What is frontotemporal dementia?

Dementia develops when there is progressive damage to the brain. Frontotemporal dementia is the term used to describe a number of different conditions, in which particular areas of the brain are affected leading to characteristic personality and behavioural changes.

Frontotemporal dementia is also known as Pick’s disease or frontal lobe dementia

The name refers to the parts of the brain that are affected in this form of the condition. The damage happens to the frontal and the temporal lobes. The connections between the lobes are affected, some of the chemical messengers that allow the nerve cells to communicate are lost and the brain cells in these areas die. With time, progressively more nerve cells die so that the brain tissue shrinks. This brain damage leads to signs and symptoms that can differ from those usually seen in Alzheimer’s disease.

What to expect with frontotemporal dementia?

Here is what you can expect from frontotemporal dementia:

Brains and behaviour

The frontal lobes, as the name suggests, are found at the front of the brain behind the forehead. This area controls behaviour, planning, problem solving and emotional control. One particular area also facilitates speech.

There are two temporal lobes, one on each side of the brain. They help with communication, knowing names and the meaning of words. They are also involved in recognising people and objects.

Because a different area of the brain is damaged, people with frontotemporal dementia may present with different symptoms from people with Alzheimer’s, especially in the early stages. Instead of problems with forgetfulness and short-term memory loss, damage to the frontotemporal areas can lead to changes in personality and struggles with speech and language.

Dementia of these parts of the brain can lead to individuals behaving rashly or irresponsibly, this can cause problems with driving, so it is particularly important for people with frontotemporal dementia to inform the DVLA.

There are three types of frontotemporal dementia, with each showing different symptoms especially in the early stages. As with all forms of dementia, the signs can be very subtle at first. With time, there is increasing brain damage and the differences between the types of the disease become less evident. Eventually, the disease will affect the individual’s ability to function and live independently.

Behavioural variant frontotemporal dementia

People with this type of frontotemporal dementia lack control over their behaviour. Even in the very early stages they may not understand or appreciate what is happening to them, so problems are usually picked by friends, family or work colleagues. Early symptoms include:

• Loss of inhibitions and behaving in a rude or socially inappropriate way.
• Apathy and loss of interest in people and things they used to like, without appearing sad.
• Loss of empathy and becoming selfish, unfeeling and unresponsive to the emotions of others.
• Compulsive behaviours, obsessive new interests and repetitive use of words and phrases.
• Deterioration in social skills and poor table manners.
• Change in appetite. People affected may crave sweet, fatty or fast foods and may eat, drink or smoke too much.
• Problems with planning, organisation and decision making. These can cause difficulties with work and home lives.

Progressive non-fluent aphasia

People with this form of the disease have gradually increasing language difficulties and problems with speech. Early symptoms include:

• Hesitant, slow or stuttering speech. They may struggle to get their sentences out or mispronounce words.
• People affected may develop grammatical errors. Some people speak in a pattern called ‘telegraphic speech’, in which linking words like ‘to’ or ‘but’ or ‘and’ are left out.
• Problems understanding complex sentences.

Semantic dementia

People with semantic dementia also have language problems. However, unlike aphasia, their speech is fluent but may indicate problems with understanding what objects are and finding the right words, confusion about the meaning of very familiar words, or difficulties finding the right word, so that they may describe objects or use generic terms.
Problems recognising familiar people and things.

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, it’s most often diagnosed between forty-five and sixty-five years of age. In fact, The Alzheimer’s Society says that it’s the second or third most common dementia in people under sixty-five and that it affects men and women roughly equally.

How to diagnose frontotemporal dementia?

There are no specific tests for diagnosing frontotemporal dementia. However, clinical history and some non-specific tests may help. The diagnosis of dementia is difficult because of overlapping symptoms. Non-specific tests helpful in diagnosis include:

• Blood tests: to rule out any other major pathology in the body.
• The sleep study test: it helps find out the cause of obstructive sleep apnea, which some patient with frontotemporal dementia may experience.
• Neuropsychological test: it may be conducted to determine what type of dementia the patient has.
• Brain imaging studies: these are also of great importance in helping reach the diagnosis of frontotemporal dementia. Imaging techniques include MRI scans and Fluorodeoxyglucose positron emission tracer scan (FDG-PET). With the help of an FDG-PET scan, areas of brain degeneration can be identified easily.

Frontotemporal dementia diagnostic criteria

Clinical history that is more consistent with frontotemporal dementia includes certain characteristic features. The diagnostic criteria require three of the following six clinical features:

1. Disinhibition
2. Apathy/inertia
3. Loss of sympathy/empathy
4. Perseverative/compulsive behaviours
5. Hyperorality
6. Dysexecutive neuropsychological profile

What is the treatment for frontotemporal dementia?

The treatment of frontotemporal dementia is both pharmacologic and supportive. Medical drugs include some antidepressants such as trazodone and selective serotonin reuptake inhibitors (SSRIs).

The variants of frontotemporal dementia

Frontotemporal dementia can occur in three clinical variants. These are:

1. Behavioral variant
2. Semantic variant (SD)
3. Progressive non-influent aphasia

Which variant is SD of frontotemporal dementia?

In the SD variant, the patient gradually loses the ability to comprehend the meaning of a word and develops difficulty in recognizing the face of a familiar person.

How to help someone with frontotemporal dementia?

The caregiver to a patient with frontotemporal dementia needs to have a lot of patience. The first step is to educate yourself. Learn the symptoms of disease and how to cope with them. Spread the awareness and discuss with all the people around the expected outcomes of the disease. Providing care to a person with frontotemporal dementia is not easy. Always be ready to ask for help.

Frontotemporal dementia complications:

A patient with frontotemporal dementia can survive for quite a long period of time as this condition is not fatal. Certain complications may confound this disease. The problem arises when an individual gets involved in dangerous activities and often ends up hurting himself/herself. Not being able to take good care of yourself is another reason for increased mortality in patients with frontotemporal dementia. These patients require good nursing care and assisted living facility.

At the end-stage of disease, frequent falls and infections are very common. The most common cause of death in a patient with frontotemporal dementia is pneumonia.

Frequently Asked Questions (FAQs)

What are the signs of frontotemporal dementia?

The signs of frontotemporal dementia can be increased sweating, deterioration in personal hygienic habits, impaired judgment, decreased self-awareness, and apathy.

How long do you live with frontotemporal dementia?

People with frontotemporal dementia usually live for about 5 to 8 years.

How quickly does frontotemporal dementia progress?

Frontotemporal dementia may progress rapidly to death in a period of just eight years!

What are the stages of frontotemporal dementia?

Frontotemporal dementia occurs in three different stages:

Early-stage frontotemporal dementia:

The early-stage causes the patient to show some extreme behavioral changes. The patient becomes selfish, callous, and irresponsible. The impulsive behavior becomes prominent. The patient develops an unusual craving for sweet food and often overeats. In the semantic variant, language is affected first.

Mid-stage frontotemporal dementia:

At this stage, the patient may show some additional symptoms. For instance, a person with a behavioral variant may develop language symptoms.

Late-stage frontotemporal dementia:

The symptoms here mimic Alzheimer’s disease. Memory loss may also become noticeable at this stage.

Can you drive with frontotemporal dementia?

In the course of early disease, a patient with frontotemporal dementia can drive, while during the later stages, driving becomes restricted.

Can you inherit frontotemporal dementia?

Sometimes frontotemporal dementia runs in genes and can be inherited.

Can you prevent frontotemporal dementia?

Certain lifestyle modifications such as playing brain strengthening games, taking good sleep, and exercising regularly can decrease the risk of frontotemporal dementia.

What is the prevalence of frontotemporal dementia?

Frontotemporal dementia is regarded as a common cause of dementia in young adults with a prevalence of about 15-22 individuals per 100,000 people.

What is the prognosis for frontotemporal dementia?

The prognosis for frontotemporal dementia is not good. The disease usually progresses in a steady manner ranging from about 2 years in some patients to 10 years in others. Many of the patients with frontotemporal dementia need 24-hour assistance and home care.

Who discovered frontotemporal dementia?

Frontotemporal dementia was first discovered by Arnold pick in 1892 in a patient in whom he noticed some unusual symptoms related to language.

Will someone with frontotemporal dementia have any vascular problems?

Yes, frontotemporal dementia may sometimes cause vascular issues.