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Fronto-temporal dementia is a specific form of dementia in which symptoms revolve around language disorders and disorders of patient behavior. Fortunately, frontotemporal dementia is quite a rare disease - the word "happily" is used here because modern medicine, apart from relieving treatments, is unable to offer patients causal treatment for frontotemporal dementia.

Fronto-temporal dementia , along with such diseases as Alzheimer's disease and Lewy body dementia, belongs to the group of degenerative dementias. The symptoms of frontotemporal dementia typically appear in people between the ages of 50 and 65. However, this is not the only age range at which frontotemporal dementia may develop. These are rather isolated cases, however, the development of this disease has been observed even in 20-year-olds.

Fronto-temporal dementia: causes

Frontotemporal dementia belongs to the so-called tauopathy. In the course of the individual, there is an excessive accumulation of tau proteins in the brain of patients, which is why the described dementia is included in the group of neurodegenerative diseases. The name "frontotemporal dementia" comes from the parts of the brain where pathological proteins accumulate - in this case the locations of the deposits are the frontal and temporal lobes of the brain.

Proteins accumulating in the brain have many negative effects on the nervous tissue. Their presence can result in both disturbances in the transmission of signals between neurons and increased death of nerve cells. Thus, in all likelihood, the mechanism behind the symptoms in frontotemporal dementia is known, but it is often not known why protein actually accumulates in the brain. In some patients (up to 50% of them), frontotemporal dementia has a family history. Scientists have even been able to detect some of the mutations that lead to frontotemporal dementia. As an example of genetic disorders that result in the development of the described neurodegenerative disease, one can mention mutations of the MAP-tau genes or the genes coding for TDP-43 proteins. Fronto-temporal dementia can certainly still behowever, it is considered a rather enigmatic disease - after all, in as many as half of the patients suffering from it, it is not possible to determine what caused the onset of the disease.

Frontotemporal Dementia: Symptoms and Types

Fronto-temporal dementia is quite a specific form of dementia, because in its course the main problem of patients is not memory impairment. The clinical picture of the degenerative processes involved in the frontal and temporal lobes of the brain is exceptionally diverse, which distinguishes three main forms of disorders from the spectrum of frontotemporal degeneration. In the course of typical frontotemporal dementia, patients developbehavioral disordersandmental disorders . They consist, for example, in neglecting personal hygiene, excessive psychomotor activity and the loss of the ability to maintain interpersonal contacts. Patients with frontotemporal dementia may present provocative sexual behaviors, and may also develop various compulsions (e.g. smoking large amounts or drinking alcohol). Patients may experience difficulty concentrating attention as well as excessive impulsiveness. In the case of mental disorders,affective disorders(mainly in the form of depression) andanxiety disordersare dominant in patients with frontotemporal dementia. The second, less frequent a subtype of dementia included in the spectrum of disorders from the group of frontotemporal dementias isprimary progressive aphasia . In her case, the main problem is the patients' speech disorders. Patients may often stutter while speaking, and the ability to freely form statements is clearly difficult for them. In the course of primary progressive aphasia, agramaticisms can also appear in the speech of sick people, and they can also distort words. Speech understanding is usually preserved in such patients, but problems may arise when trying to understand more difficult, more complicated statements. A third form of disorders associated with degenerative processes in the temporal and frontal lobes of the brain issemantic dementia. In its course, symptoms of two main groups occur: the first symptoms relate to speaking, while others are related to agnosia. Patients with semantic dementia can even talk a lot, but it is sometimes difficult to understand the meaning of what they say. This is because patients have a tendency to overuse uninformative words (eg "this", "that thing", "this"). Patients' speech may include the so-called paraphases - the sick name it wronglyobjects, eg instead of talking about the hand-held object "spoon", they describe it as "knife". In addition to the problems already mentioned, patients also develop agnosia. It can take the form of prosopagnosia (impossibility to recognize faces) or difficulties in the proper identification of objects - for this reason, patients may misuse certain devices, e.g. brushing themselves with a fork.

Worth knowing

Dementia is a group of diseases in which various cognitive functions are disturbed mainly. Diseases of this type are grouped, inter alia, according to their causes - hence the division into degenerative and vascular dementia.

Fronto-temporal dementia: recognizing

The diagnosis of frontotemporal dementia is made primarily on the basis of the symptoms presented by the patient. Neuroimaging is also used in the diagnosis of the disease - for example, during brain magnetic resonance imaging, it is possible to find atrophies within the frontal and temporal lobes. It is also possible to perform imaging functional tests in patients. In the case of frontotemporal dementia, for example, a reduction in blood flow in the temporal and frontal lobes or a reduction in glucose uptake in these regions of the brain can be seen. Imaging diagnostics is also important because it allows you to exclude other possible causes of the patient's symptoms (such as a stroke or a tumor of the central nervous system).

Frontotemporal dementia: prognosis

Fronto-temporal dementia is progressive - symptoms that occur from the beginning of the disease gradually increase in intensity, in addition, patients develop new, completely different ailments. It may be, inter alia, to disturbances of motor functions in the form of tremors or stiffness. Patients may present with deliberative symptoms, e.g. the Marinesco-Radovici symptom. Problems such as urinary or faecal incontinence can develop fairly quickly. As for memory impairment, it is rare in frontotemporal dementia, and even when it does occur, it is mild. it is influenced by many different factors, ranging from the age of onset to the general he alth condition of the patient. The median survival time for frontotemporal dementia is 8 years from onset.

Frontotemporal Dementia: Treatment

There is no causal treatmentfrontotemporal dementia - this means that the disease progression cannot be slowed down. In order to alleviate the symptoms associated with behavioral disorders, attempts are made to use psychotropic drugs from the group of serotonin or norepinephrine reuptake inhibitors. Drugs that are used to improve the cognitive function of patients with other forms of dementia - that is, acetylcholinesterase inhibitors - in frontotemporal dementia are unfortunately ineffective.

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Neurology