CHAPTER 15 Jennifer Merrilees1, Cynthia Barton1, Amy Kuo2 and Robin Ketelle1 1 University of California, San Francisco, San Francisco, CA, USA 2 On Lok Lifeways, San Francisco, CA, USA The management of a patient with an atypical dementia presents a variety of challenges to health-care providers. Such patients often have diverse symptoms and uncommon clinical presentations, which can lead to incorrect diagnoses and delay appropriate treatment. By the time a correct diagnosis is obtained, the family might have spent months or even years searching for answers from various providers. This can result in significant financial and emotional stress, leaving families closer to crisis with urgent need of resources. Health-care providers, therefore, need to be aware of these issues when managing atypical dementia patients and their families. This chapter identifies issues pertinent to the management of patients diagnosed with an atypical dementia, although most topics discussed here are common to many dementias. Key considerations (functional changes, safety concerns, and psychosocial adjustments) for specific diseases and issues to consider for promoting caregiver health and coping are discussed. A partial list of resources for patients and families also is provided in Appendix . Functional skills are traditionally divided into two broad categories—instrumental activities of daily living (IADLs) and basic activities of daily living (ADLs). IADLs include complex tasks of managing finances, working, driving, providing childcare, managing medications, and household duties. ADLs encompass basic abilities of dressing, hygiene, walking, and eating. Each dementia syndrome has a variable impact on functional abilities. For example, the patient with dementia with Lewy bodies (DLB) might be unable to cook safely or manage bill paying due to visuospatial deficits and executive dysfunction rather than the short-term memory loss associated with typical Alzheimer’s disease (AD). In most dementias, capacity for instrumental tasks is typically affected first, and basic skills are affected later in the disease course. Changes in the patient’s cognition, insight, and judgment create challenges in obtaining an accurate functional assessment [1]. For this reason, the majority of functional assessment scales rely on ratings by an informant (someone knowledgeable about the patient, typically a family member or caregiver). In atypical cases or when there is not an informant, direct observation of ADL performance may be helpful. Validated measures include the Assessment of Motor and Process Skills (AMPS) [1], the Kohlman Evaluation of Living Skills (KELS) [2], and the Texas Functional Living Scale [3]. Multiple issues contribute to a patient’s vulnerability regarding safety issues, and it is necessary to periodically review risk factors as they can change over the course of the illness. Common concerns involve driving, communication, judgment and decision-making, and the impact of neuropsychiatric symptoms on personal and social conduct. Patient and caregivers might lack insight about potential hazards and can better prepare for the future with appropriate anticipatory education. Discussions often center on issues of maintaining patient autonomy while simultaneously ensuring safety. Table 15.1 lists common areas of concern along with examples of interventions. Table 15.1 Safety concerns. Compromised work performance common in atypical dementias can occur for a variety of reasons. Patients with Huntington’s disease (HD) might be unable to work due to emotional liability, irritability, or depression, whereas patients with frontotemporal dementia (FTD) might have decreased performance secondary to social misconduct or executive dysfunction. The language and speech deficits common in progressive aphasias may interfere with comprehension and effective communication. Patients with dementia often stop working much earlier than they intended. This has repercussions beyond income; nonworking individuals no longer make contributions into social security and retirement plans and might lose employer-sponsored health insurance. Additionally, family caregivers might have to enter or return to the work force or, conversely, not be able to work due to their caregiving responsibilities. Some caregivers assume the responsibility of managing a business on the patient’s behalf to maintain financial solvency and contractual obligations. Families sometimes deplete their savings or retirement accounts to cover the costs involved in caring for a family member with dementia. Occupational concerns can be addressed during the initial evaluation. Patients and family members might not be aware of performance issues. In some cases, families prefer that the patient continue working out of a desire for income or concern about the effect of job loss on self-esteem. Some patients enjoy a supportive work environment in which colleagues cover for the patient’s reduced work performance or have supervisors who are willing to make appropriate accommodations to allow the patient to perform modified duties. A realistic and pragmatic approach to employment is recommended, given that patients might be putting themselves, their families, and their employers or clients at legal and/or financial risk. Providing assistance with a disability application or the retirement process might be necessary. Families will need to identify whether the patient is eligible for Social Security Disability Insurance (SSDI) and Supplemental Security Income (SSI) in the United States or equivalent in other countries. Health-care providers are typically asked by governmental support agencies to provide documentation of the patient’s expected progressive deficits and reasons why certain work is not feasible. Health-care providers can also provide counseling resources to facilitate decision-making regarding family caregivers returning to work. In some situations, families might be eligible for medical leave in order to care for the patient. In some countries or US states, certain diagnoses allow for automatic disability. When a patient’s occupational capacity is compromised, it may be possible for them to contribute in volunteer or work-related tasks that match their functional level. For example, patients with rigid and obsessive habits (e.g., FTD) might be able to sort objects (and may excel at such tasks). Motor symptoms (e.g., poor balance, falls, coordination deficits, rigidity, slowed movements, and supranuclear gaze palsy) are early symptoms in some atypical dementias, and almost all the dementias are associated with some motor impairment near the end of the disease course. Due to the high prevalence of falls in atypical dementia conditions, safety and fall prevention must always be a primary concern. Home safety assessments should be performed to identify potential safety hazards in the home and appropriate safety modifications (e.g., safe footwear choices, flooring, nonskid rugs, and grab bars) should be implemented. One-to-one care might be required, especially in bathrooms. Electronic room monitors such as those used for infants can assess auditory or visual information, allowing caregivers to monitor the patient’s activity in another room. Emergency notification systems (e.g., LifeAlert®) might also be necessary, either on the walls or worn by the patient. A variety of assistive devices can be used to help steady the gait. Four-wheeled walkers have been shown to provide increased stability to patients with gait problems [4, 5]. Patients with impulsivity might require one-to-one supervision and can also be encouraged to wear a safety helmet to prevent head injury. In a study of patients with Parkinson’s disease, performing cognitive tasks while walking was associated with changes in stride and balance that increased the risk for falling [6], suggesting that multitasking while walking should be discouraged. Exercise is an important component of maintaining strength and balance. Patients should be encouraged to work with a physical therapist or personal trainer and to exercise a few times per week (e.g., active cardiovascular exercise 30 min three times per week mixed with strength training). Additional measures, such as massage and gentle yoga, might improve range of motion, flexibility, and balance [7]. Dysphagia (difficulty swallowing liquids, food, and/or saliva) is commonly associated with advanced stages of dementia. Consequences of dysphagia include weight loss (due to the difficulty in meeting caloric and nutritional requirements) and aspiration (the inhalation of food or fluid into the lungs) pneumonia. Managing dysphagia in conjunction with other disease symptoms can be challenging. For example, patients with behavioral variant frontotemporal dementia (bvFTD) often exhibit hyperorality, characterized by food craving, eating past satiety, consuming large amounts of food without proper swallowing techniques, and impulsivity. Hyperorality, alone, presents a risk for aspiration but becomes exceptionally hazardous when coupled with dysphagia. The initial step in managing potential dysphagia is to obtain a swallowing evaluation by a speech (and swallowing) therapist to determine which mechanisms are contributing to swallowing deficits and outline strategies to minimize the risk of aspiration. Strategies often include modifying food and liquid choices, strict supervision at mealtimes, limiting distractions while eating, cutting up food in smaller pieces, proper positioning of the patient during feeding and drinking, limiting the amount of oral medications, and a schedule for checking weight (for more information, see http://youtu.be/H6ooxyGpj4o and http://youtu.be/6Fv9B4t9L8A). Behavioral symptoms are very common in dementia and manifest in ways often specific to the type of dementia. For example, visual hallucinations are common early in DLB and Parkinson’s disease with dementia (PDD) but might occur in late stages of AD and are uncommon in most other dementias. Management of problematic behaviors can be one of the most challenging aspects of caring for a patient with dementia. Employing an “Antecedent, Behavior, and Consequences (ABC)” method of assessment is helpful in the assessment and management of behavioral symptoms [8]. The “A” refers to the potential triggers for the behavior. Examples could include pain or environmental demands exceeding patient tolerance. The “B” refers to the behavior and should be described in specific terms. For example, “Walks up and stands uncomfortably close to strangers and touches their shoulders” is more specific than “is embarrassing in public.” The “C” refers to consequences following the behavior. For example, did the response by the caregiver improve the behavior? An assessment of behavioral symptoms should always include identification of the onset of the behavior as abrupt changes might reflect medical illness (e.g., a urinary tract infection), pain, or side effect from medication, rather than an expected feature of advancing disease. Generally, there are four categories of interventions designed to manage behavioral symptoms ranging from least restrictive to the most restrictive. The first, and least restrictive, type of intervention involves modifications to the patient’s environment. Scheduling activities at a certain time of day more suitable to the patient’s tolerance, removing visually disruptive objects from the house, and simplifying communication to enhance comprehension are examples of environmental modifications. The second type of intervention is behavioral modification. Some examples include distraction from an inappropriate behavior toward a more acceptable activity or providing a reward for more acceptable behavior. The third category of intervention involves strategies internal to the caregiver, such as coaching the caregiver to accept that the behaviors are an expected feature of the disease. The fourth, and most restrictive, type of intervention involves chemical or physical restraints. Although medications might treat specific behavioral symptoms, there are many instances in which a medication is not appropriate, is ineffective, and/or is associated with negative side effects. Polypharmacy and the inappropriate use of medications often potentiate many behavioral symptoms [9]. Physical restraints frequently are associated with negative consequences for patients and should also be avoided whenever possible. An individualized assessment of the behavior(s) and the creation of tailored interventions have the best chance of success at ameliorating the negative impact of symptoms. New models of care have been proposed and include dyadic interventions with patients and caregivers or referrals to experts in managing behavioral symptoms [10]. Setting appropriate and realistic goals is another critical feature. A reasonable goal might be a reduction in the severity of the behavior and/or the caregiver experiencing reduced levels of distress, rather than a goal of eliminating the behavior entirely. A frequent reassessment of behaviors and interventions (including medications) is required, as prior behaviors often will disappear over the course of the disease, whereas other new behaviors might arise that require different interventions. It is inevitable that families will require assistance to provide care. Formal (paid) and informal (unpaid and typically refers to care provided by family) support is usually needed and may include hiring caregivers from an agency, enrolling in adult day programs, or identifying facilities for placement. The majority of these services were established for frail elders, and finding a good “fit” for younger individuals or those with challenging physical or behavioral symptoms is often difficult. Working with a placement advisor or local disease associations to identify resources that can meet the needs of these populations may be helpful. The most common cause of death in atypical dementias is similar to those in other forms of dementia, usually complications related to aspiration and immobility. Problems with swallowing and movement occur earlier in many of the atypical dementias and therefore contribute to an earlier morbidity and mortality [11]. A discussion of end-of-life choices should occur early in the disease course to capitalize on the patient’s ability to participate in decision-making. Establishing a durable power of attorney (DPOA) and identifying personal preferences are a critical first step in this process. The introduction of a palliative approach to care can also be addressed with the patient and family at various times along the course of the illness [12]. A palliative approach covers everything from counseling to caregiver respite and attention to symptom management and thus may ensure a more peaceful passing by allowing families to avoid the psychological stress of involving emergency services at the time of death. Due to the complexity and rarity of some of these conditions, however, arranging palliative care can be a challenge [13]. Patients might not be deemed eligible for hospice care due to the differences between their conditions and more common dementias. The motor and behavioral components of HD, progressive supranuclear palsy (PSP), and corticobasal degeneration (CBD) might complicate the referral in that patients may still benefit from ongoing physical therapy for comfort reasons but also need concurrent hospice care. Providers should provide support and documentation to facilitate appropriate referrals. The National Hospice and Palliative Care Organization has a helpful website for resources (www.nhpco.org). Neuropathological diagnosis can be helpful to clinicians and family members after death in cases of atypical dementia. This diagnostic confirmation can serve as closure for family members who have struggled with the disease for years. For clinicians, it provides a determinative correlation between the clinical syndrome and specific neuropathological findings. Brain autopsy can be sensitively included in any end-of-life decision-making session with the family [14]. See videos about what is involved in, and the benefits are of, an autopsy: http://youtu.be/_yL2OlkuNSc and http://youtu.be/q1wXypAlAEQ). Atypical AD refers to a disease in which memory loss is not the predominant deficit at presentation and/or when the disease occurs in people under the age of 65 (see Chapter 3 for review). This includes early-onset Alzheimer’s disease (EOAD) affecting those under the age of 65, as well as patients in whom symptoms are associated with changes in the frontal or more posterior lobes—frontal variant AD (FAD) or posterior cortical atrophy (PCA). The changes associated with these atypical dementias have particular implications for care. Patients with EOAD often have earlier and increased relative impairment in the domains of attention [15], language [16], and visuospatial abilities [17]. Because these deficits result in greater effects on working, driving, and other instrumental tasks, EOAD carries special significance for occupational and family issues. The executive deficits in the FAD patient produce more functional impairment than typical AD [18]. PCA has been associated with EOAD [17], and the significant visuospatial deficits impair basic function early in the disease. Patients may see several eye specialists before diagnosis; unfortunately, traditional visual aids are not helpful as it is not a problem with visual acuity. The younger age of onset predicts an increased need for ADL assistance, and progression of EOAD is more rapid with higher rates of institutionalization than in typical AD [19]; therefore, careful attention to providing training to in-home caregivers or incorporating in-home help early may be necessary. The visual impairments associated with PCA and executive impairments in FAD put these individuals at particular risk for unsafe behaviors. Home safety evaluations are necessary to provide specific alterations to minimize risk and encourage independence. Patients with EOAD and their families are often contending with traditional midlife issues such as caring for aging parents, caring for children, and planning for retirement while also managing the symptoms of a neurodegenerative disease. FAD patients often have more neuropsychiatric symptoms, and caregivers report more stress than typical AD [18, 20]. Younger children might become the primary caregiver for a parent or might lose a parent at a critical time. Targeted support groups or referrals to individual therapists may provide more personalized family-oriented approach in these cases [21, 22]. Strategies for modifying the environment have been developed to assist in management (http://memory.ucsf.edu/caregiving/tips#safety). Vascular dementia (VaD) refers to cognitive impairment from strokes and/or cerebrovascular disease (see Chapter 4). The degree of impairment depends on the extent of disease and specific regions affected, and interventions should be targeted to an individual’s symptoms. For example, patients with a stroke or ischemic vascular disease in the frontal lobes might show behavioral impulsivity and need interventions to manage these issues, whereas someone with occipital involvement might need more visuospatial assistance. Motor impairment is common in VaD, tends to occur earlier in the disease process than in AD, and requires earlier intervention and activation of supportive services. Autonomic dysfunction (fatigue, urinary symptoms, constipation, and postural dizziness) is also common, leading to increased fall risk and a greater need for ADL and IADL assistance [23]. Patients with VaD typically have less anomia than patients with AD [24], and speech may be easier to understand [25]. They also have been reported to have preserved insight about their condition [26] and therefore might benefit from verbal activities and support groups. In VaD, impairments may be global or focal depending on the location of injury, so there is not one consistent functional profile [27]. Families often report more fluctuation in cognition than in AD [28], and this variability requires flexibility in approach. Deficits in attention, speed of processing, and executive function [29] may present specific safety concerns, especially in regard to driving and independent function. The frequency of gait and balance disorders in VaD increases the risk for falls [30]. Providers can consider referral for balance and gait training, encourage more ambulation, and assess for appropriateness of assistive devices. Although neuropsychiatric profiles in VaD overlap with other dementias, there is an increased risk of depression in VaD [31, 32]. Assessment and treatment of depression and other neuropsychiatric symptoms (e.g., anxiety), including therapy, support group, and medications, should be considered. Some studies report more sleep disturbance in patients with cortical VaD than in AD or subcortical VaD [33]. Attention to proper sleep hygiene including maintenance of day/night rhythms through a structured daily routine and minimizing daytime napping is important. bvFTD presents with early and prominent changes in behavior, emotion, and personality (see Chapter 5). Compared to the other FTD subtypes and typical AD, bvFTD is associated with more severe and rapid impairment in functional abilities [34]. The loss of a job, inability to master new tasks, personality conflicts with coworkers or employers, and difficulty in keeping up with executive responsibilities are often some of the first signs of the illness. Function around the home often declines; patients might participate less with chores and exhibit a lower standard of personal hygiene and clothing selection while demonstrating little concern for these changes. Patients with profound apathy often exhibit a complete loss of concern or initiative in maintaining hygiene. FTD can occur concomitantly with ALS [35], causing a more rapid decline [11, 36]. The ability to engage responsibly in certain activities typically is compromised in bvFTD. For example, driving skills, supervision of children, participation in finances, and legal planning are commonly affected. Due to loss of insight, patients typically do not acknowledge how troubling the behavioral changes are to others nor do they show remorse for the negative consequences of their actions.
Comprehensive management of the patient with an atypical dementia
After the diagnosis
Evaluating functional abilities
Vulnerability and safety
Risk areas
Suggested interventions
Legal and financial decision-making
Driving
Social misconduct
Communication and comprehension deficits
Getting lost
Occupational issues for the patient and caregiver
Managing motor symptoms
Gait instability and falls
Dysphagia
Managing behavioral symptoms
Care across the disease trajectory
End-of-life care
Autopsy decisions
Key considerations for the clinical management of atypical dementias
Atypical AD
Functional changes
Safety concerns
Psychosocial adjustments
Vascular dementia
Functional changes
Safety concerns
Psychosocial adjustments
FTD (behavioral variant)
Functional changes
Safety concerns
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