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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’s SUBCORTICAL - VASCULAR, PD, Wilson’s arousal, attn, mood, motivation, depression WHITE MATTER - MS, NPH, HIV

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dementia

DEMENTIA

Anne M. Lipton, M.D., Ph.D.

Department of Neurology

Presbyterian Hospital of Dallas

classification of dementias
Classification of Dementias
  • CORTICAL - AD, FTD/Pick’s
  • SUBCORTICAL - VASCULAR, PD, Wilson’s
    • arousal, attn, mood, motivation, depression
  • WHITE MATTER - MS, NPH, HIV
    • apathy, forgetfulness, inattention, depression
  • COMBINATION - CJD, LBD
diagnostic work up for dementia
Diagnostic Work-up for Dementia
  • Diagnostic Interview with patient and family
  • Exam, including Neurologic and Mental Status exam
  • Labs
  • Neuroimaging
  • Neuropsychological evaluation
  • Language evaluation, LP, genetics - specialist referral
neurobehavioral history and exam
Neurobehavioral History and Exam
  • Attention and concentration
  • Visuospatial skills
  • Language
  • Memory
  • Executive Functions
  • Personality/Behavior
memory
Memory
  • Registration/Encoding
  • Storage
  • Retrieval
    • Recent versus remote memory
    • Recall versus recognition
executive functions
Executive functions
  • Insight/judgment
  • IADL’s (Instrumental ADL’s)
  • Clock drawing
  • Similarities/proverbs
personality and behavior
Personality and Behavior
  • ADLs/Continence
  • Agitation/Aggression
  • Appetite/Sleep
  • Apathy/Depression
  • Hallucinations/Delusions
neurologic examination
Neurologic Examination
  • Focal signs
  • Parkinsonian signs
  • Myoclonus
  • Neuropathy
  • Gait Apraxia
prevalence of ad with increasing age
Prevalence of AD with Increasing Age

45

40

35

30

25

20

15

10

5

0

Percent of Patients With AD

65-69

70-74

75-79

80-84

85-89

90-94

95-99

Age (Years)

Adapted from Ritchie K, Kildea D. Lancet. 1995;346:931-934.

the 5 a s of alzheimer s disease
The 5 A’s of Alzheimer’s disease
  • Amnesia
  • Agnosia
  • Aphasia
  • Apraxia
  • Abstraction
early symptoms of ad
Early symptoms of AD
  • Gradual memory loss/poor recent memory
  • Poor insight
  • Apathy
  • “Empty” speech/dysnomia
  • Decline in ability to perform routine tasks
memory loss in ad
Memory loss in AD
  • “Memory leads the way”
  • Memory worst and first
  • More problems with new (recent) info than with old (remote)
cholinesterase inhibitors
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
Cholinesterase Inhibitors: ABC’s
  • Maintain activities of daily living
  • Help behavior problems
  • Slow cognitive decline
  • Delay nursing home placement
memantine namenda
Memantine (Namenda)
  • NMDA antagonist
  • NMDA = type of glutamate receptor
  • Approved for moderate-to-severe AD
  • Improves or slows cognitive and functional decline
  • Decreases caregiver burden
vitamin e
Vitamin E
  • Disease-modifying agent
  • Benefits proven in double-blind study (Sano et al., 1997)
  • Vitamin E 1000 International Units BID
  • Blood thinner
dementia with lewy bodies1
Dementia with Lewy bodies
  • Dementia
  • Parkinsonism
  • Cognitive fluctuations
  • Prominent hallucinations
  • Neuroleptic sensitivity
dementia with lewy bodies treatment
Dementia with Lewy bodies - Treatment
  • Cholinesterase Inhibitors
    • Rivastigmine has been shown to improve cognition and behavioral symptomatology
  • AVOID TYPICAL NEUROLEPTICS
    • Avoid haloperidol, risperidone
    • quetiapine OK
    • try trazodone, other Rx first
vascular dementia1
Vascular dementia
  • Stepwise progression
  • Focal neurological deficits
  • Retrieval memory deficit
  • Psychomotor slowing, apathy
  • Neuroimaging
  • Vasculitis/hypercoagulable/stroke workup
vascular dementia treatment
Vascular dementia - Treatment
  • Treat hypertension
  • Stroke prevention
    • ASA, clopidogrel, warfarin
    • Vitamin E
    • Cholesterol-lowering agents - statins
  • SSRI’s
  • Cholinesterase inhibitors?
frontotemporal dementia consensus criteria
Frontotemporal dementia consensus criteria
  • Common features
    • Gradual and insidious
    • Aphasia +/- agnosia
  • Supportive features
    • Onset before 65
    • Positive family hx
    • Motor Neuron Disease
frontotemporal dementia1
Frontotemporal dementia
  • Neurobehavioral syndrome
    • Frontotemporal Dementia (FTD)
  • Language Presentation
    • Primary progressive aphasia
    • Semantic Dementia
ftd behavioral syndrome
FTD BEHAVIORAL SYNDROME
  • Apathy, social withdrawal +/- disinhibition
  • Decreased executive function, poor self care
  • Kluver-Bucy
    • hyperphagia, hypermetamorphosis, aggression +/- changes in sexuality
  • Compulsions
  • Perception, memory, praxis, and visuospatial skills relatively well preserved
primary progressive aphasia
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 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
Neurological Examination
  • Frontal reflexes
  • Motor neuron signs
    • Weakness, fasiculations, etc.
  • Parkinsonism
  • Apraxia
  • Alien limb syndrome
work up
Work-up
  • Neuropsychological Evaluation
  • Language evaluation
  • Brain imaging: MRI, SPECT, PET
  • LP
  • EMG/NCS
treatment for ftd
Treatment for FTD
  • Cholinesterase Inhibitors
    • No cholinergic deficit
    • No effect, bad effect (increase irritability), or ?help - low doses
  • SSRI’s
  • Trazodone
  • Prefer atypical neuroleptics if necessary
head trauma and dementia
Head Trauma and Dementia
  • Usually head injury with LOC
  • Chronic Subdural Hematoma
    • can occur even after minor head trauma
    • EtOH, AED’s, anticoagulants, seizures
  • Repeated head trauma
    • Dementia Pugilistica
dementia syndrome of depression
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
Normal Pressure Hydrocephalus
  • Dementia
  • Urinary Incontinence
  • Gait Apraxia
  • Workup
    • CT or MRI
    • LP
    • Cisternogram
  • Treatment
alcoholic dementias
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
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
Creutzfeldt-Jakob Disease
  • Rapidly progressive dementia
  • Myoclonus
  • EEG clinches diagnosis
  • No treatment
  • Neuropatholgy - spongiform changes
  • Iatrogenic transmission
  • Atypical cases associated with BSE
pearls on dementia
Pearls on dementia
  • Few are reversible, but almost all are treatable
  • Distinguish from delirium
  • Atypical presentation = think atypical (non-AD) dementia