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2.1
Behavioral Variant of Frontotemporal Dementia
DEM

Presentation

The present study excluded patients presenting with progressive aphasia. [es.slideshare.net]

Method: A 33-year-old man presented with 1.5 years of progressive behavioral and cognitive changes. [academic.oup.com]

[…] project present a novel way to define criteria regarding cognition profile through language analysis in all: AD and FTDL all variants. [theses.fr]

Early apathy or inertia [one of the following symptoms (B.1–B.2) must be present]: B.1. Apathy B.2. Inertia C. Early loss of sympathy or empathy [one of the following symptoms (C.1–C.2) must be present]: C.1. [radiopaedia.org]

Entire Body System

  • Falling

    BACKGROUND: Patients with autonomic failure may experience postural dizziness, syncope, and falls. Identifying symptomatic dysautonomia in dementia is of importance to ensure appropriate management and reduce risk of falls. [ncbi.nlm.nih.gov]

    Galas & Events Fisher After Hours Every opening night this fall, celebrate pathbreaking Next Wave performance with complimentary drinks and snacks in the lower lobby of the BAM Fisher. [bam.org]

    It does, however, predispose patients to serious complications such as pneumonia, infection, or injury from a fall. The most common cause of death is pneumonia. [psp.org]

    Behavioral variant frontotemporal dementia (bvFTD) is one of several types of dementias that fall into the "frontotemporal degeneration" category. This category affects approximately 50,000 to 60,000 Americans. [verywell.com]

    Progressive supranuclear palsy (PSP): Causes problems with walking and balance (leading to frequent falls), muscle stiffness (mainly in the neck and upper body) and issues with eye movement. [silverado.com]

  • Disability

    CONCLUSION: Functional disability was similar in FTD and AD patients. Nevertheless, the caregiver burden was higher in FTD than in AD, a result that has important implications for caregiver help. Copyright 2008 S. Karger AG, Basel. [ncbi.nlm.nih.gov]

    Functional disability differences across bvFTD phenotypes. [neurology.org]

    Please enable JS and disable any ad blocker [cairn.info]

    Over the course of a few years, the symptoms seen in the mild stage will become more pronounced and disabling. [memory.ucsf.edu]

  • Movement Disorder

    In addition to muscle and movement problems, FTD movement disorders have also shown to impair language and behavior. Corticobasal degeneration (CBD): Causes tremors, lack of coordination, and muscle rigidity/spasms. [silverado.com]

    disorder, unspecified G26 Extrapyramidal and movement disorders in diseases classified elsewhere G30 Alzheimer's disease G30.0 Alzheimer's disease with early onset G30.1 Alzheimer's disease with late onset G30.8 Other Alzheimer's disease G30.9 Alzheimer's [icd10data.com]

    Corticobasal syndrome (CBS) Corticobasal syndrome is a movement disorder that can cause slowness of movements or rigid movements. It usually affects one side of a person’s body. [aftdkidsandteens.org]

    Progressive supranuclear palsy, corticobasal syndrome Corticobasal syndrome and progressive supranuclear palsy are rare movement disorders that are associated with the broad group of FTLD disorders. [rarediseases.org]

    Movement Disorders Two rare neurological disorders associated with FTLD, corticobasal syndrome (CBS) and progressive supranuclear palsy (PSP), occur when the parts of the brain that control movement are affected. [dementiatoday.com]

Respiratoric

  • Yawning

    Apraxia of other orofacial movements or swallowing often accompanies speech apraxia and is tested by asking the patient to yawn or cough, which they are unable to do to command, although they can as a reflex. [bmj.com]

Gastrointestinal

  • Dysphagia

    Individuals may exhibit weakness, muscle loss (atrophy), tiny involuntary muscle twitches (fasciculation), difficulty speaking (dysarthria) and difficult swallowing (dysphagia). [rarediseases.org]

    Wandering and intrusive behaviours, apathy, incontinence, mutism, and dysphagia are common issues toward the end of life and important sources of caregiver distress. [bmj.com]

Cardiovascular

  • Vascular Disease

    There is a need for prospective studies involving a wider range of disorders, including vascular disease. [es.slideshare.net]

  • Hypertension

    Say there's a new study that proved these vitamins are great for diabetes/hypertension, etc. (one of the other conditions he has). If needed, get the doctor to go along with your story. [thealzheimerspouse.com]

Psychiatrical

  • Suggestibility

    Disruption of orbitofrontal-amygdala circuit, as well as the frontal, temporal, and insular atrophy in bVFTD, suggests a relationship between context-sensitive social cognition and SCNM. [ncbi.nlm.nih.gov]

    Growing evidence suggests spatial orientation is a reliab le cognitive marker able to differentiate these two clinical syndromes. [content.iospress.com]

  • Delusion

    Results: In total, 22.7% of bvFTD patients suffered from delusions, hallucinatory behavior, and suspiciousness, although the majority of the patients exhibited negative psychotic symptoms such as social and emotional withdrawal and blunted affect (95.5% [dovepress.com]

    Hallucinations and delusions are relatively common as Alzheimer's progresses, but relatively uncommon in FTD. [alz.org]

    The person with bvFTD may experience false thoughts (delusions) that are jealous, religious or bizarre in nature. Or they can develop a euphoria – excessive or inappropriate elation or exaggerated self-esteem. [memory.ucsf.edu]

    Experiencing false thoughts (delusions) i.e. believing they have unusual powers Someone with this type of FTD may begin to do things that are very out of character. [aftdkidsandteens.org]

    Actual values from Day 1 in Neurophsychic Inventory (NPI) [ Time Frame: Up to 12 Months ] A 12-item participant and caregiver interview based questionnaire assessing behavioral domains which are common in dementia: hallucinations, delusions, agitation [clinicaltrials.gov]

  • Stereotyped Behavior

    When he was 70 years old, the patient had presented with stereotyped behavior, dietary changes, and a decline in social interpersonal conduct in clinical settings, and it was thought that these symptoms were consistent with a behavioral variant of frontotemporal [ncbi.nlm.nih.gov]

    […] disorder, with decline in personal hygiene; distractibility and impersistence; hyperorality; dietary changes; repetitive stereotypic behavior; utilization behavior Behavioral changes with loss of sympathy and empathy; narrowed preoccupations; parsimony [aafp.org]

    Another scale, called Cambridge Behavioral Inventory, is an inventory that explores 13 domains (memory, orientation and care, daily activities, autonomy, mood, beliefs, behavioral disorders, disinhibition, diet, sleep, stereotyped behavior, motivation [karger.com]

  • Anger

    Behaviors may include: Blaming others for consequences of socially unacceptable behavior; e.g., job loss Anger at limitations on activities Diagnosis Diagnosis is challenging in the early stages of bvFTD, and it is commonly misdiagnosed— for example as [theaftd.org]

    Liberals, on the other hand, seem to harbor a lot of anger. If I disagree with a liberal, they usually start right off by calling me names. [bvftd.blogspot.com]

    They may express feelings of anger or sadness more often. There are many things you can do to help a person with FTD to make them more comfortable. [aftdkidsandteens.org]

  • Abnormal Behavior

    Despite their abnormal behavior, just over half of bAD patients initially presented with cognitive symptoms such memory and executive problems. [alzforum.org]

    My husband Charles,was finally diagnosed two years ago.I can remember abnormal behavior way back eight years ago.Then it was going the wrong way in traffic,not being able to figure out how to do things,both at work and at home.Deep Depression,and sleeping [thealzheimerspouse.com]

Neurologic

  • Personality Change

    Behavior and personality changes The frontal lobes of the brain are the parts that control how well a person can plan, organize and make decisions. This part of the brain also controls social behavior. [aftdkidsandteens.org]

    Diagnosis Challenges Given the gradual behavioral and personality changes typical of bvFTD, diagnosis of the syndrome can be challenging. [ftd.med.upenn.edu]

    They may present with focal neurologic deficits, early gait disturbances, changes in personality and mood, and a history of frequent falls or unsteadiness. [aafp.org]

    The hallmarks of bvFTD are personality changes, apathy, and a progressive decline in socially appropriate behavior, judgment, self-control, and empathy. Unlike in Alzheimer’s disease, memory is usually relatively spared in bvFTD. [theaftd.org]

    Behavioural or personality changes may at first suggest a primary psychiatric disorder, particularly if accompanied by psychotic features: clues that such features are harbingers of FTD may include a lack of any prior psychiatric history and emergence [bmj.com]

  • Confusion

    It manifests most clearly in changes of social behaviour and is easy to confuse with mood disorders such as depression. [vitorzimmerer.net]

    Diagnosis is therefore largely based on clinical judgment and FTD can be confused with other disorders in which there are problems with behaviour (e.g. some psychiatric disorders) and with other dementias. [raredementiasupport.org]

    shaking, difficulty walking, frequent falls, and poor coordination (called corticobasal syndrome, progressive supranuclear palsy, or amyotrophic lateral sclerosis ) One of the challenges shared by patients, families, clinicians, and researchers is confusion [psp.org]

    Ossenkoppele and colleagues wanted to conduct a larger study that would cut through the confusion of previous small ones. They drew upon a large database of patient records at both UCSF and VUMC going back to 1999. [alzforum.org]

    The road to diagnosis is long and confusing, and what starts off as perplexing for the family then becomes frightening. The man they love is changing, and no one seems to know why. He no longer turns up to his sons’ high school events. [dementiaresources.org.au]

  • Behavior Problem

    Common treatments include: Medications Some antidepressants can help reduce the behavioral problems caused by changes to the brain. Antipsychotic medications are also used to treat behavioral problems. [healthline.com]

    Other common symptoms include loss of speech and language, compulsive or repetitive behavior, increased appetite, and motor problems such as stiffness and balance problems. [stanfordhealthcare.org]

    Symptoms include movement problems similar to those of Parkinson’s disease, such as slowed movement, stiffness, and balance problems, and changes in behavior or language. [dementiatoday.com]

    10 Jul 2015 As if grappling with memory loss isn’t hard enough, a subset of people with Alzheimer’s disease have behavioral problems that rival their cognitive ones. [alzforum.org]

    The caregiver's characteristics such as young age, low level of education, depression and spending a lot of time with the person with cognitive impairment have been shown to be potentially associated with an overestimation of behavior problems [ 48 ]. [karger.com]

  • Stroke

    It is associated with a disease process that causes atrophy in the frontal and temporal areas of the brain and is distinct from aphasia resulting from a stroke. [psp.org]

    Other brain diseases that can cause dementia include Alzheimer’s disease and multiple strokes. [dementiatoday.com]

    Other useful Web sites include the National Institute on Aging ( http://www.nia.nih.gov ); National Institute of Neurological Disorders and Stroke ( http://www.ninds.nih.gov ); Alzheimer's Association ( http://www.alz.org ); and Family Caregiver Alliance [aafp.org]

    Stroke 32: 1318-1322. doi: https://doi.org/10.1161/01.STR.32.6.1318. PubMed: 11387493. View Article Google Scholar 31. Jenkinson M, Smith S (2001) A global optimisation method for robust affine registration of brain images. [journals.plos.org]

  • Excitement

    If this doesn’t excite you, I don’t know what would. Where can you download the MOLA DEM of Mars? The USGS Astrogeology Science Center is the DEM data hub for Mars. [gisgeography.com]

    Apathy is defined as a general indifference or lack of interest in life, including to things or activities that were once considered exciting or emotionally moving. [rarediseases.org]

    I welcome and make use of this new free data source, but I invite readers to read my initial review of these data before getting too excited. [viewfinderpanoramas.org]

Treatment

Treatment is aimed at managing and alleviating symptoms. Common treatments include: Medications Some antidepressants can help reduce the behavioral problems caused by changes to the brain. [healthline.com]

Treatment and outcomes There are no specific treatments for any of the frontotemporal subtypes. There are medications that can reduce agitation, irritability and/or depression. These treatments should be used to help improve quality of life. [alz.org]

Treatment There is no specific cure for FTD; treatment is focused on symptom management and support for patients, families, and caregivers. [aafp.org]

Psychological treatments such as cognitive stimulation and behavioural therapy can help maintain memory function address anxiety. [psychologized.org]

As symptoms progress, however, families usually seek a clinician's assistance for diagnosis and treatment. [verywell.com]

Prognosis

What is the prognosis of bvFTD? The rate of progression is very variable and can range from two to twenty years. As the disease progresses, people will need help with most aspects of daily life. [ftdtalk.org]

Prognosis and Life Expectancy (Long-Term Outlook) Average life expectancy from onset of symptoms is approximately eight to nine years, though some people may live 20 years or so with the disease. [verywell.com]

These phenotypes could also support understanding of prognosis and clinical management. [neurology.org]

Management and Prognosis The progression of behavioral variant FTD can range from 3 to 17 years, with a mean course of 8 years from the time of diagnosis to death. [bvftd.blogspot.com]

Prognosis [ edit ] Symptoms of frontotemporal dementia progress at a rapid, steady rate. Patients suffering from the disease can survive between 2–15 years. [en.wikipedia.org]

Etiology

Keywords Adult — Aged — Feeding and Eating Disorders/etiology/physiopathology — Female — Frontotemporal Dementia/complications/genetics/pathology/physiopathology — Humans — Hypothalamus/pathology — Magnetic Resonance Imaging — Male — Middle Aged Identifiers [archive-ouverte.unige.ch]

Neuroimaging (magnetic resonance imaging) usually demonstrates focal atrophy in addition to excluding other etiologies. [aafp.org]

The diagnosis and understanding of apraxia of speech: Why including neurodegenerative etiologies may be important. Journal of Speech, Language, and Hearing Research, 55(5), S1518-S1522. doi: 10.1044/1092-4388(2012/11-0309). Duffy, J. R., Strand, E. [infad.eu]

Patients with dementia of any presumed etiology would have to be recruited and followed to death and autopsy confirmation of diagnosis. [es.slideshare.net]

Epidemiology

Frontotemporal lobar degeneration: epidemiology, pathology, diagnosis and management. CNS Drugs 2012 ; 26 : 841 -70. ↵ Vercelletto M, Boutoleau-Bretonnière C, Volteau C, Puel M, Auriacombe S, Sarazin M, et al. [bmj.com]

Epidemiology, Diagnosis, Management and Outcomes. Drugs & Aging, 1999; 15: 81-89. [ Links ] 36. Seo SW, Thibodeau M-P, Perry DC, Hua A, Sidhu M, Sible I, et al. [scielo.org.pe]

Bernardi L, Frangipani F, Smirne N et al (2012) Epidemiology and genetics of frontotemporal dementia: a door-to-door survey in Southern Italy. Neurobiol Aging 33(12):2948 e1–2948 e10 CrossRef Google Scholar 5. [link.springer.com]

Although epidemiologic studies are limited, approximately 20 to 50 percent of persons younger than 65 years with dementia have FTD, a prevalence similar to that of Alzheimer disease in persons 45 to 64 years of age (15 per 100,000). 1 – 3 FTD is underdiagnosed [aafp.org]

Pathophysiology

Our findings suggest different pathophysiological mechanisms in these 2 separate neurodegenerative disorders. Copyright © 2016 Elsevier Inc. All rights reserved. [ncbi.nlm.nih.gov]

Abstract BACKGROUND AND PURPOSE: Brain iron deposition has been implicated as a major culprit in the pathophysiology of neurodegeneration. [ajnr.org]

[…] capture pathophysiological changes become available. [bmj.com]

B 30 Pathophysiology Common pathologic findings in FTD are atrophy and neuronal loss affecting the frontal and temporal lobes of the brain. 10 There is histologic heterogeneity in patients with FTD, but about 55 percent of patients have FTD with ubiquitin-positive [aafp.org]

Prevention

[…] and adaptive feedback. a loop for dementia clinicians to provide objective observations regarding the health progression of the person with dementia and medication effectiveness, warn about trends closely related to dementia (e.g. apathy), and support preventive [demcare.eu]

Researchers don’t know why these proteins develop or how to prevent them. Frontotemporal dementia has one known risk factor: genetics. Scientists have found several genes related to the disease. [healthline.com]

Most of the patients at Patton were transferred there from prisons after their mental illness prevented them from following institutional rules, Sfera said. [clbb.mgh.harvard.edu]

Further potential for in vivo distinction of patients with differing pathologic processes could, in turn, help prevent the dilution of positive effects in therapies that occurs when patients are grouped under the single umbrella of one overarching clinical [neurology.org]

SRTM data were captured in February 2000, when the snow-laden pine forests that dominate the area would have prevented the penetration of the SRTM radar to ground level. [viewfinderpanoramas.org]

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