The Frontotemporal dementia Is the most frequent early dementia. It usually begins between 40 and 50 years and produces great changes, progressively, in the personality, affection and behavior of the people who suffer from it.
The exact cause of frontotemporal dementia is not yet known, although it appears that the genetic component plays an important role. Current treatments focus on treating behavioral symptoms and helping the person manage the disease in the best possible way.
In atypical cases, this disease can occur after a midlife crisis, depression, an outbreak of schizophrenia Or a Traumatic brain injury .
Early diagnosis of this disease is essential, because the sooner it is detected, the earlier the treatment will begin and the better prepared the relatives and caregivers will be to care for the person who suffers from the disease when necessary.
Characteristics of frontotemporal dementia
The term dementia encompasses a number of neurodegenerative diseases. These cause cognitive deficits, which can be from mild to moderate, and that significantly affect the daily functioning of the person.
The most prevalent dementia is the Alzheimer disease , Followed by the Dementia by Lewy bodies And the frontotemporal. The latter occurs in approximately 10% of diagnosed cases of dementia and is the most common early dementia (Onyike & Diehl-Schmid, 2013; Association for Frontotemporal Degeneration, 2011).
Frontemporal dementia is a category of diseases that includes those dementias that occur with an atrophy in the frontal and temporal lobes. There are two types of clinical presentation of this dementia: the behavioral variant and the language variant.
The most common variation is behavior, with a prevalence of 60% of the cases diagnosed with frontotemporal dementia. It is characterized by behavioral, emotional and the personality , Mainly due to deterioration of the frontal lobe.
Variation in language would include typical symptoms of aphasia Primary progression, such as deficits in the production of language and inability to understand the meaning of some words.
Differences between frontotemporal dementia and Alzheimer's disease
Frontotemporal dementia is often confused with Alzheimer's disease because of its symptoms or with schizophrenia or Bipolar disorder By the time of onset of the disease (between 40 and 50 years) (Rascovsky & Hodges, 2011).
Alzheimer's disease and frontotemporal dementia differ in that the major deficits of the former are in the memory And language, while those of the second have more to do with affection, personality and social behavior. Sometimes they may also have memory gaps in the early stages of the disease.
The main symptoms of frontotemporal dementia include apathy , Antisocial behavior, loss of inhibition and lack of insight or self-awareness (Onyike & Diehl-Schmid, 2013).
The life expectancy of people who have frontotemporal dementia is 6.6 to 9 years from the onset of the disease. However, due to the delay in the diagnosis, when these people are diagnosed, they only have about 3 or 4 years to live, therefore, it becomes evident the need to expedite the diagnosis of dementias of this type (Knopman & Roberts, 2011).
symptom
To diagnose a person with frontotemporal dementia, according to DSM-5 (Diagnostic and Statistical Manual), the person must meet the criteria for a major or minor neurocognitive disorder.
In addition, the disease must appear in an insidious manner and have a gradual progression and must meet the criteria for at least one of the two variants, behavior or language.
Diagnostic Criteria for Major or Minor Neurocognitive Disorders:
- Evidence of significant cognitive decline compared to the previous level of performance in one or more cognitive domains (complex attention, executive function, learning and memory, language, motor perceptual ability or social cognition) based on:
- Concern about the individual himself, an informant who knows him or the clinician, because there has been a significant decline in cognitive function, and
- A substantial impairment of cognitive performance, preferably documented by a standardized neuropsychological test or, failing this, by another quantitative clinical evaluation.
- Cognitive deficits interfere with the individual's autonomy in everyday activities (that is, at least he needs assistance with complex instrumental activities of daily living, such as paying bills or complying with treatments).
- Cognitive deficits do not occur exclusively in the context of delirium.
- Cognitive deficits are not best explained by another mental disorder (eg, major depressive disorder, schizophrenia).
Specify If because of:
Alzheimer disease
Frontotemporal lobe degeneration
Lewy Body Disease
Vascular disease
Traumatic brain injury
Consumption of substance or medication
HIV infection
Prion disease
Parkinson's Disease
Huntington's disease
Other medical condition
Multiple etiologies
Not specified
Specify
No change in behavior: If the cognitive disorder is not accompanied by any clinically significant behavioral alterations.
With Behavioral change (Specify alteration): If the cognitive disorder is accompanied by a change in clinically significant behavior (eg, psychotic symptoms, mood disturbance, agitation, apathy or other behavioral symptoms).
Specify The current gravity:
Mild: Difficulties with everyday instrumental activities (eg, household chores, money management).
Moderate : Difficulties with basic daily activities (eg, eating, dressing).
Serious : Totally dependent.
Behavior variation
This variant of dementia has an almost imperceptible beginning and is gradually causing behavioral and / or cognitive impairments.
For a person to be diagnosed behavioral variation must present at least 3 of the following 5 symptoms during most days and also must have a prominent decline in social cognition or executive abilities.
The symptoms are:
- Disinhibition of behavior . The person presents an inappropriate social behavior, loss of manners and performs impulsive acts. Some examples of such behaviors might be: kissing or grabbing strangers, inappropriate sexual acts or propositions, urinating in public, saying unkind words, not respecting each other's space, lack of hygiene...
- Apathy or Inertia . Lack of interest, motivation, initiation or maintenance of any activity that previously satisfied. Relatives may notice a lack of interest in their own appearance, grooming, and daily life activities.
- Loss of sympathy and / or empathy . Your family members and caregivers may notice a lack of interest in others, loss of eye contact and affection, and indifference to the feelings of others, for example, by giving them hurtful talk.
- Conservative, stereotyped or compulsive and ritualistic behavior . Patients present repetitive gestures such as throbbing or rubbing. They may also have more complex behaviors that resemble those suffered by people with an obsessive-compulsive disorder, such as over-cleaning, counting repeatedly, completing a task over and over, or reading the same book several times in a row. As for verbal behavior, repetitions can be observed, for example, always asking the same question.
- Hypermorality and Dietary Changes . The impulsiveness It is also reflected in behaviors such as carrying in the mouth inedible objects or the continuous cravings of foods with carbohydrates and sugars. Also, it is easy for them to lose control with food, alcohol and / or tobacco.
Language variant
The most frequent subtype of this variant is progressive primary aphasia. This type of aphasia implies a progressive deterioration of the language with a subtle, almost imperceptible beginning. The person begins to have difficulty naming objects and people.
As the disease progresses problems begin to appear in reading and writing, the person can talk less and less until it ends up making it almost impossible to communicate.
The alterations of the language are manifested through lack of production of the language, deficits in the denomination of objects, problems of grammar and understanding of some words
Motor problems
Apart from these two variants, some types of frontotemporal dementias are also characterized by the presence of motor problems similar to those of Parkinson's disease wave Amyotrophic Lateral Sclerosis .
The symptoms that can be observed are tremors, stiffness, muscle spasms, poor coordination, difficulty swallowing, muscle weakness...
Prevalence
According to the American Psychiatric Association (APA), frontotemporal dementia has a global prevalence of approximately 2 to 10 cases per 100,000 people. Between 20 and 25% of cases occur in people over 65 years of age.
The behavioral variant, with behavioral problems and deficits in semantics, is more common in men, whereas the aphasic variant with problems related to language fluency occurs more in women (APA, 2015).
Risk factor's
The main risk factor is a family history of frontotemporal dementia, as 40% of patients with this type of dementia have a family history.
In 10% of the cases, a pattern of Autosomal dominant inheritance . Half of our genetic material is from our father and the other half is from our mother. Therefore, if one of our parents has this gene and passes it to us, we will present the disease, which would not happen if the gene were recessive.
A number of genetic factors are being investigated, such as gene mutations encoding protein Tau (MAFT), related to microtubules and the structure of neurons, in the granulin gene (GRN) and in the gene C90RF72 (APA, 2015).
Diagnostic tests
In order to establish a clear diagnosis, a series of tests must be carried out, as well as a history of the patient and a physical examination. The basic tests are the analysis of the thyroid And blood to rule out other possible causes of symptoms such as Hyperthyroidism wave anemia .
A neuropsychological evaluation is carried out to check the cognitive and functional status of the patient. The most common tests are the Mini-Mental (MMSE) and the Mini-Cog.
Finally, neuroimaging tests are also available to buy if there are lesions or physical reasons that can cause the symptoms, such as tumors. The usual neuroimaging tests are the Functional magnetic resonance imaging (MRI), the Computed tomography and the Positron emission tomography (PET).
Treatment
There is as yet no treatment specifically approved for curing frontotemporal dementia. There are treatments to alleviate the symptoms, but not to cure or slow the course of the disease.
To improve behavioral, cognitive and motor symptoms, drugs are administered including stimulants as agonists of the NMDA receptors , Acetylcholinesterase inhibitors and Antidepressants As selective serotonin reuptake inhibitors.
Recent studies have indicated that there is a significant improvement in behavioral symptoms with antidepressants Trazodone HCL and SSRIs (Selective serotonin reuptake inhibitors such as floxetine, paroxetine, fluvoxamine and sertraline (Seltman & Matthews, 2012; Nardell & Tampi, 2014; Portugal Mda, Marinho, & Laks, 2011).
This medication also helped with food problems and decreased the cravings frequently associated with hyper-morality (Nardell & Tampi, 2014).
Stimulants such as Dextroamphetamine and the Methylphenidate Have been shown to be effective in improving cognitive problems, such as Problem resolution , Lack of apathy and disinhibition (Nardell & Tampi, 2014; Portugal Mda, Marinho, & Laks, 2011).
The benefits of other non-pharmacological treatments have also been demonstrated in the improvement of emotional, mental and physical symptoms, such as Cognitive stimulation Through the performance of cognitive exercises routinely (Portugal Mda, Marinho, & Laks, 2011).
References
- APA. (2015). Diagnostic and Statistical Manual of Mental Disorders DSM-5. Washington: APA.
- Association for Frontotemporal Degeneration. (2011). Fast facts about frontotemporal degeneration . Retrieved on June 10, 2016, from Association for Frontotemporal Degeneration.
- Knopman, D., & Roberts, R. (2011). Estimating the number of persons with frontotemporal lobar degeneration in the US population. J Mol Neurosci, 45 , 330-335.
- Nardell, M., & Tampi, R. (2014). Pharmacological treatments for frontotemporal dementias: a systematic review of randomized controlled trials. Am J Alzheimers Dis Other Demen, 29 (2), 123-132.
- Onyike, C., & Diehl-Schmid, J. (2013). The epidemiology of frontotemporal dementia. Int Rev Psychiatry, 25 (2), 130-137.
- Portugal Mda, G., Marinho, V., & Laks, J. (2011). Pharmacological treatment of frontotemporal lobar degeneration: systematic review. Rev Bras Psiquiatr, 33 (1), 81-90.
- Rascovsky, K., & Hodges, K. K. (2011). Sensitivity of revised diagnostic criteria for the behavioral variant of frontotemporal dementia. Brain, 134 , 2456-2477.
- Seltman, R., & Matthews, B. (2012). Frontotemporal lobar degeneration: epidemiology, pathology, diagnosis and management. CNS Drugs, 26 (10), 841-870.
- Wilfong, L., Edwards, N.E., Yehle, K.S., & Ross, K. (2016). Frontotemporal Dementia: Identification and Management. The Journal for Nurse Practitioners, 12 (4), 277-282.