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How Does Frontotemporal Dementia Progress

Driving And Work Problems

What is frontotemporal dementia?

In 2009 Pat began having problems with her driving. In September she hit a car pulling out from a driveway and worryingly didnt stop. In December she drove through a roundabout and somehow rolled her new car over.

In 2010 problems increased at work. On July 27 she noted in her diary: J told me that my colleagues didnt have confidence in my abilities. Definitely going to resign in Dec. Sadly even working as a nurse in the NHS her employer didnt think that a rapid decline in her abilities after 35 years nursing might be due to illness.

Symptoms Of Frontotemporal Dementia

Signs of frontotemporal dementia can include:

  • personality and behaviour changes acting inappropriately or impulsively, appearing selfish or unsympathetic, neglecting personal hygiene, overeating, or loss of motivation
  • language problems speaking slowly, struggling to make the right sounds when saying a word, getting words in the wrong order, or using words incorrectly
  • problems with mental abilities getting distracted easily, struggling with planning and organisation
  • memory problems these only tend to occur later on, unlike more common forms of dementia, such as Alzheimer’s disease

There may also be physical problems, such as slow or stiff movements, loss of bladder or bowel control , muscle weakness or difficulty swallowing.

These problems can make daily activities increasingly difficult, and the person may eventually be unable to look after themselves.

Read more about the symptoms of frontotemporal dementia.

Semantic Dementia And Progressive Nonfluent Aphasia

The uniqueness of SD has been underlined in the modern genetic FTD era. In contrast to bvFTD where a strong family history is found in up to 40% of affected individuals , genetic mutations are rarely found in SD patients . Moreover, the pathology is distinct. Although sharing with other syndromes a deposition of the protein TDP-43, the histopathological appearance of the deposition is unique and termed FTLD TDP-43 type C .

In contrast to SD, PNFA is a much more heterogeneous disorder in that patients share the characteristic of halting speech with pauses and distortion but this may or may not be accompanied by distortion of speech, phonological errors, or syntactic deficits. The early literature identified a range of underlying pathology including FTD and AD .

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What Happens In Bvftd

Due to their impaired judgment, people with bvFTD can fall prey to scams, online or in person. As the disease progresses, this lack of judgment may lead to criminal behavior . At the extreme, the impulsivity can be self-destructive, as when patients try to get out of a moving car. In some people, inappropriate sexual behavior occurs.

There may also be repetitive or compulsive behaviors that may include hoarding, doing the same thing over and over , pacing, or repeating particular catch phrases over and over.

The person with bvFTD may experience false thoughts that are jealous, religious or bizarre in nature. Or they can develop a euphoria excessive or inappropriate elation or exaggerated self-esteem.

Even though they might complain of memory disturbance, people with bvFTD can usually keep track of day-to-day events and understand what is going on around them. Their language skills and memory usually remain intact until late in the disease.

Problems with language and other thinking skills can also happen in bvFTD. People may start speaking in shorter phrases and may eventually lose their ability to speak. Sometimes, people with bvFTD may have weakness in their muscles, difficulty swallowing, or difficulty walking.

These behaviors have a physical cause and are not something that the person can usually control or contain. Indeed, often the person has little or no awareness of the problem behaviors.

Stage 3 Mild Changesquality Of Life: Very Little Impact

What is Frontotemporal Dementia??

You likely will start to notice changes in your loved ones thinking and reasoning. You also will see some memory loss. You may see your loved one:

  • Show some signs of forgetfulness, such as losing an item and not being able to retrace steps to find it.
  • Have some difficulty finding the right words or names.
  • Take more effort to remember appointments, manage money, and manage medicines.
  • Have trouble paying attention all the time.
  • Begin to have problems at work.

How You Can Help:

If you havent already done it, help your loved one plan for when he or she might have severe dementia. Use our tools to create a document that list his or her values and priorities at different stages of dementia.

If you have, help the health care team follow your loved ones care preferences.

You can also help with:

  • Organizing appointments.
  • Managing medicines, such as using a pill organizer.
  • Helping to put legal and financial documents in order.
  • Start to do more of the driving, if possible.

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Stage : Mild Cognitive Impairment

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.

Late Stage Frontotemporal Dementia

In the late stages of FTD, symptoms become closer to those of Alzheimers disease. While behavioral changes and language problems may develop early, memory loss generally does not occur until the late stages. In the late stages of all types of dementia, it can be difficult to discern one type from the others as symptoms become much more consistent. Because early stages of FTD can be difficult to recognize right away, many people living with late stage frontotemporal dementia are misdiagnosed as having Alzheimers. After the onset of FTD, the average person lives six to eight years but the disease currently has no cure and will eventually be fatal.

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Early Onset Frontotemporal Dementia

Frontotemporal dementia is one of the types of dementia that can affect younger people. Although it can strike in the elderly, its most often diagnosed between forty-five and sixty-five years of age. In fact, The Alzheimers Society says that its the second or third most common dementia in people under sixty-five and that it affects men and women roughly equally.

Behavioral Variant Frontotemporal Dementia

Frontotemporal dementia progression FTD (Pick’s Disease) – Time for Care

People with behavioral variant frontotemporal dementia often have trouble controlling their behavior. They may say inappropriate things or ignore other peoples feelings. bvFTD may affect how a person deals with everyday situations. bvFTD can also affect language or thinking skills. Unfortunately, people with bvFTD rarely notice these changes.

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Stages Of Picks Disease

There is no specific staging scale for Picks disease, but there are several scales for dementia.

The scale that doctors most commonly use is the Global Deterioration Scale , also called the Reisberg Scale.

The GDS specifies:

  • Stages 13: People in these stages do not meet the criteria for a dementia diagnosis. They have either no cognitive decline, very mild cognitive decline, or mild cognitive decline.
  • Stage 4: The average duration of this stage is 2 years, and it involves moderate cognitive decline or early stage dementia.
  • Stage 5: Moderately severe cognitive decline or mid-stage dementia occurs. The average duration of this stage is 1.5 years.
  • Stage 6: Severe cognitive decline marks this stage, which lasts an average of 2.5 years.
  • Stage 7: This stage involves very severe cognitive decline or late stage dementia. The average duration of this stage is 1.5 to 2.5 years.

Vascular Dementia: A Problem With Blood Vessels

Vascular dementia occurs when damage to blood vessels blocks blood flow to the brain, depriving brain cells of the oxygen and nutrients they need.

The progression of the resulting dementia depends in part on where and how the blockage occurred.

Damage to small blood vessels deep in the brain can cause dementia that worsens gradually, like Alzheimers disease.

When damage is due to a major stroke or a series of small strokes, symptoms occur suddenly. Instead of becoming worse gradually, symptoms plateau for long periods, followed by short, intense periods of change.

A person with early-stage vascular dementia will have difficulty planning and organizing, completing multistep tasks, and making decisions. Their thinking will also slow and they will have problems concentrating, with brief periods of confusion.

Mood swings, apathy, and heightened emotions are common. People with vascular dementia are also vulnerable to depression and anxiety.

As the disease progresses, symptoms are more likely to resemble those of middle and eventually later-stage Alzheimers disease: increasing memory loss, confusion, disorientation, and problems with reasoning and communication.

As with Alzheimers, irritability and agitation tend to increase, as do delusions and hallucinations.

Although each person with vascular disease will have a unique experience, patients live, on average, for five years after the onset of symptoms. Death is often due to a stroke or heart attack.

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What Affects The Speed Of Progression

The speed at which dementia progresses varies a lot from person to person because of factors such as:

  • the type of dementia for example, Alzheimers disease tends to progress more slowly than the other types
  • a persons age for example, Alzheimers disease generally progresses more slowly in older people than in younger people
  • other long-term health problems dementia tends to progress more quickly if the person is living with other conditions, such as heart disease, diabetes or high blood pressure, particularly if these are not well managed
  • delirium a medical condition that starts suddenly .

There is no way to be sure how quickly a persons dementia will progress. Some people with dementia will need support very soon after their diagnosis. In contrast, others will stay independent for several years.

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Scales For Rating Dementia

Pin on Alz News

Rather than simply using early stage,middle-stage, and late-stage dementia as descriptors, there are scales that provide a more comprehensive description. These scales help better understand the different stages of Alzheimers disease based on how well a person thinks and functions . These scales are the Global Deterioration Scale for Assessment of Primary Degenerative Dementia, the Functional Assessment Staging Test, and the Clinical Dementia Rating.

Did You Know?

Global Deterioration Scale / Reisberg Scale

The most commonly used scale is often referred to simply as GDS, or by its more formal name, the Reisberg Scale . The GDS divides into seven stages based on the amount of cognitive decline. This test is most relevant for people who have Alzheimers disease because some other types of dementia do not always include memory loss.

Someone in stages 1-3 does not typically exhibit enough symptoms for a dementia diagnosis. By the time a diagnosis has been made, a dementia patient is typically in stage 4 or beyond. Stage 4 is considered early dementia, stages 5 and 6 are considered middle dementia, and stage 7 is considered late dementia.

Global Deterioration Scale / Reisberg Scale
Diagnosis

Clinical Dementia Rating

Clinical Dementia Rating Scale
Stage
Average duration is 1 year to 2.5 years.

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The Behavioral Variant Of Frontotemporal Dementia

The bvFTD is the most common presentation of the different forms of FTD. At postmortem, most bvFTD patients show bilateral frontotemporal atrophy characterized by neuronal loss, microvacuolation, and a variable degree of astrocytic gliosis . Besides, early structural frontal lobe changes in bvFTD patients can be observed in MRI. These changes include atrophy of the mesial frontal, orbitofrontal and anterior cingulate cortices . When the disease progresses, atrophy is also present in the dorsolateral prefrontal cortex . In addition, characteristically, bvFTD patients show hypometabolism of the frontal cortex frequently extending into the anterior temporal regions . In bvFTD, the frontal lobe degeneration and disrupted connectivity with subcortical regions underlies the characteristics abnormal behavior and personality changes.

Table 1. Clinical criteria for the bvFTD diagnosis, according to the international consensus

Possible bvFTD
Three of the following cognitive-behavioral symptoms must be present. They must be persistent or recurrent, rather than unique events:

1.

Histopathological evidence of FTD in biopsy or postmortem.

3.

Presence of a known pathogenic mutation

There Are Two Broad Types Of Frontotemporal Dementia:

  • Behavioural variant FTD where damage to the frontal lobes of the brain mainly causes problems with behaviour and personality. These lobes are found behind the forehead and process information that affects how we behave and the control of our emotions. They also help us to plan, solve problems and focus for long enough to finish a task.
  • Primary progressive aphasia occurs when damage to the temporal lobes on either side of the head nearest the ears causes language problems. This part of the brain has many roles. A key function of the left temporal lobe is to store the meanings of words and the names of objects. The right temporal lobe helps most people recognise familiar faces and objects.

The first noticeable symptoms for a person with FTD will be changes to their personality and behaviour and/or difficulties with language.

These are very different from the early symptoms of more common types of dementia. For example, in Alzheimers disease, early changes are often problems with day-to-day memory. In the early stages of FTD, many people can still remember recent events.

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What Are The Symptoms Of Frontotemporal Dementia

Symptoms of FTD start gradually and progress steadily, and in some cases, rapidly. They vary from person to person, depending on the areas of the brain involved. These are common symptoms:

  • Behavior and/or dramatic personality changes, such as swearing, stealing, increased interest in sex, or a deterioration in personal hygiene habits
  • Socially inappropriate, impulsive, or repetitive behaviors
  • Impaired judgment
  • Agitation
  • Increasing dependence

Some people have physical symptoms, such as tremors, muscle spasms or weakness, rigidity, poor coordination and/or balance, or difficulty swallowing. Psychiatric symptoms, such as hallucinations or delusions, also may occur, although these are not as common as behavioral and language changes.

The Seven Stages Of Dementia

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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.

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Stage : Very Mild Cognitive Decline

Stage 2 can vary between typical age-related memory problems that most seniors face, such as forgetting specific dates or slower recall of a name or word. Or this stage could include some of the beginning signs of dementia that are often not obvious to doctors and loved ones. Some of the side effects that correspond with stage 2 include:

  • Forgetting everyday phrases or names
  • Forgetting the location of important objects

Pick’s Disease Vs Alzheimer’s Disease

Pick’s disease has many of the same causes and symptoms that Alzheimer’s does. But there are key differences.

Unlike people with Alzheimer’s disease, people with Pick’s disease:

  • Are diagnosed earlier in life
  • Don’t have hallucinations or delusions
  • Don’t tend to get lost in familiar places
  • Have a harder time making sense of their words or the words of others
  • Have behavior problems early on
  • Don’t have as many memory loss problems

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Diagnosing Frontal Lobe Dementia

No one test is able to diagnose frontal lobe dementia. Instead physicians are able to use the balance of evidence to diagnose frontal lobe dementia based on their best judgment. Because there is no foolproof test and diagnosis depends on the physicians knowledge, judgment, and observation of the patient, frontal lobe dementia is notoriously difficult to diagnose in its early stages. Although as the disease progresses, it becomes easier to definitely distinguish it between other disorders.

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Misconceptions About Ftd Lead To Underdiagnosis

PPT

In this retrospective study, we calculated an FTD age-standardized incidence rate of 2.9/100.000 py in our region. Our results stand in-between the ones of two recent studies using updated FTD criteria that found an incidence of 1.6/100.000 py in the UK and 3.05/100.000 py in Italy . However, while we used the same European reference population as our British colleagues, Logroscino et al. used the Italian population for standardization. Standardization of their incidence rate with the same European population yields an FTD age-standardized incidence rate of 2.78/100.000 py, strikingly similar to ours .

We found that FTD syndromes represented 3% of the Méotis network caseload. Similar MC surveys in Netherlands and Sweden had 7% and 3.6% of FTD syndromes, respectively. However, all patients in the Dutch cohort were followed in the Alzheimer center of the VU University Medical Center , a tertiary center where atypical dementias are likely to be addressed, possibly leading to an overrepresentation of FTD patients. Likewise, there was a 8.1% proportion of FTD patients in Lille tertiary center. A recent review on the epidemiology of FTD highlighted three studies with high methodological standards . In these publications using the Lund and Manchester or Neary criteria, FTD syndromes accounted for 1.1% , 3% , and 3.8% of dementia cases, which is consistent with our findings.

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