marsel mesulam, md - social security administration · marsel mesulam, md center director...
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Marsel Mesulam, MD Center Director
Northwestern Alzheimer’s Disease Center Northwestern University Feinberg School of Medicine Funded by the National Institute On Aging (NIA)
Website: http://www.brain.northwestern.edu
Alzheimer’sdisease(AD)isthemostwidelyrecognizedformofdemen9a.Itismostfrequentlyseenintheelderlyandtypicallycausesprofoundmemoryimpairments.
ManyofusthereforedeveloptheimpressionthatADanddemen9aarethesamething,thatdemen9acanonlyoccurintheelderlyandthatadiagnosisofdemen9acannotbemadeintheabsenceofmemoryloss.
ThemajormessagethatmycolleaguesandIwouldliketobringtothishearingistheneedtocri9callyre‐examinetheseassump9ons.
1.
2.
3.
ADCANARISEINTHEYOUNG.THEYOUNGESTPATIENTISAWWAS24.
DEMENTIACANBECAUSEDBYDISEASESOTHERTHANAD.THEMOSTFREQUENTYOUNG‐ONSET,NON‐ADDEMENTIAISKNOWNASFRONTOTEMPORALLOBARDEGENERATION(FTLD).
FTLDANDUNUSUALFORMSOFADCANCAUSEDISABILITYBYIMPAIRINGLANGUAGE,SPATIALORIENTATIONORBEHAVIORRATHERTHANMEMORY.INSOMEOFTHESEPATIENTSADIAGNOSISOFDEMENTIAISMADEALTHOUGHMEMORYISNORMAL.
• • • •
ALZHEIMER’SDISEASEHASAMYLOIDINTHEBRAIN,FTLDDOESNOT.HEREDITARYADISLINKEDTOCHR1,14,21.FTLDISLINKEDTOCHR3,9,17.TYPICALALZHEIMER’SLEADSTOANAMNESTICDEMENTIA,FTLDUSUALLYSPARESMEMORY.FTLDCANCAUSETWODIFFERENTCLINICALSYNDROMESOFDEMENTIA‐bvFTDANDPPA.
AMYLOID
TANGLE
ALZHEIMER
PICKBODY
TDP‐43
FRONTOTEMPORAL
bvFTD(akaFTD)causesagraduallossofjudgment,insightandcomportmenttothepointwherethepersoncannotmakesounddecisionsandengagesin
inappropriateimpulsivebehaviors.
PPAresultsinagraduallossofwordusageandcomprehensiontothepointwherethepersonlosestheabilitytocommunicate
thoughtsorunderstandwhatothersaresaying.Theproblemisnotinar9cula9ngorhearingspeechbutinthebrain’sabilitytouseandinterpretwords.
BOTHbvFTDANDPPAUSUALLYSTARTBEFORETHEAGEOF60ANDCANSPAREMEMORYFORMANYYEARS
bvFTDHASBEENINCLUDEDINTHECOMPASSIONATEALLOWANCESLISTBUTPPAHASNOT
SURGICALLYCAUSEDMEMORYLOSSNORMALBRAIN
TOP
LEFT RIGHT
TYPICALAMNESTICAD
atrophy NORMAL
bvFTD
FRONT
BACK
PPA
RIGHT
LEFT
1. DOCUMENTEDHISTORYOFDISABLINGANDPROGRESSIVEIMPAIRMENTIN‐• word‐finding• wordusage• orderingwordsintomeaningfulsentences• wordcomprehensionandreading• wriOngandspelling
2.OBJECTIVENEUROPSYCHOLOGICALEVIDENCEOFLANGUAGEIMPAIRMENTANDRELATIVESPARINGOFOTHERCOGNITIVEABILITIES.
3.IMAGINGEVIDENCEOFPROGRESSIVEBRAINDAMAGEINLANGUAGEAREAS
LEFT RIGHTPPACOMPUTERIZEDMAPPINGOFATROPHY
LEFT
SPECT
LEFT RIGHT
LOWBRAINFUNCTION