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Frontotemporal Degeneration

Course #96101 - $15 • 2 Hours/Credits

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  1. The age of onset for frontotemporal degeneration (FTD) is typically

    EPIDEMIOLOGY OF FTD

    It is estimated that FTD affects approximately 60,000 people in the United States [3]. As noted, the age of onset for FTD is typically 45 to 64 years, with a mean of 58.5 years and a reported range between 21 and 80 years of age [2,3,4,5]. In the United States, the prevalence in people 45 to 64 years of age is estimated at 15 to 22 per 100,000 population; the incidence in this group is 2.7 to 4.1 per 100,000 [5]. It is estimated that 60% of those with FTD have onset between 45 and 64 years of age; 10% have onset before 45 years of age, and 30% have onset after 64 years of age [5]. FTD is now considered by some to be the most common form of pre-senile dementia in patients younger than age 60, even more common than AD in this group [6,7,8].

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  2. Which areas of the brain are typically atrophied in patients with FTD?

    PATHOGENESIS AND PATHOPHYSIOLOGY OF FTD

    Patients with FTD experience a progressive loss of neurons in the frontal and anterior temporal lobes, resulting in atrophy in these areas of the brain. They may also develop gliosis in the frontal and temporal lobes where neurons have been lost or damaged.

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  3. The most common presentation of FTD is

    CLINICAL PRESENTATION

    FTD causes a gradual, progressive decline in behavior and/or language; movement disorders may also be involved. Behavior or language problems are typically the first and most prominent symptoms of FTD, whereas memory problems are the first symptoms of AD [5]. Subtypes of FTD have been identified based on clinical presentation (Table 1). Behavioral variant FTD (bvFTD) is the most common form and involves changes in behavior, personality, and emotions. Language presentations are referred to as primary progressive aphasia (PPA) and can take one of three forms: nonfluent/agrammatic variant PPA (nfvPPA), semantic variant PPA (svPPA), or logopenic variant PPA (lvPPA) [11]. Nonfluent/agrammatic PPA begins with problems in speech production, while svPPA involves impaired word comprehension and object recognition and lvPPA involves word-finding problems. A movement presentation may appear as progressive supranuclear palsy (PSP), corticobasal syndrome (CBS), or motor neuron disease (MND). Some patients may present with symptoms that overlap the different subtypes of FTD or may develop symptoms of other subtypes of FTD as the disease evolves. As more is learned about FTD, the terminology and classification of the subtypes may be revised.

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  4. Which of the following personality traits or behaviors is typically seen with behavioral variant FTD (bvFTD)?

    CLINICAL PRESENTATION

    As noted, bvFTD is the most common type of FTD, estimated to account for more than half of all cases [12]. The prominent features include disinhibition, apathy/inertia, loss of empathy, compulsive behaviors, hyper-orality, and impaired executive function (Table 2) [13]. People with FTD may become socially withdrawn, inflexible, and impulsive. They may have a shortened attention span and a tendency to be easily distracted. Behavior may become socially inappropriate. People with bvFTD are usually unaware of the changes in their personality and behavior and the impact these changes have on others. Memory and visual-spatial functioning are initially relatively spared in bvFTD. Some individuals with bvFTD may develop symptoms similar to Parkinson disease, such as bradykinesia, rigidity, postural instability, and masked face.

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  5. A person diagnosed with nonfluent/agrammatic variant primary progressive aphasia would have difficulty

    CLINICAL PRESENTATION

    Nonfluent/agrammatic variant PPA, also referred to as progressive non-fluent aphasia (PNFA), accounts for about 25% of all FTD cases and involves problems with language expression [12]. People with nfvPPA have difficulty producing speech but retain the meaning of words and know what they want to say. As a result, speech may become hesitant, slow, and labored. Speech patterns may be "agrammatic" or "telegraphic," meaning that only the most important content words are used, without connecting words. For example, a patient might say "Tuesday…hospital…sister." Patients with nfvPPA have difficulty talking on the telephone and tend to talk progressively less. Eventually, some may become mute. While in the early stages, these patients continue to understand the speech of others, but this comprehension is eventually lost also. Reading and writing skills are better preserved than speech, although these abilities are also eventually lost. As the disease progresses, patients may develop behavioral symptoms. Some individuals with nfvPPA may develop extrapyramidal symptoms of rigidity and tremors, as seen in corticobasal syndrome (CBS) and PSP.

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  6. Which of the following best describes the differences between the early symptoms of FTD and Alzheimer disease (AD)?

    CLINICAL DIAGNOSIS OF FTD

    EARLY SIGNS AND SYMPTOMS OF FTD VERSUS ALZHEIMER DISEASE

    Clinical FeaturesFTDAlzheimer Disease
    HallmarkDecline in behavior, language, and/or movement; memory is retained initiallyDecline in memory; socially appropriate behavior is retained initially
    Initial language problemsMay involve speech production, understanding words and recognizing familiar objects, or retrieving wordsWord-finding or name recall
    Age at onsetUsually 45 to 64 years of ageUsually 65 years of age or older
    Movement problemsMay have early movement disorder, with gait and balance problems, rigidity, apraxia, or muscle weaknessUsually no movement problems early in disease
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  7. If a parent carries a genetic mutation in the MAPT gene causing FTD, what is the risk to his/her child of inheriting the same genetic mutation?

    GENETICS AND FTD

    Clinical genetic testing is available for the MAPT, GRN, and C9orf72 genetic mutations causing hereditary FTD, as well as some of the other very rare genetic mutations. Genetic testing may be ordered after informed consent and clear discussion of the implications with the patient and his/her family. For patients with FTD, identification of a genetic mutation confirms the diagnosis of FTD and provides information about risk to other family members. Each of the patient's siblings and each of the patient's children would be at 50/50 risk for having inherited the genetic mutation causing FTD. If a genetic mutation causing FTD is identified, other at-risk family members may be tested.

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  8. Which of the following medications has been approved by the U.S. Food and Drug Administration (FDA) for the treatment of FTD?

    MANAGEMENT OF FTD

    There is presently no treatment to slow the progression of or cure FTD. No medication has been approved by the U.S. Food and Drug Administration (FDA) for the treatment of FTD; however, medications used to treat other disorders may be prescribed off-label for the management of FTD symptoms. Their use may be limited by the potential adverse effects. Antidepressants, particularly selective serotonin reuptake inhibitors (SSRIs), may be prescribed for behavioral symptoms of FTD, and low-dose trazodone has been used for agitation and aggression [9]. While the anticholinesterase inhibitors donepezil, galantamine, and rivastigmine are beneficial for some patients with AD, they generally have not been helpful for patients with FTD [9]. The glutamate NMDA receptor antagonist memantine, used for moderate-to-severe AD, has been used for patients with FTD as well, but a 2013 study showed that it provided no benefit to patients with FTD and that it may be harmful to cognition [23]. Antipsychotics are occasionally used to treat significant agitation and behavioral symptoms, but only with caution, as antipsychotics can have serious adverse effects such as extrapyramidal adverse effects (parkinsonism), depression, sedation, falls, incontinence, and disinhibition, and patients with FTD may have an increased susceptibility to the these effects [9]. Elderly patients with dementia who take antipsychotics have a 1.6- to 1.7-fold increase in mortality secondary to cardiac problems or infection, prompting the FDA to issue a warning about their use in older patients with behavioral disturbances [9]. L-DOPA has shown a minimal response for parkinsonism in patients with PSP and CBD [28]. Research is being done to further evaluate the use of available medications for the management of FTD and to find new, more effective treatments.

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  9. In developing a plan to manage symptoms in a patient with bvFTD, it is important to consider which of the following?

    MANAGEMENT OF FTD

    Apathy is a common symptom in patients with bvFTD, often resulting in neglect of their personal hygiene and grooming. Supervision, encouragement, and help with bathing, dressing, and grooming may be needed. For behavioral problems, simple interventions like distraction (e.g., introducing a new activity) may help interrupt the troublesome behavior. For some patients, modification of the environment or behavior may help minimize the potential for harm. For example, if the patient is pacing, creating a safe route for him or her to walk can be helpful. Physical therapists may be able to help develop an exercise program to maintain mobility. Exercise has also been shown to improve mood and cognition and may improve behavior management in patients with dementia [24]. If behaviors such as agitation or aggression become severe, a medication may be prescribed off-label to control difficult or dangerous behaviors. Supervision may be necessary to ensure patients take medications as prescribed.

    Some individuals with FTD have eating problems, such as overeating, eating just one type of food, or craving sweets. For these patients, it may be necessary to monitor weight and provide help with meal preparation to provide a balanced, nutritious diet. Access to additional foods, drinks, or sweets should be limited.

    Caring for a patient with FTD includes maintaining a safe environment for the patient and for those around him/her. A structured environment and keeping the daily routine the same is often helpful. In addition, persons with FTD should no longer drive, and safety measures should be taken at home, especially in the kitchen and bathroom. If a patient with FTD shows aggression, disinhibition, or poor judgment, close supervision is necessary when he/she is around others, especially children or the frail elderly, to prevent them from being inadvertently harmed.

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  10. All of the following are goals of current research on FTD, except:

    RESEARCH RELATED TO FTD

    The goals for research on FTD include gaining a better understanding of the pathology; identifying causes and risk factors (genetic and environmental); improving the diagnosis of FTD through enhanced neuroimaging, biomarkers, and characterization of clinical features; developing therapies to treat, cure, or prevent FTD; and exploring new ways to support family caregivers. However, research on FTD is challenging. It is an uncommon disease, so awareness is low, there are fewer potential subjects available for research studies, and there is a relatively small market for medications. FTD is a complicated disease with a wide variety of presentations (behavior, language, and movement problems) and underlying causes (sporadic and genetic). Pathologically, microscopic brain inclusions may consist of different abnormal proteins (e.g., tau, TDP-43, FUS), but there are no good biomarkers to diagnose FTD or to monitor the progression of the disease. Drug development faces the challenge of creating a medication to treat FTD that can cross the blood/brain barrier.

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