Ronald C. Hamdy, MD, FRCP, FACP Professor of Medicine

1. The Many Faces of DementiaMarch 26, 2013 Ronald C. Hamdy, MD, FRCP, FACP Professor of Medicine Cecile Cox Quillen Chair of Geriatric Medicine East Tennessee State University

2. The Many Faces of DementiaMarch 26, 2013 NO DISCLOSURES

3. The Many Faces of DementiaMarch 26, 2013 • OBJECTIVES • Recognize the different presentations of various types of dementia: Alzheimer’s disease, Lewy Body Dementia, Normal pressure hydrocephalus, Multi-infarct dementia, Fronto-temporal dementia • Develop a clinical diagnostic approach to identify various dementias.

4. Dementia • Clinical Syndrome, not disease • Different types • Decline of acquired cognitive abilities, leading to impairments in: • Attention • Language • Memory • Visuo-spacial skills • Executive abilities • Personality • Interferes with daily activities.

5. Mr. DAW, 75 yrs - retired accountant brought in by concerned wife and daughter

6. Mr. DAW, 75 yrs - retired accountant brought in by concerned wife and daughter • Impaired memory: • Shopping lists, phone calls, house chores

7. Mr. DAW, 75 yrs - retired accountant brought in by concerned wife and daughter • Impaired memory: • Shopping lists, phone calls, house chores • Part-time job at WalMart, forgets to check-in, checks-in wrong time/day • Cannot remember where to go when he gets to shop.

8. Mr. DAW, 75 yrs - retired accountant brought in by concerned wife and daughter • Impaired memory: • Shopping lists, phone calls, house chores • Part-time job at WalMart, forgets to check-in, checks-in wrong time/day • Cannot remember where to go when he gets to shop. • Serious errors tax return forms, second year, refuses to admit it • Generous donations, cannot afford, to charitable organizations, • Checks bounced twice • Gets quite irritable and at times verbally aggressive

9. Mr. DAW, 75 yrs - retired accountant brought in by concerned wife and daughter • Impaired memory: • Shopping lists, phone calls, house chores • Part-time job at WalMart, forgets to check-in, checks-in wrong time/day • Cannot remember where to go when he gets to shop. • Serious errors tax return forms, second year, refuses to admit it • Generous donations, cannot afford, to charitable organizations, • Checks bounced twice • Gets quite irritable and at times verbally aggressive • Onset: daughter: 3 years ago when he retired, wife: earlier • Gradual deterioration

10. Mr. DAW, 75 yrs - retired accountant brought in by concerned wife and daughter • Impaired memory: • Shopping lists, phone calls, house chores • Part-time job at WalMart, forgets to check-in, checks-in wrong time/day • Cannot remember where to go when he gets to shop. • Serious errors tax return forms, second year, refuses to admit it • Generous donations, cannot afford, to charitable organizations, • Checks bounced twice • Gets quite irritable and at times verbally aggressive • Onset: daughter: 3 years ago when he retired, wife: earlier • Gradual deterioration • He denies these allegations • He accuses his wife of stealing his money and being unfaithful to him

11. Mr. DAW, 75 yrs - retired accountant brought in by concerned wife and daughter 1. Deterioration from previous level of cognitive functions? • Yes: Successful accountant

12. Mr. DAW, 75 yrs - retired accountant brought in by concerned wife and daughter 2. Memory impairment: Global or selective? • Shopping lists, phone calls, house chores

13. Mr. DAW, 75 yrs - retired accountant brought in by concerned wife and daughter 2. Memory impairment: Global or selective? • Shopping lists, phone calls, house chores • Forgets to check-in at work, checks-in at wrong time/day • Cannot remember where to go when he gets to work

14. Mr. DAW, 75 yrs - retired accountant brought in by concerned wife and daughter 3. Memory impairment interfering with daily activities? Forgets to check-in, checks-in at wrong time/day Cannot remember where to go when he gets to work

15. Mr. DAW, 75 yrs - retired accountant brought in by concerned wife and daughter 4. Impaired judgment? • Checks bounced twice • Serious errors tax return forms, second year, refuses to admit it • Generous donations, cannot afford, to charitable organizations

16. Mr. DAW, 75 yrs - retired accountant brought in by concerned wife and daughter 5. Onset: Insidious or Sudden ? Daughter: 3 years ago when he retired Wife: earlier ? 5 years

17. Mr. DAW, 75 yrs - retired accountant brought in by concerned wife and daughter 6. Insight ? No insight – denies allegations Blames wife and others

18. Mr. DAW, 75 yrs - retired accountant brought in by concerned wife and daughter 7. Impairment of other cognitive domains? • Amnesia: Global

19. Mr. DAW, 75 yrs - retired accountant brought in by concerned wife and daughter MMSE: 22/30 Anomia Date, day of week, month Remembered only 1 of the 3 words Refused to write a sentence Refused to copy geometrical shape

20. Mr. DAW, 75 yrs - retired accountant brought in by concerned wife and daughter MMSE: 23/30 Anomia, Date, day of week, month Remembered only 1 of the 3 words Refused to write a sentence Refused to copy geometrical shape

21. Mr. DAW, 75 yrs - retired accountant brought in by concerned wife and daughter MMSE: 23/30 Anomia, Apraxia Date, day of week, month Remembered only 1 of the 3 words Refused to write a sentence Refused to copy geometrical shape 11:10

22. Mr. DAW, 75 yrs - retired accountant brought in by concerned wife and daughter MMSE: 23/30 Anomia, Apraxia Date, day of week, month Remembered only 1 of the 3 words Refused to write a sentence Refused to copy geometrical shape 11:10 Unable to tie shoe laces Wife helps him get dress

23. Mr. DAW, 75 yrs - retired accountant brought in by concerned wife and daughter MMSE: 23/30 Anomia, Apraxia Date, day of week, month Remembered only 1 of the 3 words Refused to write a sentence Refused to copy geometrical shape 11:10 Not aware of current events; “I don’t really care!” Cannot recall any of the TV programs he watches Thought he had about $175 in his wallet, only had $22, accuses his wife

24. Mr. DAW, 75 yrs - retired accountant brought in by concerned wife and daughter 7. Impairment of other cognitive domains? • Amnesia: Global • Anomia: Inability to name objects • Apraxia: Inability to carry out voluntary activities, no paralysis

25. Mr. DAW, 75 yrs - retired accountant brought in by concerned wife and daughter • Amnesia • Anomia • Apraxia

26. Mr. DAW, 75 yrs - retired accountant brought in by concerned wife and daughter • Amnesia • Anomia • Apraxia Impaired judgment

27. Mr. DAW, 75 yrs - retired accountant brought in by concerned wife and daughter • Amnesia • Anomia • Apraxia Impaired judgment No insight

28. Mr. DAW, 75 yrs - retired accountant brought in by concerned wife and daughter Insidious onset • Amnesia • Anomia • Apraxia Impaired judgment No insight

29. Mr. DAW, 75 yrs - retired accountant brought in by concerned wife and daughter Insidious onset • Amnesia • Anomia • Apraxia Probable Alzheimer’s Disease Impaired judgment No insight

30. Mr. DAW, 75 yrs - retired accountant brought in by concerned wife and daughter Insidious onset Paranoid delusions • Amnesia • Anomia • Apraxia Probable Alzheimer’s Disease Impaired judgment No insight

31. Mr. DAW, 75 yrs - retired accountant brought in by concerned wife and daughter Insidious onset Agnosia Paranoid delusions • Amnesia • Anomia • Apraxia Probable Alzheimer’s Disease Impaired judgment No insight

32. Stages of Alzheimer’s Disease MILD 2 to 4 Years

33. Stages of Alzheimer’s Disease MILD 2 to 4 Years Protect from others

34. Stages of Alzheimer’s Disease MILD 2 to 4 Years Protect from others MODERATE 2 to 10 Years

35. Stages of Alzheimer’s Disease MILD 2 to 4 Years Protect from others MODERATE 2 to 10 Years Protect from self

36. Stages of Alzheimer’s Disease MILD 2 to 4 Years Protect from others MODERATE 2 to 10 Years Protect from self SEVERE 1 TO 3 Years

37. Stages of Alzheimer’s Disease MILD 2 to 4 Years Protect from others MODERATE 2 to 10 Years Protect from self SEVERE 1 TO 3 Years Nursing Care

38. Mr. LMD, 65 years, CEO – 8 months Wants zolpidem sublingual tablets to prevent jet-lag • Chronic jet-lag – Constantly tired • Poor quality sleep • Often somnolent during day

39. Mr. LMD, 65 years, CEO – 8 months Wants zolpidem sublingual tablets to prevent jet-lag • Chronic jet-lag – Constantly tired • Poor quality sleep • Often somnolent during day • At times difficulties concentrating • Needs “power naps”

40. Mr. LMD, 65 years, CEO – 8 months Wants zolpidem sublingual tablets to prevent jet-lag • Chronic jet-lag – Constantly tired • Poor quality sleep • Often somnolent during day • At times difficulties concentrating • Needs “power naps” • At a recent meeting: • Hallucinations • Became confused • Similar episode few years ago

41. Mr. LMD, 65 years, CEO – 8 months Wants zolpidem sublingual tablets to prevent jet-lag • Chronic jet-lag – Constantly tired • Poor quality sleep • Often somnolent during day • At times difficulties concentrating • Needs “power naps” • At a recent meeting: • Hallucinations • Became confused • Similar episode few years ago • Frequent nightmares • often wake him up

42. Mr. LMD, 65 years, CEO – 8 months Wants zolpidem sublingual tablets to prevent jet-lag Cognitive impairment interfering with activities • Chronic jet-lag – Constantly tired • Poor quality sleep • Often somnolent during day • At times difficulties concentrating • Needs “power naps” • At a recent meeting: • Hallucinations • Became confused • Similar episode few years ago • Frequent nightmares • often wake him up

43. Mr. LMD, 65 years, CEO – 8 months Wants zolpidem sublingual tablets to prevent jet-lag Cognitive impairment interfering with activities • Chronic jet-lag – Constantly tired • Poor quality sleep • Often somnolent during day • At times difficulties concentrating • Needs “power naps” • At a recent meeting: • Hallucinations • Became confused • Similar episode few years ago • Frequent nightmares • often wake him up Fluctuations: alertness confusion

44. Mr. LMD, 65 years, CEO – 8 months Wants zolpidem sublingual tablets to prevent jet-lag Cognitive impairment interfering with activities • Chronic jet-lag – Constantly tired • Poor quality sleep • Often somnolent during day • At times difficulties concentrating • Needs “power naps” • At a recent meeting: • Hallucinations • Became confused • Similar episode few years ago • Frequent nightmares • often wake him up Fluctuations: alertness confusion Hallucinations: formed

45. Mr. LMD, 65 years, CEO – 8 months Wants zolpidem sublingual tablets to prevent jet-lag Cognitive impairment interfering with activities • Chronic jet-lag – Constantly tired • Poor quality sleep • Often somnolent during day • At times difficulties concentrating • Needs “power naps” • At a recent meeting: • Hallucinations • Became confused • Similar episode few years ago • Frequent nightmares • often wake him up Fluctuations: alertness confusion Hallucinations: formed REM Behavioral Disorder

46. Mr. LMD, 65 years, CEO – 8 months Wants zolpidem sublingual tablets to prevent jet-lag Cognitive impairment interfering with activities • Chronic jet-lag – Constantly tired • Poor quality sleep • Often somnolent during day • At times difficulties concentrating • Needs “power naps” • At a recent meeting: • Hallucinations • Became confused • Similar episode few years ago • Frequent nightmares • often wake him up • Fine tremors hands Fluctuations: alertness confusion Hallucinations: formed REM Behavioral Disorder

47. Mr. LMD, 65 years, CEO – 8 months Wants zolpidem sublingual tablets to prevent jet-lag Cognitive impairment interfering with activities • Chronic jet-lag – Constantly tired • Poor quality sleep • Often somnolent during day • At times difficulties concentrating • Needs “power naps” • At a recent meeting: • Hallucinations • Became confused • Similar episode few years ago • Frequent nightmares • often wake him up • Fine tremors left hand Fluctuations: alertness confusion Hallucinations: formed REM Behavioral Disorder Extra-pyramidal signs

48. Mr. LMD, 65 years, CEO – 8 months Wants zolpidem sublingual tablets to prevent jet-lag Cognitive impairment interfering with activities • Chronic jet-lag – Constantly tired • Poor quality sleep • Often somnolent during day • At times difficulties concentrating • Needs “power naps” • At a recent meeting: • Hallucinations • Became confused • Similar episode few years ago • Frequent nightmares • often wake him up • Fine tremors left hand Fluctuations: alertness confusion Hallucinations: formed REM Behavioral Disorder Extra-pyramidal signs Lewy Body Dementia

49. Dementia with Lewy BodiesThird Report – DLB Consortium – 2005Neurology 2005;65(12):1863-72 • Central Feature:Dementia • Core Features: Fluctuations, hallucinations, extrapyramidal signs (ONE-Year Rule) • Suggestive Features: • REM sleep behavior disorder (RBD) • Severe neuroleptic sensitivity • Dopamine transport (DAT) imaging, various ligandsNigrostriataldopaminergic system - SPECT/PET Occipital lobes: 1ry, 2ndry visual cortex # Alzheimer’s disease • Sympathetic Skin Response & Heart Rate Variability BMJ 2013;3:e001796

50. Mr. DTF, 65 years WM – English Literature ProfessorInappropriate behavior • Sexual harassment complaint • Profane language • Impatient • Walked out of classroom & meetings • Pornographic material • Explicit sexual remarks • Thrown out of night club • Difficulties finding correct word • His appointment may be terminated