The number of people with dementia, a disease that causes progressive decline in cognitive function, is increasing worldwide (Myhre et al, 2018; Thoma-Lurken et al, 2018). Dementia is considered to be a terminal condition that shortens an individual's life span. Therefore, dementia meets the World Health Organization's (WHO) definition of a condition in need of palliative care via an ‘approach that improves the quality of life of patients and their families facing the problems associated with life-threatening illness, whether they are malignant or not’(WHO, 2019).
There are 13 types of diseases that cause dementia symptoms (Heerema, 2019). Depending on the specific disease, the overall health of the individual, general treatment response and average life expectancy can range from a few weeks (i.e. Creutzfeldt-Jakob's disease) to 20 years (i.e. Huntington's chorea, fronto-temporal dementia (FTD) and Alzheimer's disease). Differentiating among the types is often determined by initial presentation, especially in those under the age of 60 years.
FTD is the second most common form of early-onset dementia with a prevalence equal to that of Alzheimer's dementia (Shi et al, 2018). There are three forms or phenotypes of FTD—behavioural variant FTD (bvFTD), primary progressive aphasia and movement disorders associated with FTD—of which bvFTD is the most common.
The signs and symptoms of bvFTD are typically identified in mid-life, at around 50–60 years of age, and they progress requiring increasingly greater caregiver support (Moheb et al, 2017) (Table 1). Diagnosis is often significantly delayed because symptoms are insidious and appear as personality and behavioural changes, such as lack of inhibition, apathy, depression, emotional withdraw and being socially inappropriate, rather than marked reductions in memory (Johannessen et al, 2017). Further contributing to the diagnostic challenge is the frequent lack of recognition of the signs and symptoms of, and behavioural management strategies for, bvFTD by GPs.
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This article describes the clinical progression and nonpharmacological behavioural management strategies for bvFTD through a case study. When providing care to home-bound patients with bvFTD, community nurses can educate family members in implementing these strategies. Management strategies may vary by country and region. This article provides general information for diagnosis and management strategies that should add to the body of nursing knowledge.
Case presentation
JA was a 51-year-old divorced woman with one daughter and worked as a loan officer at a local credit union. One day, she committed an error involving a significant amount of money. On recognising the mistake, JA panicked. While not a core component of FTD, her panicking led to what was later determined to be a psychogenic seizure. Psychogenic seizures are not caused by abnormal brain electrical activity but are usually a reflection of a psychological conflict or psychiatric disorder. They are often precipitated by an acute life event in a person who was previously traumatised, most commonly during childhood.
As a result, JA was sent to the hospital for evaluation. The team started by conducting a CT scan of the head, which was unremarkable, except for some generalised atrophy, predominantly in the frontal and temporal lobes. Laboratory studies to rule out infection and other potential causes yielded normal results.
JA was admitted for further evaluation including an MRI, 24-hour and EEG. During the neurologist's initial visit, a comprehensive neurological examination, including the Mini Mental Status Exam (MMSE) revealed that JA was unable to complete several components: attention and calculation, recall and complex commands (Table 1). This type of deficit, in a patient as young as JA, was unusual and, therefore, very concerning.
After discharge, JA was referred to several neurological speciality clinics for further evaluation. Further neurological assessments, an EEG, lumbar puncture and additional laboratory tests proved inconclusive. Due to limitations placed by third-party payers (i.e. insurance companies and government-funded programmes such as Medicare and Medicaid in the US), biomarker tests, genetic testing and tests for tau and amyloid proteins were not completed.
Over the next 2 years, JA was referred by her GP to multiple other psychologists, psychiatrists and neurologists. These specialists reviewed JA's health history provided by her daughter and her clinical presentation, which now included frequent repetitive yawning despite not being tired. Neuropsychiatric testing revealed cognitive deficits in date, recall, digit span, word list generation, similarities questions, verbal fluency and written questions. Attempts to improve JA's apathy and executive dysfunction were unsuccessful despite prescribing escalating doses of antidepressants and anti-anxiolytics. Eventually, a neurologist specialising in dementia diagnosed JA with early-onset dementia.
On reflection, JA's family remembered many symptoms of FTD appearing years before the calculation incident at work. However, it is unknown when JA's symptoms of FTD first began. For example, she would frequently forget things, such as her glasses and purse, and her dietary changes included wanting to eat one food item every day. She also exhibited sexual promiscuity and self-isolation. Her mother recalled JA frequently saying about her siblings, ‘They might be your daughters, but they will never be my sisters’.
As JA's disease progressed, the behavioural and personality changes became more apparent, and she began spending excessive amounts of money on things that were not typical, such as 38 tropical birds with individual cages costing greater than $1500 a piece and large amounts of clothing. She would spend days eating popcorn, pretzels and crushed ice with lemonade powder. Her interest in friends and family became almost non-existent, to the extent that she missed her daughter's 16th birthday. She never invited her daughter and grandchildren to visit. Conversely, her interest in men escalated, and she started inviting strange men home to not only stay but live with her.
Once the family obtained a medical power of attorney, further work-up determined that JA had bvFTD; she exhibited many of its core clinical features, including an insidious onset, gradual progression, early decline in social interpersonal skills, emotional blunting and loss of insight. Supportive features such as perseverative/stereotypical behaviour, inability to manage money and speech and language difficulties were also present. Treatment included discontinuing the antidepressants and anxiolytics.
About 2.5 years later, JA's cognitive status declined further, and she was unable to recognise new people or places and tell the time, season or year. She had stopped adjusting her clothing for the weather, was no longer able to cook and did not show signs of hunger. JA wandered frequently and went on walks but would almost immediately return home. She did not initiate conversations and, when she did speak, she would only use one-syllable words.
Eight years after symptom onset, disease progression led to JA becoming unable to be cared for at home. She had stopped talking but cried frequently, became bedridden and, ultimately, required two caregivers around the clock. During hospitalisation for an upper respiratory infection, the family decided it was time to place JA in a nursing home. JA never bore weight on her feet or spoke again. She cried almost constantly. About 6 months later, she began to aspirate, stopped eating and died subsequently.
Pathophysiology
bvFTD primarily involves the frontal and temporal lobes of the brain. Progressive atrophy extends to the middle and upper parts of the frontal and anterior temporal lobes and lateral ventricles, with relative sparing of the parietal and occipital lobes, cerebellum and brain stem (Bertoux et al, 2015; Che et al, 2018). Myelination of nerve fibres in the frontal and temporal lobes allows for highly synchronised action potential timing, which is responsible for an individual's cognitive and behavioural functioning. These lobes also have fewer oligodendrocytes to support a large number of axons (Bertoux et al, 2015; Che et al, 2018). Because axons in the cortical association areas of the brain have a large distribution network, the oligodendrocytes have a greater metabolic need to maintain this area (Bertoux et al, 2015; Che et al, 2018). As a result of their abundant distribution and greater resource demands, these axons are more vulnerable to pathological processes and breakdown (Bertoux et al, 2015; Che et al, 2018).
The orbito-frontal cortex, anterior temporal lobe and insula appeared to suffer the most atrophy, while the posterior gyri are relatively preserved (Bertoux et al, 2015). This atrophy is associated with a gradual loss of approximately 4% brain volume loss/year (Rohrer et al, 2016). Regardless of the underlying cause, atrophy in bvFTD has consistently been found to involve the anterior cingulate cortex, anterior insula, striatum, amygdala, hypothalamus and thalamus. In adults, these regions of the brain are interconnected and form the salience network, which is responsible for executing the appropriate visceral-emotional-autonomic, behavioural and cognitive responses. Because these diverse regions of the brain require a dynamic, time-sensitive action potential that is in sync with rapidly evolving social processing, breakdown in these systems can occur in multiple ways with a common end product— bvFTD. As the disease process evolves, changes in cerebral neurochemistry and neuroanatomy are responsible for bvFTD symptoms. While asymmetric atrophy can occur, at autopsy, patients with bvFTD were observed to have bilateral cerebral atrophy in the white and grey matter of both lobes (Bertoux et al, 2015; Che et al, 2018).
Symptoms
The extreme and overwhelming changes in personality and behaviour are the most significant clinical feature of bvFTD and foreshadow disease onset (Table 1). Typically, other symptoms include progressive, debilitating personality and behavioural alterations and impaired communication and interpersonal skills. If only the right frontal lobe is involved, patients present poor insight and lack of inhibition. Conversely, if only the left frontal lobe is involved, they are likely to be depressed (Everhart et al, 2015). With right temporal degeneration, patients may present some psychiatric symptoms, such as obsessive-compulsive behaviours, lack of interest in family and lack of empathy. Delusions are frequently inappropriate, bizarre and may be associated with jealousy. There can also be inappropriate feelings of euphoria (Everhart et al, 2015). Prosopagnosia—loss of the ability to recognise faces, even one's own—also typically occurs. Finally, the speech centre located in the left temporal lobe degenerates, resulting in difficulty with communication.
The individual's usual self-constraint and social behaviour may be hampered. Because the personality and behavioural changes occur early in the disease process, while memory impairment occurs much later, the condition is frequently mistaken for a psychiatric disorder. The individual's family and friends are often overwhelmingly disturbed by the changes. As a result, there can be a considerable disruption in the home and workplace. Sometimes criminal behaviours (stealing, sexual impropriety and unethical business activity) are the initial signs that gain attention. Because the frontal lobe is primarily responsible for higher level executive functions as well, the individual may have issues with planning and sequencing, prioritising, multi-tasking and self-monitoring and correcting behaviour. Conversely, when an individual has Alzheimer's disease, they experience impaired memory, language and visuo-spatial functions (Rascovsky et al, 2011).
The behavioural symptoms associated with bvFTD include irritability, lack of inhibition, apathy, depression, emotion withdrawal, repetitive behaviours, euphoria and social impropriety. Additionally, the patient may also be self-centred, have poor self-hygiene and rigid stereotypical behaviours. Weight gain occurs due to carbohydrate craving and gorging. In the early stages, because the behaviours may appear as an atypical depression or mood disorder, bvFTD is often mistaken for a mood or affective disorder (Pospos et al, 2018). In the later stages, physical deficits and limitations become severe, causing most patients to experience severe physical disabilities. Memory and cognitive deficits also become more prominent. Individuals may have difficulty speaking or even become mute. They may also become incontinent, have problems walking, resulting in frequent falls, and develop dysphagia. The progressive disability results in the inability to self-care and perform basic activities of daily living, ultimately leading to dependency on long-term care.
Diagnosis
Disease duration in FTD is approximately 6–8 years from the onset of clinical symptoms (Valente et al, 2019). Various assessment tools are available for neuropsychological testing, which should include a thorough patient and family history as well as tests to specifically detect the primary symptoms, behavioural changes and language deficits associated with bvFTD (Merrilees et al, 2014; Sadak et al, 2014; Garre-Olmo et al, 2016; Lautenschlager et al, 2016).
An individual is considered to have a diagnosis of possible bvFTD based on the presence of clinical hallmarks. A probable bvFTD diagnosis requires the presence of both functional decline and abnormalities that can be seen on neuroimaging tests, such as MRI. Atrophy occurs bilaterally in the frontal and anterior temporal cortical cortex (Agosta et al, 2012). Some patients with possible bvFTD go on to develop neuroimaging changes, or a genetic mutation is identified, re-classifying them as having probable or definite bvFTD.
FTDs are clinically, genetically and pathologically heterogeneous neurodegenerative disorders (Zetterberg et al, 2019). As many as 40% of patients with FTD have a positive family history of dementia with an autosomal dominant pattern of inheritance (Zetterberg et al, 2019). About one-third of occurrences are autosomal dominantly inherited as a result of mutations in progranulin, microtubule-associated protein tau or chromosome 9 open reading frame 72 (Zetterberg et al, 2019).
Management
While there is no treatment or cure for bvFTD, managing symptoms is helpful and, therefore, should be the primary treatment goal (Sivasathiaseelan et al, 2019). Symptom management requires thorough, careful evaluation and monitoring.
Treatment of cognitive symptoms has been relatively ineffective. There have been some attempts to use selegiline, which is typically used for attention deficit hyperactivity disorder, because dopamine levels in the brain are often decreased, but this has not proven to be beneficial (Logroscino et al, 2019). Donepezil is also not effective because the cholinergic system remains relatively intact in FTD (Liu et al, 2019). Depression, disinhibition, carbohydrate craving and obsessive-compulsive behaviours may be improved with the use of selective serotonin re-uptake inhibitors (SSRIs), such as fluoxetine, sertraline, paroxetine and citalopram (Logroscino et al, 2019). However, fluoxetine is often avoided due to its long half-life. Benzodiazepines have been found to significantly increase delusions, hallucinations, agitation, depression, apathy in patients with dementia and are, therefore, typically avoided (Pariente et al, 2016).
Sodium valproate and valproic acid may be useful for managing behaviours such as irritability, aggressiveness or disinhibition. If the individual experiences delusions, hallucinations or becomes severely aggressive, antipsychotics may be useful. Caution should be exercised due to the side effects associated with antipsychotics, which includes increased drowsiness, falls and paradoxical disinhibition. Therefore, frequent monitoring is recommended.
Non-pharmacological interventions and strategies
Ensuring patient, family and staff safety and wellbeing are always a priority. Therefore, individualised care planning in collaboration with family is needed (Woods et al, 2012). Safety measures are necessary to prevent injury while maintaining as much freedom as possible. The use of multimodal interventions may provide better symptom and behaviour management, delaying transitions to long-term care (Sadak et al, 2014; Moheb et al, 2017). While there may be impairments in verbal communication, non-verbal communication remains intact (Mulkey, 2019). As such, the individuals with bvFTD frequently use non-verbal communication (Machiels et al, 2017). Formal and informal caregivers should consider the behaviour's purpose. Using short sentences, making eye contact and active listening strategies may assist caregivers in communicating with the individual (Mulkey, 2019). Correcting or confronting patients should be avoided (Machiels et al, 2017; Pfeifer et al, 2018). It is important to remain calm, use positive non-verbal communication and move away from the individual when challenging behaviour occurs. Use of distraction and re-direction is likely to increase agitation in individuals with bvFTD (Mulkey, 2019). To calm the agitation, talking in a calm, lowered voice may be helpful (Machiels et al, 2017; Pfeifer et al, 2018). Caregivers should try not take the patient's remarks or behaviour personally.
Having consistent routines may reduce the likelihood of negative outbursts. Consideration regarding the amount and type of stimulation and removing or decreasing stimuli may reduce agitation (Mulkey, 2019). Roaming behaviour serves a purpose for the individual with bvFTD and should, therefore, not be completely discouraged but may require supervision. The individual will typically follow the same pattern or routine and go to the same place. Providing a safe protected area for roaming that is free of clutter and obstacles while having the patient wear non-skid footwear will help reduce injuries. Finally, roaming will increase caloric and hydration needs, and this should be considered when planning meals (Mulkey, 2019).
Caregiver strategies and support
In the UK, there are approximately 670 000 unpaid carers of people with dementia (Carers Trust, 2019). Due to the younger age of symptom onset, family caregivers of individual's with FTD are often working spouses and school-aged children. These caregivers have been shown to have higher burden and different needs than those of caregivers with other types of dementias (Rosness et al, 2008).
According to Mullins et al (2016), health literacy includes having the requisite knowledge and understanding to perform clinical, prevention and health system navigation tasks. Therefore, it is vital that caregivers receive information about dementia from a qualified clinician. Patient and family education and training should include information related to the disease process and symptom management, including behavioural and psychological symptoms. Information regarding available services and medications for symptom management is critical, as is information related to risk factors. Due to the high level of stress they experience, caregivers often benefit from education related to managing their stress and developing effective coping strategies (Whitlatch and Orsulic-Jeras, 2018).
Caregivers are frequently exposed to aggressive behaviour from individuals with bvFTD. A behaviour log may help identify precipitators and patterns of aggression and warning signs. Individuals with bvFTD often lack awareness of the discomfort they illicit when they invade someone's personal space (Talerico, 1999). Allowing the patient to drive a vehicle is dangerous and should be prevented (Piersma et al, 2018). This is often challenging for the family and caregivers to address, because the ability to drive means freedom.
Caregivers also experience feelings of inadequacy. When they are not able to provide adequate care, they feel guilty, which increases the risk of depression (Rosness et al, 2008). When counselling and support are provided, caregivers are better prepared to care for the individual with bvFTD. As a result, the length of time these individuals are able to stay in the home increases, and the caregiver's quality of life is improved (Rosness et al, 2008). These benefits are typically realised during the middle stage, when the individual's condition may not require 24-hour nursing care. The Aging Well Hub, founded by the Global Social Enterprise Initiative, published a short book called ‘The journey map’ that is particularly helpful to support caregivers throughout all stages of dementia (Aging Well Hub, 2018).
Formal training programmes for caregivers have been shown to not only increase health literacy, but also improve care capacity, confidence and caregiver health outcomes (Mullins et al, 2016). Extended family and friend caregivers may also benefit from nurse-led education. Interacting with other caregivers and participating in support groups have been found to decrease caregiver emotional and social burden. The reduction in caregiver burden is a direct result of providing them with the knowledge they need to understand and accept the disease, as well as how to care for their loved one (Küçükgüçlü et al, 2018). Use of online discussions and mobile device applications may be also be helpful, but further research is needed to confirm this.
Conclusion
bvFTD is a type of early-onset dementia that initially presents as behavioural changes that may be mistaken as a personality disorder or depression. As the disease progresses, the individual's speech and language become impaired. Simultaneously, as physical impairments increase, the individual becomes incontinent and bedridden. Due to the early age of onset and behavioural impairments, the burden is often higher on caregivers. Therefore, it is critical that clinicians provide caregivers education regarding disease progression and the available resources as well as encouraging participation in support groups. Nurses play a vital role in assisting family and caregivers to develop safe and effective strategies for care delivery. Another very important role nurses is as patient advocates and educators. Therefore, they should understand the peculiarities FTD presents and be prepared to assume these critical roles.