{"id":16927,"date":"2022-05-10T14:50:14","date_gmt":"2022-05-10T12:50:14","guid":{"rendered":"https:\/\/neurologiaclinica.es\/neurological-diseases-and-disorders\/frontotemporal-degeneration\/"},"modified":"2025-04-28T12:27:08","modified_gmt":"2025-04-28T10:27:08","slug":"frontotemporal-degeneration","status":"publish","type":"page","link":"https:\/\/neurologiaclinica.es\/en\/neurological-diseases-and-disorders\/frontotemporal-degeneration\/","title":{"rendered":"Frontotemporal degeneration"},"content":{"rendered":"\t\t
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Frontotemporal degeneration<\/h1>\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t<\/section>\n\t\t\t\t
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What is frontotemporal degeneration?<\/h2>

Frontotemporal degeneration is a group of neurodegenerative diseases<\/strong> characterized by progressive neuronal loss<\/strong> in the anterior regions of the brain (frontal and temporal lobes).<\/p>

Microscopic and molecular analysis of brain tissue shows several types of alterations:<\/p>

– Deposits of tau protein, similar but not identical to those seen in Alzheimer’s disease,<\/strong> are found in half of the cases.<\/p>

– In the remaining cases, deposits of other proteins, such as TDP-43 and neurofilament-associated proteins,<\/strong> are detected .<\/strong><\/p>

What is the frequency of frontotemporal degeneration?<\/h2>

Frontotemporal degeneration causes about 10% of dementia cases. In relative terms, it is more frequent in cases of early onset (<65 years) and of genetic origin. <\/p>

What are the main causes of frontotemporal degeneration?<\/h2>

Most cases have no apparent cause (sporadic forms). Up to 40% of cases in some series may have a genetic cause, dependent on mutations in several genes (MAPT, PGRN, VCP, CHMP2B…). Genetic forms are usually inherited in an autosomal dominant pattern, but the age of onset and clinical manifestations can vary widely, even among relatives carrying the same mutation. <\/p>

What are the symptoms of frontotemporal degeneration?<\/h2>

Three classic clinical pictures<\/strong> have been described:<\/p>

– Frontal dementia<\/strong> is characterized by behavioral disturbances (apathy, disinhibition, loss of awareness of the disease) and cognitive disturbances of the dis-executive type (problems in planning and sequencing tasks, abstraction deficits). In these cases brain atrophy predominates in the anterior and medial regions of the frontal lobes. <\/p>

– Semantic dementia<\/strong> is characterized by fluent language, even verbose, but with little content. Patients progressively lose the notion of the meanings of words, starting at the most general levels. Brain atrophy in these patients is more intense in the left temporal lobe. <\/p>

– Primary progressive aphasia<\/strong> presents with slow and slurred speech. In these cases a marked cerebral atrophy is observed in the left perisylvian region. <\/p>

In addition to these classic pictures, other forms of presentation have been described in recent years, such as primary progressive prosopagnosia, in which face recognition is altered early, and logopenic aphasia.<\/p>

DIAGNOSIS<\/h2>

How is frontotemporal degeneration diagnosed?<\/h2>

The diagnosis of frontotemporal degeneration is established with reasonable certainty in patients who present with a compatible clinical picture and a pattern of brain atrophy or hypometabolism of frontal or temporal predominance and do not present with the findings of a number of processes that can produce similar symptoms. Fluorodeoxyglucose (FDG) positron emission tomography (PET) is particularly useful in these cases, as it can show a characteristic pattern of hypometabolism even before signs of atrophy are apparent. However, other factors that can produce similar patterns, such as sedative drugs or severe depression, must be taken into account when interpreting FDG PET. <\/p>

As in Alzheimer’s disease and other neurodegenerative diseases, definitive diagnosis would require analysis of brain tissue, but at present the risks of a brain biopsy versus the benefits obtained mean that it is not indicated except in exceptional situations.<\/p><\/div><\/div>

TREATMENT<\/h2>

What is the treatment of frontotemporal degeneration?<\/h2>

At present we do not have treatments capable of modifying the course of the disease, but we can use various symptomatic treatments.<\/p>

– First of all, in these cases it is advisable to seek early legal advice to protect the patient and his or her estate. Many patients with frontotemporal degeneration are still active at the onset of the disease, so they are at risk of making serious errors at work and in their personal lives. The problem is compounded by the fact that they are often unaware of their cognitive problems and may engage in disinhibited behavior. <\/p>

– Secondly, they should maintain a healthy lifestyle regimen, including a Mediterranean diet, regular physical exercise, regular monitoring of vascular risk factors and cognitive stimulation exercises under the guidance of a neuropsychologist.<\/p>

– Thirdly, it is important that the relatives and caregivers of these patients receive adequate training and the necessary support from the healthcare personnel involved (neurologists, psychiatrists, neuropsychologists, social workers, physiotherapists, occupational therapists, speech therapists, etc.).<\/p>

– Fourth, serotonin reuptake inhibitor drugs or neuroleptics may be used to improve the affective and behavioral symptoms of<\/p>

patients. Specific treatments for Alzheimer’s disease are often not effective in these cases, and may even worsen their behavior. <\/p><\/div><\/div>

FREQUENT QUESTIONS<\/h2>

How can I access research studies on frontotemporal degeneration?<\/h2>

Information about clinical trials and other research studies on this pathology can be obtained through various websites(clinicaltrials<\/a>, Medlineplus<\/a>) and scientific publications<\/a>.<\/p>

AUTHOR AND DATE OF ISSUE<\/strong><\/p>

Dr. ADOLFO JIM\u00c9NEZ HUETE<\/strong><\/p>

19\/05\/2020<\/strong><\/p><\/div><\/div>\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t

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What is frontotemporal degeneration? Frontotemporal degeneration is a group of neurodegenerative diseases characterized by progressive neuronal loss in the anterior regions of the brain (frontal and temporal lobes). Microscopic and molecular analysis of brain tissue shows several types of alterations: – Deposits of tau protein, similar but not identical to those seen in Alzheimer’s disease, […]<\/p>\n","protected":false},"author":1,"featured_media":0,"parent":16593,"menu_order":0,"comment_status":"closed","ping_status":"closed","template":"","meta":{"footnotes":""},"class_list":["post-16927","page","type-page","status-publish","hentry"],"_links":{"self":[{"href":"https:\/\/neurologiaclinica.es\/en\/wp-json\/wp\/v2\/pages\/16927","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/neurologiaclinica.es\/en\/wp-json\/wp\/v2\/pages"}],"about":[{"href":"https:\/\/neurologiaclinica.es\/en\/wp-json\/wp\/v2\/types\/page"}],"author":[{"embeddable":true,"href":"https:\/\/neurologiaclinica.es\/en\/wp-json\/wp\/v2\/users\/1"}],"replies":[{"embeddable":true,"href":"https:\/\/neurologiaclinica.es\/en\/wp-json\/wp\/v2\/comments?post=16927"}],"version-history":[{"count":4,"href":"https:\/\/neurologiaclinica.es\/en\/wp-json\/wp\/v2\/pages\/16927\/revisions"}],"predecessor-version":[{"id":17917,"href":"https:\/\/neurologiaclinica.es\/en\/wp-json\/wp\/v2\/pages\/16927\/revisions\/17917"}],"up":[{"embeddable":true,"href":"https:\/\/neurologiaclinica.es\/en\/wp-json\/wp\/v2\/pages\/16593"}],"wp:attachment":[{"href":"https:\/\/neurologiaclinica.es\/en\/wp-json\/wp\/v2\/media?parent=16927"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}