Signs Of Frontotemporal Dementia
Signs Of Frontotemporal Dementia
A group of disorders caused by cell degeneration, frontotemporal dementia affects the brain, specifically its areas associated with personality, behavior and language. Once considered a rare disease, FTD may account for 20-50% of dementia cases in people younger than age 65, according to the Alzheimers Association.
FTD causes cell damage that shrinks the brains frontal and temporal lobes. The disease generally starts with personality and behavior changes and may eventually lead to severe memory loss.
Often miscategorized as psychiatric illness, frontotemporal dementia typically strikes between the ages of 45 and 65. However, the Association for Frontotemporal Dementia Degeneration indicates that cases have occurred as early as age 21 and as late as age 80.
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
What Are The Complications Of 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. People are also at increased risk for infections and fall-related injuries.
As FTD progressively worsens, people may engage in dangerous behaviors or be unable to care for themselves. They may need 24-hour nursing care or to stay in an assisted living facility or nursing home.
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Can You Fully Recover From Frontal Lobe Damage
Treating frontal lobe injuries arent easy, because everyone responds differently. The key is for both the patient and their family to have patience. Full recovery can take weeks, months, years or may never occur, so you need to be patient and take pride in the progress being made. It also may not be easy.
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
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Why Is Ftd Hard To Diagnose
Frontotemporal dementia is much less common than other types of dementia and often has different early symptoms. This means FTD can be hard for doctors to diagnose as they may not recognise its symptoms as dementia.
Most changes in behaviour or personality caused by FTD may not be very obvious at first. These kinds of symptoms for example, risk-taking, loss of social or sexual inhibitions, or obsessive behaviour can sometimes look more like the person is going through a difficult or emotionally-challenging time.
Also, if the person is under 65, doctors may not expect to see dementia in someone so young.
Even if changes in behaviour are understood as medical symptoms, they may be mistaken for depression, schizophrenia or obsessive-compulsive disorder. Problems with language or movement may also be misdiagnosed.
If a person is showing some of the symptoms mentioned in this factsheet, they may want to ask their doctor to explore FTD as a possible diagnosis especially if they have family members with the condition.
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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Language Variants Of Ftd
In the other two types of FTD, the early symptoms are difficulties with language that progressively get worse. These difficulties become apparent slowly, often over two or more years.
In progressive non-fluent aphasia, these problems are with speech aphasia means loss of language. Common early symptoms may include:
slow, hesitant speech which may seem difficult to produce a person may stutter before they can get the right word out, or may mispronounce it when they do
- errors in grammar a person may have telegraphic speech, leaving out small link words such as to, from or the
- impaired understanding of complex sentences, but not single words.
In semantic dementia, speech is fluent but people begin to lose their vocabulary and understanding of what objects are. Common early symptoms may include:
- asking the meaning of familiar words
- trouble finding the right word, leading to descriptions instead , or use of less precise words
- difficulty recognising familiar people or common objects.
In the early stages of both language forms of FTD, other mental abilities tend to be unaffected.
People With Ftd May Develop Curious Obsessions
One of the key symptoms often seen in FTD is changes in personality and behaviour. This can be an aspect of the condition that is particularly upsetting for those around the person with FTD, as it is difficult to understand why the person you know so well is acting so differently.
These changes can also bring about new obsessions including: hoarding, refusing to throw anything away fiddling and touching everything around a symptom called utilisation behaviour and disinhibition, which can take many forms but commonly includes a loss of social graces. You can find an A-Z of FTD obsessions on the Rare Dementia Support Group website, www.raredementiasupport.org/ftd/what-is-ftd/a-z-of-picks-ftld-obsessions.
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What Is The Prognosis In Frontotemporal Dementia
The clinical course of all FTD syndromes is steadily progressive, with declining function in everyday life and accumulation of social, cognitive, and neurological disabilities leading to complete dependency requiring institutional care. However, survival duration based on meta-analyses is highly variable across the FTD spectrum22: the most aggressive course is associated with FTD with motor neurone disease, leading to death typically within three to five years of symptom onset, whereas a substantial proportion of patients survive for well over a decade. There tends to be convergence of syndromes over time,23 with emergence of non-cognitive neurological features in many cases, and the precise clinical trajectory is difficult to predict in individual cases. Wandering and intrusive behaviours, apathy, incontinence, mutism, and dysphagia are common issues toward the end of life and important sources of caregiver distress. The burden placed on those who care for patients with FTD is great, even in comparison with other neurodegenerative diseases.24
Distinguishing Features Of Other Common Types Of Dementia
More than 90 percent of patients with dementia have Alzheimer disease, dementia with Lewy bodies, vascular dementia, or FTD.24 Parkinson disease with dementia may be part of the spectrum of disease progression associated with dementia with Lewy bodies.25 Primary signs and symptoms associated with Alzheimer disease are memory and visuospatial loss. Patients who have dementia with Lewy bodies also have memory loss, fluctuating cognition, and visual hallucinations, and develop spontaneous parkinsonism motor features. Those with vascular dementia usually have a history of cerebrovascular events and white matter changes on neuroimaging. They may present with focal neurologic deficits, early gait disturbances, changes in personality and mood, and a history of frequent falls or unsteadiness.
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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.
What Are The Risk Factors For Frontotemporal Dementia
Frontotemporal dementia has one known risk factor: genetics. Scientists have found several genes related to the disease. If one of your family members is diagnosed with frontotemporal dementia, you have a greater risk.
However, not everyone with a family history will develop a problem. Its also estimated that more than half of the individuals diagnosed with frontotemporal dementia dont have a family history associated with the disease.
Doctors cant diagnose frontotemporal dementia with a single test. Instead, your doctors will try to rule out other conditions or diseases that cause similar symptoms.
Some of the tests used to diagnose frontotemporal dementia include:
- blood tests: These can help identify other possible causes.
- neuropsychological testing: These tests check your judgment and memory skills, and help determine what type of dementia you may have.
- brain imaging: Doctors will check for tumors or blood clots.
- MRI: A magnetic resonance imaging test gives doctors a detailed image of your brain.
- CT scan: A computerized tomography scan creates images of your brain in layers.
Frontotemporal dementia cannot be cured. Treatment is aimed at managing and alleviating symptoms.
Common treatments include:
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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 Alzheimer’s 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 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
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Is There A Neuropsychologic Test For Executive Function
Given the pervasive nature of executive deficit, assessment of executive functions is of crucial importance in neuropsychiatry, child and adolescent psychiatry, geriatric psychiatry, and other related areas. A number of neuropsychologic tests of executive function commonly are used in assessing seve
The Role Of Specialists
Many primary care physicians are uncomfortable making the diagnosis of FTD. Given the uncommon nature of the condition, and the implications of an incorrect diagnosis, it is reasonable to refer those suspected of having FTD to a specialty center in cognitive disorders. Most of these are directed by neurologists, though an interested geriatric psychiatrist or geriatrician may also be appropriate. Psychiatrists are helpful when behavioral or emotional problems are predominant. Geriatricians are desirable in older FTD patients with concurrent medical comorbidities.
Some individuals seek rehabilitation services. These are appropriate when there are functional disabilities in communication , mobility or self-care . Cognitive therapies are sometimes appropriate when specific tasks need to be learned. A referral for behavioral management strategies can also be helpful for caregivers since most individuals with FTD are more functional in a structured, consistent environment.
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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
Arterial Spin Labeling
Tau PET Imaging
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.
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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.
What Are The Stages Of Frontotemporal Dementia
James M. Ellison, MD, MPH
Swank Center for Memory Care and Geriatric Consultation, ChristianaCare
- Expert Advice
Learn about the symptoms associated with the early, middle, and late stages of frontotemporal dementia.
On the surface, Joseph, Barbara, and Lloyd may not seem to be experiencing related changes. Each of them, though, turned out to be in the early stage of frontotemporal dementia . FTD is the diagnosis for about 5 percent of people with major neurocognitive disorders . About 70 percent of cases begin before age 65, so it is a more common dementia among the young old. FTD involves degeneration of the frontal and temporal lobes of the brain. The frontal lobes are important regulators of behavior and the temporal lobes assist in our understanding and expression of language. The symptoms of FTD, therefore, include major changes in behavior, impairment of language, or both.
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Who Gets Frontotemporal Dementia
FTD can affect anybody. It typically affects people at a younger age than Alzheimers disease, with symptoms beginning in the 50s or 60s, and sometimes younger.
Almost a third of people with FTD have a family history of dementia. However, only about 10-15% of cases have familial FTD, in which a gene mutation is passed on that causes the disease.
The genetic basis of FTD is not fully understood and is actively being researched.
Frontotemporal Dementia Versus Psychiatric Disorders
When behavioral symptoms predominate, people with FTD who become ill in mid-life may be confused with patients who have late life depression. When the onset is in younger persons, the FTD may be confused with schizophrenia or bipolar disorder. Repetitive compulsive behaviors are very common in bvFTD, and some patients may initially be given the diagnosis of obsessive-compulsive disorder. Since the history and exam of a person with a psychiatric disorder and a person with FTD may look very similar, neuropsychological testing and a brain image may help clarify the picture. MRI can help rule out other diseases and support a diagnosis of FTD.
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