Dementia (Case 53)
Jessica L. Israel MD
Case: The patient is a 76-year-old woman who managed a small restaurant with her husband for many years. He died 2 years ago, and now her son does most of the day-to-day work at the restaurant. She has a history of osteoarthritis and well-controlled diabetes mellitus. During the history and physical exam at the office visit, she is pleasant and cooperative. She tells you that she does not know why her son brought her to see you. She says she feels fine.
When you step out of the examination room, her son and his wife are waiting to talk with you. They tell you that they are worried about Mom and that she just hasn’t been the same since her husband died. She now lives alone. She vehemently resists accepting any help and she argues with them if they try to help her. Her son has noticed that her pocketbook is full of bills that she hasn’t paid and old receipts for things she bought many months ago. She has been forgetting appointments and sometimes even forgetting the names of their regular customers at the restaurant. She seems to repeat herself, asking the same question over and over. Some evenings, they believe, she doesn’t bother to cook or eat dinner. They are very worried that she might have a problem with her memory.
Differential Diagnosis
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Alzheimer disease |
Vascular dementia |
Lewy body dementia |
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Frontotemporal dementia |
Mild cognitive impairment |
Delirium |
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Depression |
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Speaking Intelligently
PATIENT CARE
Clinical Thinking
History
• Comparing the person’s present function to his or her previous level of function is important. Commonly, instrumental activities of daily living (IADLs) are impacted by the cognitive changes. IADLs include such activities as using the telephone and preparing food.
Physical Examination
• Physical findings, if present, may suggest other types of dementia.
• Focal neurologic findings might be found in vascular dementia.
• Rigidity, tremor, or gait disturbance can be seen in patients with Lewy body dementia.
Tests for Consideration
| Clinical Entities | Medical Knowledge |
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Alzheimer Disease |
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Pφ |
A diagnosis of Alzheimer disease can be confirmed at autopsy. The pathognomonic sign is an increased number of neuritic plaques in the cerebral cortex. These plaques are tortuous neuritic processes around a central amyloid core. There may also be neurofibrillary tangles, amyloid angiopathy, and granulovacuolar degeneration. Grossly, cerebral atrophy with ventricular dilatation is often present. |
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TP |
Alzheimer disease is a clinical diagnosis based on progressive memory loss and increasing inability to participate in activities of daily living (ADLs). Generally, motor and sensory functional compromise does not occur until late-stage disease. Memory impairment, particularly the inability to learn and recall new information, is the main symptom. Early-stage disease commonly presents with compromises in executive functioning skills and in judgment. Apraxia, aphasia, disorientation, and visuospatial abnormalities are also common. |
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Dx |
Rule out “reversible causes of dementia” (hypothyroidism, vitamin B12 deficiency, and neurosyphilis). Carefully test for evidence of concomitant depression. CT of the head, if obtained, should be normal or show atrophy. MMSE score of <24 is diagnostic. There will be impairment on the clock-drawing test and on three-item recall. |
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Tx |
Pharmacologic treatment includes the use of cholinesterase inhibitors (donepezil, rivastigmine, galantamine, and tacrine). These medications slow the breakdown of acetylcholine in the synaptic cleft. They slow the progression of the disease but are not curative. Some patients will experience a modest improvement in function and cognition. Cholinesterase inhibitors may also be helpful in controlling behavioral symptoms. An N-methyl-D-aspartate antagonist (memantine) is also an indicated pharmacologic treatment in moderate-to-severe disease. It reduces glutamate-mediated excitotoxicity and is thought to be neuroprotective. Again, the medication is not curative but contributes to slowed disease progression, some modest improvement in cognitive function, and, possibly, behavioral control. See Cecil Essentials 116. |
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Pφ |
Cognitive and neurologic impairments will correlate with areas of ischemic damage in the brain. Most patients also have significant vasculopathic risk factors, including hypertension, diabetes mellitus, peripheral arterial disease, coronary artery disease, and hypercholesterolemia. |
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TP |
The typical presentation of vascular dementia may be similar to that of Alzheimer disease. It is the timing, however, or the presence of neurologic compromise in addition to cognitive changes that makes this diagnosis more likely. Vascular dementia is usually described as a stepwise decline in function rather than a gradual one. Many patients describe a history of “mini-strokes.” |
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Dx |
Imaging findings on CT scan will be consistent with cerebrovascular events: there is evidence of two or more infarcts outside the cerebellum or evidence of white-matter disease exceeding 25% of the white-matter volume. |
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Tx |
Cholinesterase inhibitors are helpful in slowing disease progression. A significant number of patients with vascular dementia may also have Alzheimer dementia, and the effects can be additive. Optimizing blood pressure control and cholesterol management is important, as is counseling patients in smoking cessation. See Cecil Essentials 116 |
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Lewy Body Dementia |
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Pφ |
Pathologically, Lewy bodies are present on biopsy specimens at autopsy. Lewy bodies are spherical intracytoplasmic neuronal inclusion bodies made of neurofilament material. In many cases Lewy bodies exist along with the classic neuropathologic changes typical of Alzheimer disease. |
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TP |
Cognitive changes are similar to those seen in Alzheimer disease, along with the presence of detailed visual hallucinations, parkinsonian signs, or fluctuation of alertness and attention. Parkinsonian signs usually do not pre-date the onset of the dementia, and rigidity is more common than tremulousness. A history of falls and an unsteady gait may also be present. Visuospatial deficits are often significant. |
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Dx |
The diagnostic workup is similar to the workup for Alzheimer dementia in terms of cognitive testing and ruling out possible reversible disorders. Lewy body dementia may be difficult to differentiate from Alzheimer dementia or from dementia associated with Parkinson disease. |
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Cholinesterase inhibitors have been shown to be useful in treating hallucinations. See Cecil Essentials 116. |
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Frontotemporal Dementia |
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Pφ |
Pathologic changes are similar to those in patients with Alzheimer disease, with more significant changes in the frontal and temporal cortex. |
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TP |
Presents with the typical cognitive decline seen in dementia. Patients are typically younger than the average patient with Alzheimer disease at diagnosis. They present commonly with disturbed executive function and losses in language expression or comprehension. This dementia is marked by serious and significant behavioral disturbance. These more “frontal” symptoms include disinhibition and impulsiveness, and they are present at the time of initial diagnosis. |
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Dx |
The initial diagnostic workup is similar to that for Alzheimer disease. The memory loss component may not be as obvious. More intensive neuropsychiatric testing may be needed, especially to elucidate language impairments in early-stage disease. |
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Tx |
Treatment of the significant behavioral disturbance often requires the administration of antipsychotic medications and sometimes antidepressants. Behavior journals can assist with pinpointing patterns and guiding treatment. An experienced geriatric psychiatrist may also be helpful with medication management. See Cecil Essentials 116. |
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Mild Cognitive Impairment |
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Pφ |
The pathology of this disorder is not well understood; many patients will progress to have the changes found in patients with Alzheimer disease. |
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TP |
These patients have significant problems with memory, but no other cognitive domains are affected. They have no clear functional compromise. |
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Dx |
The MMSE score is >25. Diagnostic workup is similar to that for patients with Alzheimer disease. |
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Tx |
Data suggest that patients with mild cognitive impairment often progress to Alzheimer dementia. This occurs at a rate of ~12% per year. See Cecil Essentials 133. |
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Pφ |
Research on the neuropathology of delirium has examined alterations in many neurotransmitter systems, including acetylcholine, serotonin, dopamine, and γ-aminobutyric acid (GABA), as well as alterations in certain cytokines including tumor necrosis factor-α. |
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TP |
Delirium is very common in older adults. It is characterized by an acute change in mental status and a lack of attention. Delirium can present as an agitated change or a more hypoactive or quiet change. |
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Dx |
The Confusion Assessment Method (CAM) is the most clinically useful diagnostic tool, with >95% sensitivity and specificity. It requires an acute change in mental status or a fluctuating course, along with inattention, to make a diagnosis, along with either disorganized thinking or an altered level of consciousness. |
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Tx |
The treatment of delirium requires treatment of the underlying problem (e.g., electrolyte disturbance, untreated pain, or medication interactions). Occasionally, when the delirium threatens a patient’s safety or dignity, low-dose antipsychotic medication may be needed. Modifying the risk factors that contribute to the development of delirium is also appropriate; these are very common in hospitalized patients (as a result of sleep deprivation, immobility, visual impairment, hearing impairment, and dehydration). See Cecil Essentials 133. |
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Depression |
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Pφ |
The pathophysiology of depression is related to disturbed neurotransmitter balance in serotonergic and noradrenergic systems. Various other neurohormonal systems have been implicated as well. |
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TP |
Symptoms at presentation may include difficulty with concentration or decision making, lack of motivation, loss of interest, apathy, sleep disturbance, psychomotor retardation, and impaired memory. Often symptoms overlap or appear very similar to those of patients with early dementia. The response to treatment often is what confirms the diagnosis. Depression has been referred to as a “pseudodementia.” However, it is important to keep in mind that patients often present with concurrent depression and early-stage dementia and may require concurrent treatment for both these problems. |
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A diagnosis of depression requires the presence of at least one core symptom that has lasted for >2 weeks with significant effect on the patient’s everyday life and functioning; these core symptoms are loss of interest or pleasure, appetite change, weight loss, psychomotor agitation or retardation, energy loss, feelings of worthlessness or guilt, difficulty concentrating or making decisions, and recurrent thoughts of death or suicide. Older adults tend to present with more somatic, physical symptoms related to depression. Therefore, the diagnosis may be difficult because of the overlap of these symptoms with those of other physical illnesses. Using a geriatric depression scale questionnaire may be helpful. |
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Tx |
Treatment varies but may include administration of selective serotonin reuptake inhibitors, tricyclic antidepressants, or other antidepressants. Generally, the side effect profile and/or the possibility of drug–drug interactions with the patient’s other medications will dictate the treatment. It may take as long as 3 months of treatment for the patient to begin to see a response to medication. Electroconvulsive therapy should be considered for patients with serious risk of suicide or poor oral intake related to their depression. See Cecil Essentials 117, 133. |
Practice-Based Learning and Improvement: Evidence-Based Medicine
Title
Current pharmacologic treatment of dementia: a clinical practice guideline from the American College of Physicians and the American Academy of Family Physicians
Authors
Qaseem A, Snow V, Cross JT, Jr, et al., and the Joint American College of Physicians/American Academy of Family Physicians Panel on Dementia
Institution
American College of Physicians’ Clinical Efficacy Assessment Subcommittee and the Commission on Science of the American Academy of Physicians
Reference
Ann Intern Med 2008;148:370–378
Problem
The American College of Physicians and the American Academy of Family Physicians developed this guideline to present available evidence on current pharmacologic treatment of dementia.
Intervention
Targeted literature review
Quality of evidence
Moderate- and low-quality evidence
Outcome/effect
Specific recommendations were made to guide pharmacologic intervention in dementia patients. The first recommendation is to initiate a trial of therapy with either an acetylcholinesterase inhibitor or memantine based on individualized patient assessment. The second recommendation is to base the choice of medication on tolerability, side effect profile, ease of use, and medication cost. There is no evidence at present to compare the effectiveness of different available agents.
Historical significance/comments
The final recommendation of this treatment guideline stresses the urgent need for further research into the clinical effectiveness of pharmacologic treatment in dementia. This area of research and development is expected to grow significantly in the future as the U.S. population of older adults, or geriatric patients, continues to increase.
Interpersonal and Communication Skills
Somewhere along the continuum of the patient’s illness, more than half of primary caregivers for patients with dementia (spouses, children, and siblings) will meet the Diagnostic and Statistical Manual of Mental Disorders (DSM-IV) criteria for a major depressive disorder. Families caring for patients with dementia bear the brunt of the financial burden, as Medicare, Medicaid, and private insurers pay only direct costs. Many families will spend a significant portion of their life savings to care for a patient with this type of chronic, progressive disease. Family members often leave their jobs to become full-time at-home caregivers. At the same time, the person for whom they are caring continues to lose function and deteriorate. Eventually, patients with dementia will be unable to recognize the caregiver who has sacrificed so much to provide care. Clear and open discussion of these caregiver losses and stresses is an important priority in optimizing patient and family care. The involvement of an interdisciplinary team can be extraordinarily helpful to ensure appropriate support.
Professionalism
Demonstrate Candor with Patients and Families
Carrying a diagnosis of dementia is a significant risk factor for impaired ability to drive a motor vehicle and for accidents. Many accidents happen close to home, on small roads, or when running simple errands. There are multiple issues involved, including respect for patient autonomy, doctor–patient confidentiality, and the duty to protect both the patient and others from potential harm. This is often a difficult discussion to have for all concerned, and a complicated decision-making process as well. For the physician, certainly, any report of a traffic accident or violation involving the patient as driver should trigger conversation and a more in-depth evaluation of the problem. A referral for a formal driving evaluation may be warranted. These services are often available through occupational therapists or in local rehabilitation centers. Doctors should also be aware of the laws in their state concerning the reporting of unsafe drivers; most states encourage this type of reporting to the division of motor vehicles, but some states mandate this practice. It is the physician’s responsibility, along with counseling and following up, to disclose and explain to the patient the steps that have been taken to report unsafe driving. The Physician’s Guide to Assessing and Counseling Older Drivers is an excellent resource guide developed by the American Medical Association and the National Highway Traffic Safety Administration. It lists all of the states’ requirements on reporting and helpful tips to assess patients’ driving ability and to counsel them effectively.
Identify a Health-Care Proxy and Discuss End-of-Life Care
Dementia is a terminal illness, usually within 7 to 10 years of diagnosis. The medical literature supports the idea that patients with moderate dementia, even if they cannot make medical decisions themselves, can still clearly verbalize whom they trust to make medical decisions for them. Early in the course of treating a patient with dementia, it is important to help the patient appoint a health-care proxy. The physician should elicit from the patient and the patient’s medical decision maker/family their personal ideas and expectations of the health-care system and should discuss the stages of the disease and the likely outcomes that can be anticipated. Many families find themselves in the hospital facing decisions about providing their loved ones with hydration, artificial nutrition, mechanical ventilation, and nursing home placement. End-of-life care in the hospital may significantly compromise patient dignity and comfort, and these discussions are best conducted in the outpatient setting. In addition, significant medical costs occur during the last weeks of a person’s life, when hospital resources are often unwittingly invested in futile medical scenarios. Although advanced-care planning must be discussed within the context of a patient’s particular belief systems, when presented with possible outcomes, many patients will choose to extend their home-care options and perhaps consider hospice, with an approach focused on comfort and dignity in the last stages of their illness. Currently, on average, patients are referred to hospice only in the last days of an illness. For patients who are declining and meet the criteria for advanced dementia earlier, referrals may be of tremendous benefit. Hospice offers an interdisciplinary approach (including physician, nurse, social worker, chaplain, volunteer services personnel, and physical therapists), attention to the caregivers, and even eventual bereavement support. With proper planning, goals of care can occur in the patient’s home under realistic and well-supported expectations.
Suggested Readings
American Medical Association. Physician’s guide to assessing and counseling older drivers. Available at: http://www.ama-assn.org/ama1/pub/upload/mm/433/older-drivers-guide.pdf.
American Psychiatric Association. Diagnostic and statistical manual of mental disorders. (DSM-IV) 4th ed Washington, DC: American Psychiatric Association; 1994.
Inouye SK, van Dyke CH, Alessi CA, et al. Clarifying confusion: the Confusion Assessment Method: a new method for detection of delirium. Ann Intern Med. 1990;113:941–948.
