatricAssessment(Ryan,Sunada,1997)� Results
� 75%had>1undiagnosed/untreatedmedicalproblems� AlmostallhadNOdiagnosisotherthanIDbeforethestudy� Mostcommonpsychiatricdiagnosis-anxiety,depression
� Comments� Commonconditionspresentatypically� Conditionsconsidered“uncommon”mayoccurmorefrequently� Greaternumberoftestsmaybenecessarybecausehistoryoftennotavailableorhelpful� Workupconsideredcompletewhenpersonisimprovingorhasaspecificterminaldiagnosis
andiscomfortable
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http://www.ddhealthinfo.org/
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Generalpain/discomfort(highac2vity,rocking,head-banging,orotherbehavior)� Arthritis� Bonefracture� Cervicalbodysubluxation
� Cardiacdisease
� Constipation� Intestinalobstruction� Gastroesophagealreflux(w/orw/oesophagitis)� Hernia� Rectalfissure
� Dentalpathology
� Dehydration� Electrolyteorglucoseabnormality
� Pneumonia
� Headacheormigraine� Hydrocephalus� Ocularandvisionproblems� Seizures� Changeofconsciousness
� Sepsis� Occultinfection(sinusitis,otitismedia,dental,
urinarytractinfection,vaginitis,prostatitis)
� Medicationtoxicity
� Traumaincludingabuseorneglect� Psychiatricdisordersincludingdepression
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Hands/FingersinMouth� Sinusproblem� Eustachiantube� Middleearproblem� Dentalpathology� Gastroesophagealreflux� Asthma� Nausea
� Cardiacproblems� Seizures� Syncopeororthostasis� Vertigo� Atlanto-axialdislocation
SuddenSiWng
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Medicalcausesofbehavior-Allergies� Environmental/hayfever,foodallergies,atopicdermatitis,allergicasthma� Canoccurforthefirsttimewhensettingorenvironmentischanged
� Changeinweather/seasons� Potentiallyundiagnosedinnonverbalindividualsasmanysymptomsmaynotbeapparentandcouldimpactbehavior� Symptoms(runnynose,itchiness,redeyes,shortnessofbreath,swelling,etc.)&associateddiscomfortcanimpactbehavior
� Uncomfortability>irritability,anxiety,agitation,SIB� Associatedmedicationsalsohavesideeffects
� Antihistamines-sedation+moodchanges(especiallyZyrtec)
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Medicalcausesofbehavior-Allergies� Correlationofallergies/asthmawithbehavioralissuesandASDhasnotbeenexaminedindepth
� Medicalcomorbiditieslikeallergiesinthegeneralpopulationareoftenrelatedtoincreasedirritabilityandpoorfunctionaloutcomesinchildren(Jyonouchi,2010)
� Asthma&AllergiesinChildrenwithAutismSpectrumDisorders:ResultsfromtheCHARGEStudy(Lyall,etal.,2015)� OverallallergyinchildrenwithASDwasassociatedwithhigherstereotypyscoresasmeasuredbytheAberrantBehavioralChecklist(ABC)
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Medicalcausesofbehavior-allergies� Caseexample� 45-year-oldnonverbalmalewithprofoundintellectualdisability+autismwhowouldrepeatedlyforciblyinserthishandsinhismouth/throatandalsohadsevereSIB(banginghisheadwithhishands)
� Discoveredthathehadsevereenvironmentalallergieswhichcausedairritation/itchingofhispalette(roofofhismouth)whichwasthetriggerfortheinsertionofhishandsinhismouth
� AntihistaminehydroxyzineandotherrelevantinterventionshasproducedsomebenefitincludingtheeliminationofthisbehaviorbutcontinuestohaveSIB
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Medicalcausesofbehavior-cardiac� Caseexample� 60-year-oldverbalmalewithmildID,autism,possiblemooddisorderincludingbipolarwithsuddenonsetofdroppingtothefloorincludinginpublic
� Viewedbystaffasnoncompliancepossibleassociatedwithanxiety� Ultimatelydiagnosedwithbradycardia(slowheartrate)whichrequiredplacementofapacemaker.
� Followingthis,nofurtherdroppingbehaviorornoncomplianceandanxietywasoverallreduced
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Medicalcausesofbehavior-pain� Manymedical/dentalissuescangoundiagnosedinindividualswithdevelopmentaldisabilitiesandleadtothedevelopmentofpain
� Painmaybeexperiencedverydifferentlyinindividualswithdevelopmentaldisabilities� Diminishedorhigherpainthresholdmany� Heightenedsensitivityinsomeindividualsperhapsrelatedtosensoryissues
� Awarenessbutlackofunderstandingandstillimpactbehavior/functioning� Discomfort,anxiety,irritability,agitation,aggression,SIB,insomnia
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Medicalcausesofbehavior-pain� Dentalissues
� Dentalvisits/cleaningsareoftenverylimited/infrequentornotpossibleinsomeindividuals
� Asaresultvariousissuescandevelop(i.e.impactedteeth,infection,gumissues,etc.)whichcancausediscomfort/painandassociatedbehavioralmanifestations
� Loseteeth+bracescanalsobeproblematic� Sinus/ear/nasalissues
� Canbeacuteorchronicandsignificantlyimpactbehavior� Bothcouldpotentiallycontributetodiscomfortandassociatedirritability,SIB(headbanging,slapping)and/oraggressiontowardsothers
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Medicalcausesofbehavior-gastrointes2nal
� StudiessuggestthatchildrenwithASDareatincreasedriskforgastrointestinalproblems(Ibrahimetal,2009)
� FrequentG.I.complaintsincludingconstipation,reflux,foodallergies/sensitivities
� GastrointestinalSymptomsinAutismSpectrumDisorder:AMetaAnalysis,McElhanon,etal,2015
� Analysisof15peer-reviewedstudies(1980-2012)� ChildrenwithASDexperiencedsignificantlymoreG.I.symptoms� Diarrhea(OR,3.63/95%CI1.82-7.23),constipation(OR3.86/2.23-6.71),abdominalpain(OR2.45/1.19-5.07).
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Medicalcausesofbehavior-gastrointes2nal� StudiessuggestthatchildrenwithASDareatincreasedriskforgastrointestinalproblems(Ibrahimetal,2009)andmaybemoredifficulttoevaluate(Buieet.al.,2015)
� FurthersuggestedthatcertainbehavioralproblemsinchildrenwithASDmaybeindicativeofachild’sresponsetooranattempttocommunicatethepainordiscomfortofthisunderlyingG.I.issue(Horvath,etal.1999,Williamsatall2010,Bauman,2010)
� Specificsuggestedbehaviorsincludesleepdisturbance,stereotypicalrepetitivebehaviors,selfinjuriousbehaviors(SIB),aggression,oppositionality,irritability,tantrums,andothermooddisturbances
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Medicalcausesofbehavior-gastrointes2nal� Briefreport:Associationbetweenbehavioralfeatures&gastrointestinalproblemsamongchildrenwithautismspectrumdisorder,Maener,etal,2011
� Cross-sectionalstudyofchildrenwithASD35/487(7.2percent)haddocumentedhistoryofG.I.issues� Constipation,abdominalpain,diarrhea,encopresis,gastroesophagealrefluxdisease(GERD),gastritis,abdominalbloating,disaccharideacedeficiencies,inflammationofGItract,abnormalitiesofentericnervoussystem,functionalabdominalpain,irritablebowelsyndrome(IBS),flatulence,celiacdisease
� Constipation,encopresis,GERD-mostcommondocumented� Significantassociation-Unusualsleeping(p<0.01)oreatinghabits(p<.02),oppositionalbehavior(P<0.04)
� Associationbutnotsignificant-Mooddisturbance(P<.08),tantrums(p<0.05)� Noassociation-stereotypic/repetitivebehaviors+SIB
� UnusualsleepingoreatinghabitsandoppositionalbehaviorweresignificantlyassociatedwithGIproblems
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Medicalcausesofbehavior-gastrointes2nal� Pica
� Persistenteatingofnon-nutritive,nonfoodsubstancesoveraperiodof1month(DSMV)
� Paper,soap,cloth,hair,string,wool,soil,chalk,paint,gum,metal,pebbles,ice,coins
� Occursinthecontextofintellectualdisability,autism,schizophreniaorothermedicalconditionincludingpregnancy
� Caseexample� 39-year-oldmalewithahistoryofsevereintellectualdisabilityandchildhoodTBIwithachronichistoryofpica
� WouldoftenswallowballoonsorrubbergloveswhichcouldcauseG.I.irritationincludingconstipation
� Constipation/holdingofstooling� Perhapsrelatedtosensory/painissuesindividualsmaynotbeawareoftheassociateddiscomfortandimpactonbehavior
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Medicalcausesofbehavior-hormonal� Mostmenstruatingwomenexperiencesomepremenstrualsymptoms� Approximately20-40%considerthemsevereenoughtoseekmedicalhelp
� Premenstrualdysphoricdisorder� includesmoodlability,irritability,dysphoriaandanxietysymptomsthatoccursrepeatedlyduringthepremenstrualphaseofthecycleandremitaroundtheonsetofmensesorshortlythereafter
� Alsoincludesphysicalandbehavioralsymptoms� 12monthprevalence=1.8-5.8%ofmenstruatingwomen
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Medicalcausesofbehavior-hormonal� Prevalenceofpremenstrualsyndromeandautism:aprospectiveobservatorratedstudy,Obaydi+Puri2008
� Comparedwomenwithautism+learningdisability(26)withagroupwithlearningdisabilityonly(36)
� 24/26(92%)metcriteriaforlatelutealphasedysphoricdisordervs11%ofcontrols(p<.000001)
� Increasedsymptoms-affectivelability,angerorirritability,clumsiness,anxietyortension,depressedmood,impairmentofworkperformance,socialactivitiesrelationship,socialwithdrawal,isolationanddecreasedinterestinusualactivities,decreasedconcentration,tempertantrums,physicalaggression,selfharm,stereotypiesorrepetitivemovements,destructivebehavior,hypersomnia,insomnia,changeinappetiteorspecificfoodcraving,headache
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Medicalcausesofbehavior-hormonal� Caseexample� 23-year-oldlargelynonverbalfemalewithautism,priordiagnosisofADHDandbipolardisorderbeginninginlateteens
� Developedcyclicalmoodchangesinadditiontohormonalchangesinmood
� Respondedwelltomoodstabilizers/antipsychoticsaswellastheadditionoftransdermalbirthcontrol
� Withthiscombinationcyclicalandhormonalmoodchangesarelargelyundercontrol
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Medicalcausesofbehavior-sleepdisorders� Sleepwakecycleinindividualswithautismappearstobeabnormal� Sleepproblems
� CommoninchildrenwithASD-45-86%Liu,Hubbard,Fabes&Adam,2006,Maskeyetal.,2008
� Morechronic–remission8.3%vs52.4%Silversenetal.,2012� ConsideredasacoexistingsymptomofASDnotsignificantlyinfluencedbyrace,gender,ageorIQMayes&Calhoun2009
� Impactontheindividualandthefamily/careprovidersisprofound� Significantstresstofamilies� Shortsleepdurationassociatedwithhigherratesofstereotypicalbehavior33aswellassocialdeficits32
� Mayworsendaytimebehaviorsincludinginattention/hyperactivity28
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Medicalcausesofbehavior-insomnia� Insomnia=Difficultyinitiatingormaintainingsleep� Types-Prolongedsleeplatency(timetofallasleep),bedtimeresistance,decreasedsleepefficiency,decreasedsleepduration+continuityandincreasedawakenings
� Sleeponsetinsomniaismoreprevalentthansleepmaintenanceinsomnia17,34� Manypossiblecontributingfactors
� Aberrationsofneurotransmittersystemsthatarerelatedtosleep+regularsleepwakecycle(e.g.melatonin)
� Psychiatricdisorders-anxiety/depression,ADHD,obsessive/repetitivebehavior� Medicalissuesthatdisruptsleepcontinuity-epilepsy,G.I.disorders,pain,constipation,breathingissues/asthma
� Neurologicissues-sleepapnea,periodiclimbmovement,restlesslegsyndrome� Difficultyestablishingaproperbedtimebehaviorandroutine� Sensoryissues
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Medicalcausesofbehavior-sleepapnea� Sleepdisorderedbreathingincludesdisordersrelatedtoairwayobstruction/obstructivesleepapnea
� Commoninthegeneralpediatricpopulationincludingthosewithautism� Contributingfactors-allergies,tonsillitis,hypotonia,medicationrelatedsedation
� Contributestodaytimesleepinesswhichcanimpactbehavior,mood,attention,etc.
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Medicalcausesofbehavior-headaches� Prevalence
� 37-51%duringelementaryschoolyears,57-82%-highschool� Frequent/severeheadachesincludingmigraine-17%ofchildren+adolescence
� Migraineheadaches=moderate/severerecurrentheadaches� otherssymptomsincludingnausea,vomiting,sensitivities(light,sound,smell),visual/visiondisturbances,frequenttriggers
� Familyhistory� Hormonal+othertriggers
� Inthosewithlanguage/communicationdifficultiesheadachesmaybehardtodiagnosebutcancausesignificantimpactonpain
� Empiricaltreatment?
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Medicalcausesofbehavior-neurological� PrevalenceofepilepsyinASDranges11-39%� Moreseverecognitivedysfunctionassociatedwithhigherriskofseizures� EEGabnormalitiescanbefoundin8-31%ofthosewithoutepilepsy� Morecommonlyassociatedwithregression–seeninabout30%ofASD,usually18-24mos� Inthissubset,upto80%inonestudyhadepileptiformactivityonEEGduringsleep(ESESorCSWS)
� OnsetofESEScommonlyassociatedwithonsetofseizures,mostfrequentlyabsence
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Medicalcausesofbehavior-neurological� Epilepsy=Recurrentseizures
� Complexpartialseizures/temporallobeepilepsy� Absenceseizures/petitemal� Frontallobeseizures
� Mixedseizuretypes/syndromes� LennoxGastautSyndrome
� Continuousspikewavesinslowwavesleep(CSWS)� LandauKleffnerSyndrome
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Medicalcausesofbehavior-neurological� Epilepsy=Recurrentseizures� Frequentseizuresespeciallyatnightcanimpactcognition/development� Whenveryfrequentcancontributetothedevelopmentofachronicconfusedstate(encephalopathy).
� Theadditionofrequiredmedicationcanfurthercontributetothisconfusedstate
� Thelocationofbeingabnormalelectricalactivitycanberelevanttofunctioningofthebraininthatareaincludingbehavior.
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Medicalcausesofbehavior-neurological� CSWS–Continuousspikewavesinslowwavesleep
� Presentwithregression–lossoflanguageandtemporospatialskills,hyperactivity,aggression
� Mayhavemotordeficits–ataxia,apraxia,dyspraxia� Mostlyexpressiveaphasia(receptiveusuallyspared)� Seizuresarepresentingsymptominupto80%� Ageofonsetvariable,mean4-8years
� Youngeronset,worseprognosis
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Medicalcausesofbehavior-neurological-LandauKleffnerSyndrome� Onset-3-8yearswithpeak4-5years� Acquiredaphasia
� Normaldevelopmentuntil4-6yrs� Languagearrest>jargonizedspeech
� Othersymptoms� Hyperactive/impulsive,autisticlike
� Seizures� Presentand70-80%� EEG-Bilateralindependenttemp/parietalspike/waveorfocaltemporal
� Differential-Autism
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Medicalcausesofbehavior-neurological-PANDAS� PediatricAutoimmuneNeuropsychiatricDisordersAssociatedwithStreptococcalInfections� Specifically,groupAbeta-hemolyticStreptococcus(GABHS)
� Firstdescribedin1998withacaseseriesof50casesbySusanSwedoattheNIH.� SwedoSE,LeonardHL,GarveyM,etal.PANDAS:ClinicalDescriptionoftheFirst50Cases.AmericanJournalof
Psychiatry1998;155:264-271.
� AntibodiesgeneratedagainstGABHScross-reactwithself-antigensexpressedinthebasalgangliaand/orotherbrainstructures(molecularmimicry).
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PANDASCriteria1. TicdisorderorObsessiveCompulsiveDisorder
meetingDSMcriteria2. Onsetpriortopuberty(usually3-12)3. Abruptonsetofsymptomsorepisodiccourse(severe)4. Temporalassociationofsymptomexacerbationand
streptococcalinfections(onset+oneexacerbation,oratleasttwoexacerbationsconfirmedbycultureorrepeatedrisinganti-streptolysinO/antiDNAseBtiters)
5. Presenceofneurologicalabnormalitiesduringperiodsofsymptomsexacerbation(e.g.-choreiformmovements(butnotchorea),tremor,cognitivedeficits,motorichyperactivity)
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Medicalcausesofbehavior-neurological-PANDASs AdditionalresearchrevealedassociationbetweenPANDASinthepresenceofcomorbidADHD,separationanxiety,vocalandmotortics,frequenturination,handwritingdeterioration,anddeclineinschoolperformance.
s ChildrenwithPANDASweremoreliketopresentwithdramaticonsetofsymptoms,completeremission,temporalassociationofsymptomswithGASinfectionandclumsiness.
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Medicalcausesofbehavior-neurological-PANS
� PediatricAcuteonsetNeuropsychiatricSyndrome� ConsensusconferenceMay2013� JournalofAcademyofChild&AdolescentPsychopharmacology
� PANDAS“morphed”asresearchidentifiedpotentialinfectioustriggersbeyondGroupAstreptococcusaswellasnoninfectiousormetabolicfactors
� CliniciansconfrontedwiththedifficultydiagnosingyouthwhomeetallbutonecriteriaofthePANDASsubtype,evidenceofGASinfectionbeforesymptomonset.� IncreasingevidencesuggestingabsenceofsignificantroleforGASinfection
� Proposedabroadertermthatencompassesacuteonsetneuropsychiatricsymptomswithoutaspecificenvironmentalorimmunerelatedtrigger(Swedoetal.,2012)
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Medicalcausesofbehavior-neurological-PANS
� CliniciansconfrontedwiththedifficultydiagnosingyouthwhomeetallbutonecriteriaofthePANDASsubtype,evidenceofGASinfectionbeforesymptomonset.
� IncreasingevidencesuggestingabsenceofsignificantroleforGASinfection
� Proposedabroadertermthatencompassesacuteonsetneuropsychiatricsymptomswithoutaspecificenvironmentalorimmunerelatedtrigger(Swedoetal.,2012)
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Medicalcausesofbehavior-neurological-PANS
� PANSCriteria� Abrupt,dramaticonsetofOCDORseverelyrestrictedfoodintake+� 2ormoreconcurrentneuropsychiatricsymptomsalsowithacuteonset
� Sensorysymptoms� Handwritingdeterioration� Separationanxiety� Emotionallability
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Medicalcausesofbehavior-neurological-PANS
� Otherfeatures� Anxiety� Emotionallylability,depression,orboth� Irritability,aggressionorsevereoppositionalbehaviororcombination� Behavioralregression� Deteriorationinschoolperformance� Sensorymotorabnormalities� Somaticsignsorsymptomsincludingsleepdisturbance,enuresisorurinaryfrequency
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Medicalcausesofbehavior-neurological-PANS
� Laboratorytests–PANS� ASOtiterelevationsbeganoneweekafterinfectionandpeakafter2-3weeks
� Anti-DNAseBtiterelevationsafter4-8weeks� Useofbothreducesthepossibilityofobtainingfalsenegatives
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Thank you