dementia anne m. lipton, m.d., ph.d. department of neurology presbyterian hospital of dallas
TRANSCRIPT
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DEMENTIA
Anne M. Lipton, M.D., Ph.D.
Department of Neurology
Presbyterian Hospital of Dallas
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Classification of Dementias
CORTICAL - AD, FTD/Pick’sSUBCORTICAL - VASCULAR, PD, Wilson’s
arousal, attn, mood, motivation, depressionWHITE MATTER - MS, NPH, HIV
apathy, forgetfulness, inattention, depression
COMBINATION - CJD, LBD
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Diagnostic Work-up for Dementia
Diagnostic Interview with patient and familyExam, including Neurologic and Mental
Status exam LabsNeuroimagingNeuropsychological evaluationLanguage evaluation, LP, genetics -
specialist referral
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Neurobehavioral History and Exam
Attention and concentrationVisuospatial skillsLanguageMemoryExecutive Functions Personality/Behavior
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Memory
Registration/EncodingStorageRetrieval
Recent versus remote memoryRecall versus recognition
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Executive functions
Insight/judgment IADL’s (Instrumental ADL’s)Clock drawingSimilarities/proverbs
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Personality and Behavior
ADLs/ContinenceAgitation/AggressionAppetite/SleepApathy/DepressionHallucinations/Delusions
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Neurologic Examination
Focal signsParkinsonian signsMyoclonusNeuropathyGait Apraxia
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Alzheimer’s disease
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Prevalence of AD with Increasing Age
Adapted from Ritchie K, Kildea D. Lancet. 1995;346:931-934.
45
40
35
30
25
20
15
10
5
0
Per
cen
t o
f P
atie
nts
Wit
h A
D
65-69 70-74 75-79 85-89 95-99
Age (Years)80-84 90-94
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The 5 A’s of Alzheimer’s disease
Amnesia
AgnosiaAphasiaApraxiaAbstraction
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Early symptoms of AD
Gradual memory loss/poor recent memoryPoor insightApathy “Empty” speech/dysnomiaDecline in ability to perform routine tasks
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Memory loss in AD
“Memory leads the way”
Memory worst and first
More problems with new (recent) info than with old (remote)
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Cholinesterase Inhibitors
Donepezil (Aricept), rivastigmine (Exelon), galantamine (Reminyl)
All approved for use in mild-moderate AD (MMSE ~10-26), donepezil also approved for moderate-severe AD
Start low, go slow GI side effects Expected outcome of therapy - to SLOW decline May be helpful in treatment of other dementias
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Cholinesterase Inhibitors: ABC’s
Maintain activities of daily living
Help behavior problems
Slow cognitive decline
Delay nursing home placement
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Memantine (Namenda)
NMDA antagonistNMDA = type of glutamate receptorApproved for moderate-to-severe AD Improves or slows cognitive and functional
declineDecreases caregiver burden
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Vitamin E
Disease-modifying agent Benefits proven in double-blind study (Sano
et al., 1997)Vitamin E 1000 International Units BIDBlood thinner
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Dementia with Lewy bodies
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Dementia with Lewy bodies
DementiaParkinsonismCognitive fluctuationsProminent hallucinationsNeuroleptic sensitivity
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Dementia with Lewy bodies - Treatment
Cholinesterase InhibitorsRivastigmine has been shown to improve
cognition and behavioral symptomatology
AVOID TYPICAL NEUROLEPTICSAvoid haloperidol, risperidonequetiapine OK try trazodone, other Rx first
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Vascular Dementia
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Vascular dementia
Stepwise progressionFocal neurological deficitsRetrieval memory deficitPsychomotor slowing, apathyNeuroimagingVasculitis/hypercoagulable/stroke
workup
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Vascular dementia - Treatment
Treat hypertensionStroke prevention
ASA, clopidogrel, warfarinVitamin ECholesterol-lowering agents - statins
SSRI’s Cholinesterase inhibitors?
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Mixed dementia
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Frontotemporal dementia
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Frontotemporal dementia consensus criteria
Common featuresGradual and insidiousAphasia +/- agnosia
Supportive featuresOnset before 65Positive family hxMotor Neuron Disease
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Frontotemporal dementia
Neurobehavioral syndrome Frontotemporal Dementia (FTD)
Language Presentation Primary progressive aphasia Semantic Dementia
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FTD BEHAVIORAL SYNDROME
Apathy, social withdrawal +/- disinhibitionDecreased executive function, poor self careKluver-Bucy
hyperphagia, hypermetamorphosis, aggression +/- changes in sexuality
CompulsionsPerception, memory, praxis, and visuospatial
skills relatively well preserved
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PRIMARY PROGRESSIVE APHASIA
Insidious onset and gradual progression Nonfluent spontaneous speech w/at least one of
the following:agrammatism, phonemic paraphasias, anomia
Other aspects of cognition are relatively well preserved
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SEMANTIC DEMENTIA
Semantic aphasia and associative agnosia Insidious onset and gradual progression Language +/- perceptual disorder Other aspects of cognition, including memory,
are relatively preserved Preserved perceptual matching and drawing
reproduction Preserved single-word repetition, reading, taking
dictation
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Neurological Examination
Frontal reflexesMotor neuron signs
Weakness, fasiculations, etc.ParkinsonismApraxiaAlien limb syndrome
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Work-up
Neuropsychological EvaluationLanguage evaluationBrain imaging: MRI, SPECT, PETLPEMG/NCS
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Treatment for FTD
Cholinesterase Inhibitors No cholinergic deficit No effect, bad effect (increase irritability), or ?
help - low dosesSSRI’sTrazodonePrefer atypical neuroleptics if necessary
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Head Trauma and Dementia
Usually head injury with LOC
Chronic Subdural Hematomacan occur even after minor head traumaEtOH, AED’s, anticoagulants, seizures
Repeated head trauma Dementia Pugilistica
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Dementia Syndrome of Depression
Usually called Pseudodementia of Depression Dementia
Insidious, progressive, pt unaware with variable affect Sundowning
Depression Abrupt, stable, pt depressed with multiple vegetative
symptoms and somatic complaints.
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Normal Pressure Hydrocephalus
DementiaUrinary IncontinenceGait ApraxiaWorkup
CT or MRI LP Cisternogram
Treatment
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Alcoholic Dementias
Pellagra - 4 D’s Dementia, Depression, Diarrhea, and Dermatitis
Marchiafava Bignama Red wine
Elderly Italian men Necrosis of the corpus callosum
Korsakoff’s Really an amnestic syndrome May be reversible with abstinence
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Neoplastic Disease and Dementia
Cerebral Neoplasm focal signs, headache, and seizure neuroimaging with contrast
Neoplastic meningitis CSF cytology
low yield
Treatment radiation intrathecal cytararabine
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Creutzfeldt-Jakob Disease
Rapidly progressive dementiaMyoclonusEEG clinches diagnosisNo treatment
Neuropatholgy - spongiform changes Iatrogenic transmissionAtypical cases associated with BSE
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Pearls on dementiaFew are reversible, but almost all are
treatable
Distinguish from delirium
Atypical presentation = think atypical (non-AD) dementia