Demencja czołowo-skroniowa – Etiologia i przyczyny

Demencja czołowo-skroniowa
Etiologia i przyczyny

Demencja czołowo-skroniowa (FTD) to grupa neurodegeneracyjnych zaburzeń charakteryzujących się postępującą atrofią płatów czołowych i skroniowych, prowadzącą do zmian w zachowaniu, osobowości oraz funkcjach językowych. Patologicznie FTD wiąże się z nieprawidłowym gromadzeniem białek tau (około 50% przypadków), TDP-43 (około 50%) oraz FUS (około 5%). W niektórych wariantach, np. chorobie Picka, obecne są ciała Picka zawierające patologiczne formy tau. Genetyka odgrywa istotną rolę – 30-40% pacjentów ma historię rodzinną, a 10-30% przypadków ma potwierdzoną przyczynę genetyczną, z dominującym autosomalnie dziedziczonym wzorcem. Kluczowe mutacje dotyczą genów C9orf72 (najczęstsza przyczyna rodzinnej FTD, powiązana także z ALS), MAPT (białko tau) oraz GRN (progranulina). Rzadziej występują mutacje w TBK1, TARDBP, VCP i CHMP2B. Współwystępowanie FTD i ALS wskazuje na wspólne mechanizmy patogenetyczne, szczególnie u pacjentów z mutacją C9orf72.

Etiologia demencji czołowo-skroniowej

Nieprawidłowe białka w FTD
Czynniki genetyczne
FTD a stwardnienie zanikowe boczne
Czynniki ryzyka środowiskowe
Mechanizm uszkodzenia mózgu w FTD

Podsumowanie etiologii FTD

Kolejne rozdziały

Etiologia demencji czołowo-skroniowej

Demencja czołowo-skroniowa (FTD) to grupa zaburzeń neurodegeneracyjnych wynikających z uszkodzenia neuronów głównie w płatach czołowych i skroniowych mózgu. Charakteryzuje się ona postępującą atrofią tych regionów, co prowadzi do zmian w zachowaniu, osobowości i zdolnościach językowych pacjenta.¹² W przeciwieństwie do choroby Alzheimera, FTD zazwyczaj pojawia się w młodszym wieku, najczęściej między 40. a 65. rokiem życia, choć może wystąpić również później.³⁴

Nieprawidłowe białka w FTD

Główną cechą patologiczną demencji czołowo-skroniowej jest nieprawidłowe gromadzenie się określonych białek w komórkach nerwowych płatów czołowych i skroniowych. Badacze zidentyfikowali kilka kluczowych białek związanych z rozwojem FTD:⁵⁶

Białko tau – w około 50% przypadków FTD obserwuje się nieprawidłowe formy tego białka, które tworzą splątki wewnątrz neuronów, prowadząc ostatecznie do ich zniszczenia⁷⁸
Białko TDP-43 (TAR DNA-binding protein 43) – występuje w około 50% przypadków FTD; jego nieprawidłowe gromadzenie się prowadzi do dysfunkcji i ostatecznie śmierci komórek nerwowych⁹¹⁰
Białka FUS (FET proteins) – odpowiadają za około 5% przypadków FTD¹¹¹²

W niektórych przypadkach FTD, szczególnie tych związanych z chorobą Picka, obserwuje się charakterystyczne struktury zwane ciałami Picka, które zawierają nieprawidłowe formy białka tau.¹³ Te nieprawidłowe białka gromadzą się w neuronach, uszkadzając je i zakłócając ich normalne funkcjonowanie, co prowadzi do postępującego niszczenia komórek mózgowych i charakterystycznych objawów klinicznych FTD.¹⁴

Czynniki genetyczne

Genetyka odgrywa znaczącą rolę w etiologii demencji czołowo-skroniowej. W przeciwieństwie do innych form demencji, FTD wykazuje silniejszy komponent genetyczny. Dane pokazują, że:¹⁵¹⁶

Około 30-40% osób z FTD ma historię rodzinną tego zaburzenia¹⁷¹⁸
Około 10-30% przypadków FTD ma ściśle określoną przyczynę genetyczną¹⁹²⁰
W przypadkach rodzinnych, FTD jest zazwyczaj dziedziczona w sposób autosomalny dominujący, co oznacza, że dziecko rodzica z mutacją genetyczną ma 50% szans na odziedziczenie tej mutacji²¹²²

Naukowcy zidentyfikowali kilka genów, których mutacje mogą prowadzić do rozwoju FTD:²³²⁴

Główne geny związane z FTD

C9orf72 – najczęstsza genetyczna przyczyna rodzinnej FTD; mutacja w tym genie jest również związana z stwardnieniem zanikowym bocznym (ALS). Powtórzenie sekwencji GGGGCC w tym genie prowadzi do produkcji toksycznych białek²⁵²⁶
MAPT (gen białka tau) – mutacje prowadzą do nieprawidłowości w białku tau, zwiększając jego tendencję do tworzenia toksycznych agregatów; związany głównie z behawioralnym wariantem FTD²⁷²⁸
GRN (gen progranulin) – mutacje prowadzą do zmniejszonej produkcji białka progranulin, co z kolei powoduje nieprawidłowe funkcjonowanie białka TDP-43²⁹³⁰

Rzadziej występujące geny związane z FTD obejmują TANK-binding kinase 1 (TBK1), transactive response DNA binding protein (TARDBP), valosin-containing protein (VCP) i charged multivesicular body protein 2B (CHMP2B).³¹³²

FTD a stwardnienie zanikowe boczne

Istnieje znaczące powiązanie między demencją czołowo-skroniową a stwardnieniem zanikowym bocznym (ALS, choroba Lou Gehriga). Badania genetyczne potwierdziły, że niektóre mutacje genowe, szczególnie w genie C9orf72, są wspólne dla obu tych chorób.³³³⁴

Niektóre osoby z FTD rozwijają objawy ALS w miarę postępu choroby, szczególnie pacjenci z behawioralnym wariantem FTD i ci, u których choroba jest spowodowana pojedynczą mutacją genetyczną.³⁵ Związek między tymi dwiema chorobami sugeruje wspólny mechanizm patogenetyczny, który obecnie jest przedmiotem intensywnych badań.³⁶³⁷

Czynniki ryzyka środowiskowe

Oprócz czynników genetycznych, badacze zidentyfikowali kilka potencjalnych czynników ryzyka środowiskowych, które mogą zwiększać prawdopodobieństwo rozwoju FTD:³⁸³⁹

Uraz głowy – historia urazów głowy może ponad trzykrotnie zwiększać ryzyko rozwoju FTD⁴⁰⁴¹
Choroby tarczycy – zwiększają ryzyko FTD 2,5-krotnie⁴²⁴³
Choroby autoimmunologiczne – mogą być związane ze zwiększonym ryzykiem pierwotnej postępującej afazji, podtypu FTD⁴⁴
Cukrzyca – niektóre badania wskazują na związek między cukrzycą a zwiększonym ryzykiem FTD⁴⁵
Stan zapalny w mózgu – przewlekły stan zapalny może przyśpieszać gromadzenie się nieprawidłowych białek i uszkodzenie neuronów⁴⁶⁴⁷

Należy jednak podkreślić, że dowody na wpływ tych czynników są wciąż ograniczone, a badania w tym kierunku są kontynuowane. Nie ma obecnie wystarczających dowodów, aby zalecać konkretne zmiany stylu życia w celu zapobiegania FTD na poziomie populacyjnym.⁴⁸

Mechanizm uszkodzenia mózgu w FTD

Proces neurodegeneracyjny w demencji czołowo-skroniowej obejmuje kilka wzajemnie powiązanych mechanizmów:⁴⁹⁵⁰

Śmierć komórek nerwowych – charakterystyczną cechą FTD jest postępująca utrata neuronów, szczególnie w płatach czołowych i skroniowych mózgu⁵¹
Uszkodzenie synaps – zaburzenie komunikacji między komórkami nerwowymi, co prowadzi do dysfunkcji sieci neuronalnych⁵²
Zmniejszenie poziomu neuroprzekaźników – wpływa na zdolność komórek nerwowych do przesyłania sygnałów⁵³
Zaburzenia naprawy DNA – uszkodzenia DNA i defekty w mechanizmach naprawczych zostały etiologicznie powiązane z FTD⁵⁴
Zaburzenia w reaktywnych cząsteczkach azotu – badania sugerują, że reaktywne cząsteczki azotu mogą przyczyniać się do rozwoju FTD i ALS⁵⁵

W wyniku tych procesów obserwuje się postępujące zmniejszanie się tkanki mózgowej w płatach czołowych i skroniowych, co prowadzi do charakterystycznych objawów klinicznych FTD, takich jak zmiany osobowości, zaburzenia zachowania i trudności językowe.⁵⁶⁵⁷

Podsumowanie etiologii FTD

Demencja czołowo-skroniowa stanowi złożone zaburzenie neurodegeneracyjne, którego dokładna etiologia pozostaje w znacznej mierze nieznana. Badania wskazują na wieloczynnikową naturę tej choroby, obejmującą zarówno komponenty genetyczne, jak i środowiskowe.⁵⁸⁵⁹

W przeciwieństwie do choroby Alzheimera, FTD wykazuje silniejszy komponent dziedziczny, z około 30-40% przypadków mających wyraźną historię rodzinną. Zidentyfikowano kilka kluczowych genów, których mutacje zwiększają ryzyko rozwoju FTD, szczególnie C9orf72, MAPT i GRN.⁶⁰⁶¹

Patologicznie, FTD charakteryzuje się gromadzeniem nieprawidłowych białek (tau, TDP-43, FUS) w komórkach nerwowych płatów czołowych i skroniowych, co prowadzi do ich uszkodzenia i ostatecznie śmierci. Interesujący jest również związek między FTD a stwardnieniem zanikowym bocznym (ALS), sugerujący wspólne mechanizmy patogenetyczne.⁶²⁶³

Chociaż zidentyfikowano pewne czynniki ryzyka środowiskowego, takie jak urazy głowy czy choroby tarczycy, ich bezpośredni wpływ na rozwój FTD wymaga dalszych badań. Obecnie nie ma skutecznych strategii zapobiegania ani leczenia modyfikującego przebieg choroby, co podkreśla potrzebę dalszych intensywnych badań nad jej etiologią.⁶⁴⁶⁵

Zrozumienie złożonej etiologii FTD stanowi klucz do opracowania skutecznych metod diagnostycznych, terapeutycznych i ewentualnie profilaktycznych, które mogłyby znacząco poprawić rokowanie pacjentów z tym ciężkim zaburzeniem neurodegeneracyjnym.⁶⁶⁶⁷

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Materiały źródłowe

#1 Frontotemporal Disorders: Causes, Symptoms, and Diagnosis | National Institute on Aging
https://www.nia.nih.gov/health/frontotemporal-disorders/what-are-frontotemporal-disorders-causes-symptoms-and-treatment
Frontotemporal disorders result from damage to neurons primarily in the frontal and temporal lobes of the brain. […] Scientists are beginning to understand the biological and genetic basis for the changes observed in brain cells that lead to FTD. […] Scientists describe FTD using the patterns of change in the brain seen in an autopsy after death. These changes include loss of neurons and abnormal amounts or forms of proteins called tau and TDP-43. […] In most cases, the cause of FTD is unknown. Individuals with a family history of the disease are more likely to develop FTD. […] FTD that runs in a family is often related to variants (permanent changes) in certain genes. […] An estimated one-third of FTD cases are inherited, meaning the genetic variant is passed from parent to child.
#2 What Is Frontotemporal Dementia?
https://www.alzheimers.gov/alzheimers-dementias/frontotemporal-dementia
Frontotemporal dementia is caused by a group of disorders that gradually damage the brains frontal and temporal lobes. […] The underlying causes of frontotemporal dementia are not yet fully understood. People with FTD have abnormal amounts or forms of proteins called tau and TDP-43 inside nerve cells, or neurons, in their brain. The neurons die because the buildup of these proteins causes damage. In some cases, changes or differences in genes can be identified as the cause of FTD. For example, about 10% to 30% of bvFTD cases can be attributed to genetic causes. People with a family history of FTD are more likely to have a genetic form of the disease.
#3 Frontotemporal dementia – Symptoms and causes – Mayo Clinic
https://www.mayoclinic.org/diseases-conditions/frontotemporal-dementia/symptoms-causes/syc-20354737
Frontotemporal dementia can be misdiagnosed as a mental health condition or as Alzheimer’s disease. But FTD tends to occur at a younger age than does Alzheimer’s disease. It often begins between the ages of 40 and 65, although it can occur later in life as well. FTD is the cause of dementia about 10% to 20% of the time. […] In frontotemporal dementia, the frontal and temporal lobes of the brain shrink and certain substances build up in the brain. What causes these changes is usually not known. […] Some genetic changes have been linked to frontotemporal dementia. But more than half of the people with FTD have no family history of dementia. […] Researchers have confirmed that some frontotemporal dementia gene changes also are seen in amyotrophic lateral sclerosis (ALS). More research is being done to understand the connection between the conditions.
#4 Frontotemporal dementia – Dementia UK
https://www.dementiauk.org/information-and-support/types-of-dementia/frontotemporal-dementia/
Frontotemporal dementia (FTD) is an umbrella term for a group of dementias that mainly affect the frontal and temporal lobes of the brain, which are responsible for personality, behaviour, language and speech. […] In FTD, there is an abnormal build-up of proteins within the brain, which damages the cells. It is not known why this occurs, but it is thought to have a genetic link in about one third of people with the diagnosis. […] FTD is most common in people aged 40 to 60 but can also affect younger or older people.
#5 What is frontotemporal dementia? | Alzheimer’s Research UK
https://www.alzheimersresearchuk.org/dementia-information/types-of-dementia/frontotemporal-dementia/
Frontotemporal dementia also called FTD, is a rarer type of dementia caused by a build up of proteins, tau, FUS and TDP-43, in the frontal and temporal lobes of the brain. […] FTD is caused by damage to cells in areas of the brain called the frontal and temporal lobes. These areas of our brains control our personality, emotions and behaviour. […] In FTD, there is a build-up of proteins in the frontal and temporal lobes. Some of the proteins linked to FTD are called: tau, TDP-43, a group called FET proteins. […] FTD is sometimes described as an umbrella term because it can be caused by several different underlying diseases.
#6 What Is Frontotemporal Dementia?
https://www.alzheimers.gov/alzheimers-dementias/frontotemporal-dementia
Frontotemporal dementia is caused by a group of disorders that gradually damage the brains frontal and temporal lobes. […] The underlying causes of frontotemporal dementia are not yet fully understood. People with FTD have abnormal amounts or forms of proteins called tau and TDP-43 inside nerve cells, or neurons, in their brain. The neurons die because the buildup of these proteins causes damage. In some cases, changes or differences in genes can be identified as the cause of FTD. For example, about 10% to 30% of bvFTD cases can be attributed to genetic causes. People with a family history of FTD are more likely to have a genetic form of the disease.
#7 Frontotemporal Dementia and Other Frontotemporal Disorders | National Institute of Neurological Disorders and Stroke
https://www.ninds.nih.gov/health-information/disorders/frontotemporal-dementia-and-other-frontotemporal-disorders
Frontotemporal disorders are the result of damage to neurons primarily in the in the frontal and temporal lobes of the brain. […] Scientists are beginning to understand the biological and genetic basis for the changes observed in brain cells that lead to FTD. […] In most cases, the cause of FTD is unknown. Individuals with a family history of FTD are more likely to develop FTD. […] FTD that runs in a family is often related to variants (permanent changes) in certain genes. […] An estimated one-third of FTD cases are inherited, meaning the genetic variant is passed from parent to child. […] Scientists have discovered several different genes that, when changed or mutated, can lead to FTD: […] A change in this gene causes abnormalities in tau proteins, which then form tangles inside neurons and ultimately leads to the destruction of brain cells.
#8 Frontotemporal dementia | Stanford Health Care
https://stanfordhealthcare.org/medical-conditions/brain-and-nerves/dementia/types/frontotemporal-dementia.html
Frontotemporal dementia (FTD) describes a group of diseases characterized by degeneration of nerve cells – especially those in the frontal and temporal lobes of the brain. […] In approximately 50% of people with FTD, there is an abnormal form of tau protein in the brain and about 50% of people with FTD have TDP-43 protein accumulation. […] A small percentage, about 5%, have FUS protein accumulation. This disrupts normal cell activities and may cause the cells to die. […] In many cases, people with FTD have a family history of dementia, suggesting that there is a strong genetic factor in the disease. […] The cause of Pick’s disease is unknown, but it runs in some families and thus it is probably due at least in part to a faulty gene or genes. […] In some cases, familial FTD is linked to a mutation in a gene called C9ORF72. This mutation is thought to be the most common cause of familial FTD and familial ALS (amyotrophic lateral sclerosis or Lou Gherig’s disease). […] A mutation in the tau gene (MAPT) or progranulin gene can also cause familial FTD.
#9 Frontotemporal Dementia and Other Frontotemporal Disorders | National Institute of Neurological Disorders and Stroke
https://www.ninds.nih.gov/health-information/disorders/frontotemporal-dementia-and-other-frontotemporal-disorders
A change in this gene can lead to lower production of the protein progranulin, which in turn causes another protein, TDP-43, to go awry in brain cells. […] An unusual change in this gene appears to be the most common genetic abnormality in familial frontotemporal disorders and familial ALS. […] Scientists are continuing to study these genes and to search for other genes and proteins, as well as nongenetic risk factors, that may play a role in FTD.
#10 Frontotemporal dementia | Stanford Health Care
https://stanfordhealthcare.org/medical-conditions/brain-and-nerves/dementia/types/frontotemporal-dementia.html
Frontotemporal dementia (FTD) describes a group of diseases characterized by degeneration of nerve cells – especially those in the frontal and temporal lobes of the brain. […] In approximately 50% of people with FTD, there is an abnormal form of tau protein in the brain and about 50% of people with FTD have TDP-43 protein accumulation. […] A small percentage, about 5%, have FUS protein accumulation. This disrupts normal cell activities and may cause the cells to die. […] In many cases, people with FTD have a family history of dementia, suggesting that there is a strong genetic factor in the disease. […] The cause of Pick’s disease is unknown, but it runs in some families and thus it is probably due at least in part to a faulty gene or genes. […] In some cases, familial FTD is linked to a mutation in a gene called C9ORF72. This mutation is thought to be the most common cause of familial FTD and familial ALS (amyotrophic lateral sclerosis or Lou Gherig’s disease). […] A mutation in the tau gene (MAPT) or progranulin gene can also cause familial FTD.
#11 Frontotemporal dementia | Stanford Health Care
https://stanfordhealthcare.org/medical-conditions/brain-and-nerves/dementia/types/frontotemporal-dementia.html
Frontotemporal dementia (FTD) describes a group of diseases characterized by degeneration of nerve cells – especially those in the frontal and temporal lobes of the brain. […] In approximately 50% of people with FTD, there is an abnormal form of tau protein in the brain and about 50% of people with FTD have TDP-43 protein accumulation. […] A small percentage, about 5%, have FUS protein accumulation. This disrupts normal cell activities and may cause the cells to die. […] In many cases, people with FTD have a family history of dementia, suggesting that there is a strong genetic factor in the disease. […] The cause of Pick’s disease is unknown, but it runs in some families and thus it is probably due at least in part to a faulty gene or genes. […] In some cases, familial FTD is linked to a mutation in a gene called C9ORF72. This mutation is thought to be the most common cause of familial FTD and familial ALS (amyotrophic lateral sclerosis or Lou Gherig’s disease). […] A mutation in the tau gene (MAPT) or progranulin gene can also cause familial FTD.
#12 What is frontotemporal dementia? | Alzheimer’s Research UK
https://www.alzheimersresearchuk.org/dementia-information/types-of-dementia/frontotemporal-dementia/
Frontotemporal dementia also called FTD, is a rarer type of dementia caused by a build up of proteins, tau, FUS and TDP-43, in the frontal and temporal lobes of the brain. […] FTD is caused by damage to cells in areas of the brain called the frontal and temporal lobes. These areas of our brains control our personality, emotions and behaviour. […] In FTD, there is a build-up of proteins in the frontal and temporal lobes. Some of the proteins linked to FTD are called: tau, TDP-43, a group called FET proteins. […] FTD is sometimes described as an umbrella term because it can be caused by several different underlying diseases.
#13 Pickâs Disease: What It Is, Causes, Symptoms & Diagnosis
https://my.clevelandclinic.org/health/diseases/22637-picks-disease
Picks disease is a specific type of frontotemporal dementia, a degenerative brain disease that usually affects people under 65. […] Picks disease is a type of frontotemporal dementia with a very specific cause. All nerve and brain cells contain a specific protein, tau (named for a Greek alphabet letter). Proteins like tau need to have a specific shape, or they wont work properly. […] When tau proteins malfunction and tangle together, they build up inside neurons, damaging and destroying those cells. These destroyed neurons, known as Pick cells, have a swollen, balloon-like shape. Tangles of tau proteins inside cells, known as Pick bodies, are also visible under a microscope. […] Researchers have linked Picks disease to certain genetic mutations, but most cases of the disease arent inherited.
#14
https://www.nhs.uk/conditions/frontotemporal-dementia/
Frontotemporal dementia is caused by clumps of abnormal protein forming inside brain cells. These are thought to damage the cells and stop them working properly. […] The proteins mainly build up in the frontal and temporal lobes of the brain at the front and sides. These are important for controlling language, behaviour, and the ability to plan and organise. […] It’s not fully understood why this happens, but there’s often a genetic link. People who get frontotemporal dementia may have relatives who were also affected by the condition. […] There’s a lot of research being done to try to improve understanding of the causes of frontotemporal dementia so treatments can be discovered.
#15 What is frontotemporal dementia? – Mayo Clinic News Network
https://newsnetwork.mayoclinic.org/discussion/what-is-frontotemporal-degeneration/
Frontotemporal dementia (FTD) is a group of neurologic disorders associated with changes in personality, behavior, language or movement. Some FTD forms are inherited, and some are not. […] Genetic mutations and dysfunctional proteins in the brain have been linked to FTD. […] „Roughly 20% to 40% of individuals with FTD have a genetic cause or contributor, and that is more common than in Alzheimer’s disease, Lewy body disease and other neurologic disorders,” says Dr. Boeve, The Little Family Foundation Professor of Lewy Body Dementia.
#16 Frontotemporal Disorders: Causes, Symptoms, and Diagnosis | National Institute on Aging
https://www.nia.nih.gov/health/frontotemporal-disorders/what-are-frontotemporal-disorders-causes-symptoms-and-treatment
Frontotemporal disorders result from damage to neurons primarily in the frontal and temporal lobes of the brain. […] Scientists are beginning to understand the biological and genetic basis for the changes observed in brain cells that lead to FTD. […] Scientists describe FTD using the patterns of change in the brain seen in an autopsy after death. These changes include loss of neurons and abnormal amounts or forms of proteins called tau and TDP-43. […] In most cases, the cause of FTD is unknown. Individuals with a family history of the disease are more likely to develop FTD. […] FTD that runs in a family is often related to variants (permanent changes) in certain genes. […] An estimated one-third of FTD cases are inherited, meaning the genetic variant is passed from parent to child.
#17 Frontotemporal dementia | UK DRI
https://www.ukdri.ac.uk/conditions/frontotemporal-dementia
During frontotemporal dementia, clumps of abnormal protein, including tau, TDP-43 and FUS form inside brain cells. These are thought to damage the cells and stop them working properly. This causes the connections between the cells and other parts of the brain to break down. The levels of chemical messengers in the brain also reduce over time. These messengers allow nerve cells to send signals to each other and the rest of the body. The proteins mainly build up in the frontal and temporal lobes of the brain at the front and sides. These are important for controlling language, behaviour, and the ability to plan and organise. As more and more nerve cells are damaged and die, the brain tissue in the frontal and temporal lobes starts to get smaller. […] It is not fully understood why this happens, but in around 30-40% of cases, a person with frontotemporal dementia has a family history of the condition in which a parent or sibling has been affected. In these cases, the cause of frontotemporal dementia is likely to be genetic. However, most cases of frontotemporal dementia do not have a genetic basis.
#18 Insights Into The Causes Of Frontotemporal Dementia – Discovery Village
https://www.discoveryvillages.com/senior-living-blog/insights-into-the-causes-of-frontotemporal-dementia/
Frontotemporal dementia (FTD) stands as a notable neurological condition characterized by the progressive degeneration of the frontal and temporal lobes of the brain. […] A combination of genetic predisposition, environmental influences, and biological factors contributes to its development. […] One of the primary causes of FTD is genetics. In about 30-50% of cases, there is a family history of the disorder, suggesting a genetic predisposition. […] While genetics play a significant role, environmental and lifestyle factors can also contribute to the development of FTD. […] Another key factor in the development of FTD is the misfolding of proteins in the brain. […] Emerging research suggests that inflammation and abnormal immune responses may play a significant role in the complex progression of Frontotemporal Dementia (FTD).
#19 Frontotemporal dementia risk factors – Alzheimer’s Research UK
https://www.alzheimersresearchuk.org/dementia-information/types-of-dementia/frontotemporal-dementia/risk-factors/
Some people with FTD have a family history of dementia and the condition may be inherited in some of these families. […] Although some people with frontotemporal dementia have a family history of the condition, around seven in every 10 cases of FTD do not have a genetic basis. […] Directly inherited dementia is rare, but in around three in every 10 people with FTD a strong family history of the condition is known. In these cases the cause is likely to be genetic. This is known as familial frontotemporal dementia. […] Research has found a number of faulty genes that can cause inherited forms of FTD. […] Faulty genes are passed directly from a person who is affected by a condition like familial FTD to their children. […] In cases where there is no family history, the risk factors for frontotemporal dementia are not yet fully understood. Because FTD is a rarer type of dementia it is harder for researchers to study how risk factors develop over time to cause it.
#20 Understanding fFTD – Rare Dementia Support
https://www.raredementiasupport.org/familial-frontotemporal-dementia/understanding-fftd/
Familial frontotemporal dementia (fFTD) is an inherited form of frontotemporal dementia (FTD). […] In around 30-40% of people diagnosed with frontotemporal dementia (FTD), there is a family history of the condition and it is likely to have a genetic cause we call this familial FTD (fFTD). […] In some cases FTD appears to run in families and there is likely to be a genetic cause. […] In 30-40% of all people with FTD, the condition is known to be caused by an abnormality in a single gene. Three genes cause the majority of genetic FTD cases progranulin, tau and C9orf72. At least six other genes can cause familial FTD, and more may be discovered. […] If someone has one of the gene abnormalities that cause FTD, then each of their children has a 50% chance of inheriting the faulty gene. Each sibling of someone with the faulty gene also has a 50% chance of inheriting it. […] If someone is tested and has inherited one of these gene abnormalities, then they are highly likely to develop FTD. Only a very small minority of people who have inherited a faulty gene live without developing FTD in their lifetime.
#21 Exploring Causes of Frontotemporal Dementia—and Potential Treatments
https://www.brainandlife.org/articles/frontotemporal-dementia-causes-treatments-alzheimers-disease
About one in five cases of FTD is inherited. Most genetic cases are caused by a mutation in the C9orf72 gene, the tau (MAPT) gene, or the GRN gene, although genetic abnormalities can be in any of some 20 genes that are linked to the presentation. […] In cases of an autosomal dominant gene meaning that a single disease-associated mutated gene causes FTD there’s a 50 percent chance of passing that gene down to each one of your children. […] Once the initial shock of the diagnosis is over, families should behave as facilitators. […] Currently no treatments approved by the US Food and Drug Administration exist for FTD. Some antidepressants and selective serotonin reuptake inhibitors can help with symptoms such as depression, irritability, or agitation, since FTD is linked to serotonin and dopamine deficits in the brain.
#22 Understanding fFTD – Rare Dementia Support
https://www.raredementiasupport.org/familial-frontotemporal-dementia/understanding-fftd/
Familial frontotemporal dementia (fFTD) is an inherited form of frontotemporal dementia (FTD). […] In around 30-40% of people diagnosed with frontotemporal dementia (FTD), there is a family history of the condition and it is likely to have a genetic cause we call this familial FTD (fFTD). […] In some cases FTD appears to run in families and there is likely to be a genetic cause. […] In 30-40% of all people with FTD, the condition is known to be caused by an abnormality in a single gene. Three genes cause the majority of genetic FTD cases progranulin, tau and C9orf72. At least six other genes can cause familial FTD, and more may be discovered. […] If someone has one of the gene abnormalities that cause FTD, then each of their children has a 50% chance of inheriting the faulty gene. Each sibling of someone with the faulty gene also has a 50% chance of inheriting it. […] If someone is tested and has inherited one of these gene abnormalities, then they are highly likely to develop FTD. Only a very small minority of people who have inherited a faulty gene live without developing FTD in their lifetime.
#23 Frontotemporal Disorders: Causes, Symptoms, and Diagnosis | National Institute on Aging
https://www.nia.nih.gov/health/frontotemporal-disorders/what-are-frontotemporal-disorders-causes-symptoms-and-treatment
Scientists have discovered several different genes that, when changed or mutated, can lead to FTD. […] In recent years, researchers have discovered several other genetic changes that lead to rare familial types of frontotemporal disorders. […] Scientists are continuing to study these genes and to search for other genes and proteins, as well as nongenetic risk factors, that may play a role in FTD.
#24 Frontotemporal Dementia and Other Frontotemporal Disorders | National Institute of Neurological Disorders and Stroke
https://www.ninds.nih.gov/health-information/disorders/frontotemporal-dementia-and-other-frontotemporal-disorders
Frontotemporal disorders are the result of damage to neurons primarily in the in the frontal and temporal lobes of the brain. […] Scientists are beginning to understand the biological and genetic basis for the changes observed in brain cells that lead to FTD. […] In most cases, the cause of FTD is unknown. Individuals with a family history of FTD are more likely to develop FTD. […] FTD that runs in a family is often related to variants (permanent changes) in certain genes. […] An estimated one-third of FTD cases are inherited, meaning the genetic variant is passed from parent to child. […] Scientists have discovered several different genes that, when changed or mutated, can lead to FTD: […] A change in this gene causes abnormalities in tau proteins, which then form tangles inside neurons and ultimately leads to the destruction of brain cells.
#25 Frontotemporal Dementia | Stanford Health Care
https://stanfordhealthcare.org/medical-conditions/brain-and-nerves/frontotemporal-dementia.html
Frontotemporal dementia is a group of disorders that occur when the nerve cells in the frontal and temporal lobes of the brain are damaged, causing the lobes to shrink. […] The cause of frontotemporal dementia is unknown. Researchers have linked certain subtypes of frontotemporal dementia to mutations on several genes. The most common is a mutation called C9ORF72, which was discovered in 2011. It can cause amyotrophic lateral sclerosis (ALS), often referred to as Lou Gherig’s disease, and a combination of both diseases. Other common mutations include MAPT and progranulin. A family history of frontotemporal dementia is the only known risk for these diseases. Although experts believe that some cases of frontotemporal dementia are inherited, the majority of people with frontotemporal dementia have no family history of it or other types of dementia.
#26 Frontotemporal (Frontal Lobe) Dementia: Causes and Symptoms
https://www.webmd.com/alzheimers/frontotemporal-dementia
Frontotemporal dementia (FTD) is a group of diseases that damage the frontal and temporal lobes of the brain. […] Scientists believe that certain proteins made by your body play a role in this damage. Abnormal (misshapen and malfunctioning) forms of these proteins, called tau and TDP-43, build up in the damaged brain regions. […] About 40% of people with FTD have a family history of the disease. In some cases, FTD has been linked to specific genetic mutations that run in families. […] Genes that can cause FTD include: Tau gene (also called the MAPT gene), linked most often to the behavioral type of FTD; GRN gene, also mostly linked to behavioral FTD; C9orf72 gene, linked to both FTD and ALS. […] Other factors may raise your risk of developing FTD: A history of head injury, which more than triples your risk; Thyroid disease, which increases your risk 2.5 times.
#27 Pulsenotes | Frontotemporal dementia
https://app.pulsenotes.com/medicine/neurology/notes/frontotemporal-dementia
Frontotemporal dementia shows a strong genetic predisposition. […] The exact cause of FTD is unknown, but is characterised by tissue deposition of aggregated proteins including phosphorylated tau or transactive response DNA-binding protein 43 (TDP-43). […] In 10-25% of cases, there is an autosomal dominant pattern of inheritance. […] Commonly implicated genes include: Microtubule associated protein tau (MAPT): found on chromosome 17. Multiple identified mutations. Leads to a propensity of tau to form neurotoxic aggregates. […] Granulin precursor (GRN): found on chromosome 17. Multiple identified mutations. Role in FTD is unclear. […] C9ORF72 gene: found on chromosome 9. Most common genetic cause of inherited FTD. Also implicated in hereditary motor neuron disease. Usually results in bvFTD with or without MND. […] These three genes explain approximately 15% of familial cases.
#28 Frontotemporal dementia | Stanford Health Care
https://stanfordhealthcare.org/medical-conditions/brain-and-nerves/dementia/types/frontotemporal-dementia.html
Frontotemporal dementia (FTD) describes a group of diseases characterized by degeneration of nerve cells – especially those in the frontal and temporal lobes of the brain. […] In approximately 50% of people with FTD, there is an abnormal form of tau protein in the brain and about 50% of people with FTD have TDP-43 protein accumulation. […] A small percentage, about 5%, have FUS protein accumulation. This disrupts normal cell activities and may cause the cells to die. […] In many cases, people with FTD have a family history of dementia, suggesting that there is a strong genetic factor in the disease. […] The cause of Pick’s disease is unknown, but it runs in some families and thus it is probably due at least in part to a faulty gene or genes. […] In some cases, familial FTD is linked to a mutation in a gene called C9ORF72. This mutation is thought to be the most common cause of familial FTD and familial ALS (amyotrophic lateral sclerosis or Lou Gherig’s disease). […] A mutation in the tau gene (MAPT) or progranulin gene can also cause familial FTD.
#29 Frontotemporal Dementia and Other Frontotemporal Disorders | National Institute of Neurological Disorders and Stroke
https://www.ninds.nih.gov/health-information/disorders/frontotemporal-dementia-and-other-frontotemporal-disorders
A change in this gene can lead to lower production of the protein progranulin, which in turn causes another protein, TDP-43, to go awry in brain cells. […] An unusual change in this gene appears to be the most common genetic abnormality in familial frontotemporal disorders and familial ALS. […] Scientists are continuing to study these genes and to search for other genes and proteins, as well as nongenetic risk factors, that may play a role in FTD.
#30 Exploring Causes of Frontotemporal Dementia—and Potential Treatments
https://www.brainandlife.org/articles/frontotemporal-dementia-causes-treatments-alzheimers-disease
About one in five cases of FTD is inherited. Most genetic cases are caused by a mutation in the C9orf72 gene, the tau (MAPT) gene, or the GRN gene, although genetic abnormalities can be in any of some 20 genes that are linked to the presentation. […] In cases of an autosomal dominant gene meaning that a single disease-associated mutated gene causes FTD there’s a 50 percent chance of passing that gene down to each one of your children. […] Once the initial shock of the diagnosis is over, families should behave as facilitators. […] Currently no treatments approved by the US Food and Drug Administration exist for FTD. Some antidepressants and selective serotonin reuptake inhibitors can help with symptoms such as depression, irritability, or agitation, since FTD is linked to serotonin and dopamine deficits in the brain.
#31 What is Frontotemporal Dementia? – FTD Talk
https://www.ftdtalk.org/what-is-frontotemporal-dementia/
Frontotemporal dementia, or FTD, is a neurodegenerative disease that affects adults, with an average age of onset between 50 and 60. […] In about a third of people with FTD the disease is caused by an abnormal gene that has been passed on from one generation to another, a pattern of inheritance known as autosomal dominant. It remains unclear exactly why the other two thirds of people with FTD develop the condition. […] About a third of people with FTD will have a family history of the disorder with around a quarter having an autosomal dominant inheritance pattern. […] There are quite a few genes that are associated with FTD but only three are common. These are called microtubule-associated protein tau or MAPT, progranulin or GRN, and C9orf72. […] Mutations in other genes are only very rare causes of FTD. These include TANK-binding kinase 1 or TBK1, transactive response DNA binding protein or TARDBP, valosin-containing protein or VCP, and charged multivesicular body protein 2B or CHMP2B. […] A number of genes do not directly cause FTD but can modify the type of symptoms or age at which those symptoms develop. These include TMEM106B.
#32 Pulsenotes | Frontotemporal dementia
https://app.pulsenotes.com/medicine/neurology/notes/frontotemporal-dementia
Frontotemporal dementia shows a strong genetic predisposition. […] The exact cause of FTD is unknown, but is characterised by tissue deposition of aggregated proteins including phosphorylated tau or transactive response DNA-binding protein 43 (TDP-43). […] In 10-25% of cases, there is an autosomal dominant pattern of inheritance. […] Commonly implicated genes include: Microtubule associated protein tau (MAPT): found on chromosome 17. Multiple identified mutations. Leads to a propensity of tau to form neurotoxic aggregates. […] Granulin precursor (GRN): found on chromosome 17. Multiple identified mutations. Role in FTD is unclear. […] C9ORF72 gene: found on chromosome 9. Most common genetic cause of inherited FTD. Also implicated in hereditary motor neuron disease. Usually results in bvFTD with or without MND. […] These three genes explain approximately 15% of familial cases.
#33 Frontotemporal dementia – Symptoms and causes – Mayo Clinic
https://www.mayoclinic.org/diseases-conditions/frontotemporal-dementia/symptoms-causes/syc-20354737
Frontotemporal dementia can be misdiagnosed as a mental health condition or as Alzheimer’s disease. But FTD tends to occur at a younger age than does Alzheimer’s disease. It often begins between the ages of 40 and 65, although it can occur later in life as well. FTD is the cause of dementia about 10% to 20% of the time. […] In frontotemporal dementia, the frontal and temporal lobes of the brain shrink and certain substances build up in the brain. What causes these changes is usually not known. […] Some genetic changes have been linked to frontotemporal dementia. But more than half of the people with FTD have no family history of dementia. […] Researchers have confirmed that some frontotemporal dementia gene changes also are seen in amyotrophic lateral sclerosis (ALS). More research is being done to understand the connection between the conditions.
#34 Frontotemporal dementia (FTD) | Alzheimer’s Society
https://www.alzheimers.org.uk/about-dementia/types-dementia/frontotemporal-dementia
Frontotemporal dementia (FTD) occurs when disease damages nerve cells in these lobes. This causes the connections between them and other parts of the brain to break down. […] FTD and MND are both progressive conditions that get worse over time. Having the two conditions can reduce a person’s life expectancy more than having FTD on its own. […] Some people with FTD start to have symptoms of MND as their condition progresses. This is more likely to happen to people with behavioural variant FTD, particularly if their condition is caused by a single gene. Its less likely for people who have a language-led dementia and very rare for those with semantic variant PPA.
#35 Frontotemporal dementia (FTD) | Alzheimer’s Society
https://www.alzheimers.org.uk/about-dementia/types-dementia/frontotemporal-dementia
Frontotemporal dementia (FTD) occurs when disease damages nerve cells in these lobes. This causes the connections between them and other parts of the brain to break down. […] FTD and MND are both progressive conditions that get worse over time. Having the two conditions can reduce a person’s life expectancy more than having FTD on its own. […] Some people with FTD start to have symptoms of MND as their condition progresses. This is more likely to happen to people with behavioural variant FTD, particularly if their condition is caused by a single gene. Its less likely for people who have a language-led dementia and very rare for those with semantic variant PPA.
#36 Frontotemporal Dementia | Conditions | UCSF Health
https://www.ucsfhealth.org/conditions/frontotemporal-dementia
Frontotemporal dementia (FTD) is a group of degenerative diseases that gradually damage or shrink the brain’s frontal and anterior temporal lobes, located in the front of the brain. […] About 40 percent of patients have a clear family history. The remaining 60 percent of cases occur randomly. If a parent has frontotemporal dementia, one in two of the children are likely to develop the disease. In some cases where the disorder appears to be inherited, there is an apparent change or mutation in a gene on chromosome 3, 9 or 17. Other genes may be involved as well. The cause of the illness, however, is unknown. […] Some patients with FTD develop Lou Gehrig’s disease, also known as amyotrophic lateral sclerosis (ALS). Doctors don’t yet fully understand the connection between the two diseases but are studying the trend.
#37 A new clue to the cause of ALS and frontotemporal dementia | Scripps Research
https://www.scripps.edu/news-and-events/press-room/2021/20210310-lipton-als.html
Scientists at Scripps Research have discovered a plausible trigger for the most common form of amyotrophic lateral sclerosis (ALS, also commonly called Lou Gehrigs disease), and a related form of dementia. […] The scientists, whose study is published in the Proceedings of the National Academy of Sciences of the USA, found evidence that key features of ALS and frontotemporal dementia can be triggered by reactive nitrogen molecules in the human brain. […] Liptons team found evidence that this process can be caused by highly reactive molecules that contain nitrogen. […] These findings support the hypothesis that aging or environmental factorsor bothcan trigger ALS by increasing the levels of reactive nitrogen molecules, Lipton says.
#38 4 Underlying Frontotemporal Dementia Causes
https://addingtonplaceofdesmoines.seniorlivingnearme.com/blog/4-underlying-frontotemporal-dementia-causes?hs_amp=true
Frontotemporal dementia (FTD) stands out in the landscape of cognitive disorders due to its unique impact on personality, behavior and language. […] One of the primary frontotemporal dementia causes is genetic mutations. Research has identified certain genetic changes that significantly increase the risk of developing FTD. These mutations often affect the production and function of specific proteins in the brain, leading to the degeneration of frontal and temporal lobe neurons. […] An additional crucial aspect contributing to the progression of Frontotemporal Dementia (FTD) is the abnormal accumulation of proteins in the brain. Tau proteins and TDP-43 are frequently linked with FTD, causing disruption to regular cellular processes, ultimately resulting in cell demise and brain degeneration.
#39 4 Underlying Frontotemporal Dementia Causes
https://addingtonplaceofdesmoines.seniorlivingnearme.com/blog/4-underlying-frontotemporal-dementia-causes?hs_amp=true
While much attention is given to genetic and biological factors, environmental influences and lifestyle choices may also play a role in the onset of frontotemporal dementia. […] Emerging evidence suggests that inflammation within the brain could be a contributing factor to frontotemporal dementia. Neuroinflammation, the brain’s response to injury or disease, may accelerate the progression of FTD by damaging neural pathways and exacerbating protein accumulation. […] Frontotemporal dementia presents a complex interplay of genetic, biological and possibly environmental factors.
#40 Frontotemporal Dementia: What It Is, Symptoms & Treatment
https://my.clevelandclinic.org/health/diseases/21075-frontotemporal-dementia
Frontotemporal dementia causes brain tissue to deteriorate in the frontal (front) and temporal (side) lobes of the brain. […] FTD happens when neurons, a key type of brain cell, deteriorate. This usually happens when there’s a malfunction in how your body creates certain proteins. […] Experts have linked misfolded proteins with FTD and its related conditions, such as Picks disease. […] These protein malfunctions happen with certain DNA mutations. […] Some DNA mutations run in families, which is why about 40% of FTD cases involve a family history of the disease. […] While they aren’t causes, two other factors can increase the risk of developing FTD. One is having a history of head trauma, which more than triples your risk of developing FTD. Thyroid disease is also linked, making FTD 2.5 times more likely to develop.
#41 Risk factors for frontotemporal dementia | Tidsskrift for Den norske legeforening
https://tidsskriftet.no/en/2018/09/oversiktsartikkel/risk-factors-frontotemporal-dementia
Risk factors for frontotemporal dementia are poorly understood. The purpose of this article is to provide an up-to-date review of modifiable risk factors for frontotemporal dementia and to evaluate the evidence base for clinical recommendations on how to reduce risk. […] Some studies showed an association between modifiable risk factors and the development of frontotemporal dementia. One study found that diabetes gives rise to increased risk. Three studies showed that head injury can increase the risk of frontotemporal dementia and that the prevalence of traumatic brain injury is significantly higher in patients with frontotemporal dementia than with other forms of dementia. Autoimmune disease may be associated with increased risk of primary progressive aphasia, a subtype of frontotemporal dementia.
#42 Frontotemporal Dementia: What It Is, Symptoms & Treatment
https://my.clevelandclinic.org/health/diseases/21075-frontotemporal-dementia
Frontotemporal dementia causes brain tissue to deteriorate in the frontal (front) and temporal (side) lobes of the brain. […] FTD happens when neurons, a key type of brain cell, deteriorate. This usually happens when there’s a malfunction in how your body creates certain proteins. […] Experts have linked misfolded proteins with FTD and its related conditions, such as Picks disease. […] These protein malfunctions happen with certain DNA mutations. […] Some DNA mutations run in families, which is why about 40% of FTD cases involve a family history of the disease. […] While they aren’t causes, two other factors can increase the risk of developing FTD. One is having a history of head trauma, which more than triples your risk of developing FTD. Thyroid disease is also linked, making FTD 2.5 times more likely to develop.
#43 Frontotemporal (Frontal Lobe) Dementia: Causes and Symptoms
https://www.webmd.com/alzheimers/frontotemporal-dementia
Frontotemporal dementia (FTD) is a group of diseases that damage the frontal and temporal lobes of the brain. […] Scientists believe that certain proteins made by your body play a role in this damage. Abnormal (misshapen and malfunctioning) forms of these proteins, called tau and TDP-43, build up in the damaged brain regions. […] About 40% of people with FTD have a family history of the disease. In some cases, FTD has been linked to specific genetic mutations that run in families. […] Genes that can cause FTD include: Tau gene (also called the MAPT gene), linked most often to the behavioral type of FTD; GRN gene, also mostly linked to behavioral FTD; C9orf72 gene, linked to both FTD and ALS. […] Other factors may raise your risk of developing FTD: A history of head injury, which more than triples your risk; Thyroid disease, which increases your risk 2.5 times.
#44 Risk factors for frontotemporal dementia | Tidsskrift for Den norske legeforening
https://tidsskriftet.no/en/2018/09/oversiktsartikkel/risk-factors-frontotemporal-dementia
Risk factors for frontotemporal dementia are poorly understood. The purpose of this article is to provide an up-to-date review of modifiable risk factors for frontotemporal dementia and to evaluate the evidence base for clinical recommendations on how to reduce risk. […] Some studies showed an association between modifiable risk factors and the development of frontotemporal dementia. One study found that diabetes gives rise to increased risk. Three studies showed that head injury can increase the risk of frontotemporal dementia and that the prevalence of traumatic brain injury is significantly higher in patients with frontotemporal dementia than with other forms of dementia. Autoimmune disease may be associated with increased risk of primary progressive aphasia, a subtype of frontotemporal dementia.
#45 Risk factors for frontotemporal dementia | Tidsskrift for Den norske legeforening
https://tidsskriftet.no/en/2018/09/oversiktsartikkel/risk-factors-frontotemporal-dementia
Risk factors for frontotemporal dementia are poorly understood. The purpose of this article is to provide an up-to-date review of modifiable risk factors for frontotemporal dementia and to evaluate the evidence base for clinical recommendations on how to reduce risk. […] Some studies showed an association between modifiable risk factors and the development of frontotemporal dementia. One study found that diabetes gives rise to increased risk. Three studies showed that head injury can increase the risk of frontotemporal dementia and that the prevalence of traumatic brain injury is significantly higher in patients with frontotemporal dementia than with other forms of dementia. Autoimmune disease may be associated with increased risk of primary progressive aphasia, a subtype of frontotemporal dementia.
#46 4 Underlying Frontotemporal Dementia Causes
https://addingtonplaceofdesmoines.seniorlivingnearme.com/blog/4-underlying-frontotemporal-dementia-causes?hs_amp=true
While much attention is given to genetic and biological factors, environmental influences and lifestyle choices may also play a role in the onset of frontotemporal dementia. […] Emerging evidence suggests that inflammation within the brain could be a contributing factor to frontotemporal dementia. Neuroinflammation, the brain’s response to injury or disease, may accelerate the progression of FTD by damaging neural pathways and exacerbating protein accumulation. […] Frontotemporal dementia presents a complex interplay of genetic, biological and possibly environmental factors.
#47 Insights Into The Causes Of Frontotemporal Dementia – Discovery Village
https://www.discoveryvillages.com/senior-living-blog/insights-into-the-causes-of-frontotemporal-dementia/
Frontotemporal dementia (FTD) stands as a notable neurological condition characterized by the progressive degeneration of the frontal and temporal lobes of the brain. […] A combination of genetic predisposition, environmental influences, and biological factors contributes to its development. […] One of the primary causes of FTD is genetics. In about 30-50% of cases, there is a family history of the disorder, suggesting a genetic predisposition. […] While genetics play a significant role, environmental and lifestyle factors can also contribute to the development of FTD. […] Another key factor in the development of FTD is the misfolding of proteins in the brain. […] Emerging research suggests that inflammation and abnormal immune responses may play a significant role in the complex progression of Frontotemporal Dementia (FTD).
#48 Risk factors for frontotemporal dementia | Tidsskrift for Den norske legeforening
https://tidsskriftet.no/en/2018/09/oversiktsartikkel/risk-factors-frontotemporal-dementia
The literature suggested an association between diabetes, head injury, autoimmune disease and frontotemporal dementia. […] A family history is one of the major risk factors for frontotemporal dementia, but up to 60 % of those affected have no known family members with the condition. This indicates that 6 out of 10 cases are sporadic (non-hereditary). Frontotemporal dementia is linked to chromosome 17 in some families, with an autosomal dominant inheritance pattern, and to chromosomes 3 and 9 in other cases. Mutations in the tau gene have also been detected in certain cases. […] The literature suggests associations between diabetes, head injury and autoimmune disease, and frontotemporal dementia, but the current evidence base is too narrow to be able to draw any conclusions. There is insufficient evidence to support recommendations for specific lifestyle changes aimed at preventing frontotemporal dementia at the population level.
#49 Frontotemporal dementia – Wikipedia
https://en.wikipedia.org/wiki/Frontotemporal_dementia
Frontotemporal dementia (FTD), also called frontotemporal degeneration disease or frontotemporal neurocognitive disorder, encompasses several types of dementia involving the progressive degeneration of the brain’s frontal and temporal lobes. […] FTDs are mostly early onset syndromes linked to frontotemporal lobar degeneration (FTLD), which is characterized by progressive neuronal loss predominantly involving the frontal or temporal lobes, and a typical loss of more than 70% of spindle neurons, while other neuron types remain intact. […] The International Classification of Diseases recognizes the disease as causative to disorder affecting mental and behavioural aspects in humans. […] With regard to the genetic defects that have been found, repeat expansion in the C9orf72 gene is considered a major contribution to FTLD, although defects in the GRN and MAPT genes are also associated with it. […] DNA damage and the defective repair of such damages have been etiologically linked to various neurodegenerative diseases including FTD.
#50 Frontotemporal Dementia | Memory and Aging Center
https://memory.ucsf.edu/dementia/ftd
Frontotemporal dementia (FTD) is a group of related conditions resulting from the progressive degeneration of the temporal and frontal lobes of the brain. […] The clinical symptoms of FTD are caused by degeneration in the parts of the brain that control decision-making, behavior, emotion, and language (typically the frontal, temporal, and insular regions). […] While each type of FTD produces different symptoms, all forms cause a steady decline in the ability to think and function, eventually leaving the person dependent on caregivers to get through the day.
#51 Frontotemporal Dementia (FTD) | Symptoms & Treatments | alz.org
https://www.alz.org/alzheimers-dementia/what-is-dementia/types-of-dementia/frontotemporal-dementia
Frontotemporal dementia (FTD) or frontotemporal degeneration refers to a group of disorders caused by progressive nerve cell loss in the brain’s frontal lobes (the areas behind your forehead) or its temporal lobes (the regions behind your ears). […] There are a number of different diseases that cause frontotemporal degeneration. The two most prominent are 1) a group of brain disorders involving the protein tau and 2) a group of brain disorders involving the protein called TDP43. For reasons that are not yet known, these two groups have a preference for the frontal and temporal lobes that cause dementia. […] Frontotemporal degeneration is inherited in about a third of all cases. Genetic counseling and testing are available now for individuals with family histories of frontotemporal degeneration. There are no known risk factors for any frontotemporal degeneration except for a family history or a similar disorder.
#52 Frontotemporal dementia | UK DRI
https://www.ukdri.ac.uk/conditions/frontotemporal-dementia
During frontotemporal dementia, clumps of abnormal protein, including tau, TDP-43 and FUS form inside brain cells. These are thought to damage the cells and stop them working properly. This causes the connections between the cells and other parts of the brain to break down. The levels of chemical messengers in the brain also reduce over time. These messengers allow nerve cells to send signals to each other and the rest of the body. The proteins mainly build up in the frontal and temporal lobes of the brain at the front and sides. These are important for controlling language, behaviour, and the ability to plan and organise. As more and more nerve cells are damaged and die, the brain tissue in the frontal and temporal lobes starts to get smaller. […] It is not fully understood why this happens, but in around 30-40% of cases, a person with frontotemporal dementia has a family history of the condition in which a parent or sibling has been affected. In these cases, the cause of frontotemporal dementia is likely to be genetic. However, most cases of frontotemporal dementia do not have a genetic basis.
#53 Frontotemporal dementia | UK DRI
https://www.ukdri.ac.uk/conditions/frontotemporal-dementia
During frontotemporal dementia, clumps of abnormal protein, including tau, TDP-43 and FUS form inside brain cells. These are thought to damage the cells and stop them working properly. This causes the connections between the cells and other parts of the brain to break down. The levels of chemical messengers in the brain also reduce over time. These messengers allow nerve cells to send signals to each other and the rest of the body. The proteins mainly build up in the frontal and temporal lobes of the brain at the front and sides. These are important for controlling language, behaviour, and the ability to plan and organise. As more and more nerve cells are damaged and die, the brain tissue in the frontal and temporal lobes starts to get smaller. […] It is not fully understood why this happens, but in around 30-40% of cases, a person with frontotemporal dementia has a family history of the condition in which a parent or sibling has been affected. In these cases, the cause of frontotemporal dementia is likely to be genetic. However, most cases of frontotemporal dementia do not have a genetic basis.
#54 Frontotemporal dementia – Wikipedia
https://en.wikipedia.org/wiki/Frontotemporal_dementia
Frontotemporal dementia (FTD), also called frontotemporal degeneration disease or frontotemporal neurocognitive disorder, encompasses several types of dementia involving the progressive degeneration of the brain’s frontal and temporal lobes. […] FTDs are mostly early onset syndromes linked to frontotemporal lobar degeneration (FTLD), which is characterized by progressive neuronal loss predominantly involving the frontal or temporal lobes, and a typical loss of more than 70% of spindle neurons, while other neuron types remain intact. […] The International Classification of Diseases recognizes the disease as causative to disorder affecting mental and behavioural aspects in humans. […] With regard to the genetic defects that have been found, repeat expansion in the C9orf72 gene is considered a major contribution to FTLD, although defects in the GRN and MAPT genes are also associated with it. […] DNA damage and the defective repair of such damages have been etiologically linked to various neurodegenerative diseases including FTD.
#55 A new clue to the cause of ALS and frontotemporal dementia | Scripps Research
https://www.scripps.edu/news-and-events/press-room/2021/20210310-lipton-als.html
Scientists at Scripps Research have discovered a plausible trigger for the most common form of amyotrophic lateral sclerosis (ALS, also commonly called Lou Gehrigs disease), and a related form of dementia. […] The scientists, whose study is published in the Proceedings of the National Academy of Sciences of the USA, found evidence that key features of ALS and frontotemporal dementia can be triggered by reactive nitrogen molecules in the human brain. […] Liptons team found evidence that this process can be caused by highly reactive molecules that contain nitrogen. […] These findings support the hypothesis that aging or environmental factorsor bothcan trigger ALS by increasing the levels of reactive nitrogen molecules, Lipton says.
#56 Frontotemporal Dementia | Memory and Aging Center
https://memory.ucsf.edu/dementia/ftd
Frontotemporal dementia (FTD) is a group of related conditions resulting from the progressive degeneration of the temporal and frontal lobes of the brain. […] The clinical symptoms of FTD are caused by degeneration in the parts of the brain that control decision-making, behavior, emotion, and language (typically the frontal, temporal, and insular regions). […] While each type of FTD produces different symptoms, all forms cause a steady decline in the ability to think and function, eventually leaving the person dependent on caregivers to get through the day.
#57 What is frontotemporal dementia? | Alzheimer’s Research UK
https://www.alzheimersresearchuk.org/dementia-information/types-of-dementia/frontotemporal-dementia/
Frontotemporal dementia also called FTD, is a rarer type of dementia caused by a build up of proteins, tau, FUS and TDP-43, in the frontal and temporal lobes of the brain. […] FTD is caused by damage to cells in areas of the brain called the frontal and temporal lobes. These areas of our brains control our personality, emotions and behaviour. […] In FTD, there is a build-up of proteins in the frontal and temporal lobes. Some of the proteins linked to FTD are called: tau, TDP-43, a group called FET proteins. […] FTD is sometimes described as an umbrella term because it can be caused by several different underlying diseases.
#58
https://continentalhospitals.com/diseases/frontotemporal-dementia/
Unlike other types of dementia, such as Alzheimer’s disease, FTD typically affects individuals at a younger age, often between the ages of 40 and 65. […] The exact cause of FTD is still not fully understood, but it is believed to involve a combination of genetic and environmental factors. […] While the exact cause of FTD is still not fully understood, researchers have identified several factors that may contribute to its development. One potential cause is genetic mutations, as certain genes have been found to be associated with an increased risk of developing FTD. […] Another possible cause of FTD is abnormal protein accumulation in the brain. In some cases, proteins such as tau or TDP-43 clump together and form aggregates, disrupting normal brain function. […] Furthermore, environmental factors may also play a role in the development of FTD. Exposure to certain toxins or chemicals over a prolonged period may increase the risk of developing this condition.
#59 8 Underlying Causes of Frontotemporal Dementia | SummerHouse Senior Living
https://www.summerhouseseniorliving.com/senior-living-blog/8-underlying-causes-of-frontotemporal-dementia/
Frontotemporal dementia is a multifaceted condition influenced by a combination of genetic predisposition, protein abnormalities, environmental factors, and lifestyle choices. […] By understanding the underlying causes of FTD, we can better tailor interventions and treatments to address the specific needs of individuals affected by this condition.
#60 Frontotemporal Disorders: Causes, Symptoms, and Diagnosis | National Institute on Aging
https://www.nia.nih.gov/health/frontotemporal-disorders/what-are-frontotemporal-disorders-causes-symptoms-and-treatment
Frontotemporal disorders result from damage to neurons primarily in the frontal and temporal lobes of the brain. […] Scientists are beginning to understand the biological and genetic basis for the changes observed in brain cells that lead to FTD. […] Scientists describe FTD using the patterns of change in the brain seen in an autopsy after death. These changes include loss of neurons and abnormal amounts or forms of proteins called tau and TDP-43. […] In most cases, the cause of FTD is unknown. Individuals with a family history of the disease are more likely to develop FTD. […] FTD that runs in a family is often related to variants (permanent changes) in certain genes. […] An estimated one-third of FTD cases are inherited, meaning the genetic variant is passed from parent to child.
#61 Frontotemporal dementia | UK DRI
https://www.ukdri.ac.uk/conditions/frontotemporal-dementia
During frontotemporal dementia, clumps of abnormal protein, including tau, TDP-43 and FUS form inside brain cells. These are thought to damage the cells and stop them working properly. This causes the connections between the cells and other parts of the brain to break down. The levels of chemical messengers in the brain also reduce over time. These messengers allow nerve cells to send signals to each other and the rest of the body. The proteins mainly build up in the frontal and temporal lobes of the brain at the front and sides. These are important for controlling language, behaviour, and the ability to plan and organise. As more and more nerve cells are damaged and die, the brain tissue in the frontal and temporal lobes starts to get smaller. […] It is not fully understood why this happens, but in around 30-40% of cases, a person with frontotemporal dementia has a family history of the condition in which a parent or sibling has been affected. In these cases, the cause of frontotemporal dementia is likely to be genetic. However, most cases of frontotemporal dementia do not have a genetic basis.
#62 Frontotemporal Dementia (FTD) | Symptoms & Treatments | alz.org
https://www.alz.org/alzheimers-dementia/what-is-dementia/types-of-dementia/frontotemporal-dementia
Frontotemporal dementia (FTD) or frontotemporal degeneration refers to a group of disorders caused by progressive nerve cell loss in the brain’s frontal lobes (the areas behind your forehead) or its temporal lobes (the regions behind your ears). […] There are a number of different diseases that cause frontotemporal degeneration. The two most prominent are 1) a group of brain disorders involving the protein tau and 2) a group of brain disorders involving the protein called TDP43. For reasons that are not yet known, these two groups have a preference for the frontal and temporal lobes that cause dementia. […] Frontotemporal degeneration is inherited in about a third of all cases. Genetic counseling and testing are available now for individuals with family histories of frontotemporal degeneration. There are no known risk factors for any frontotemporal degeneration except for a family history or a similar disorder.
#63 Frontotemporal Dementia | Stanford Health Care
https://stanfordhealthcare.org/medical-conditions/brain-and-nerves/frontotemporal-dementia.html
Frontotemporal dementia is a group of disorders that occur when the nerve cells in the frontal and temporal lobes of the brain are damaged, causing the lobes to shrink. […] The cause of frontotemporal dementia is unknown. Researchers have linked certain subtypes of frontotemporal dementia to mutations on several genes. The most common is a mutation called C9ORF72, which was discovered in 2011. It can cause amyotrophic lateral sclerosis (ALS), often referred to as Lou Gherig’s disease, and a combination of both diseases. Other common mutations include MAPT and progranulin. A family history of frontotemporal dementia is the only known risk for these diseases. Although experts believe that some cases of frontotemporal dementia are inherited, the majority of people with frontotemporal dementia have no family history of it or other types of dementia.
#64 Risk factors for frontotemporal dementia | Tidsskrift for Den norske legeforening
https://tidsskriftet.no/en/2018/09/oversiktsartikkel/risk-factors-frontotemporal-dementia
The literature suggested an association between diabetes, head injury, autoimmune disease and frontotemporal dementia. […] A family history is one of the major risk factors for frontotemporal dementia, but up to 60 % of those affected have no known family members with the condition. This indicates that 6 out of 10 cases are sporadic (non-hereditary). Frontotemporal dementia is linked to chromosome 17 in some families, with an autosomal dominant inheritance pattern, and to chromosomes 3 and 9 in other cases. Mutations in the tau gene have also been detected in certain cases. […] The literature suggests associations between diabetes, head injury and autoimmune disease, and frontotemporal dementia, but the current evidence base is too narrow to be able to draw any conclusions. There is insufficient evidence to support recommendations for specific lifestyle changes aimed at preventing frontotemporal dementia at the population level.
#65 Exploring Causes of Frontotemporal Dementia—and Potential Treatments
https://www.brainandlife.org/articles/frontotemporal-dementia-causes-treatments-alzheimers-disease
Clinical research into disease-modifying therapies medications that target the underlying disease process is just starting. […] Scientists have deepened their understanding of the disease. For a long time, FTD was the neglected stepchild of dementia research and was poorly understood. But we’re making great strides in finding specific ways to treat the disease. […] Research into the causes of frontotemporal dementia (FTD) and possible targets for treatment is in high gear.
#66 Frontotemporal Dementia
https://www.caregiver.org/resource/frontotemporal-dementia/
Frontotemporal dementia (FTD), sometimes also known as frontotemporal lobar degeneration, is one of the most common causes of early-onset dementia. […] Approximately 40 percent of people diagnosed with FTD have a family history that includes at least one other relative with dementia. There is a strongly inherited genetic link in at least 10 percent of people diagnosed with FTD. […] Additionally, a form of dementia in persons with motor neuron disease (amyotrophic lateral sclerosis, commonly known as ALS or Lou Gehrig’s Disease) may be associated with FTD. […] Researchers are studying ways to diagnose frontotemporal disorders earlier and more accurately. They are developing biomarkers (new blood tests, spinal fluid tests, and advanced brain imaging) to improve diagnosis, measure rate of disease progression, and measure efficacy of treatment.
#67 Exploring Causes of Frontotemporal Dementia—and Potential Treatments
https://www.brainandlife.org/articles/frontotemporal-dementia-causes-treatments-alzheimers-disease
Clinical research into disease-modifying therapies medications that target the underlying disease process is just starting. […] Scientists have deepened their understanding of the disease. For a long time, FTD was the neglected stepchild of dementia research and was poorly understood. But we’re making great strides in finding specific ways to treat the disease. […] Research into the causes of frontotemporal dementia (FTD) and possible targets for treatment is in high gear.