Amyotrophic Lateral Sclerosis With Frontotemporal Dementia
Toms illness was clearly not simply a psychiatric depression because depression does not include muscle weakness and fasciculations. Evaluation of his nerve conduction velocity and neuropsychological status revealed that he had frontotemporal dementia along with disease of the motor cortex brain cells, the neurons that control muscles. This is one way that amyotrophic lateral sclerosis can present. When FTD and motor neuron disease are both present, a person can develop muscle weakness leading to difficulty walking, grasping a pen, lifting their arms over their head, or performing precise small muscle movements. Physical examination reveals muscle twitching, spasticity, and atrophy as well as weakness. Over time, difficulty with speech and swallowing can be noticed.
As yet, we have no definitive treatment for FTD, but there are valuable information resources for caregivers and patients through the Association for Frontotemporal Dementia .
How Is Ftd Diagnosed
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.
Trouble With Balance And Mobility
At Easter 2016 we reached another crisis when her weight dropped drastically and a PEG tube was considered, but it was decided that she would be at great risk if she underwent the operation. However, once she came off her medication for osteoporosis she began to take food again and regained weight.
Don’t Miss: Alzheimers Dement
What Are The Signs And Symptoms Of Frontotemporal Dementia
The signs and symptoms of frontotemporal dementia vary from person to person and the order in which they appear can also vary. Changes in the frontal lobe of the brain are generally associated with behavioral symptoms and may also lead to movement symptoms. Changes in the temporal lobe generally lead to disorders of language and emotion.
Symptoms of frontotemporal dementia and associated disorders may include:
What Is Frontotemporal Dementia
Frontotemporal dementia , a common cause of dementia, is a group of disorders that occur when nerve cells in the frontal and temporal lobes of the brain are lost. This causes the lobes to shrink. FTD can affect behavior, personality, language, and movement.
These disorders are among the most common dementias that strike at younger ages. Symptoms typically start between the ages of 40 and 65, but FTD can strike young adults and those who are older. FTD affects men and women equally.
The most common types of FTD are:
- Frontal variant. This form of FTD affects behavior and personality.
- Primary progressive aphasia. Aphasia means difficulty communicating. This form has two subtypes:
- Progressive nonfluent aphasia, which affects the ability to speak.
- Semantic dementia, which affects the ability to use and understand language.
A less common form of FTD affects movement, causing symptoms similar to Parkinson disease or amyotrophic lateral sclerosis .
Recommended Reading: Does Diet Coke Cause Dementia
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.
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
You May Like: What Is The Difference Between Senility And Dementia
First Thing Doctors Will Do Is Eliminate Other Possible Diagnoses
Dr. Lorenzo states, “In my experience, many of the people that come to me reporting memory difficulties, trouble concentrating and other cognitive issues have a relatively benign explanation for their complaints. Big offenders in this first stage of eliminating possible diagnoses include things like:
Medications that cause side effects that affect cognitive functions. This also includes recreational substances such as alcohol, ‘downer’ type drugs, cannabis. Now that cannabis is being used so much more frequently by people trying to treat a myriad of conditions, we are starting to see what is called cannabis encephalopathy among heavy users. This is associated with severe and long-lasting memory impairment and changes in the structure of the brain similar to what happens in an Alzheimer’s dementia patient.
Pseudodementia. There has been such an upswing in people who are depressed and anxious with everything that’s going on with Covid, and it is well known that one of the cardinal symptoms for depression includes trouble concentrating and forgetfulness.
Poor sleep. Whether it be volitional such as people ‘burning the candle at both ends’ or due to insufficient sleep in people with a sleep disorder, such as obstructive sleep apnea or chronic insomnia , sleep is crucial for the brain to recharge and to function at its optimal capacity. “
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.
Recommended Reading: Aphasia Alzheimer’s Disease
Treating Language Problems In Ftd
Treatment of PPA has two goals maintaining language skills and using new tools and other ways to communicate. Treatment tailored to a persons specific language problem and stage of PPA generally works best. Since language ability declines over time, different strategies may be needed as the illness progresses. The following strategies may help:
- Use a communication notebook , gestures, and drawings to communicate without talking.
- Store lists of words or phrases in a computer or phone to point to.
- Speak slowly and clearly, use simple sentences, wait for responses, and ask for clarification if needed.
- Work with a speech-language pathologist familiar with PPA to determine the best tools and strategies to use. Note that many speech-language pathologists are trained to treat aphasia caused by stroke, which requires different strategies from those used with PPA.
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 .
You May Like: Does Prevagen Help With Dementia
What Are Frontotemporal Disorders Causes Symptoms And Treatment
On this page:
Frontotemporal disorders , sometimes called frontotemporal dementia, are the result of damage to neurons in the frontal and temporal lobes of the brain. Many possible symptoms can result, including unusual behaviors, emotional problems, trouble communicating, difficulty with work, or difficulty with walking. FTD is rare and tends to occur at a younger age than other forms of dementia. Roughly 60% of people with FTD are 45 to 64 years old.
FTD is progressive, meaning symptoms get worse over time. In the early stages, people may have just one symptom. As the disease progresses, other symptoms appear as more parts of the brain are affected. It is difficult to predict how long someone with FTD will live. Some people live more than 10 years after diagnosis, while others live less than two years after they are diagnosed.
There is currently no cure for FTD, and no treatments slow or stop the progression of the disease, but there are ways to help manage the symptoms.
Frontotemporal Dementia Treatment And Life Expectancy
Unfortunately, like other dementias, FTD is a progressive disease with no cure.
That means the symptoms will worsen over time. The speed of decline will be different for each person.
Treatment optionsCurrent treatments focus on easing symptoms, but cant slow the progression.
There are medications that can be used to improve quality of life by reducing behavioral symptoms.
- Selective serotonin reuptake inhibitors like Zoloft or Luvox
- Antipsychotics like Zyprexa or Seroquel
Important: These medications must be used with caution because the side effects include an increased risk of death in people with dementia.
Speech therapy could also help with language issues by teaching alternate communication strategies.
Life expectancyFrontotemporal dementia shortens a persons life span. Each person is different, but most people with FTD live 6 to 8 years after the first symptoms appear.
FTD will eventually cause a person to have difficulty with essential bodily functions like chewing, swallowing, moving around, and controlling the bladder and bowels.
These changes can cause serious infections in the lungs, urinary tract, and skin leading to death.
Read Also: Alzheimer’s Purple Color Code
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:
Can Lifestyle Changes Help Frontotemporal Dementia
Medication can be effective for treating dementia, but lifestyle treatments can help, too. Helping people find a comfortable environment can help them cope with the symptoms of dementia.
Environment is important. Maintaining an environment that doesnt upset someone is vital. Make sure your home is well-lit and has minimal noise. People with behavior problems need to be in environments that are familiar. They may also need to avoid large crowds.
People with speech problems may need to be in environments where communication is easier. They may wish to keep tools for communicating, like a pen and paper, with them all the time.
Recommended Reading: At What Age Alzheimer’s Start
Clinical Genetics General Characteristic And Parkinsonian Features In Familial Ftd With Parkinsonism
Molecular genetic studies in patients with FTD associated with Parkinsonism have identified mutations in several genes: MAPT, PGRN, C9ORF72, CHMP2B, and VCP. Mutations found in common ALS genes, TARDBP and FUS, may also contribute to FTD associated with Parkinsonism . Parkinsonism seen in FTD patients carrying different autosomal dominant gene mutations can have a diverse clinical manifestation. In we provide a summary of parkinsonian features and other motor and non-motor manifestations that could be present in patients with FTD.
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:
Also Check: What Is The 7th Stage Of Alzheimer’s
Dementia Signs That Worry Doctors Most
A surprisingly high 5.8 million Americans live with dementia according to the Centers for Disease Control and Prevention. “Dementia is the loss of cognitive functioning thinking, remembering, and reasoning to such an extent that it interferes with a person’s daily life and activities. Some people with dementia cannot control their emotions, and their personalities may change,” the National Institute on Aging states. While diagnosing dementia can be a challenge at times, particularly in the early stages, there are signs to watch out for that indicate someone has the condition. Dr. Dalia Lorenzo, neurologist at Baptist Health’s Miami Neuroscience Institute says, “Many of the signs of dementia can be subtle and of insidious onset. It is not unusual for the signs to be attributed to the mild forgetfulness that can accompany normal aging and truth be told, early on, it can be difficult to distinguish what accompanies normal aging from what will develop into the progressive neurodegeneration that we know as Alzheimer type dementia. Many times, doctors are left with watchful waiting to see how the symptoms evolve over time.”Eat This, Not That! Health talked to experts who explained signs that worry doctors the most. Read onand to ensure your health and the health of others, don’t miss theseSure Signs You’ve Already Had COVID.
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.
You May Like: Diet Soda And Alzheimer’s
Signs And Symptoms Of Frontotemporal Dementia
The most common signs and symptoms of frontotemporal dementia are extreme changes in behavior and personality.
In the early stages of FTD, people typically have one type of symptom. As the disease progresses, more types of symptoms will appear as more parts of the brain are affected.
Its important to know that these behaviors are caused by physical damage inside the brain and arent things the person can control or contain.
Often, they arent even aware that their behavior has changed or that its become a problem.
Common behavioral and personality symptoms
- Changes in personality and mood like becoming depressed, self-centered, or withdrawn
- Avoiding socializing or being unwilling to talk
- Repetitive or obsessive behavior
- Apathy like no longer paying attention to hobbies and interests
- Lack of awareness of thinking or behavioral changes
- Loss of empathy and other interpersonal skills
- Increasingly inappropriate actions like unusual verbal, physical or sexual behavior
- Changes in eating habits, especially overeating
- Weight gain due to overeating
- Putting things in the mouth or trying to eat inedible objects
However, people with FTD can usually keep track of day-to-day events and understand whats going on around them.
And people with bvFTD usually keep their language skills and memory until late in the disease.
Common speech and language symptoms
Some people with FTD could have severe problems recalling and understanding words, but still be able to speak fairly normally.
How Lack Of Sleep Is Connected To Dementia
Dr. Lorenzo explains, “Although most people think of sleep as seven hours of downtime and their 24-hour day, the truth is that sleep has a very important function in refreshing and recharging the brain. For the majority of the population, purposeful curtailment of sleep hours is seen as a good way to squeeze a few more hours of work and play in the day. But the studies say otherwise. We know that people who get less than six hours of sleep nightly have a higher risk of developing dementia later in life.”
Recommended Reading: What Is Senility
How To Spot Pick’s Disease
Picks disease is one of three dementias classed as frontotemporal dementias they are all associated with shrinking temporal and frontal anterior lobes of the brain. The other two frontotemporal dementias are semantic dementia and primary progressive aphasia. All three dementias have some genetic causes, for they often run in families. Picks disease can also be sporadic, which means it can have no known cause.
The symptoms of Picks disease fall into two broad categories: difficulties with language and behavioral changes. Unlike other dementias, such as Alzheimers disease, memory is not affected until the disease has become quite advanced. The patients spatial awareness also remains intact. Get to know some of the major warning signs of Pick’s disease now.