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What Causes Death In Frontotemporal Dementia

Primary Progressive Aphasia = Speech And Language Problems

What is frontotemporal dementia?
  • Some subtypes of frontotemporal dementia are evidenced by a loss of speech and language difficulties. For example, this might include increasing difficulty in using and understanding written and spoken the language – known as progressive non-fluent aphasia. This type of language variant of FTD is characterized by effortful, halting speech with poor grammar. This is the most slowly progressing type of FTD.

People with another subtype, semantic dementia, utter grammatically correct speech that has no meaning or may have difficulty with single-word comprehension.

Diagnosis And Treatment Of Ftd

While there currently are no treatments specifically for FTD, approaches to diagnosis and treatment of frontotemporal dementia are similar to other forms of dementia. Follow the links below to learn more:

Treating Dementia

How Long Can Someone Live With FTD? A person with frontotemporal dementia usually lives between six to eight years after diagnosis, though some will hold on longer. The cause of death is not the illness itself, but complications from its symptoms. The inability to swallow correctly may result in food or liquid getting into the lungs, causing an infection that turns into aspiration pneumonia. Injuries from falling, due to balance problems, might also kill someone in the late stages of FTD.

Differences Between Ftd And Other Dementias

FTD differs markedly in several ways when compared to other dementias, especially Alzheimerâs disease:

  • FTD is characterized by cerebral atrophy in the frontal and anterior temporal lobes of the brain, while Alzheimerâs affects the hippocampal, posterior temporal, and parietal regions.
  • The neurofibrillary tangles, senile plaques, and Lewy bodies present in the brains of Alzheimerâs and other dementia patients are absent.
  • Alzheimerâs patients experience severe memory loss. While FTD patients exhibit memory disturbances, they remain oriented to time and place and recall information about the present and past.
  • FTD patients, even in late stages of the disease, retain visuo-spatial orientation, and they negotiate and locate their surroundings accurately.
  • Intellectual failure in FTD is distinctly different from that of Alzheimerâs patients. Results of intelligence tests are normal in those with FTD until the point in the disease when disinterest results in lower scores.
  • Life expectancy is slightly longer for FTD.

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What To Do If Your Loved One Has Dementia

It can be extremely difficult to watch someone you care about slowly lose themselves over time to dementia. Worldwide, there still tends to be some major misunderstandings about what exactly dementia is and how it affects people. Unfortunately, every type of dementia not only affects the individual diagnosed with it but their family members and loved ones as well.

If your loved one has dementia, being aware of what to expect is the first step. Coming to terms with the disease is necessary for your loved one and your well-being.

Seeking the professional care your loved one needs is crucial as it can keep them as comfortable as possible throughout all stages of dementia. Up until death, its important to appreciate as many moments as possible while they are still here and functioning.

Although dementia is fatal, there are plenty of healthcare and support resources to ensure you and your family enjoy the remaining lifespan of the dementia patient to the best of your ability.

How Does Ftd Progress

Frontotemporal Dementia (Pick

The progression of symptoms in behavior, language, and/or movement varies by individual, but FTD brings an inevitable decline in functioning. The length of progression varies from 2 to over 20 years.

As the disease progresses, the person affected may experience increasing difficulty in planning or organizing activities. They may behave inappropriately in social or work settings, and have trouble communicating with others, or relating to loved ones.

Over time, FTD predisposes an individual to physical complications such as pneumonia, infection, or injury from a fall. Average life expectancy is 7 to 13 years after the start of symptoms . The most common cause of death is pneumonia.

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Common Frontotemporal Dementia Symptoms

The signs and symptoms of FTD generally dont align with those associated with Alzheimers disease because damage to different areas in the brain produces different symptoms. FTD is primarily characterized by changes in:

  • Behavior: Family and friends notice unusual changes in their loved ones personality or behavior. Someone with FTD may become more impulsive, apathetic, or disinhibited.
  • Speech & language: This symptom can include difficulty finding the right words, decreased vocabulary, trouble with grammar and syntax, or difficulty understanding verbal communication.
  • Motor skills: FTD is characterized by the deterioration of basic motor skillssuch as difficulty walking and swallowing.

Subtypes And Related Disorders

The main subtypes of frontotemporal dementia are behavioral variant FTD, semantic dementia, progressive nonfluent aphasia, and FTD associated with amyotrophic lateral sclerosis . Two distinct rare subtypes are neuronal intermediate filament inclusion disease, and basophilic inclusion body disease. Related disorders are corticobasal syndrome, and progressive supranuclear palsy.

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How Is Dementia Diagnosed

Confirming the diagnosis of dementia can be difficult due to the many diseases and conditions that cause it as well as because its symptoms are common to many other illnesses. However, doctors are able to make the diagnosis based on the results of personal medical history, review of current symptoms, neurological and cognitive tests, laboratory tests, imaging tests and by interacting with the patient.

Current general symptoms that would indicate dementia are, by definition, a decline in such mental functions as memory, thinking, reasoning, personality, mood or behavior that are severe enough to interfere with the ability to accomplish everyday tasks. Patients undergo mental function testing to identify problems in these areas. Interviews with family members and/or close friends who may have noticed changes in these areas are helpful as well.

Laboratory tests rule out other diseases and conditions as the cause of dementia, such as thyroid problems and vitamin B12 deficiency. Similarly, brain scans can look for signs of a stroke or tumor that may be the source of the dementia. A PET scan can determine if amyloid proteins are present in the brain, a marker for Alzheimers disease.

Oftentimes, neurologists and geriatricians assist in making the diagnosis.

An Overview Of Frontotemporal Dementia

Frontotemporal dementia | Symptoms, diagnosis, treatment, prognosis

FTD is a progressive neurological disorder that affects the frontal and temporal lobes of the brain. The frontal lobe manages executive functionincluding planning, organization, and impulse controlwhile the temporal lobe helps process language, speech comprehension, and emotion.

There are three subtypes of frontotemporal dementia:

  • Behavioral variant: Someone with this FTD subtype will experience changes in their personality, language, social skills, and behavior.
  • Primary progressive aphasia: Someone with this FTD subtype will experience a decline in fluency when speaking.
  • Semantic variant: Someone with this FTD subtype will experience a decline in semantic memory, including the ability to understand vocabulary and general knowledge.

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Other Complications Of Dementia

Dementia and pneumonia often go hand in hand. As the ability to properly swallow declines, liquids can tragically end up down the wrong pipe, often leading to infection in the lungs.

Other conditions that may lead to death include heart failure, blood clots, and dehydration. Additionally, individuals with pre-existing health conditions can be at higher risk of death with dementia.

Can Dementia Be Prevented

Although dementia cannot be prevented, living a health-focused life might influence risk factors for certain types of dementia. Keeping blood vessels clear of cholesterol buildup, maintaining normal blood pressure, controlling blood sugar, staying at a healthy weight basically, staying as healthy as one can can keep the brain fueled with the oxygen and nutrients it needs to function at its highest possible level. Specific healthful steps you can take include:

  • Follow a Mediterranean diet, which is one filled with whole grains, vegetables, fruits, fish and shellfish, nuts, beans, olive oil and only limited amounts of red meats.
  • Exercise. Get at least 30 minutes of exercise most days of the week.
  • Keep your brain engaged. Solve puzzles, play word games, and try other mentally stimulating activities. These activities may delay the start of dementia.
  • Stay socially active. Interact with people discuss current events keep your mind, heart, and soul engaged.

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The Seven Stages Of Dementia

One of the most difficult things to hear about dementia is that, in most cases, dementia is irreversible and incurable. However, with an early diagnosis and proper care, the progression of some forms of dementia can be managed and slowed down. The cognitive decline that accompanies dementia conditions does not happen all at once – the progression of dementia can be divided into seven distinct, identifiable stages.

Learning about the stages of dementia can help with identifying signs and symptoms early on, as well as assisting sufferers and caretakers in knowing what to expect in further stages. The earlier dementia is diagnosed, the sooner treatment can start.

Trajectories Of Clinical Features

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During the last six months until death, in both groups there was an increase in prevalence of features including pressure ulcers, decrease of eating, being bedridden, pain, fever, nausea/vomiting, dysphagia, gait disorders, myoclonus, and anxiety.

In particularly bvFTD, there was an additional increase of 10% in daytime sleepiness, autonomic disorders, and epilepsy, whereas in yoAD these features included, hypertonia, restlessness,and intertrigo. On the other hand, features that decreased solely in bvFTD included disinhibition and hyperorality. In yoAD there was a decrease of wander .

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Living With Frontotemporal Dementia

Coping with FTD can be frightening, frustrating, and embarrassing for the patient and family members. Since some symptoms cant be controlled, family members shouldn’t take their loved ones behaviors personally. Families need to maintain their own well-being, while ensuring that their loved one is treated with dignity and respect.

Caregivers should learn all they can about FTD and gather a team of experts to help the family meet the medical, financial, and emotional challenges they are facing.

Its important to find a healthcare provider knowledgeable about FTD. Other healthcare specialists who may play a role on the team are home care nurses, neuropsychologists, genetic counselors, speech and language therapists, as well as physical and occupational therapists. Social workers can help the patient and caregivers find community resources, such as medical supplies and equipment, nursing care, support groups, respite care, and financial assistance.

Attorneys and financial advisors can help families prepare for the later stages of the disease.

Advanced planning will help smooth future transitions for the patient and family members, and may allow all to participate in the decision-making process.

Frontotemporal Dementia Causes And Risk Factors

Scientists donât know for sure what causes FTD. But it starts when nerve cells in two parts, or âlobes,â of the brain — the front and the side — die. That causes the lobes to shrink.

Doctors believe FTD may be genetic. About 4 out of 10 people who have it also have a relative who had it or some other form of dementia.

Genetic FTD happens when you have a mutation, or change, in one of three genes:

  • C9ORF72

The type of mutation you have affects the type of symptoms you have.

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Why Is Dementia Fatal

So, does dementia cause death?

Ultimately yes, dementia can lead to death. But, its often not the sole culprit.

As seen from the list above, dementia causes complications that contribute to death. In addition, dementia affects brain functions that support life.

When the condition affects higher-level brain functions that support life, dementia may be considered a direct cause of death such as when it causes a loss of appetite or difficulty swallowing.

How Is Ftd Diagnosed

Wnt signaling in Frontotemporal Dementia

FTD can be hard to diagnose because the symptoms are similar to those of other conditions. For example, bvFTD is sometimes misdiagnosed as a mood disorder, such as depression. To make matters more confusing, a person can have both FTD and another type of dementia, such as Alzheimer’s disease. Also, because these disorders are rare, physicians may be unfamiliar with the signs and symptoms.

To help diagnose frontotemporal dementia, a doctor may:

  • Perform an exam and ask about symptoms
  • Look at personal and family medical history
  • Use laboratory tests to help rule out other conditions
  • Order genetic testing
  • Conduct tests to assess memory, thinking, language skills, and physical functioning
  • Order imaging of the brain

A psychiatric evaluation can help determine if depression or another mental health condition is causing or contributing to the condition. Only genetic tests in familial cases or a brain autopsy after a person dies can confirm a diagnosis of FTD.

Researchers are studying ways to diagnose FTD earlier and more accurately and to distinguish them from other types of dementia. One area of research involves biomarkers, such as proteins or other substances in the blood or cerebrospinal fluid which can be used to measure disease progression or the effects of treatment. Researchers are also exploring ways to improve brain imaging and neuropsychological testing.

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What Is The Outlook For Frontotemporal Dementia

In the early stages, the symptoms and signs of frontotemporal dementia can be cared for and treated with good results. Late-stage frontotemporal dementia can take years to develop. As the disease progresses, 24-hour care may become necessary.

Frontotemporal dementia shortens a persons life span. The condition will eventually cause a person to have difficulty with bodily functions such as:

  • swallowing

How Common Is Ftd

FTD is the most common cause of dementia in people under 60. Typically the condition first appears in their mid-40s to early 60s. On average, the disease lasts eight years from diagnosis to death.

Due to similarities with other conditions, FTD is often misdiagnosed as Alzheimer’s disease, vascular dementia, Parkinson’s disease or another psychiatric problem. An MRI will determine whether or not a patient has FTD, since specific areas of the brain are damaged.

Although there is no cure for FTD, lifestyle changes and medications can ease symptoms.Mayo Clinic suggests speech therapy for patients dealing with language difficulties.

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What Are The Types Of Dementia

Dementias are often broken down into two main categories — Alzheimer type or non-Alzheimer type. Dementias of the Alzheimers disease type are defined by the symptoms of memory loss plus impairment in other brain functions, such as language function inability to move the muscles associated with speech or perception, visual or other inabilities to recognize speech or name objects .

Non-Alzheimer dementias include the frontotemporal lobar degenerations, which are further broken down into two main types. One type primarily affects speech. An example is primary progressive aphasia syndromes. The other type is defined by changes in behavior, including lack of feeling, emotion, interest or concern loss of a social filter personality change and loss of executive functions . In both of these frontotemporal lobe dementias, memory loss is relatively mild until later in the course of the disease.

Other non-Alzheimers disease dementias include vascular disorders , dementia with Lewy bodies, Parkinson’s dementia, and normal pressure hydrocephalus.

Is It Genetic Can It Be Passed On To Family Members

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  • A combination of genetic, environmental and lifestyle factors have been suggested by researchers. While a variety of changes on multiple genes have been linked to specific subtypes of frontotemporal dementia, more than half of people who develop frontotemporal dementia have no family history of dementia.
  • A persons risk of developing frontotemporal dementia is higher if they have a family history of dementia. Roughly 10-15 per cent of persons living with the disease have a strong family history of the disease. Typically, in these cases, FTD is inherited from a parent as a defect in one of three genes: MAPT, GRN or C9ORF72.
  • Currently, there are no other known risk factors.

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Signs Of The Dying Process

As someones condition gets worse and they are within a few days or hours of dying, further changes are common. The person may:

  • deteriorate more quickly than before
  • lose consciousness
  • develop an irregular breathing pattern
  • have a chesty or rattly sound to their breathing
  • have cold hands and feet.

These changes are part of the dying process when the person is often unaware of what is happening.

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Are There Any Treatments For Dementia

At this time there is no treatment for dementia. There is only medical care that can help manage symptoms and support people through their gradual decline.

The options for proper medical care with the diagnosis often include specialty caregivers, individual and family support groups, healthy diet and exercise, and frequent check-ins with your doctor.

Depending on which stage of dementia you or your loved one is in, the level of care required will vary. Someone in the earlier stages might need little to no care if symptoms are mild and not affecting daily life.

On the other hand, someone in the final stages of dementia will most certainly require 24/7 caregiving and constant supervision. If they dont have the proper care they need to avoid a risk factor such as choking or falling, it could lead to death.

Stage : Mild Cognitive Impairment

Reporting on frontotemporal dementia

Clear cognitive problems begin to manifest in stage 3. A few signs of stage 3 dementia include:

  • Getting lost easily
  • Noticeably poor performance at work
  • Forgetting the names of family members and close friends
  • Difficulty retaining information read in a book or passage
  • Losing or misplacing important objects
  • Difficulty concentrating

Patients often start to experience mild to moderate anxiety as these symptoms increasingly interfere with day to day life. Patients who may be in this stage of dementia are encouraged to have a clinical interview with a clinician for proper diagnosis.

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