Specific Learning Objectives
A. Introduction
Before reviewing the learning objectives and content, please take the Pre-Test.
Please review the Objectives, Content material, and Cases before our class session. We will apply the tasks in the Skills Objectives to these cases, and you should think about them ahead of time.
B. Attitudes - medical students will:
C. Knowledge - Medical students should be able to:
D. Skills - Medical students should be able to:
E. Readings
Additional resources and readings are suggested at the end of each section.
G. Cases
Prevalence and Impact
Dementia affects about 6-8% of people older than 65 years but about 50% of those are older than 85 years of age. The prevalence doubles every five years after aged 60. Alzheimer’s disease affects 4 million people in the United States. By 2040, 14 million people will be diagnosed with Alzheimer’s disease in the U.S. Vascular dementia consists of 15-25% of the progressive dementia types. The anticipated changes in the US population indicate a massive increase in cases of dementia as the baby-boom generation ages.
Dementia has a multifactorial impact on society. The financial burden derives from medical costs, long term care, home care, and lost productivity of the caregivers. In 1998, the estimated cost for Alzheimer’s disease was $200 million which increased to $100 billion in 2003
Aside from the financial cost, the caregivers, family and patients suffer from emotional turmoil with dynamic nature of dementia.Suggested Reading: (http://alzheimers.infopop.cc/eve/ubb.x) or by reading The 36-Hour Day : A Family Guide to Caring for Persons With Alzheimer Disease, Related Dementing Illnesses, and Memory Loss in Later Life by Nancy L. Mace, Peter V. Rabins
MCI can be defined as a transitional state between the cognition of normal aging and mild dementia without functional impairment.
There is a risk of progression to Alzheimer’s disease at 12% per year as compared to 2% for the general population. Symptoms may be present for a maximum of 7 years prior to progressing to moderate-severe dementia
Physical activity and higher levels of education could potentially decrease the risk of Alzheimer’s. Antioxidants have been studied with beneficial results from the use of high dose Vitamin E, but there are risks of cardiovascular adverse events on these doses.
Estrogen therapy has been used for the reduction in the overall risk of Alzheimer’s Disease in postmenopausal women. More recent studies have not demonstrated any protective effect from hormonal therapy on cognition in older women.
NSAIDs have also been evaluated with new data supporting benefit from COX-2 inhibitors, but the risk versus benefit is unclear.
Pick’s Disease and Creutzfeldt-Jacob Disease (CJD) are rare conditions that possess unique features including young onset. CJD is a rapidly progressive condition that affects about one in every million people per year worldwide.
Huntington’s Disease, Wilson’s Disease, and Parkinson’s Disease can lead to cognitive impairment in the late stages of the diseases.
Brain insults may sufficiently severe to cause dementia from trauma with risk of subdural hematoma, normal pressure hydrocephalus, infection (HIV, syphilis, viral encephalitis), and toxins (alcohol)
This is a neurodegenerative disorder with the following symptoms: resting tremor, rigidity, bradykinesia, postural instability, festinating gait and overall lack of voluntary movement.
Cognitive impairment can have a late onset in Parkinson’s patients, thus the diagnosis should differentiate dementia from Parkinson’s disease.
This form of dementia is an atypical presentation of depression. The features include altered sleep, change in appetite, social isolation, irritable, easy agitation, and withdrawal from personal interests.
Numerous conditions could contribute to the presentation of dementia. The conditions are thyroid disorder (hyper or hypo), hyperparathyroidism, tertiary syphilis, B12 deficiency or hepatic disease.
A. Criteria and DefinitionsDiagnostic criteria for all types of dementia are based on the type and extent of deficits.
- Must have MULTIPLE cognitive deficits with BOTH features:
- AMNESIA; memory impairment (impaired ability to learn new information or to recall previously learned information
- COGNITIVE DISTURBANCE; one (or more) of the following findings click here
- Aphasia (language disturbances)
- Apraxia (impaired ability to carry out motor activities despite intact motor function)
- Agnosia (failure to recognize or identify objects despite intact sensory function)
- Disturbance in executive functioning (i.e., planning, organizing, sequencing, abstracting)
- Cognitive deficits must be a decline from previous functioning AND significantly impact function. The diagnostic process evaluates the impact of the functional impairments on one’s social or occupational environments.
This implies that physician must find out about previous status (from family, other informants, medical records etc) in addition to documenting cognitive deficits in order to make the diagnosis of any dementia.
- Additional diagnostic criteria for Alzheimer’s Dementia
- Exclusion of other conditions that can cause progressive deficits in memory and cognition (e.g. cerebrovascular disease, Parkinsonism, normal pressure hydrocephalus, intracranial pathology)
- Exclusion of delirium and medical conditions that could account for symptoms (e.g. thyroid abnormalities, vitamin B12 or folate deficiency, metabolic abnormalities, infections)
- Exclusion of substance-induced conditions (i.e. alcohol)
- Exclusion of Axis I disorder (e.g. major depressive disorder, schizophrenia)
B. Stages of Alzheimer’s Dementia
Level of Functional Impairment
Ability to Perform Specific Functions
Mild Cognitive Impairment
MMSE 26-30
Preclinical state with potential risk of progressing to dementia
Mild
MSSE 22-28
Likely to have difficulties with balancing a checkbook, preparing a complex meal, or managing multiple medications
Moderate
MSSE 10-21
Occurs 2-8 years from symptom onset
Difficulties with simpler food preparation, household cleanup, and yard work and may require assistance with some aspects of self-care (e.g., reminders to use the bathroom, help with fasteners or shaving)
Severe
MSSE 0-9
Occurs 6-12 years from symptom onset
Requires considerable assistance with personal care, including feeding, grooming, and toileting. Unintelligible verbal output
Profound
Largely oblivious to surroundings and almost completely dependent on caregivers
Terminal
Generally bed bound, requires constant care, and may be susceptible to accidents and infectious diseases which lead to one’s death
Current practice recommendations (see references):
- Detailed history from the patient and outside sources about usual level of functioning, type and rate of decline, current medical symptoms, previous medical history, medications (including non-prescription and “alternative” medications) and substance abuse.
- Use the history to detect conditions that can cause or exacerbate cognitive decline
- Complete physical examination focusing on signs of conditions related to dementia such as vascular disease, Parkinsonism, neurological conditions.
- Documenting cognitive function with screening tests:
- Mini Mental Status Exam (MMSE) - need a form that is adequately visible to the examinee
- Clock drawing is a test of visuospatial abilities.
- Language can be tested with use of naming set number of items (i.e. animals, fruits, vegetables) in a timed period
- Geriatric Depression Scale (GDS) is helpful in providing diagnostic evaluation of pseudodementia and depression.
- Documenting baseline ADLs and IADLs
- Screening for depression Geriatric Depression Scale (GDS)
- Laboratory testing. Evidence-based review currently only supports
- complete blood count
- folate
- serum B12
- serum electrolytes
- glucose
- thyroid function tests
- liver function tests
- RPR if the patient has a specific risk factor
- CT or MRI. Although the yield is small and the benefit controversial, guidelines currently recommend noncontrast CT or MRI for most patients to identify pathology such as neoplasm, subdural hematoma, or normal pressure hydrocephalus.
- Alzheimer’s disease – hippocampal & cortical atrophy
- Vascular – multiple vascular lesions
- Fronto-temporal dementia – frontal and /or temporal atrophy
- Pet Scan or SPECT can be useful it CT or MRI demonstrate uncertain diagnosis, but not recommended for routine use
- EEG is indicated for atypical presentation or if history of head trauma
- Lewy Body Dementia demonstrates transient slow wave activity in the temporal regions.
Suggested Readings
References: Primary Care Geriatrics: a case-based approach. Chapter 16
Adelman AM, Daly MP. Initial evaluation of the patients with suspect dementia. Am Fam Physician 2005;71:1745-50 (http://www.aafp.org/afp/20050501/1745.html)
Evidence-based guidelines for diagnosis of dementia are regularly updated (http://www.aan.com/go/practice/guidelines)
Review article http://www.aafp.org/afp/20050501/1745.html
See AAN Dementia Encounter Kit http://www.aan.com/go/practice/quality/dementia
- Confirm that patients meets criteria for dementia
- Establish stage of dementia
- Define the cause of dementia if possible.
- Address coexisting conditions and impairments. Identify conditions that can be reversed or improved
- Individualize assessment (work-up)
Diagnostic criteria for all types of dementia are based on the type and extent of deficits.
1. Must have MULTIPLE cognitive deficits with BOTH features:
- AMNESIA; memory impairment (impaired ability to learn new information or to recall previously learned information
- COGNITIVE DISTURBANCE; one (or more) of the following findings (link to chart below)
- Aphasia (language disturbances)
- Apraxia (impaired ability to carry out motor activities despite intact motor function)
- Agnosia (failure to recognize or identify objects despite intact sensory function)
- Disturbance in executive functioning (i.e., planning, organizing, sequencing, abstracting).
2. Cognitive deficits must be a decline from previous functioning AND significantly impact function. The diagnostic process evaluates the impact of the functional impairments on one’s social or occupational environments.
- This implies that physician must find out about previous status (from family, other informants, medical records etc) in addition to documenting cognitive deficits in order to make the diagnosis of any dementia.
Neurological Signs Behavioral Consequences Possible misinterpretation by the Caregivers Amnesia (memory loss)
- Repeating questions
- Misplace objects
“Frustrating”
“Paranoid”Aphasia (inability to speak or understand spoken language)
- Unable to follow directions
- Does not engage in conversation
“Uncooperative”
“Withdrawn”Apraxia (loss of ability to coordinate learned movements despite intact motor function)
- Unable to use utensils
- Unable to dress oneself
- Unable to use the toilet
“Won’t eat”
“Uncooperative”
“Incontinent”Agnosia (inability to recognize what is seen despite intact sensory function)
- Cannot recognize faces
- Cannot recognize home or familiar objects
“Frightened, combative”
“Wandering”
“Stealing others’ belongings”Altered Executive Function
- Unable to plan or organize
- Unable to think abstractly
“Leaves the stove on or the water in the faucet running”
Chart Source: Alzheimer’s Association. Key Elements of Dementia Care. Chicago, IL; Alzheimer’s Disease and Related Disorders Association, Inc. 1997B. Differential Diagnoses
Physicians should be especially alert for these conditions in “dementia” that presents suddenly, has new neurological or physical findings, or does not fit the classical presentation of the major dementia syndromes.
**Medication overuse or interactions are probably the most common source of dementia-like presentations.
Other conditions include
- Depression
- Delirium (due in turn to medications, medical conditions etc)
- Hypo or hyperthyroidism
- Metabolic upsets (e.g. electrolyte imbalances, renal failure)
- Infections (esp. HIV, neurosyphilis)
- Normal pressure hydrocephalus
- Trauma (including subdural hematoma)
- B12 deficiency
- Joint pain
- Constipation
- Insomnia
- Social isolation
On average, patients live for 6-10 years after the diagnosis of dementia is made. Living up to 20 years with dementia is possible. Overall, the impact of the disease should be minimized by anticipating and addressing factors that alter the individual’s quality of life. Thus, the physician’s responsibility is to work with patients, family members, and other providers to:
A. Goals of Care
- Preserve & optimize function & quality of life
- Preserve long term memory
- Prolong residence in familiar surroundings or their own home
- Establish plan for preferences in residences in the future
- Address patient’s preferences for ongoing care and end of life care, including advance directives, living will and resuscitation wishes
- Establish health care proxy
Suggested Readings
References: Primary Care Geriatrics: a case-based approach. Chapter 16
Review article: Cummings JL, Cherry D, Kohatsu ND et al. guidelines for managing Alzheimer’s disease: Part II Treatment. Am Fam Physic 2002;65:2525-34 http://www.aafp.org/afp/20020615/2525.html
Evidence-based guidelines for management of dementia are regularly updated e.g. http://www.neurology.org/cgi/reprint/56/9/1154.pdf
B. Management of Cognitive Impairment
1. Non-pharmacologic.
A patient with dementia benefits from a familiar environment and established daily routines. The environment can be enhanced with visual cues including labeled pictures or drawers for specific items. Additional environmental adjustments are to ensure good light during the day and to remove obstructing hazards. Regular routines for meals, sleeping, self-care, and daily activities should be consistent. Ideally, the patient’s independence should be maintained as much as possible in his/her daily routines.
Other common therapies include music therapy, “white noise” (low level background noise) reminiscence therapy (family video or audio tapes), pet therapy, and formal cognitive training such a repetitive practicing of tasks or memory games.
Suggested Link:
Alzheimer’s Friendly Home http://www.environmentalgeriatrics.com/home_mod/alz_friendly_home.html
Suggested Readings
(Ref: Clare L, Woods RT, Moniz Cook ED, Orrell M, Spector A. Cognitive rehabilitation and cognitive training for early-stage Alzheimer's disease and vascular dementia. Cochrane Database of Systematic Reviews 2006 ;(4):CD003260 (http://www.mrw.interscience.wiley.com/cochrane/clsysrev/articles/CD003260/frame.html)
2. Pharmacologic
The two classes of medications with evidence of benefit for cognitive problems currently available are:
1. Cholinesterase inhibitors (ChEIs) e.g. donepezil, rivastigmine, galantamine
- FDA Indications include mild to moderate dementia of Alzheimer’s type with the benefit of slowing the disease progression and preserve current functional status. The medications do not reverse the disease.
- Need to titrate up the dose slowly to avoid side effects
- Side effects include gastrointestinal symptoms, dizziness, leg cramps, bradycardia, insomnia
- Costs
- Rivastigmine 3 mg (60): $170.01 or 6 mg (60): $169.74
- Donepezil 5 mg (30): $159.99 or 10 mg (30): $149.99
- Razadyne
- Short acting 4 mg (30): $79.98 or 12 mg (30): $79.98
- Extended Release – 8 mg (30): $167.45 or 24 mg (30): $167.45
2. N-methyl-D-aspartic acid receptor agonists (NMDAs) e.g. memantine.
Practice recommendations emphasize:
- FDA Indications are for moderate to severe dementia of Alzheimer’s type. The medication does not alter or prevent the disease.
- Administration can be monotherapy or combination therapy with donepezil or razadyne
- Need slow titration over weeks and renal dosing
- Cost - 5 mg (60): $149.30 or10 mg (60): $136.36
- Medications can only slow the progression of dementia. Evidence of long-term benefit is limited and controversial
- Individual medications are only approved for specific forms of dementia (e.g. Alzheimer’s disease) and certain stages (usually mild-moderate).
- Start earlier in the disease to maintain higher levels of function
- Consider medication cessation if loss of basic ADLs despite the medication
- Consider cost and expense balances given the high cost of the medications
- Potential benefit must be balanced against side-effect and cost
- Need to reassess every 3-4 months
Current practice guidelines support a trial of treatment in mild to moderate Alzheimer’s disease. Although medications can offer hope, the rate of decline without therapy and therefore the net benefit of therapy, cannot be known. Ending a trial of treatment can be very difficult for families, physicians, and care staff. See reference: (http://www.aan.com/professionals/practice/pdfs/dementia_guideline.pdf)
Suggested Reference:
Information on individual medications is summarized in: Cummings JL, Cherry D, Kohatsu ND et al. guidelines for managing Alzheimer’s disease: Part II Treatment. Am Fam Physic 2002;65:2525-34 http://www.aafp.org/afp/20020615/2525.html
C. Management of Functional Disabilities
Functional abilities are monitored by reports from the patient and caregivers plus documenting ADLs and IADLs. This information is subjective and should be augmented by periodic formal functional assessment.
Addressing functional disabilities and minimizing decline requires:
- Optimizing cognitive abilities (see above)
- Identifying and addressing physical limitations (vision, hearing, mobility, pain, anxiety etc.)
- Providing the optimal environment and equipment to facilitate function and to reduce the risk of injury.
- Providing the patient and caregivers with resources
- Occupational and physical therapists for home safety and individual functional evaluations
- Area Agencies of Aging: (http://www.n4a.org/aboutn4a.cfm)
- Alzheimer’s Association (http://www.alz.org/)
D. Management of Behavioral/Psychological Symptoms
Behavioral and psychological problems are extremely common in dementia and cause the greatest distress to caretakers. Problems may range from withdrawal and self neglect to wandering, aggression, combativeness, and inappropriate sexual behavior. It is important to stress that the behavior is a symptom of the disease and probably not a deliberate, reasoned act by the patient.
The general approach to management is to determine the following aspects of the behavior.
- Description of Difficult Behaviors
- What was the individual doing?
- What made the individual better or worse?
- What was happening prior to behavior change?
- Were there any changes in the environment?
- Do certain individuals induce behavior changes?
- Determine the frequency of behaviors; and determine if treatment is indicated. --- Treatment may be as needed or chronic for behavioral management
- Severity of behaviors can be assessed by the extent of individual and social disruption.
- Precipitating or exacerbating factors
A full description of the problem plus an appropriate medical history and examination may identify factors that cause or exacerbate the behavioral problem. Common factors include:
- Environmental stressors – change in living arrangement or new admission, recent hospitalization, disruption of routines, new difficulties/barriers in daily functioning
- Change in coping abilities of caregiver – overwhelmed by additional responsibilities, health problems, inability to accept the change in the patient (especially if the behavior is very different from previous personality)
- Medical problems or symptoms – any condition causing pain or distress may be expressed as a behavioral problem (including constipation, fecal impaction, urinary retention with bladder distention, exacerbation of arthritis, or indigestion)
- Exacerbation of conditions such as heart failure, cardiac dysrhythmia, or develop new physical such as stroke, cancer, hypo/hyperthyroidism or myocardial infarction
- Medication side effect
One can determine the cause of a new behavioral problem in patients with dementia, the mnemonic SEDIMENT is useful.
For list of non-pharmacological measure see Cummings JL, Cherry D, Kohatsu ND et al. guidelines for managing Alzheimer’s disease: Part II Treatment. Am Fam Physic 2002;65:2525-34 (http://www.aafp.org/afp/20020615/2525.html)
- Nonpharmacologic of Behavior
- Environmental adjustments can include optimize sleep with limited light at night, reduce noise and distractions at night
- Situational adjustments may be indicated as in the case of aggression during bathing. The adjustments could be reassurance, background music, and flexible bathing methods.
- Pharmacologic Interventions
Psychotropic medications should be used in dementia only for specific indications and only after other approaches have not altered the behavior. Justification for psychotropic medications should be carefully documented because of drug interactions, side-effects, increased mortality, and potential over-medication of elderly patients.
- Indications for psychotropic medications:
- Violent behavior dangerous to patient or others
- Distressing hallucinations, delusions, or paranoid ideation
- Abrupt worsening of condition such as mental illness, e.g., schizophrenia
- Depression with impaired function
- Abrupt changes in behavior associated with an acute medical condition
- Medication options
- Neuroleptics or mood stabilizers
- There is not a FDA indication for dementia related psychosis. However, neuroleptics are commonly used in clinical practice as off label use.
- Typical neuroleptics: Haloperidol (0.25 – 0.5 mg daily dose)
- Side effects include extrapyramidal movement signs and QT interval prolongation with subsequent potential of progression to torsades de pointes
- Atypical anti-psychotics: risperidone, quetiapine, clozapine and olanzapine
- Risk of sedation, hypotension, and weight gain
- Severe side effects include prolong QT interval
- In particular, clozapine has the severe side effect of agranulocytosis
- Anxiolytics are indicated if the patient is anxious or agitated.
- Benzodiazepines (i.e., lorazepam)
- Buspirone
- Side effects including sedation, confusion, altered balance and increased risk of falls with subsequent hip fracture risks
- Antidepressants
- Indications for treatment include anxiety, agitation, impulsivity and depression. Treatment is encouraged if symptoms are contributing to behavior changes.
- SSRIs
- Mood stabilizers
- Options are divalproex or gabapentin with limited knowledge available on the mechanism of action for behaviors.
For a full review see as suggested references.
Rayner AV, O’Brien JG, Scoenbachler B. Behavior Disorders of Dementia: Recognition and Treatment. Am Fam Physic 2006;73:647-52 (http://www.aafp.org/afp/20060215/647.pdf)
Cummings JL, Cherry D, Kohatsu ND et al. guidelines for managing Alzheimer’s disease: Part II Treatment. Am Fam Physic 2002;65:2525-34 http://www.aafp.org/afp/20020615/2525.html
Autonomy should be preserved by incorporating the demented patient’s opinions (when possible) in decision making about his/her life. The physicians and caregivers need to complete serial assessments of decision making capacity due to potential for change in the cognition.
Identity should be preserved with maintenance of family relationships and continuation of preferred activities and interests.
Advance directives need to be established for the purpose of respecting and documenting the patients’ medical care preferences in the event of becoming incapacitated. The directives need to address the health care and financial management decisions and appoint proxy for decision making.
Family members and professional caregivers require significant support to manage the heavy physical, emotional, and financial challenges of caring for patients with dementia. Coping strategies may range from complete self-sacrifice in order to care for the patient to avoidance. Old or ongoing issues (usually about relationships and/or finances) within the family may complicate how individuals adapt to an individual with dementia and may significantly complicate management decisions.
Physicians can support caregivers by:
- Being available and responsive to family concerns and values
- Monitoring the caregiver for signs of depression
- Recommending resources, websites, and organizations
- Assisting with management decisions such as insurance, long-term care and related paperwork/forms.
- Helping to advocate for necessary assistive devices
- Working with families to control costs e.g. reviewing drugs, using generics.
- Emphasizing the importance of caregiver health and respite care to avoid potential risk of decline in quality of care for the patient
- Helping caregivers to find positive meaning in their experience and to use it to help others.
Caregivers may experience significant distress after the death of the dementia patient. Many express guilt and a sense of loss of purpose even if the death removes a substantial burden of care. They often benefit by volunteering for an organization serving the elderly or a hospice.
Suggested Review Article
Parks SM, Novielli KD A Practical Guide to Caring for Caregivers. Am Fam Physician 2000;62:2613-20,2621-2. (http://www.aafp.org/afp/20001215/2613.html)
Mr. White
Mr. White is an 82 year old male who has been brought to the clinic by his daughter. He has lived alone in their family home since his wife died 5 years ago. Over the past year, his daughter has noticed slow changes in his behavior. He stopped fixing his own meals and he frequently does not eat food that she brings for him. In observation of his dirty laundry and unkempt yard, she feels that his home and lawn care have declined. Prior to a year ago, he maintained a clean home with a well manicured lawn. Mr. White argues against these criticisms by stating that his daughter is only “fussing”. From this point, he is able to clearly describe activities of interest to him especially outings to visit his family. He also provides his medical history with limited help from his daughter. In the past, he has been treated for hypertension and he was hospitalized 2 years ago for an episode consistent with unstable angina with a negative exercise stress test. For the past two years, he has maintained his own medical management with aspirin 81 mg daily, amlodipine 10 mg daily and hydrochlorothiazide 25 mg daily. Although, he has not followed up with a physician after that episode.
Questions:
Mrs. Hinck
Mrs. Hinck is a 73 year old Caucasian female presents with her daughter who provides the majority of the history due to limited responses from Mrs. H. Approximately nine months ago, Mrs. H’s husband had a sudden heart attack which led to his death. She has been progressively withdrawing from her regular activities and interests that she used to enjoy in the past. She has also decreased in her self-care which was a significant change from her past meticulous way of dressing. She needs reminders to bath and clean herself. She will occasionally forget to do tasks after her daughter has just asked her to do something. In review of her history, she does not have any major medical problems for which she has been treated in the past. She did have a total abdominal hysterectomy with unilateral salpingoophorectomy due to excessive peripartum bleeding in the past. She has never been on hormone replacement. She did smoke in the past but her daughter notes that this was for a couple of years approximately 40 years ago. There was question of intermittent alcohol use, but there was not a concern for excessive use. Her family history is negative for dementia. Physical exam reveals a thin (BMI – 19), alert 73 year old CF who appears older than her stated age. Otherwise, there are no focal findings on her exam. Labs are done to demonstrate no evidence of urinary infection and she has normal blood count with normal thyroid studies. In further discussion with Mrs. H, she has poor sleep, decreased appetite, and feelings of worthlessness.
Questions:
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