30: Dementia & Delirium

Dementia is a common disorder with a prevalence that doubles every 5 years after the age of 60 years, thereby affecting up to 45% of those aged 85 years and older. Dementia is defined as an acquired, persistent, and usually progressive impairment in intellectual function, with compromise in multiple cognitive domains, at least one of which is memory. The deficits must represent a significant decline in function, and must be severe enough to interfere with work or social life for the diagnosis to be formally applied. As the disorder progresses, individuals with dementia often fail to recognize family members, are unable to express themselves clearly and meaningfully, and often undergo dramatic personality changes. Table 30-1 lists some of the more common causes of dementia.

Despite the high prevalence of dementia, the diagnosis is often missed by clinicians, particularly in the early stages. The primary care practitioner can play an important role in diagnosing and treating dementia. An early diagnosis can offer the opportunity to involve patients in meaningful advance care planning while they are still able to express their preferences clearly. In addition, potentially treatable causes, though rare, are more likely to be partially or fully reversed if diagnosed early. Later in the disease, the practitioner can work with other team members to manage difficult behaviors, ensure that the patient is as comfortable and safe as possible, recognize when referral for specialist care is needed, educate caregivers about the condition, and support caregivers in coping with their situations to the best of their abilities. Furthermore, with several pharmacological treatments available for dementing illnesses, it has become more important for physicians to be proficient in diagnosing specific subtypes of dementia and knowing what therapies may (or may not) be helpful for that syndrome.

Alzheimer Disease

Alzheimer disease (AD) is the most common form of dementia, accounting for about 60–70% of the cases. The age of onset varies considerably, but most commonly symptoms arise after the age of 70 years. Incidence of the disease increases with age. Women may be at a slightly higher risk of developing the disorder than men. The rare patient with early onset of AD (before the age of 60 years) may have a genetic disorder with an autosomal dominant pattern of inheritance, so a thorough family history is important in these cases.

Alzheimer disease usually follows a slow, but progressive and insidious, course (Table 30-2). Life expectancy following the appearance of the disorder ranges from 3 to 15 years, but considerable variation may occur, and survival of up to 20 years has been reported. Memory deficits are prominent in all dementias, but especially so in AD. Typically, short-term episodic memory problems are the first and most obvious manifestation of the disorder. Alzheimer disease affects both the encoding and retrieval of new information to a profound degree, such that these patients do not appear to benefit from cueing and prompting of memory. For example, a patient asked to remember the words “piano,” “carrot,” and “green” may not be helped by hints such as “musical instrument,” “vegetable,” or “color.” Although diagnostic certainty is less than 100% without pathological examination, careful clinical and neuropsychological evaluation results in a reliable diagnosis of the disease in most instances. A computed tomography (CT) or magnetic resonance imaging (MRI) scan often shows cerebral atrophy and hippocampal volume loss; the electroencephalogram (EEG) may show diffuse slowing.

Dementia with Lewy Bodies

Dementia with Lewy Bodies (DLB) is reported to be the second most common cause of dementia in autopsy studies, accounting for 15–30% of the cases. It is defined by the presence of cognitive impairment, parkinsonism, prominent (often bizarre) hallucinations or other psychotic symptoms, and fluctuations in alertness, although patients may not have all of these symptoms. Patients with DLB are often intolerant of traditional antipsychotic medications, and their use can induce severe parkinsonism or even death. At least one randomized-controlled trial suggests that cholinesterase inhibitors may be useful in treating the disorder.

Vascular Dementia

Previously known as multi-infarct dementia, vascular dementia is characterized by the development of cognitive impairment in association with single or multiple areas of infarction and/or subcortical ischemia. Not surprisingly, the risk factors for vascular dementia are identical to the risk factors for cerebrovascular disease, including smoking, hypertension, diabetes mellitus, coronary artery disease (CAD), and previous history of stroke. A patchy and inconsistent pattern of deficits may be seen initially, with relative preservation of some cognitive areas. Because vascular dementia can result from insults to a variety of cortical or subcortical areas, the neuropsychological profile of patients with vascular dementia can be quite varied.

Focal neurologic signs are more common in vascular dementia than in AD, and lateralized motor and sensory findings may be seen. CT and MRI scans frequently reveal multiple areas of infarction in the brain, often with diffuse subcortical white matter changes. Later in the course of the disorder, atrophy from loss of brain tissue may be seen.

A number of diagnostic criteria for vascular dementia have been proposed, but none are sensitive or specific enough to rule the diagnosis in or out with certainty. Many elders show diffuse white matter changes on brain imaging, and yet are cognitively intact. Other demented elders, who were thought by their clinicians to have vascular dementia because of cognitive decline following cerebral infarcts, are subsequently found to have Alzheimer’s pathology at autopsy in addition to the known infarcts. Such individuals, with elements of both AD and vascular dementia, are considered to have mixed dementia. In short, current diagnostic instruments and criteria are often inadequate for clinicians confidently to employ or reject the “vascular dementia” label.

Other Causes

Frontotemporal dementias (FTDs) are a group of disorders characterized by personality change (e.g., apathy or socially inappropriate behavior), hyperorality, and cognitive decline especially involving language and executive function. Frontotemporal dementia tends to affect individuals at a younger age than AD, with many persons receiving the diagnosis in their 50s or 60s. Imaging studies may show marked atrophy in frontal and temporal lobes.

Advanced Parkinson disease commonly results in dementia. These patients frequently display mental sluggishness and a general lack of spontaneity. Language functions (compared with those of AD patients) are relatively preserved.

Chronic alcohol abuse can result in serious cognitive deficits if drinking is severe and prolonged, although it is not clear whether the dementing process occurs in the absence of coexisting thiamine deficiency. Severe head trauma can also result in permanent cognitive impairment, which might be viewed as static dementia.

Human immunodeficiency virus (HIV)-1-associated dementia (HAD) is caused by the direct infection of the brain by the HIV-1 virus. It is associated with late-stage AIDS and is generally seen in individuals whose T-cell count has fallen below 200. Features of this form of dementia are summarized in Table 30-3. HIV-1-associated dementia is discussed further in Chapter 36. Creutzfeldt–Jacob disease is a rare infectious cause that should be suspected when a nonelderly person develops a rapidly dementing illness associated with other neurologic deficits such as myoclonus.

Reversible Conditions

A number of medical conditions can result in cognitive impairment. These may be treatable, but full or partial reversal of dementia is relatively rare, even with appropriate treatment. However, because dementia is such a devastating diagnosis, assessing the patient for these conditions is an important part of the dementia workup. Fully or partially reversible dementia syndromes are usually the result of depression, medication, or a toxic or metabolic disorder. The most common types of primary and secondary treatable syndromes are listed in Table 30-4.

Differential Diagnosis

Persons suspected of having dementia are encountered in a number of medical settings. For example, a patient who presents to the emergency department with confusion, disorientation, and hallucinations should be evaluated for delirium (e.g., from infection, metabolic derangement, or use of or withdrawal from medications or recreational substances) and psychotic disorders. An older adult with a history of recent bereavement, withdrawal, and increased forgetfulness may indeed be demented, or may be suffering from cognitive deficits related to major depression. Other problems that initially may be confused with dementia include sensory deficits (severe visual or hearing impairment), aphasia, developmental disability, and low levels of literacy or education.

Normal Aging

As people age, some degree of decline in cognitive functioning is common. The presence of mild memory impairment among older adults has been reported to occur among a very wide range (11–96%) of participants in various research studies. Normal aging often brings minor word-finding difficulties, slowed memory retrieval, and some changes in the speed and efficiency of thought. These changes, however, typically do not cause significant problems in everyday functioning or produce confusion or disorientation, which are signs of impaired cognition (see Chapter 14).

Mild Cognitive Impairment

Mild cognitive impairment (MCI) is an increasingly recognized condition in which a patient complains of memory problems, demonstrates mild deficits (most commonly in short-term memory) on formal testing, but does not meet criteria for dementia. Dementia will develop in roughly 10% of people with MCI each year. Amnestic MCI, in which the deficit is primarily episodic memory, is more likely to progress to AD than nonamnestic MCI, which may be more pathophysiologically heterogeneous. At the time of this writing, no medication intended for the treatment of MCI has been approved by the FDA. Acetylcholinesterase inhibitors have shown mixed results in clinical trials. Any potentially reversible conditions, including depression, hearing loss, or B₁₂ deficiency, should be addressed in patients with MCI. They also should be advised to complete advance care planning and followed with serial clinical evaluation at least every 6 months.

New Diagnostic Criteria for Alzheimer Disease and Mild Cognitive Impairment

Research suggests that AD pathophysiology begins many years and possibly decades prior to the clinical expression of the disease. In an attempt to identify a preclinical phase of AD and to improve the accuracy of the diagnosis of AD and MCI, several working groups have suggested new guidelines and diagnostic criteria. Many of these incorporate biomarker measures (either from neuroimaging or cerebrospinal fluid) as these may provide direct evidence of the underlying pathophysiology associated with AD. How these new guidelines will be incorporated into clinical practice is uncertain. In particular, the concept of a preclinical phase of AD will require much greater research and public health policy guidelines.

Dementia versus Delirium

Delirium is a state of cognitive impairment that is acute in onset and associated with fluctuations in mental status and behavior, inattention, disorganized thinking, and an altered level of consciousness. Because dementia is a significant risk factor for delirium, the two syndromes are closely linked (Table 30-5). It is not uncommon for delirious patients to be incorrectly labeled as demented by an inpatient team with no prior knowledge of the patient. Optimized communication between inpatient and outpatient teams, good baseline mental status testing on elderly outpatients, and/or collateral information from family and friends, can often be critical in trying to determine how likely it is that the delirious patient will “clear” enough to be able to live independently following hospital discharge. Unfortunately, some patients, like George, never return to their prior level of functioning following an episode of delirium.

Delirium is usually caused by toxic and metabolic derangements or infections. In determining the cause, a clinician should consider reviewing the medication record (looking for recent changes); checking blood cell counts, electrolytes, renal function, glucose, calcium, and urinalysis; and reviewing a chest film and electrocardiogram. In general, workup does not need to include neuroimaging or lumbar puncture unless routine labs or medication review is unrevealing, or focal neurologic deficits or fever are present.

Most studies have suggested that prevention of delirium is more effective than treatment. Preventive strategies include avoiding anticholinergic medications (like diphenhydramine); treating pain adequately; promoting normal sleep patterns; keeping patients active, stimulated, socially connected, and oriented during the day; making certain vision and hearing aids are available; promoting adequate hydration; and avoiding unnecessary tubes and catheters. Established delirium, if disruptive to care, is probably best treated with low-dose haloperidol, 0.5 mg once or twice daily. If the delirium is related to alcohol or benzodiazepine withdrawal, benzodiazepines are the treatment of choice.

Dementia versus Depression

Especially among older adults, symptoms of depression may masquerade as dementia (Table 30-6) making it difficult to distinguish between “true” dementia and the dementia syndrome of depression (DSD, formerly called “pseudodementia”). It has become increasingly apparent that depressed patients who present with cognitive impairment are much more likely to go on to develop true dementia within a few years. Formal neuropsychological testing and/or an empiric trial of antidepressant medication may provide the best means of distinguishing between dementia and DSD.

Dementia versus Psychotic Disorders

The symptoms of psychotic disorders, such as schizophrenia or bipolar disorder, may resemble the dis-organization, confusion, delusions, and agitation that sometimes accompany dementia. The typical presence of more consistent and systematized delusions and the increased likelihood of previous psychosis and a family psychiatric history among individuals with nondementia-related psychotic disorders are important in making this differentiation.

There is currently no consensus on whether older persons should be screened for dementia. At present, early detection and treatment does not improve outcomes, but that may change if disease-modifying treatments become available in the future. Despite the lack of data, the authors of this chapter still support screening for cognitive impairment in older adults because early detection can allow patients to participate in advance care planning for finances and health care. We favor the Mini-Cog (a three-item recall and clock draw test) as a screening test because it is a sensitive and easy to administer tool. When a patient fails this simple screen, following up with a standardized instrument is warranted.

A dementia workup should include a careful history (Table 30-7) and physical, neurologic, and mental status examinations. In many cases, additional tests and procedures may be helpful; these are discussed later.

A complete history should be elicited from both patients and their families. In many cases, it is helpful to ask both about the same topics. Although many demented individuals are unreliable historians, they may be able to provide much useful information, including symptoms of which family members are not aware. In addition, patients’ abilities to provide a coherent and organized account of their complaints and recent history can often tell you much about their conditions.

A complete social and family history that includes native language; family history of dementia; and educational, marital, and employment background can also be useful. An educational and occupational history, for example, may provide information about a patient’s previous level of functioning. A quality of vagueness in patients’ reports or inconsistency in the information supplied may suggest less blatant memory deficits. Particular attention should be paid to patients’ accounts of more recent events. Individuals with mild or moderate dementia often show intact recall for older, overlearned information (e.g., family, education, and work), but cannot provide accurate information about the immediate past.

Ask patients about their regular activities, appetite, and sleep habits, as well as any physical or cognitive complaints. Information about their mood can be gained not only by inquiring directly, but also by asking about things that they enjoy and look forward to, any worries or concerns they may have, and their plans for the future.

Family members may be able to supply a more complete and balanced impression of the patient’s symptoms, behavioral changes, and level of functioning. Individuals with dementia typically lack insight into their cognitive problems and minimize or deny any difficulties. In fact, it is the family that most frequently raises concerns about the patient. Because family members may feel more comfortable discussing their observations away from the presence of the patient, they should be interviewed separately. It is especially important to inquire about both current and potential safety issues, such as driving (Table 30-8).

Physical & Neurologic Examinations

In addition to a routine physical examination, a complete neurologic examination should be done, although the results of the latter are generally unremarkable in cases of early AD. Parkinsonian signs may be apparent during the later course of many dementing illnesses. Individuals with a developing vascular dementia may show focal signs on the neurologic examination.

The Mental Status Examination

Because cognitive impairment is the hallmark of dementia, it is necessary to add additional screening items to the traditional mental status examination. Several brief dementia-screening instruments offer the advantages of both a structured format and a scoring system. These instruments must be used with caution, however, for the reasons discussed here.

The Mini-Mental State Examination (MMSE)^© has been the most commonly used dementia-screening instrument (Table 30-9). It takes about 5 minutes to administer and samples a wide range of functions, including orientation, immediate and delayed memory, concentration, basic language and reading functions, and visual construction abilities. Patients are asked to count backward by 7, write a sentence, follow concrete directions, and copy geometric figures. A maximum score of 30 is possible; a score lower than 24 (some clinicians recommend 27) is suspicious for dementia.

The Montreal Cognitive Assessment (MOCA) is currently being used in many settings because it is more sensitive than the MMSE for the detection of MCI and has a less restrictive copyright agreement. The web site www.mocatest.org contains MOCA forms in 37 languages, instructions for administration and scoring, and normative data for the examination. Other brief dementia-screening instruments include the Mini-Cog, the Blessed Dementia Scale Neurobehavioral Cognitive Status Examination, and the SLUMS (St. Louis University Mental State Exam). Functional scales such as the FAST (Functional Assessment Staging) can be useful in determining what site of care may be most appropriate for a patient with dementia, including when hospice might be appropriate.

Other Tests

The American Academy of Neurology recommends that structural neuroimaging (with noncontrast CT or MRI) be employed in the initial evaluation of most patients with dementia, as some 5% of such patients have been found to have important but clinically unexpected structural lesions. We believe that in cases of long-standing dementia that is consistent with AD, there may be instances when the burdens of neuroimaging outweigh the benefits, particularly in a patient who becomes agitated in unfamiliar settings. An EEG is helpful if the differential diagnosis includes status epilepticus or postictal confusion. On the other hand, the major dementing disorders, especially early in their course, do not generate characteristic EEG patterns, and a normal EEG cannot be taken as definitive evidence of the absence of brain pathology.

Lumbar punctures are indicated when there is a question of an infectious process or when a rapidly progressing condition is present. In rare instances, brain biopsy may be employed to aid in diagnosis. Table 30-10 suggests other recommended laboratory tests.

Neuropsychological Testing

Neuropsychological testing involves the use of psychometric tests designed to detect and characterize a wide variety of cognitive problems. The testing requires the patient to be able to attend for a minimum of 20–30 minutes; some patients may be too agitated, withdrawn, or resistant to comply. Consider referring a patient for neuropsychological testing in the following circumstances:

• When the reported deficits are subtle or equivocal and the impairment is mild.

• When the differential diagnosis is difficult to resolve (e.g., distinguishing between dementia and depression).

• When a baseline is needed to measure the degree of cognitive deterioration over time.

• When a greater understanding of the patient’s everyday level of functioning, limitations, potential safety issues, and the appropriate level of supervision is needed.

• When deficits or competency issues must be documented, such as in conservatorship proceedings.

• To monitor effects of treatment.

Treatment of dementia consists of identifying and addressing a treatable basis for the underlying condition (if one exists), treating symptoms of the cognitive deficits, treating secondary disorders such as anxiety or depression, educating patients and their families as to the nature of the condition, and assisting caregivers with safety and management issues. Table 30-11 summarizes overall principles of dementia management.

Management of patients with dementia involves matters of self-care, safety, and communication. Individuals with dementia are characteristically disorganized, forgetful, and inefficient in many of their activities making it difficult and unsafe for them to function independently. Complicating this, they may be impulsive, have limited insight into their abilities, and fail to learn from experience.

Neuropsychiatric symptoms are common and almost all persons with dementia will experience them at some point during the course of their illness (97% in one series of patients with dementia followed over 5 years). Providers should inquire about them with patients and caregivers at each visit. Specifically, they may be labile and unstable, irritable, or anxious; they may be fearful, easily frustrated, or apathetic and withdrawn. They may wander at night or endorse paranoid thoughts or delusions. Because of circadian rhythm disturbances, patients with AD may tend to be awake and wandering during the night, leading to sleep deprivation in caregivers.

These traits create special problems in managing and caring for patients with dementia, and many interactions with family and caregivers therefore focus on day-to-day management issues and behavioral problems. The following sections provide some general guidelines for addressing these issues. It is useful in many situations to refer patients and their families to groups that can provide counseling, home evaluations, and case management. (In some instances, there may be no more important knowledge for the primary doctor than familiarity with the capabilities of local support agencies.)

In general, nonpharmacological strategies for behavior management should be attempted first, and if unsuccessful, medication trials may be indicated. In practice, many distressing symptoms, such as agitation, prompt providers to try medications concurrently.

Self-Care

This area includes dressing, grooming, and hygiene. The best approach is to provide sufficient structure and guidance so that the patient can manage, while allowing some preservation of independence and choice. Individuals with MCI often get by with prompts and reminders. For individuals with more severe impairment, caregivers should offer a limited choice of clothing, food, or activities; use signs or pictures to identify important objects and locations; and emphasize routine and predictability. Breaking down complex and potentially overwhelming activities such as bathing into simple one-step tasks may keep these activities manageable well into the course of the illness. The caregiver may need to modify clothing, for example, by incorporating sweatshirts to avoid buttons, and using shoes with Velcro closures rather than laces. Incontinence often develops. Protective garments in clothing and timed toileting, for example, having the patient go every 2 hours while awake, may prevent frequent accidents.

Safety Issues

Individuals with dementia are at increased risk for accidents because of their problems with attention, perception, and judgment. They are also at increased risk of abuse, including physical, emotional, and financial.

To assess risk of accidents, ask caregivers and patients about conditions at home, how often the patient is left alone, and whether he/she wanders, cooks, or self-administers medications (see Table 30-8). Where safety is unclear, more detailed information can be provided by an early home safety evaluation, often done by an occupational therapist.

The following guidelines are designed to minimize the risk of household accidents:

• Carefully supervise use of medications.

• Keep the home relatively free of clutter and keep passageways clear.

• Install railings and other safety equipment, such as shower chairs and tub rails.

• Use a night-light where necessary.

• Remove stove and oven controls if necessary.

• Use safety gates or install keyless locks on exterior doors if wandering is a problem.

• Make sure patients wear medical and personal identification bracelets. Registering the patient in the Safe Return program via the Alzheimer’s Association is a common way to obtain identification bracelets or necklaces. (Access at http://www.alz.org/safetycenter/we_can_help_safety_medicalert_safereturn.asp)

Another important safety issue concerns driving and dementia. Deficits in attention, reaction time, visual spatial abilities, and judgment typically impair the ability of individuals with marked cognitive deficits to operate a motor vehicle safely. Individuals with advanced dementia who continue to operate a car seriously endanger others as well as themselves. Unfortunately, patients with dementia may insist on driving because of their lack of insight into their problems, and caregivers may be reluctant to press the issue. Because driving is symbolic of independence, this often becomes an emotional issue for everyone involved.

Confidentiality must be weighed against issues of competency, safety of the public, and the duty to warn. Legal requirements vary considerably between states. For example, in California, physicians are required by law to report patients with a diagnosis of AD or other related dementias. In the case of accident or injury, physicians who have failed to make such a report may be legally liable. Clinicians should inform themselves of the legal requirements in their own state regarding this issue.

Advance care planning should be addressed as early as possible. Evidence has shown that patients are more likely to have their care preferences followed at the end of life with these in place, and routine documentation of these preferences helps that occur. Similarly, referral to social workers and specialized legal agencies can help ensure that the patient has adequate financial and personal protections legally and medically. It may be helpful to know of legal resources for patients and families in the local community.

Communication

Individuals with cognitive impairment may have specific deficits in language comprehension and expression similar to the various forms of aphasia that occur in stroke patients. More common may be a generalized difficulty in communication secondary to a short attention span, memory problems, or confusion. Some guidelines for communicating with the cognitively impaired are presented in Table 30-12.

Another factor affecting communication may be hearing or vision impairment, which is highly prevalent and often minimized by the patient. Therefore, we recommend asking about vision loss and referring to an ophthalmologist for full evaluation if there is any suspicion for vision loss. Hearing loss compounds cognitive difficulty profoundly. When speaking with someone with dementia, portable amplifiers, called “pocket talkers,” help ensure that the person can hear the conversation. These can be kept in the clinic for regular use. Trying a pocket talker, even if the patient denies significant hearing impairment, often reveals undercorrected hearing loss and improves communication tremendously.

Drug Therapy

Cholinesterase inhibitors may confer mild benefits for patients with AD, vascular dementia, and DLB. These benefits, on average, are not clinically significant for cognition and are modest for global assessments; however, this class is approved by the FDA for mild-to-moderate AD. Anecdotal evidence suggests that a subgroup of patients may achieve clinically important improvements. The decision to initiate therapy with an acetylcholinesterase inhibitor should be based on evaluation of benefits and risks associated with an individual patient. A beneficial effect, that is, improvement or stabilization of target symptoms, would generally be expected within 6 months of initiation of therapy. Evidence is insufficient to determine the optimal timing or duration of therapy. Side effects of acetylcholinesterase inhibitors include nausea, anorexia, diarrhea, weight loss, syncope, and urinary frequency. An ECG should be checked after dose increases to monitor for bradycardia. Starting doses, respectively, of donepezil, galantamine, and rivastigmine, are 5 mg orally once daily, 4 mg orally twice daily, and 1.5 mg orally twice daily. The doses are increased gradually as tolerated.

Memantine, an N-methyl-d-aspartate (NMDA) antagonist, may be helpful in patients with moderate-to-severe AD or vascular dementia, with or without concomitant use of an acetylcholinesterase inhibitor. Clinical trials have shown a statistically significant, but most likely not a clinically significant, improvement on standard tests of cognition. Some studies have also demonstrated improvements in behavior and quality of life with use of memantine. The starting dose is 5 mg daily; the dose may be increased by 5 mg in weekly intervals up to a maximum of 20 mg per day. Table 30-13 shows medications commonly used for AD and their recommended doses.

Behavioral Interventions and Medication for Severe Behavioral and Psychiatric Symptoms

Because of limited efficacy of many medications and higher risk of complications, nonpharmacological approaches should be attempted first before psychotropic medications.

Nonpsychotic agitation deserves careful evaluation before a drug is prescribed. New agitation may represent delirium, undertreated pain, depression, a distended bladder, or fecal impaction. If these disorders are not contributing and if nonpharmacological approaches are inadequate, an empiric trial of analgesics such as acetaminophen, an antidepressant medication, or a mood stabilizer may be worthwhile. However, any empiric trial should be time limited, and consultation with a geriatric psychiatrist is recommended in recalcitrant cases if at all possible.

When behaviors are severe and may lead to harm for the patient and caregiver, medications may be indicated. There is no clear consensus about pharmacological approaches to treatment of behavioral problems in patients who have not benefited from nonpharmacological therapies. The target symptoms—depression, anxiety, psychosis, mood lability, or pain—may suggest which class of medications might be most helpful in a given patient. Patients with depressive symptoms may show improvement with antidepressant therapy. In general, benzodiazepines should be avoided, especially for more than brief periods, as they have significant potential for causing sedation, confusion, ataxia, and falls.

Despite the lack of strong evidence, antipsychotic medications are commonly used as first-line agents for the treatment of behavioral disturbances, largely because of the lack of alternatives. The newer atypical antipsychotic agents (e.g., risperidone, olanzapine, quetiapine, aripiprazole, clozapine, and ziprasidone) may be better tolerated than older agents (e.g., haloperidol), but confer an excess risk of stroke, weight gain, and hyperglycemia. Both typical and atypical antipsychotics are associated with an increased risk of death compared with placebo when used to treat elderly demented patients with behavioral disturbances. When the choice is made to use these agents, patients and caregivers should be carefully warned of the risks. It is prudent to check an ECG prior to beginning antipsychotic therapy, and to seriously consider other alternatives when a patient is found to have a prolonged QTc. Starting and target dosages should be much lower than those used in schizophrenia (e.g., haloperidol, 0.5–2 mg orally; risperidone, 0.25–2 mg orally). Federal regulations require that if antipsychotic agents are used in treatment of a nursing home patient, drug reduction efforts must be made at least every 6 months. If the problem continues, consider another agent or obtain consultation with a psychiatrist, preferably one specializing in geriatric psychiatry.

Randomized controlled trials suggest that donepezil has no benefit when used to treat agitation in AD. However, if a medication such as a cholinesterase inhibitor or memantine is being considered for cognition, it is reasonable to see if it has an impact on behavior, as well. If at all possible, we recommend delaying the prescription of a second agent (e.g., antipsychotic or other psychoactive medication) until the impact of the cognitive agent medication can be evaluated.

Caregiver involvement is essential to managing neuropsychiatric symptoms without medications. Common strategies for behavioral problems are maintaining and promoting adequate daytime physical and social activity, avoiding confrontational approaches by redirecting questions or topics of conversation, and consistently using reassuring and calming responses to a patient’s behaviors instead of anger or frustration. Guides and informational material may be a very useful resource for caregivers and can be easily downloaded from web sites such as http://www.indydiscoverynetwork.org or www.caregiver.org.

Caregivers

Caring for cognitively impaired individuals is often exhausting and stressful, especially in the presence of neuropsychiatric symptoms. Primary care providers should ensure that caregivers also attend to their own needs and guard against fatigue and burnout. Caregiver-specific inventories can be administered, such as the Revised Memory and Behavior Problems Checklist, to quantify the patient’s behaviors and assess the caregiver’s emotional burden. A number of support services, such as the Family Caregiver Alliance (http://www.caregiver.org), offer education, support groups, and information about resources. Specialized case management and home consultation services can also be very helpful in assessing patient and family needs and locating additional services. Respite-care and day-activity programs may be invaluable in allowing stressed families and caregivers time off and providing needed stimulation and increased structure for the patient. Many experienced clinicians often assess stressed caregivers at each clinic visit.

Nursing Home Placement

In the management of demented patients, the issue often arises as to whether the patient can continue to be managed at home. This is an area fraught with strong feelings on the part of patient and caregiver alike, and it is often difficult to make objective decisions. Major predictors of nursing home placement include the presence of behavioral problems, dementia severity, and extent of caregiver burden. Some important considerations related to nursing home admission are as follows:

• Can the necessary level of supervision and care required by the patient be realistically and dependably supplied at home? Such issues as wandering and the potential for neglect and abuse should be considered here.

• Is sufficient assistance available to caregivers for them to continue to care for the patient without becoming overstressed and burned out?

• What financial resources are available for the patient’s care?

All things being equal, many patients and caregivers prefer to remain in their own home. In many cases, however, doing so creates other significant problems, and however desirable this goal, it must be carefully weighed against issues of safety and practical limitations. In the advanced stage of dementia, when the patient becomes mute, bedbound, and incontinent, very often the services of a skilled nursing facility are required to ensure that the patient receives proper care. Nursing facilities should be chosen carefully; obviously, cost must be considered, but the quality of care must also be investigated. Information about both complaints and compliance with state regulations can be found through government agencies, advocacy groups, published reviews, and organizations (e.g., geriatric care programs) that frequently place patients in such facilities. Although definitive data are lacking, some studies suggest that Alzheimer’s special care units reduce behavioral disturbances and use of restraints. Some patients do better in structured environments such as Alzheimer’s special care units or Alzheimer’s day care programs than in less structured home settings.

Dementia is a terminal disease, and in late stages the patient may become bedbound, unable to recognize family, and have difficulty swallowing. In these instances, hospice care is often appropriate (see Chapter 40).

Dementia is an increasingly common syndrome characterized by a progressive loss of cognition and functioning. Given the aging of the population, it is essential that clinicians become comfortable with diagnosis, counseling, community and specialist referral, and treatment. Early diagnosis may allow for identification of (unfortunately rare) reversible causes, improved symptom management, and planning for the future.

When patients present with cognitive complaints, the clinician must consider differential diagnoses including dementia, delirium, depression, and psychosis. Diagnostic workup includes a careful history from patient and family, physical examination, mental status examination, laboratory work, brain imaging, and on occasion, referral for neuropsychological testing or other specialized assessment.

Treatment and management consist of ruling out treatable conditions, treating secondary disorders such as anxiety or depression, providing information to patients and families, and assisting caregivers with safety and management issues. Use of medications may modestly slow the rate of cognitive decline or help with management of severe psychiatric symptoms, but interventions also consist of adjusting the environment to make the patient as comfortable and safe as possible and providing support for caregivers.

Taking care of demented patients and their caregivers can be extremely rewarding. Further, with abundant research efforts under way, there is reason to be optimistic that improved methods of preventing and treating dementing disorders are just over the horizon.

David M. Pope, PhD and Alicia Boccellari, PhD, were authors of the first edition version of this chapter, and William Lyons, MD was author of the second edition version of this chapter.