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DEMENTIA

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

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  1. DEMENTIA Anne M. Lipton, M.D., Ph.D. Department of Neurology Presbyterian Hospital of Dallas

  2. 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

  3. 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

  4. Neurobehavioral History and Exam • Attention and concentration • Visuospatial skills • Language • Memory • Executive Functions • Personality/Behavior

  5. Memory • Registration/Encoding • Storage • Retrieval • Recent versus remote memory • Recall versus recognition

  6. Executive functions • Insight/judgment • IADL’s (Instrumental ADL’s) • Clock drawing • Similarities/proverbs

  7. Personality and Behavior • ADLs/Continence • Agitation/Aggression • Appetite/Sleep • Apathy/Depression • Hallucinations/Delusions

  8. Neurologic Examination • Focal signs • Parkinsonian signs • Myoclonus • Neuropathy • Gait Apraxia

  9. Alzheimer’s disease

  10. 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.

  11. The 5 A’s of Alzheimer’s disease • Amnesia • Agnosia • Aphasia • Apraxia • Abstraction

  12. Early symptoms of AD • Gradual memory loss/poor recent memory • Poor insight • Apathy • “Empty” speech/dysnomia • Decline in ability to perform routine tasks

  13. Memory loss in AD • “Memory leads the way” • Memory worst and first • More problems with new (recent) info than with old (remote)

  14. 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

  15. Cholinesterase Inhibitors: ABC’s • Maintain activities of daily living • Help behavior problems • Slow cognitive decline • Delay nursing home placement

  16. 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

  17. Vitamin E • Disease-modifying agent • Benefits proven in double-blind study (Sano et al., 1997) • Vitamin E 1000 International Units BID • Blood thinner

  18. Dementia with Lewy bodies

  19. Dementia with Lewy bodies • Dementia • Parkinsonism • Cognitive fluctuations • Prominent hallucinations • Neuroleptic sensitivity

  20. 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

  21. Vascular Dementia

  22. Vascular dementia • Stepwise progression • Focal neurological deficits • Retrieval memory deficit • Psychomotor slowing, apathy • Neuroimaging • Vasculitis/hypercoagulable/stroke workup

  23. Vascular dementia - Treatment • Treat hypertension • Stroke prevention • ASA, clopidogrel, warfarin • Vitamin E • Cholesterol-lowering agents - statins • SSRI’s • Cholinesterase inhibitors?

  24. Mixed dementia

  25. Frontotemporal dementia

  26. Frontotemporal dementia consensus criteria • Common features • Gradual and insidious • Aphasia +/- agnosia • Supportive features • Onset before 65 • Positive family hx • Motor Neuron Disease

  27. Frontotemporal dementia • Neurobehavioral syndrome • Frontotemporal Dementia (FTD) • Language Presentation • Primary progressive aphasia • Semantic Dementia

  28. 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

  29. 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

  30. 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

  31. Neurological Examination • Frontal reflexes • Motor neuron signs • Weakness, fasiculations, etc. • Parkinsonism • Apraxia • Alien limb syndrome

  32. Work-up • Neuropsychological Evaluation • Language evaluation • Brain imaging: MRI, SPECT, PET • LP • EMG/NCS

  33. 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

  34. 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

  35. 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.

  36. Normal Pressure Hydrocephalus • Dementia • Urinary Incontinence • Gait Apraxia • Workup • CT or MRI • LP • Cisternogram • Treatment

  37. 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

  38. Neoplastic Disease and Dementia • Cerebral Neoplasm • focal signs, headache, and seizure • neuroimaging with contrast • Neoplastic meningitis • CSF cytology • low yield • Treatment • radiation • intrathecal cytararabine

  39. Creutzfeldt-Jakob Disease • Rapidly progressive dementia • Myoclonus • EEG clinches diagnosis • No treatment • Neuropatholgy - spongiform changes • Iatrogenic transmission • Atypical cases associated with BSE

  40. Pearls on dementia • Few are reversible, but almost all are treatable • Distinguish from delirium • Atypical presentation = think atypical (non-AD) dementia

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