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DEMENTIA

This guide provides a comprehensive overview of the classification and diagnostic work-up for different types of dementia, including Alzheimer's disease, dementia with Lewy bodies, vascular dementia, and frontotemporal dementia. It covers various assessment methods, neurobehavioral history and exam, memory loss patterns, and treatment options.

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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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