Behavioral variant FTD

Impairment in executive functioning and ADL performance occurs early in the disease for patients with bvFTD [10]. We have shown [11, 12] a similar level of impairment in basic ADL and instrumental ADLs in bvFTD, but this finding is not universal [13]. This discrepancy is likely to be due to different methodologic approaches in instrument selection or inclusion of patients at different stages of the dementia. In general dementia, ADL decline follows a well-known pattern, in which instrumental ADLs are usually affected first, followed by late deficits in basic ADLs. However, this is usually not the case for bvFTD, where both instrumental complex tasks as well as basic ADLs are affected from disease onset. The great majority of bvFTD patients (90%) are impaired in basic ADL performance [11]. As such, impairment on basic ADLs features in the diagnostic criteria for FTD [14, 15].

In comparison with other FTD variants, bvFTD patients perform more poorly in both instrumental ADLs and basic ADLs than svPPA and non-fluent variant PPA (nfvPPA) patients in most activities, including household chores, shopping, finances, leisure, and self-care [11, 13, 16]. In comparison with AD, bvFTD patients show similar levels of instrumental ADL impairment, but more severe basic ADL impairment than AD patients when matched for length of symptoms. The rate of progression of functional disability is also marked in bvFTD, where patients decline more rapidly than other variants over a 12-month period [6, 12, 17, 18].

Assessment of functional decline in bvFTD is not only important because of its severity from the early stages, but also because it is not directly associated with cognitive deficits. In other words, high scores on standard cognitive tests, such as the Addenbrooke’s Cognitive Examination or the Mini-Mental State Examination (MMSE) can lead to an erroneous assumption that the patient is not very impaired at home or in the community. It has been demonstrated that bvFTD patients may continue to score relatively well on cognitive assessments when in reality there is marked worsening in the patient’s abilities to perform activities at home and in the community safely [19]. (Patients with PPA variants show a clear decline on these tests from an early stage, and this will be discussed below.) When the striking impairment to perform ADLs is added to a low or absent level of insight into their own changes, patients can become evidently vulnerable to scams and malicious behavior, but can also present risk to others around if not monitored closely.

Driving is usually one of the most distressing areas to address with patients and families. In most cases, speeding and risk-taking behavior have been suggested as being related to behaviors such as disinhibition and agitation [20, 21]. Arranging for a professional driving assessment allows for an objective assessment from an external party, and minimizes the chances of the patient blaming the loss of the license on the spouse or family. However, the removal of the driver’s license may not impact on their attempts to drive. Common issues with shopping are related to overspending, purchasing unnecessary items such as those shown on television commercials, paper advertisements, or even via door-to-door sellers at home – as a result of poor judgment and impulsivity [22]. In some extreme cases, patients might shoplift, which can be very distressing to the family. It is often reported that while the patient is able to go to the shops independently, they might ignore the shopping list, only returning with items which interest them (such as biscuits, lollies, or other favorite items). Impaired judgment can lead to mistakes in financial management, but the patient might be rigid about retaining their role as financial manager, refusing help, and in some cases hiding correspondences and bills received in the mail. bvFTD patients can become very prone to email scams, and many families have lost considerable amounts of money as a result of these behaviors. Inappropriate phone calls such as calling people or services multiple times a day, and disclosing credit card or personal details to telemarketers can occur quite often [22]. Changes in behavior may lead to over-medicating, or refusal to take medication [23, 24], which can become stressful scenarios for the family. Apathy and executive dysfunction are common symptoms that may impact on a person’s participation in meal preparation [24–26]. An important aspect to management of any of these behaviors is carer education about FTD and how the condition manifests in behaviors. Table 16.1 offers a number of strategies that might be useful in managing instrumental ADLs in bvFTD.

Basic ADLs are also affected in bvFTD from an early stage, contrary to most dementias, where there is usually a clear gradient of loss from the most complex to the simplest tasks.

Neglect in personal hygiene is an early symptom, and can affect routines with showering, brushing teeth, and grooming (e.g., shaving, combing hair, and using makeup). This neglect worsens as the disease progresses [12, 24]. Patients often wear a reduced selection of outfits, or tend to repeat a favorite piece (e.g., shirt, dress, underwear), which can lead to refusal to change even when washing is obviously needed. Also, wearing inappropriate clothing can be a consequence of several deficits: inability to choose appropriate outfits for a specific occasion, inability to recognize weather conditions or even potentially changes in temperature regulation. Incontinence can occur quite early in the disease and can be the result of behaviors such as apathy, decreased insight, or even disinhibition, rather than a specific physiologic cause. Examples include passing urine sitting on the couch rather than getting up; passing urine while eating cake as the patient did not want to leave the table; using pot plants and rubbish bins as urinals in the care facility. Of note, it is important to consider that incontinence can also result from health conditions such as prostate enlargement or urinary tract infections, and therefore these should be ruled out as potential causes with the patient’s doctor. Eating habits are generally affected from an early stage, with changes in food preference being very common in bvFTD, in particular increased preference for sweet foods and carbohydrates [24, 27–29], which can lead to marked weight increase. In most cases, patients tend to seek out preferred foods, rummaging through the refrigerator and pantry, or even going shopping recurrently to obtain the item. Table 16.2 shows strategies that might be helpful in managing basic ADLs in bvFTD patients.

Primary progressive aphasia: semantic variant

Impairments in semantic memory and difficulty recognizing faces, voices, or names are common in svPPA and impact on the person’s ability to engage in instrumental ADLs such as social events, using the telephone, and appropriately managing correspondence [11]. These impairments can ultimately result in early retirement if the person was in the workforce at diagnosis.

Patients with svPPA show similar rates of impairment to bvFTD and nfvPPA in instrumental ADLs; however, they differ in their performance of basic ADLs [11]. Patients with svPPA usually have preservation of basic ADLs for a number of years compared with instrumental ADLs, which are usually impaired from an early stage.

In terms of functional progression over time, svPPA patients have been reported to decline at a less rapid rate than both nfvPPA and bvFTD patients [8, 30, 31]. This is also seen when measuring dementia stages: svPPA patients are quite stable in disease progression for many years, from a functional perspective [6, 31].

On cognitive tests, patients with svPPA are often globally impaired owing to their language deficits; however, at home they often maintain their engagement in routine tasks until later into disease progression. This is likely to be associated with the rigid routines that are extremely common in patients with svPPA; future studies should address this potential relationship.

Ability to perform instrumental ADLs in svPPA is therefore well maintained for years, despite the evident semantic deficits and rigid behavior. Driving skills are often well preserved until the moderate stage. Impairments in language can pose a risk for these patients if they were to be involved in a car accident or altercation where they may have difficulty communicating with authorities. Ability to shop independently remains for some years if the patient is purchasing habitual items. Semantic impairments may eventually create difficulty with recognizing products and produce. Overspending is not a common issue in svPPA; patients in fact may become increasingly frugal with money, which often manifests as obsessive searching for “bargains.” Managing medications is also usually not a problem for svPPA patients for a number of years, which seems to be made possible by their ritualized routines. Meal preparation can be affected by deficits in semantic knowledge of kitchen implements and foods [32], and ability to perform household chores is often retained until the late stages of the dementia, often driven by compulsions such as the daily need to pack the dishwasher in a certain way [33]. Social outings tend to be greatly affected by changes in behavior, especially disinhibition. These behaviors may include (but are not limited to): approaching people or children they do not know when out in public, commenting loudly about the way people look, or providing bizarre demonstrations such as dancing or singing [34]. When disinhibition is pronounced, carers can become very embarrassed and may prefer to limit outings (see Case 2). Overall, patients with svPPA adhere to rigid, time-constrained routines, which in fact seem to facilitate their engagement in a limited range of activities [33, 35]. Narrowing of interests is another common symptom seen in svPPA patients, where they may develop a more focused, obsessive interest in only one or two activities – and will spend a large amount of time engaging within these [36], e.g., spending hours per day engaged with jigsaw puzzles [37]. Case 3 provides an example of a carer changing her own reaction to a situation, rather than trying to change the patient’s behavior. Attempting to interrupt a person’s rigid routine is likely to elicit distress, irritability, and aggression [36]. Table 16.3 contains a number of strategies that might be useful in managing instrumental ADLs in svPPA and nfvPPA.

Ability to perform basic ADLs is well maintained in svPPA until the very late stages of dementia, even though patients might not be able to name products or to verbally communicate the activities they are intending to perform. Late in the disease, difficulties in differentiating products appear, e.g., patients can mistake shaving cream for toothpaste. As with all dementias, patients might refuse to perform personal hygiene tasks at the severe stages and excessive prompting often leads to increased stress and unsuccessful outcomes. Dressing issues might also appear later in the dementia. Changes in food preferences are also common in svPPA: restrictive dieting or rigidity with specific foods are common [25, 36], which can be accompanied by fixed times to set the table and eat. Secondary to their semantic deficits, patients may also consume unsafe items such as raw meat, mouldy food, or non-food items such as soap or a kitchen sponge in the severe stages of the dementia [38]. Strategies that might be helpful in managing basic ADLs in svPPA and nfvPPA are shown in Table 16.4.

Primary progressive aphasia: non-fluent variant

Frank language dysfunction in nfvPPA leads to impairments in writing and reading and eventually mutism, with great impact on the ability of patients to participate in leisure activities such as social events and other interpersonal interactions [35]. The majority of patients with nfvPPA do not show any changes in their performance on basic ADLs for a number of years post-diagnosis, despite performing poorly on general cognitive assessments [13, 39, 40]. While patients with nfvPPA have relatively preserved basic ADLs, impairments in instrumental ADLs are common, ranging from mild to severe impairment by around two years into disease progression [39, 41].

Longitudinal changes to function over a 12-month period have recently been suggested to be more marked in nfvPPA than in svPPA [6]. In nfvPPA, clear associations between global cognition and functional disability have been demonstrated [39, 40], especially in cohorts of patients assessed longitudinally.

Instrumental ADLs are impaired from an early stage also in nfvPPA, though mostly secondary to language deficits. Driving skills are often preserved until late in the disease, unless apraxia appears. As with svPPA, impairments in language can become a risk for these patients if they were to be involved in a car accident where they may have difficulty communicating with others. Patients with nfvPPA may be able to continue managing financial tasks for a relatively long period into the disease. Telephone use tends to become an issue as patients avoid it, because of awareness of their communication limitations; many patients prefer to text or email, even if spelling mistakes are obvious. It is not exactly clear why nfvPPA patients reduce their participation in meal preparation, since they often maintain good semantic knowledge of kitchen-related items. It is likely that a combination of early apraxia and deficits in multitasking can hinder their ability to prepare meals [42]. The ability to perform household chores is often retained until late into the dementia, when other combined cognitive deficits impact on their ability to perform chores. Insight into their language deficits is a common cause of social withdrawal.

Basic ADLs are very well preserved for a large numbers of years in nfvPPA. However, once the patient reaches the severe stages, refusal to engage in personal hygiene tasks can occur as in most dementias. Incontinence does not usually appear until very late into disease progression, and can therefore be managed according to standard dementia practices. Eating and dietary changes are rarely seen in nfvPPA.