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KERMCP

Joined 6 October 2017

Frontotemporal Dementia (Pick's disease)

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With a growing aging population, there has been an increase in the prevalence of neurodegenerative disorders such as dementia. There are several types of dementia which include Alzheimer’s disease (AD), vascular dementia (VaD), dementia with lewy bodies (DLB), dementia in Parkinson’s disease (PDD) and frontotemporal dementia (FTD). All are characterised by a decline in both cognitive and functional ability. The boundaries between different sub-types of dementia are indistinct and therefore some often coexist [1].

Frontotemporal dementia is a rare form of dementia. It is divided into three major sub-types: behavioural variant frontotemporal dementia (bvFTD), semantic variant of primary progressive aphasia (svPPA), and non-fluent variant primary progressive aphasia (nfvPPA)[2].

Prevalence

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An estimated 47million people worldwide were believed to have dementia in 2016, and the figure is expected to increase to 131 million by 2050 due to the population living longer [3]. Frontotemporal dementia (FTD) is recognized as the second most common neurodegenerative dementia in patients under the age of 65 and the third most common neurodegenerative dementia in all age groups [4][5].

Diagnosis/assessment

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Methods used to assess a person suspected to have bvFTD should include behavioural and neuropsychiatric tests, assessment of everyday abilities, cognitive testing, and neuro-imaging imaging methods such as fMRI, CT scan and PET scans . Various blood and bio-markers are being developed, but are not yet available for routine clinical use. Genetic testing is advised for those at high risk of a gene mutation [6]. Behavioural assessment in patients with potential bvFTD are considered the most important assessment. These are more sensitive at distinguishing bvFTD from AD than standard cognitive testing [7][8]. Carers play a substantial role in providing information. Carer based questionnaires include the Neuropsychiatric Inventory [9], Cambridge Behavioural Inventory [10], and the Frontal Behavioural Inventory [11]. All of the features found in bvFTD can occur in other dementias, however, the behavioural symptoms that have early onset are more typical in  bvFTD [8].

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Misdiagnosis

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Early onset dementia such as FTD is frequently misdiagnosed. Reasons being include Early Onset Dementia (EOD) (onset before age 65)[12] can occur in result of frontotemporal lobe degenerations, traumatic head injury, alcohol related dementia, and is therefore more varied and differently diagnosed as it has many different symptoms in comparison to late onset dementia. Misdiagnosis also occurs because many EODs predominantly have cognitive deficits rather than memory loss, contrary to Alzheimer’s dementia (AD). EOD often has neuropsychiatric features such as psychosis, depression and anxiety as well as schizophrenia, obsessive-compulsive disorder, borderline personality disorder, and bipolar disorder, that are out of proportion to any of the cognitive defective symptoms [13][14]. Clinicians therefore often misdiagnose FTD, in order to successfully diagnose clinicians must consider cognitive and family history factors, as well as mental status and neurological examinations and neuroimaging. Patients who have the disease are often unaware of their illness or have limited insight, therefore an informant of their behalf such as their primary carer is crucial for collecting patient history [13]. Due to early onset in FTD’s people are often diagnosed with non-progressive psychiatric diseases such as schizophrenia and bipolar disorder. It has been found that 50% of patients with bvFTD had initially been diagnosed with a psychiatric disorder before being correctly diagnosed later [15]. Incorrect diagnoses are damaging as they prevent patients and families from being able to plan accordingly for the future, as well as giving patients incorrect and harmful treatments. Although memory is considerably longer sustained in FTD in comparison to AD and other dementia's, patients with FTD often lose episodic memory early in the disease, causing misdiagnosis of early AD [8]. FTD might therefore be more common than expected because older adults rarely receive the appropriate types of investigation needed to establish a confident diagnosis [8].

Stages of Disease Progression/Developmental time course

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Stages are not clearly distinguished as individual’s experiences vary significantly. Stages are broadly defined as mild, moderate and severe. In the mild stage, patients are highly functional and can manage household chores as well as being able to care for themselves without the need of caregiver’s assistance. In the moderate stage, symptoms become more pronounced, therefore the person experiences significant deficits and require increased assistance and supervision [16]. This often leads to contemplation of placing the person in a residential care home, assisted living or nursing facility [17]. At the severe stage of the disease, symptoms are profound and they are extremely compromised, requiring total care. The disease progression involves loss in executive functions such as making decisions, working memory, mental flexibility, and self-control as well as language impairments and repetitive behaviours [18]. Life expectancy of someone diagnosed with FTD is 7–13 years after on average, but can range from as little as 2 years to as long as 20years [19]. Whether development and progression of the disease is rapid or not, almost all patients will eventually develop apathy (a lack of interest and concern) and inertia (doing nothing), some may also become mute and immobile at the severe stage. Many develop movement disorders such as parkinsonism [20] [21]. It has been shown that FTD progresses faster than AD [22][23].

Behavioural problems/personality change developments

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Early on in disease progression when diagnosis is unclear, family members often do not realize that personality changes are caused by a degenerative illness. The patient might be seen by psychiatrists or marriage counselors, human resources at work, and the criminal system, however later with progression, symptoms of global dementia become more visible [24]. Early personality changes include social dis-inhibition and impulsive. People with FTD often break societal norms and are often unable to control themselves from making offensive remarks, or acting inappropriately [24]. They also lose ability to emphasize with others, and become distant and detached from people. An inappropriately subdued grief reaction is a common early symptom as well as tendency to repeat phrases, stories, or jokes [8]. Social dis-inhibition, euphoria, stereotypical and aberrant motor behaviour, and changes in eating habits are symptoms that have been the most clearly discriminative between bvFTD and AD [10]. Many patients develop a preference for sweets, and eat beyond full capacity and eat in excess therefore overindulging, some also develop hyper orality, where they attempt to eat objects that are inedible. Carers must take care that the patient does not steal in stores, and at home they may have to resort in locking kitchen cupboards. Many behave sexually inappropriately, therefore people feel uncomfortable in their presence [25].

Caregiver stress

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Studies have indicated that caregivers of people with FTD have significantly higher rates of burden and stress in comparison to caregivers of people with AD [26] [27]. The behavioural changes involved with bvFTD has been found to be more distressing for caregivers in comparison to level of disability [27]. Being a carer significantly affects a person’s health and has been found to significantly affect depression levels [28]. It has been suggested that increasing a carer’s understanding of the disease and teaching ways of being able to cope and deal with behavioural challenges are key in reducing caregiver stress, however there is lack of evidence and understanding, and therefore not enough support available for those care-giving someone with FTD [28].

Treatment/management

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Currently there is no treatment for any type of dementia, and therefore treatments are dispersed in attempt to alleviate associated symptoms. The US Food and Drug Administration (FDA) have not approved any drugs for treating NPS symptoms associated with AD dementia [29]. Anti-psychotics are commonly used for treating psychological symptoms of the disease which include agitation, aggression and psychosis, however they have shown only to have modest effectiveness [30][31] and serious side effects which include difficulty sleeping, anxiousness, restlessness, inability to keep still, vomiting, tiredness, weight gain, developing unusual movements and even sudden death [32][33][34]. There is an increasing need for new drugs that are safer and more effective in treating dementia and it's associated symptoms[35] . Several drugs are being developed to reduce aggregation of tau or TDP-43 to slow down the progress of the FTD disease, with eventual aim to treat FTD completely [36]

Future treatment potential: Cannabinoids

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Cannabinoids has been researched as a potential future treatment for dementias [37]. CB2 receptors could be involved in reducing neuro-inflamation, meaning that there is potential to provide neuro-protection and in future medicines may be able to slow down the progression of dementia[38] . Endogenous cannabinoid system (ECS) main function is believed to regulate synaptic transmition [39]. Research has also shown that cannabinoids may have potential to reduce neuropsychiatric symptoms (NPS) associated with dementias which include depression and anxiety, agitation and aggression, psychosis, eating disorders, sleep disorders, pacing and wandering [40][41]. A review found that six studies showed significant benefits of the synthetic cannabinoids dronabinol and nabilone in treating agitation and aggression symptoms; however conclusions were limited by small sample sizes, short trial duration, and lack of placebo control [42]. Dronabinol has successfully treated NPS in dementia and improved nocturnal motor activity and behaviour [43] as well as improved sleep duration, food consumption and agitation symptoms [44].

 

Training for Students complete!

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