Different Types Of Frontotemporal Dementia & Their Symptoms and Treatment Options

What is Frontotemporal Dementia?

Frontotemporal Dementia is a medical term used for describing a collection of disorders which cause cognitive decline in an individual. These disorders cause a gradual decline in executive functioning, language processing and significant behavioral issues. Frontotemporal Dementia is the third most common form of dementia or memory loss after Alzheimer Dementia and Lewy Body Dementia. The primary cause of Frontotemporal Dementia is gradual degeneration of the frontal and temporal lobes of the brain. It is further subcategorized into behavioral and language subtypes depending on the symptoms displayed by the patient.[1]

The World health Organization states that there are about 48 million people who have some form of dementia and the numbers are ever increasing to the tune of 7 million cases reported every year. Out of these, around 70% of cases are diagnosed with Alzheimer while others are diagnosed with mixed type of dementia. Study done on the prevalence of Frontotemporal Dementia suggest that this is the second most common form of dementia in people below the age of 60. There were about 25% cases of Frontotemporal Dementia diagnosed in people above the age of 65.[1]

However, it is quite tough to predict the exact number of people with underlying Frontotemporal Dementia due to the low frequency of the disease even in people who are at severe risk. The prevalence of Frontotemporal Dementia is also impaired by the difficulty that this condition poses to clinicians in accurately diagnosing it. In the United States, studies suggest that the prevalence of Frontotemporal Dementia is about 20 in every 100,000 people.[1]

What exactly causes Frontotemporal Dementia is not very well understood but researchers are of the belief that gradual shrinking of the frontal and temporal lobes in the brain with age and time is one of the factors that play a role in the development of this condition. The shrinking of these lobes are a result of buildup of abnormal proteins which become toxic and start causing damage to the brain cells causing them to shrink. There have also been several gene defects that have been associated with the development of Frontotemporal Dementia but majority of people with this defect do not have a family history of this condition.[1]

Some studies suggest a genetic and molecular similarity between Frontotemporal Dementia and ALS but more research needs to be done in this respect to verify the association. This article describes the various types of Frontotemporal Dementia, their presenting features, and the management options for this condition.[1]

Different Types Of Frontotemporal Dementia & Their Symptoms and Treatment Options

As stated, Frontotemporal Dementia is a collection of a group of neurocognitive disorders. These disorders are categorized based on the symptoms that are displayed by the patient. They include a gradual decline in behavior and personality, decline in language processing, and progressive decline in motor function. The symptom along with a careful inspection of the lobe that is damaged or affected determines the type of Frontotemporal Dementia the individual has.[2]

In cases of a behavior and personality decline, the patient will display errors in judgment. The patient will have a significant change in personality and behavior where he or she will cry or laugh at inappropriate times. Their behavior becomes unpredictable where they may become aggressive all of a sudden or may prefer to stay alone on some days. This variant is termed as Behavioral Variant Frontotemporal Dementia. This form of Frontotemporal Dementia is seen in around 60% of cases of this condition.[2]

In cases of a language decline, the patient will start having problems with reading, writing, or communicating. The patient will also have a tough time understanding what is being said to him or her. A gradual decline in the language processing can be due to two variants of Frontotemporal Dementia namely non-fluent aphasia in which the individual has a gradual decline in the ability to speak.[2]

The second variant is semantic dementia where the patient starts forgetting objects that are used in everyday life like a razor, cream, or comb. The patient may also forget the names of people close to him or her. These two variants of Frontotemporal Dementia constitute about 20% of all cases of Frontotemporal Dementia.[2]

In cases of a decline in the motor function, the patient will have problems with any attempts at movement. Ambulation becomes a challenge for them as they are not able to use their extremities in the right way. The coordination is extremely poor and there is continuous shaking of the limbs. In many cases, Frontotemporal Dementia tends to occur with other motor dysfunctions like motor neuron disease of the ALS type in which the nerves of the brain and spinal cord get affected.[2]

Supranuclear palsy is yet another motor dysfunction that is commonly seen in people with Frontotemporal Dementia where the patient has difficulty performing any type of movement whether it is walking or holding a cup of coffee.[2]

Coming to the presenting features of Frontotemporal Dementia, the symptoms are significantly variable and depend on the type of Frontotemporal Dementia the patient has. However, as the disease progresses, the function of the entire brain is affected and the patient will have symptoms of all the variants together.

In cases where the patient has Behavioral variant Frontotemporal Dementia, the following symptoms will be displayed:[2]

  • Gradual decline in ability to multitask, plan a task, prioritize tasks and have unpredictable behavior.
  • They will have repetitive behaviors like washing hands every time they touch something
  • Extremely aggressive at times and sulky behavior at other times
  • These people tend to eat a lot and always feel hungry
  • They do not take care of their personal hygiene
  • They will show complete lack of interest in doing anything
  • They are completely insensitive and will show no signs of empathy
  • Gradually, the individual will become socially withdrawn and may restrict self in a room.[2]

People with the Language Variant Frontotemporal Dementia will have:

  • Reduced ability to understand words but will be able to speak normally
  • In some cases, they may have slurred speech but will be able to understand words.
  • The vocabulary of the individual tends to decrease gradually
  • There will repetition of words quite often
  • As the disease progresses, the overall ability to comprehend and speak declines.[2]

People with Motor Variant Frontotemporal Dementia will exhibit the following symptoms:

  • They will not be able to perform complicated movements like eating with a fork and a knife
  • They will have inability to button their shirt or wear a belt
  • They will not be able to hold an object and will often drop them such as a coffee cup
  • Their gait will be shuffling or waddling and they will be prone to falls
  • Significant muscle weakness will also be observed
  • Tremors of the upper and lower extremity
  • Dysphagia

In some cases, there may be urinary incontinence due to the inability of the patient to maneuver self to go to the restroom on time.[2]

Unfortunately, as of now, there is no way to either cure Frontotemporal Dementia or slow down the progression of the disease process. The treatment is mainly aimed at controlling the symptoms and improving quality of life of the patient.[2]

For managing behavioral problems, there are certain medications that can be given which will help in controlling the behavior to some extent. They can also help in preventing other symptoms like overeating and repetitive behaviors. These medications include SSRIs like sertraline and antipsychotics like olanzapine. It is mandatory for people with Frontotemporal Dementia who are on these medications to be monitored closely as they tend to have potentially serious side effects especially in people with dementia.[2]

For managing the symptoms of language and comprehension, speech therapy can be of help since they will devise new ways of communication between the patient and the family or caregiver. In most cases, Frontotemporal Dementia patients will have to communicate through signs or through a notebook. It can also be helpful to provide such patients with photographs of common objects which they can show if they need it.[2]

It is essential for family members or the caregivers to speak solely to the patients so that they are able to grasp and comprehend what is being said to them. There should always be a change of plan on hand since the symptoms will change for the worse as the disease progresses.[2]

Unfortunately, there is no way to slow down the decline of motor function in an individual with Frontotemporal Dementia. There are certain medications which may improve the quality of life when they are used with physical therapy sessions. As of now, research is still ongoing as to find out more effective treatments for this condition. The researchers are trying to find ways to somehow target and eliminate the abnormal proteins that cluster together damaging the brain cells causing Frontotemporal Dementia.[2]

In conclusion, Frontotemporal Dementia is a progressive neurodegenerative disorder in which the affected individual gradually loses the ability to function in everyday life. They lose the ability to speak, read, and write. In some cases, patients with Frontotemporal Dementia are not able to speak anything at all. Gradually, they forget familiar people or objects they use daily like a toothbrush or a shaving cream.[2]

The primary cause of Frontotemporal Dementia is not clear but studies suggest accumulation of abnormal proteins in the brain that gradually damage the frontal and temporal lobes of the brain as the reason behind this condition. There is no cure for Frontotemporal Dementia and unfortunately researchers are still trying to find a way to at least slow down the progression. As the condition progresses, the patient starts to need 24 hour supervision or has to be admitted to a residential care facility.[2]

The overall survival rate of people with Frontotemporal Dementia is variable. The prognosis is quite poor if Frontotemporal Dementia occurs with a motor neuron dysfunction like ALS where the individual at maximum can survive for about 5 years post diagnosis. In other cases, the survival rate is quite bright and the patient may survive up to 10 years even though the quality of life may be severely compromised.[2]

It is quite a challenge for caregivers and family members to care for an individual with Frontotemporal Dementia. A strong support network is therefore required to help the patient cope up with the stress of managing a dementing illness like Frontotemporal Dementia.[2]

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