Dementia a family s feared diagnosis

1. Dementia a family�s feared diagnosis CDR Carol L. Blackwood USN (retired) Grace Cottage Hospital Family Medicine and Geriatrics

2. Rural Healthcare in Vermont

3. The dreaded �post it� from the family

4. Dementia Screening (EBM) No national organization recommends routine screening for dementia syndrome. The 2003 Recommendations from the US Preventive Services Task Force (USPSTF) found insufficient evidence to make a recommendation either for or against population based dementia screening Dementia in general population >65yrs of age is between 2-12% Dementia is difficult to to indentify without specific testing of patients. More than 50% of patients with dementia have never been diagnosed by a physician

5. If a patient complains of dementia or memory loss Evaluate, but� Consider other diagnoses: Depression Stress/Anxiety Poor registration

6. Dementia Defined as a Significant decline in 2 or more areas of cognition severe enough to affect daily life. Usually occurs after age 60yrs. Costs $100 billion annually Alzheimer�s Disease is most common type (4 million Americans now affected). Increasing incidence. Estimate: In 2040, 14 million Americans (greater than the populations of New York City and Los Angeles combined!) will have Alzheimer�s. Burden of illness is very heavy on family.

8. Dementia Prevalence

10. Primary prevention of dementia (EBM) Research has produced no effective approach for primary prevention of dementia. Chemoprevention has been advocated, but data on effectiveness are lacking. Although control of hypertension reduces the risk of cerebrovascular accidents, its role in reducing small vessel vascular dementia is less clear. The wealth of literature has been on screening for dementia with the hope of reducing its burden of suffering by earlier intervention.

11. Types of Dementia Alzheimer's (most common 60%) Vascular (2nd most common) Lewy body (5-10%) Frontotemporal Lobar dementia (including Pick disease) (<5%) Parkinson�s Disease Potentially reversible dementias: B12 deficency, hypothyroidism, medication-induced, alcohol-related, hyponatremia, hypercalcemia, depression (pseudodementia), central nervous system neoplasms, chronic subdural hematomas, chronic meningitis, normal pressure hydrocephalus

12. Alzheimer�s Disease Risk Factors Definite risk factors: age family history. Down�s Syndrome APOE*4 allele on chromosome 19. Possible risk factors: Other genes (early onset) Head trauma Low educational achievement Depression

13. Family History Risk Factors for Alzheimer�s Dementia Individuals whose parents both had Alzheimer's disease have a 54% cumulative risk of developing this condition by age 80. This risk is about 1.5 times greater than the risk faced by those with 1 parent with Alzheimer's disease and nearly 5 times greater than for those with neither parent affected. First-degree relatives of patients with Alzheimer's disease have a cumulative lifetime risk of 39%, approximately twice the risk of Alzheimer's disease in the general population.

14. Risk Factors for Alzheimer�s Dementia Some genetic mutations have been associated with Alzheimer's disease. For example, about 20% to 30% of the general population and 45% to 60% of people with late-onset Alzheimer's disease have the apolipoprotein E-4 gene. In a study of people with Down syndrome, 55% of individuals between 50 and 59 years and 75% of those 60 years of age and older had Alzheimer's disease.

15. Risk Factors for Alzheimer�s Dementia Head trauma is also associated with Alzheimer's disease. A case-control study of Alzheimer's disease found the odds ratio of Alzheimer's disease to be 3.5 when comparing patients with previous head trauma to controls.

16. Risk Factors for dementia Cardiovascular risk factors are associated with vascular dementia. The presence of lacunar infarctions leading to symptomatic change is independently related to diastolic blood pressure, serum creatinine, tobacco smoking, carotid stenosis, male sex, and a history of diabetes. A cross-sectional study found all indicators of atherosclerosis (vessel wall thickness, plaques of the carotid arteries, and the ratio of ankle-to-brachial systolic blood pressure) to be associated with all dementias, with odds ratios ranging from 1.3 to 1.9.

17. Dementia Screening Methods Mini Mental Status Exam In a population with 2% dementia: Specificity of 84% Sensitivity of 75% �Gold Standard� Language and education dependent. Clock Draw test

18. Dementia Screening Methods (EBM) the Blessed Orientation Memory Concentration Test (BOMC) the Blessed Information Memory Concentration (BIMC) the Short Test of Mental Status (STMS) the Modified Mini-Mental Status Exam (3MS) the Abbreviated Mental Test (AMT) the Chula Mental Test (CMT) the Mental State Questionnaire (MSQ) the Comprehensive Assessment and Referral Interview Cognitive Scale, Dementia Version (CARE-D) and the Short Portable Mental Status Questionnaire (SPMSQ) These tests were found to have a level of performance similar to that of the MMSE.

19. Screening�IF you do not look, you will not find The percentage of primary care patients over age 65 who have unrecognized dementia is between 2% and 12%. One-half to two-thirds of cases of early dementia are not diagnosed by a routine history and physical examination. Considerable evidence shows that the prevalence of dementia increases with age; thus, the prevalence of missed dementia cases likely increases among older individuals. Be watchful of clues of possible dementia, when choosing who to evaluate.

20. Specific Types of Dementia Alzheimer�s Dementia Vascular Dementia Lewy Body Dementia Frontotemporal lobar

21. Alzheimer�s Disease History Alois Alzheimer first observed the waxy plaques and skeins of tangles in the autopsied brain of a 51yo German woman with dementia in 1907. 100yrs later�Not much has changed. Only on autopsy can Alzheimer�s Disease be definitively diagnosed. Clinical diagnosis Average life span is 8-10yrs from time symptoms start.

23. Alzheimer�s Disease Treatment Cholinesterase Inhibitors (Aricept, Excelon, etc.) FDA indications for all stages of dementia. Modest benefit (on average), delays symptoms about 6mo. Common side effects: nausia and diarrhea. NMDA antagonist (memantine, Namenda) uncompetitive antagonist of the N-methyl-D-aspartate (NMDA) type of glutamate receptors. FDA approved for moderate-severe Alzheimer�s type dementia. Few side effects.

24. Vascular Dementia "multi-infarct dementia" A heterogeneous syndrome rather than a distinct disorder, in which the underlying cause is cerebrovascular disease in some form and its ultimate manifestation is dementia VD may share risk factors with cerebrovascular disease, the evidence is not compelling. Variable degrees of association and, in some studies, no associations have been found for hypertension, diabetes and insulin resistance, dyslipidemia, and heart disease.

25. Vascular Dementia Diagnosis Neuroimaging should be performed in patients with suspected VD by virtue of stroke history, vascular risk factors, abnormal neurologic examination, or a course or symptom complex atypical for AD. MRI is significantly more sensitive than CT however, if sufficient evidence of vascular pathology is seen on CT, an MRI may not be necessary.

26. Vascular Dementia Treatment A population-based nested case-control study revealed a substantively lowered risk of dementia among patients treated with statin therapies but did not distinguish between types of dementia. Cholinesterace inhibitors, calcium channel inhibitors, anti-platlet, HTN treatment, statins have all been studied. No clear treatment plan yet proven.

27. Lewy Body Dementia Dementia plus one of the following: Detailed visual hallucinations Parkinsonian signs (bradykinesis and rigidity) Alteration of alertness or attention May overlap Alzheimer�s dementia and Parkinson�s Disease dementia Early symptoms include driving difficulty (eg, getting lost, misjudging distances, or failing to see stop signs or other cars) and impaired job performance

28. Lewy Body Dementia= presentation 73yo man�s wife tells you when she goes to the mail box, he locks the door behind her, for fear of people breaking in. She then can not get back in the house. He was getting wood out of the garage to board up the windows thinking snipers are trying to get him. His short term memory is normal, and he recognizes his friends and family.

29. Lewy Body Treatment Can be worsened by medications. Behavioral treatment is first line. Cholinesterase inhibitors, may be helpful. No FDA indication. Consider use of antipsycotics if hallucinations are frightening or negatively affecting life. �Black box� warning on antipsycotic medications. Patents die sooner. (sudden cardiac death, age and dose related). Neuroleptics���Do not use. Sensitivity reactions, including exacerbation of parkinsonism, confusion, or autonomic dysfunction.

30. Lewy Body Treatment Treatment of parkinsonian symptoms in Lewy Body dementia is similar to that for Parkinson disease (PD), if somewhat less successful (Parkinson�s drugs may exacerbate psychotic symptoms). Start low, go slow!

31. Frontotemporal Lobar Dementia Younger age of onset than other dementia�s. In patients <65yrs of age, predominance is the same as Alzheimer�s dementia. Deterioration of personality and social functioning. Emotional blunting and loss of insight. Decline in executive functioning, with relatively well preserved short term memory. Clinical diagnosis, Criteria derived by consensus opinion.

32. Frontotemporal Lobar Dementia= presentation

33. Consensus Diagnostic Criteria for Frontotemporal Dementia Core features: Social and interpersonal decline Emotional blunting and loss of insight Insidious onset and gradual progression Supportive features: Behavioral disorder (such as; perseveration, stero-typed behaviors, poor hygiene and rigid thinking) Abnormal speech Physical findings (primitive reflexes, tremor)

34. Workup to Substantiate Frontotemporal Lobar Dementia Diagnosis Recommended: Neuropsychology testing EEG (normal) CT/MRI (frontal or temporal abnormalities)

35. Subtypes of Frontotemporal Lobar Dementia Three sub types: Frontotemporal Progressive nonfluent aphasia Semantic Patients may have symptom overlap.

36. Language Disorders of frontotemporal, and progressive non fluent aphasia Anomia-failure to retrieve words Substituting watcher instead of washer, clothes instead of pants. Agrammatism-loss of grammatical syntax Example �box fell down� not �the box fell on the floor� Phonemic paraphasia Example �cubunker� instead of �cucumber�

37. Semantic subtype features Prosopagnosia- Failure to recognize familiar faces. Associative agnosia- Failure to recognize familiar objects. Loss of empathy, rigid routines and focused preoccupations (works on hobbies all day rather than eating or performing necessary chores).

38. Parkinson�s Dementia Dementia is a common feature of Parkinson disease Depression is common Whether Parkinson�s dementia and dementia with Lewy bodies are distinct disorders, or whether they represent different presentations of the same disease, is an area of debate and investigation. Executive dysfunction is a hallmark feature. This syndrome consists of deficiencies in set shifting, attention, and planning. (driving difficulty (getting lost, misjudging distances, or failing to see stop signs or other cars) impaired job performance, difficulty planning and executing multi-step projects)

39. Parkinson�s Dementia treatment Trial of cholinesterase inhibitors.� Severe psychotic features can be treated with atypical antipsychotic agents. Severe side effects, while not as common as in dementia with Lewy bodies, do occur in a substantial portion of patients with Parkinson�s dementia. Doses should be started at the lowest possible and titrated upward gradually.

40. Patient and Family resource for patients with dementia 36-Hour Day, but Nancy Mace M.A. and Peter V. Rabins, M.D., M.P.H. Chapters on: Related medical issues Behavioral and mood Issues Advance planning Family stress Explaining what is dementia

41. Care Plan for Dementia Dementia does not fit traditional �curative� model. Chronic, progressive� Dementia (esp. Alzheimer�s) often follows a predictable course. Can plan for future events. Patient chosen, �level of care� preferences Palliative care model.

42. Palliative Care Plan for Dementia Palliative care model-maintains quality of life, and comfort for patient and family. Multidisciplinary approach Nurses, social workers, volunteers, etc. Useful throughout entire disease process Decreases costs, use of inappropriate interventions and increases patient comfort

43. Timing of Care Plan Actions for Dementia Diagnosis- Advance directives, wills, power of attorney. Basic education. Later-Stabilize living situation, identify future care providers. More detailed information for family about: burden of care, grieving, financial and emotional impact. Resource information. End stage-Hospice

44. Dementia Hospice Criteria Less than 6 different words on avg day Can not walk (without assistance) Can not sit (without chair arm rests) Loss of smile Loss of ability to hold head up independently Urinary or bladder incontinence In past 12mo, must have had one: pneumonia, septicemia, decub ulcers (multiple stage 3-4), recurrent fever after abx, poor food/fluid intake.

45. One last time� ifAnti-psycotic Medications are used to treat symptoms of Dementia, remember� No FDA indication, commonly done (17% of nursing home patients have antipsycotic meds initiated within 100 days). Risks: extra pyramidal symptoms, sudden cardiac death, falls, hip fractures. Only use if severe symptoms can not be controlled any other way.

46. References Geriatrics Review Syllabus 2006, American Geriatric Society. Geriatrics Division, East Carolina University McDuffie, Everett. A Case of Atypical Early Onset Dementia in a 54yo Female. Clinical Geriatrics. Dec 2007. MacPhee, E. Palliative Care for Patients with Dementia. Annals of Long Term Care. June 2007. USPSTF 2003 Dementia, recommendations and rationale. UpToDate Annals of Long Term Care Dec 2009