Frontotemporal dementia

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Martina Feichter studied biology with an elective subject pharmacy in Innsbruck and also immersed herself in the world of medicinal plants. From there it was not far to other medical topics that still captivate her to this day. She trained as a journalist at the Axel Springer Academy in Hamburg and has been working for since 2007 - first as an editor and since 2012 as a freelance writer.

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Frontotemporal dementia (FTD) is a rarer form of dementia. It used to be called Pick's disease or Pick's disease. In contrast to other forms of dementia, the memory is largely preserved here. Instead, FTD patients show abnormal and anti-social behavior. Only in the further course does frontotemporal dementia resemble Alzheimer's dementia. Read more about frontotemporal dementia here: causes, symptoms, diagnosis and therapy!

ICD codes for this disease: ICD codes are internationally recognized codes for medical diagnoses. They can be found, for example, in doctor's letters or on certificates of incapacity for work. G31

Frontotemporal dementia: description

The term frontotemporal dementia (FTD) is used colloquially for a group of diseases whose main symptoms are changes in personality, social behavior and language skills. The technical term for this group of diseases is actually Frontotemporal Lobar Degenerations (FTLD). Strictly speaking, the FTD is only a sub-form of the FTLD. There are three such sub-forms in total:

1. Frontotemporal Dementia (FTD)
2. Semantic dementia
3. Progressive non-fluid aphasia

In this article, too, the term frontotemporal dementia is used in simplified form for frontotemporal lobar degenerations.

Frontotemporal dementia: frequency

Frontotemporal dementia is a rare form of dementia. It is estimated that it accounts for three to nine percent of all dementias. The disease breaks out on average between the ages of 50 and 60; However, the range of the onset age is very wide: it is 20 to 85 years. Studies have shown that frontotemporal dementia is equally common in men and women.

Frontotemporal dementia: symptoms

In frontotemporal dementia, nerve cells in the frontal and temporal lobes of the brain perish. The symptoms depend, among other things, on exactly in which part of the cerebrum the nerve cells die.

Frontotemporal dementia in the narrower sense

In the sub-form of frontotemporal dementia, it is primarily the patient's personality and interpersonal behavior that change. The main symptoms here are:

• superficial, carefree, unfocused and thoughtless behavior
• Neglect of duties
• decreasing interest in one's own family and hobbies
• Apathy, listlessness and apathy
• sometimes increasing indifference to others, irritability and aggressiveness
• often violation of social norms
• sometimes strange rituals and repetitive behaviors
• Cravings (especially for sweets) and a great preference for certain foods
• Neglect of physical hygiene
• hardly any insight into the disease (that is, the patients consider themselves healthy)

In the further course, frontotemporal dementia leads to speech disorders (up to complete silence) and memory disorders. For a long time, however, the latter are not as severe as with Alzheimer's.

Patients find it increasingly difficult to cope with everyday life. Incontinence and sometimes neurological symptoms such as Parkinson's disease (gait disorder, movement disorders) and swallowing disorder (dysphagia) occur.

In the final stage, frontotemporal dementia leads to a complete need for care.

Semantic dementia and progressive non-liquid aphasia

In the case of the other two sub-forms of FTLD, disorders of language and language comprehension are in the foreground. However, the personality and behavior of the patient can also change here.

People who suffer from semantic dementia are increasingly forgetting the meaning of words: their vocabulary is decreasing, but those affected can still speak fluently and grammatically correctly. Familiar faces are no longer recognized later. Usually the personality and behavior of the patient also change.

The progressive non-liquid aphasia is characterized by pronounced word-finding disorders. Patients have difficulty speaking and often make grammar or pronunciation mistakes. On the other hand, brain functions such as memory, thinking skills, orientation and functionality are retained for a long time in everyday life.

Frontotemporal dementia: causes and risk factors

Frontotemporal dementia is characterized by the loss of nerve cells in the frontal lobes and in the two temporal lobes. In most cases it is not exactly known how this happens. In around ten percent of patients, however, frontotemporal dementia is triggered by changes (mutations) in certain genes. Then several blood relatives can show similar diseases.

Apart from such mutations, no risk factors for frontotemporal dementia are known to date.

Frontotemporal dementia: diagnosis

Frontotemporal dementia is not easy to diagnose. If it initially manifests itself primarily through changes in personality and behavior, it is often confused with mental disorders such as depression, burnout, schizophrenia, or mania. At an advanced stage, FTD can hardly be distinguished from Alzheimer's dementia.

As is generally the case with suspected dementia, the diagnosis is based primarily on taking the medical history (anamnesis), a physical examination and psychological tests (dementia tests). In addition, if possible, the doctor has relatives describe the changes in the patient's personality and behavior.

Frontotemporal dementia causes the brain tissue in the frontal and temporal lobes to shrink (through the death of nerve cells). This shrinkage (atrophy) can be demonstrated by imaging procedures. Computed tomography (CT) and magnetic resonance tomography (magnetic resonance tomography, MRT) are used.

Due to the cell death, the energy consumption (sugar consumption) in the frontal and temporal lobes decreases in the course of the disease. This changed metabolic activity can be detected with positron emission tomography (PET).

There are no special laboratory tests with which frontotemporal dementia can be reliably detected. However, a sample of the cerebral spinal cord fluid (liquor) can be taken (lumbar puncture) and proteins such as total tau or beta-amyloid can be determined. This can sometimes help to distinguish frontotemporal dementia from Alzheimer's dementia - in Alzheimer's disease, the liquor concentration of these proteins is changed in a characteristic way.

If there are already blood relatives with FTD, genetic testing can reveal a congenital cause.

Frontotemporal dementia: treatment

There are no drugs that have been shown to help the symptoms of frontotemporal dementia. Some patients benefit from so-called serotonergic antidepressants (such as citalopram, sertraline): These active ingredients have a drive-increasing effect. If frontotemporal dementia is accompanied by severe restlessness and aggressiveness, neuroleptics such as melperon, quetiapine or olanzapine can be tried.

Non-drug treatment of FTD

Non-drug measures are very important in the treatment of frontotemporal dementia. In each individual case, it is decided which measures are most sensible for a patient. For example, sport can help against increased aggressiveness. If frontotemporal dementia is accompanied by withdrawal and apathy, music, dance and art therapy are particularly recommended.

Frontotemporal dementia: course and prognosis

Frontotemporal dementia has not yet been cured. After the onset of symptoms, it takes an average of eight years for patients to die. However, frontotemporal dementia can also progress unusually quickly (duration of the disease approx. Two years) or very slowly (approx. 15 years).

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