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DEMENTIA
Anne M. Lipton, M.D., Ph.D.
Department of Neurology
Presbyterian Hospital of Dallas
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
Diagnostic Work-up for Dementia
Diagnostic Interview with patient and familyExam, including Neurologic and Mental
Status exam LabsNeuroimagingNeuropsychological evaluationLanguage evaluation, LP, genetics -
specialist referral
Neurobehavioral History and Exam
Attention and concentrationVisuospatial skillsLanguageMemoryExecutive Functions Personality/Behavior
Memory
Registration/EncodingStorageRetrieval
Recent versus remote memoryRecall versus recognition
Executive functions
Insight/judgment IADL’s (Instrumental ADL’s)Clock drawingSimilarities/proverbs
Personality and Behavior
ADLs/ContinenceAgitation/AggressionAppetite/SleepApathy/DepressionHallucinations/Delusions
Neurologic Examination
Focal signsParkinsonian signsMyoclonusNeuropathyGait Apraxia
Alzheimer’s disease
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
The 5 A’s of Alzheimer’s disease
Amnesia
AgnosiaAphasiaApraxiaAbstraction
Early symptoms of AD
Gradual memory loss/poor recent memoryPoor insightApathy “Empty” speech/dysnomiaDecline in ability to perform routine tasks
Memory loss in AD
“Memory leads the way”
Memory worst and first
More problems with new (recent) info than with old (remote)
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
Cholinesterase Inhibitors: ABC’s
Maintain activities of daily living
Help behavior problems
Slow cognitive decline
Delay nursing home placement
Memantine (Namenda)
NMDA antagonistNMDA = type of glutamate receptorApproved for moderate-to-severe AD Improves or slows cognitive and functional
declineDecreases caregiver burden
Vitamin E
Disease-modifying agent Benefits proven in double-blind study (Sano
et al., 1997)Vitamin E 1000 International Units BIDBlood thinner
Dementia with Lewy bodies
Dementia with Lewy bodies
DementiaParkinsonismCognitive fluctuationsProminent hallucinationsNeuroleptic sensitivity
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
Vascular Dementia
Vascular dementia
Stepwise progressionFocal neurological deficitsRetrieval memory deficitPsychomotor slowing, apathyNeuroimagingVasculitis/hypercoagulable/stroke
workup
Vascular dementia - Treatment
Treat hypertensionStroke prevention
ASA, clopidogrel, warfarinVitamin ECholesterol-lowering agents - statins
SSRI’s Cholinesterase inhibitors?
Mixed dementia
Frontotemporal dementia
Frontotemporal dementia consensus criteria
Common featuresGradual and insidiousAphasia +/- agnosia
Supportive featuresOnset before 65Positive family hxMotor Neuron Disease
Frontotemporal dementia
Neurobehavioral syndrome Frontotemporal Dementia (FTD)
Language Presentation Primary progressive aphasia Semantic Dementia
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
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
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
Neurological Examination
Frontal reflexesMotor neuron signs
Weakness, fasiculations, etc.ParkinsonismApraxiaAlien limb syndrome
Work-up
Neuropsychological EvaluationLanguage evaluationBrain imaging: MRI, SPECT, PETLPEMG/NCS
Treatment for FTD
Cholinesterase Inhibitors No cholinergic deficit No effect, bad effect (increase irritability), or ?
help - low dosesSSRI’sTrazodonePrefer atypical neuroleptics if necessary
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
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.
Normal Pressure Hydrocephalus
DementiaUrinary IncontinenceGait ApraxiaWorkup
CT or MRI LP Cisternogram
Treatment
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
Neoplastic Disease and Dementia
Cerebral Neoplasm focal signs, headache, and seizure neuroimaging with contrast
Neoplastic meningitis CSF cytology
low yield
Treatment radiation intrathecal cytararabine
Creutzfeldt-Jakob Disease
Rapidly progressive dementiaMyoclonusEEG clinches diagnosisNo treatment
Neuropatholgy - spongiform changes Iatrogenic transmissionAtypical cases associated with BSE
Pearls on dementiaFew are reversible, but almost all are
treatable
Distinguish from delirium
Atypical presentation = think atypical (non-AD) dementia