What Causes Frontal Lobe Dementia/frontotemporal Dementia
Scientists dont know what causes the majority of cases of frontal lobe dementia. However, abnormal accumulations of proteins, either tau or TDP-43, have been found during autopsy in the brains of people who suffered from frontal lobe dementia.
Furthermore, a number of genes linked to the abnormal production of these proteins have been identified and are responsible for the disease in the 15 to 40 percent of people who suffer from a hereditary form of it.
Overlap Between Frontotemporal Dementia And Movement Disorders
Most people with FTD do not experience significant movement or motor problems. However, motor symptoms can develop in the later stages of the illness and FTD can co-occur with other conditions characterised by motor symptoms.
These include motor neuron disease, corticobasal syndrome, progressive supranuclear palsy and FTDP-17 .
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Caring For A Loved One
Some tips for keeping the person safe and maximizing their quality of life include:
- helping them manage their finances and apply for disability payments, if appropriate
- preempting any physical, social, or psychological risks they may face
- making adaptations to reduce the risk of falls
- seeking counseling for depression and anxiety, if applicable
- maintaining a regular routine
- helping them avoid triggers of hypersexuality, overeating, and other behaviors that can lead to problems
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How Is Frontotemporal Dementia Diagnosed And Treated
Frontotemporal dementia can be hard to diagnose because symptoms are similar to other conditions. Also, because these disorders are rare, physicians may be unfamiliar with the signs and symptoms.
Talking with patients, family members, and caregivers can help doctors make a diagnosis. It is important to tell the doctor about any symptoms involving changes in personality and behavior and difficulties with speech or movement.
To diagnose frontotemporal dementia, a doctor may perform an exam and ask about symptoms, look at personal and family medical history, use tests to help rule out other conditions, order genetic testing, conduct an assessment of the mind and behavior, and order imaging of the brain. Only genetic tests in familial cases or a brain autopsy after a person dies can confirm a diagnosis of frontotemporal dementia. Researchers are studying ways to diagnose the disorders earlier and more accurately and to distinguish them from other types of dementia.
There is no cure for frontotemporal dementia and no way to slow down or prevent it. However, there are ways to help manage symptoms:
Support groups can also be a valuable resource for people with frontotemporal dementias and their caregivers, allowing them to share experiences and tips with others who may be in the same situation. Nonprofit organizations and community organizations may provide online or in-person support groups. Alzheimers Disease Research Centers may also offer education and support groups.
Can We Predict Molecular Pathologies In Frontotemporal Dementia
Despite recent histopathological and genetic advances,24 accurate prediction of underlying molecular pathologies within the FTD spectrum remains problematic . This has important implications for targeting future disease modifying therapies and, more immediately, for counselling of patients and family members. While phenotypic variation and overlap are substantial, certain clinico-anatomical profiles are consistently associated with particular molecular pathologies .21 These include FTD with motor neurone disease associated with TDP-43 pathology the semantic dementia syndrome and focal, asymmetric temporal lobe atrophy with TDP-43 type C pathology behavioural variant FTD of very young onset and prominent caudate atrophy with FUS pathology and the progressive supranuclear palsy syndrome with a specific tauopathy. Non-fluent speech breakdown and the development of parkinsonism are more commonly associated with tau than non-tau pathologies. The heritability of particular FTD syndromes varies substantially3: semantic dementia is generally sporadic, whereas behavioural variant FTD and corticobasal syndrome are highly genetically as well as pathologically heterogeneous. Useful neuroanatomical associations which may help guide genetic testing include relatively symmetric anteromesial temporal lobe atrophy with MAPT mutations and striking asymmetric hemispheric atrophy with GRN mutations .4
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Who Can Get Frontal Lobe Dementia
Anyone can get frontal lobe dementia, however according to Alzheimers Association, it typically affects people at a younger age than Alzheimers disease. Symptoms such as changes in personality and behaviour often occur in people in their 50s or 60s, but can develop as early as their 20s or as late as their 80s.
Almost one third of people with the disease have a family history of dementia, however not everyone with a family history will develop a problem. There is still much research to be done to understand the causes and risk factors of frontal lobe dementia.
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What Treatments Are Available
At this stage, there is no cure for frontal lobe dementia. Instead, treatment is aimed at managing and alleviating the symptoms associated with the disease.
Common treatments include:
- Medications which are often used to treat behavioural symptoms associated with the disease and form part of a comprehensive treatment plan in consultation with a GP or health professional.
- Speech therapy to help cope with a decline in language abilities. A speech therapist can also help you find alternative ways to communicate.
- Environmental changes maintaining an environment that doesnt agitate or upset someone is vital. People with behaviour problems are most comfortable in environments that are familiar, quiet, and away from large crowds.
Remember, only a medical practitioner such as a doctor or specialist can diagnose dementia. If an official diagnosis has been made, there is a lot of support available to help you and your loved ones manage. A good place to start is by visiting Dementia Australias website or by calling the National Dementia Helpline on 1800 100 500. They offer support, information, education and counselling.
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Behavioral Variant Frontotemporal Dementia
The most common FTD, bvFTD, involves changes in personality, behavior, and judgment. People with this disorder may have problems with cognition, but their memory may stay relatively intact. Symptoms can include:
- Problems planning and sequencing
- Difficulty prioritizing tasks or activities
- Repeating the same activity or saying the same word over and over
- Acting impulsively or saying or doing inappropriate things without considering how others perceive the behavior
- Becoming disinterested in family or activities they used to care about
Over time, language and/or movement problems may occur, and the person living with bvFTD will need more care and supervision.
Life Expectancy And Treatment
About 10 15% of dementia cases are thought to be frontal lobe dementia, the disease affecting 1 in 5000 of the population. However in those under 65 it is believed to be 20 50% of cases. Onset of frontal lobe dementia is normally identified when the patient is between 45 and 65 years of age, although it has been seen in people aged 20 to 30 years of age. Only 10% of cases are identified in those 70 years and over.
The disease takes from three to ten years to progress, although there are instances of much shorter or longer times. The average life expectancy of a person diagnosed with frontal lobe dementia is eight years. Approximately 50% of deaths are as a result of pneumonia, following complications associated with inability of the person to move or care for themselves.
As with other forms of dementia there is no current cure for the disease, but there are a range of treatments that can help to manage and deal with the symptoms, and to help people to regain some of their lost functions.
These include drugs such as SSRI antidepressants to help control the symptoms like obsession, over-eating and depression. Antipsychotics may be given to address challenging and inappropriate behaviours. Psychological treatments such as cognitive stimulation and behavioural therapy can help maintain memory function address anxiety. Rehabilitative practices such as, occupational therapy, physiotherapy and speech therapy can help the brain to learn new ways to do things.
References:
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Enhancing Healthcare Team Outcomes
FTD is a progressive neurodegenerative disorder that involves three different spectra of clinical syndromes characterized by loss of intellectual functions. To improve patient outcomes and quality of life, an interprofessional approach has to be adopted. It includes collaboration and coordination of healthcare professionals, including neurologists, psychiatrists, audiologists, nurses, social workers, physical therapy, occupational therapy, and speech therapists. Family, friends, and caregivers must meet with the primary care provider and/or local community mental health team regularly. The role of the caregiver is crucial in the management of FTD therefore, a therapeutic partnership between the healthcare team and caregivers is warranted.
For the end-of-life care, living arrangements, and decision making about financial and medical services, proper future planning regarding assessing decision capacity, surrogates decision-makers, living will, power of attorney, and medical arrangements are to be implemented.
Here is a checklist of problem-oriented management that can optimize the quality of life if implicated.
When Should I Call My Healthcare Provider
If you are diagnosed with FTD, you and your caregivers should talk with your healthcare providers about when to call them. Your healthcare provider will likely advise calling if your symptoms become worse, or if you have obvious or sudden changes in behavior, personality, or speech. This includes mood changes, such as increasing depression or feeling suicidal.It can be very stressful for a caregiver to take care of a loved one with FTD. It’s normal to have feelings of denial, anger, and irritability. Caregivers may also have anxiety, depression, exhaustion, and health problems of their own. Caregivers should contact their healthcare provider if they have any of these signs of stress.
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Treatments For Frontotemporal Dementia
There’s currently no cure for frontotemporal dementia or any treatment that will slow it down.
But there are treatments that can help control some of the symptoms, possibly for several years.
Treatments include:
- medicines to control some of the behavioural problems
- therapies such as physiotherapy, occupational therapy, and speech and language therapy for problems with movement, everyday tasks and communication
- dementia activities such as memory cafes, which are drop-in sessions for people with memory problems and their carers to get support and advice
- support groups who can offer tips on managing symptoms from dementia experts and people living with frontotemporal dementia, and their families
How Does Frontotemporal Dementia Develop
Frontotemporal dementia is a progressive condition which affects behaviour and personality, which sometimes leads to disinhibition and inappropriate social behaviour. Eating patterns can also be affected, with people suddenly bingeing on food, especially sweet foods. This form of dementia can sometimes be confused with depression, stress, anxiety, psychosis or obsessive compulsive disorder.
In behavioural variant frontotemporal dementia initial symptoms may include:
- changes in behaviour and personality
- apathy
- changes in appetite and food eaten
- difficulties with decision making, problem solving and concentration
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Can You Die From Frontotemporal Dementia
FTD is not life-threatening ? people may live with it for years. But it can lead to an increased risk for other illnesses that can be more serious. Pneumonia is the most common cause of death, with FTD.
What is the progression of frontotemporal dementia?
As frontotemporal dementia progresses, differences between these types lessen: people with the behavioural variant develop language problems and those with language problems develop behaviour changes. In the later stages, the symptoms of frontotemporal dementia become more similar to those of Alzheimers disease.
What Is The Prognosis For Someone With Frontotemporal Dementia
Frontotemporal dementia worsens over time. Eventually people with FTD will need help with their activities of everyday living. Some people may need 24-hour care at home or at living facilities or nursing homes. The speed of decline varies from person to person, but the disease course typically ranges from two to 10 years.
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How Is Frontotemporal Dementia Treated
Currently, no treatments are available to cure or slow the progression of FTD, but healthcare providers may prescribe medicine to treat symptoms. Antidepressants may help treat anxiety and control obsessive-compulsive behaviors and other symptoms. Prescription sleeping aids can help ease insomnia and other sleep disturbances. Antipsychotic medicine may reduce irrational and compulsive behaviors.
Behavior modification may help control unacceptable or risky behaviors.
Speech and language pathologists and physical and occupational therapists can help adjustment to some of the changes caused by FTD.
Is There Treatment Available
There is not yet a cure for FTD, or any disease modifying treatment. However, various therapies can help with some of the symptoms. Secondary symptoms, such as depression, may be helped by medication.
Knowing more about FTD and why the person is behaving as they are can help people to cope with the disease.
Family members and carers can, with support, develop coping strategies to work around problems rather than trying to change the behaviour of the person with FTD.
Psychological therapies are important to help manage abnormal behaviour.
Speech therapy is of benefit to people with PNFA, particularly in helping to develop alternative communication methods.
The input of an occupational therapist can help improve everyday functioning at home.
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What Are The Types Of Frontotemporal Dementia
There are several disorders that can result in frontotemporal dementia, all of which can affect middle-aged and older adults.
- Behavioral variant frontotemporal dementia is the most common frontotemporal disorder. It causes changes in personality, behavior, emotions, and judgment.
- Primary progressive aphasia involves changes in the ability to communicate to speak, read, write, and understand what others are saying.
- Movement disorders happen when the parts of the brain that control movement are affected. There are two types of rare movement disorders associated with FTD: corticobasal syndrome and progressive supranuclear palsy. Other movement-related disorders include frontotemporal dementia with parkinsonism and frontotemporal dementia with amyotrophic lateral sclerosis . Symptoms can vary among these movement disorders.
What Is The Life Expectancy With Frontotemporal Dementia
life expectancy
What is the life expectancy of someone with frontal lobe dementia?
The average survival time after a diagnosis of frontal lobe dementia is six to eight years, although patients have been recorded to live up to 20 years after a frontal lobe dementia diagnosis.
what do people with frontal lobe dementia die from?death infrontotemporal dementia
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Imaging Biomarkers In A Research Setting
Diffusion Tensor Imaging
Diffusion tensor imaging is an MRI imaging technique visualizing the diffusion of water molecules throughout the brain. It is used as a technique for white matter tractography .
Studies have shown that white matter damage is an early marker for disease in FTD, and DTI may be used as a tool to screen for such abnormalities at the presymptomatic stage . Reduced integrity of the uncinate fasciculus and anterior corpus callosum is typical for FTD, and the degree of damage is correlated to age and disease severity . Even here, specific patterns can be recognized for different clinical subtypes, and for carriers of different causal mutations. These abnormalities are consistent with characteristic brain atrophy distributions . The increase in white matter damage over time has been reported to be greater than that of gray matter atrophy, although only at the symptomatic stage, indicating the possible use of this technique as a marker for disease progression .
Studies comparing FTD cohorts with AD patients and with normal controls found significantly more white matter pathology mostly in bilateral uncinate fasciculus, cingulum bundle, and corpus callosum in FTD compared to both other groups . More studies are needed to consolidate these findings and define the diagnostic accuracy for FTD of DTI, as well as its power to distinguish FTD from other types of neurodegenerative brain diseases.
Resting-State fMRI
Tau PET Imaging
What Do The Terms Mean
One of the challenges shared by people living with these disorders, families, clinicians, and researchers is what terminology to use. Here, we have used the term frontotemporal disorders to characterize this group of diseases and the abbreviation FTD, which is commonly used to refer to them. Other terms used include frontotemporal lobar degeneration and frontotemporal dementia, but it’s important to note that with some frontotemporal disorders, the primary symptoms are problems with speech or movement, rather than dementia symptoms. Physicians and psychologists diagnose the different forms of FTD based on a persons symptoms as well as the results of brain scans and genetic tests.
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What Was Ultimately Wrong With The Patient
Temporal lobe seizures may transiently impair cognitive and memory function and result in fluctuating memory loss to reflect the ictal-post-ictal continuum. In Alzheimerâs disease, toxic accumulations of A-beta peptides underlies the memory disorder triggering synaptic degeneration, circuit remodeling, and abnormal synchronization within the same hippocampal networks to lead to progressive memory loss1. The neuronal hyperexcitability that is associated with seizures augments the synaptic release of A-beta and may further compound the cognitive deficits in compromised patients such as those with AD. While AD and epilepsy are felt to reflect independent disorders, progressive cognitive and memory deficits are known to occur in patients with TLS associated with hippocampal sclerosis. Furthermore, seizures are a common comorbidity in those with early onset AD. When seizures are subtle, nocturnal, or occur in seniors that expect memory loss, the diagnosis may be elusive. If memory loss fluctuates, subtle TLS without awareness should be considered. In our patient living alone, prolonged EEG had a greater yield than repeated EEG2.
References
What Functions Are Controlled By The Frontal And Temporal Lobe Of The Brain
The frontal lobes control executive functioning, meaning a persons ability to plan in a step-by-step process, to choose priorities, to keep track of several tasks at one time, and make adjustments to ones behavior. The frontal lobe also controls social and emotional responses. In addition, the frontal lobe helps in linking words to form sentences and is crucial making involuntary muscle movements.
The temporal lobes are the major language center, involved with speaking, reading, writing, understanding words and their meanings, and recognizing objects. The temporal lobe also plays a role in recognizing and responding to emotions and is a major memory center.
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