Demencja czołowo-skroniowa
Diagnostyka i diagnoza

Demencja czołowo-skroniowa (FTD) to postępujące schorzenie neurodegeneracyjne charakteryzujące się zanikiem płatów czołowych i/lub skroniowych mózgu. Diagnostyka FTD jest wyzwaniem klinicznym ze względu na brak pojedynczego testu diagnostycznego oraz nakładanie się objawów z innymi chorobami, takimi jak choroba Alzheimera, depresja czy choroba Parkinsona. Średni czas do prawidłowej diagnozy wynosi około 3,6 roku. Rozpoznanie opiera się na szczegółowym wywiadzie medycznym, badaniu neurologicznym, ocenie neuropsychologicznej (w tym funkcji wykonawczych, fluencji słownej i rozumienia słów) oraz badaniach obrazowych mózgu (MRI, CT, FDG-PET, SPECT). Charakterystyczne dla FTD są deficyty funkcji wykonawczych, hipometabolizm czołowy i/lub skroniowy w PET oraz zanik obustronny lub asymetryczny płatów czołowych i skroniowych w MRI/CT. Badania laboratoryjne i płynu mózgowo-rdzeniowego służą głównie wykluczeniu innych przyczyn, a biomarkery takie jak łańcuch lekki neurofilamentu są obecnie badane pod kątem diagnostyki FTD.

Diagnostyka demencji czołowo-skroniowej

Demencja czołowo-skroniowa (FTD – Frontotemporal Dementia) jest postępującym schorzeniem neurodegeneracyjnym, które charakteryzuje się degradacją płatów czołowych i/lub skroniowych mózgu. Diagnostyka FTD stanowi wyzwanie kliniczne, ponieważ nie istnieje pojedynczy test diagnostyczny pozwalający na jednoznaczne rozpoznanie tej choroby. Rozpoznanie demencji czołowo-skroniowej opiera się na kompleksowej ocenie klinicznej, badaniach obrazowych oraz wykluczeniu innych możliwych przyczyn obserwowanych objawów.12

Trudności diagnostyczne

Demencja czołowo-skroniowa jest często błędnie diagnozowana jako choroba Alzheimera, depresja, choroba Parkinsona lub schorzenie psychiatryczne. Średni czas do uzyskania prawidłowej diagnozy wynosi około 3,6 roku. Trudności w diagnostyce wynikają z kilku czynników:34

  • Objawy FTD często nakładają się na objawy innych schorzeń
  • FTD jest rzadszą formą demencji niż choroba Alzheimera
  • Wczesne objawy FTD nie obejmują typowo zaburzeń pamięci, które są powszechnie kojarzone z demencją
  • Pacjenci często nie mają wglądu we własne objawy
  • Wielu lekarzy podstawowej opieki zdrowotnej rzadko spotyka się z przypadkami FTD w swojej praktyce

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Badanie kliniczne

Podstawą rozpoznania demencji czołowo-skroniowej jest dokładne badanie kliniczne, które obejmuje:78

  • Szczegółowy wywiad medyczny, w tym wywiad rodzinny
  • Badanie neurologiczne oceniające równowagę, napięcie mięśniowe i siłę
  • Ocena neuropsychologiczna
  • Wywiad od osoby, która dobrze zna pacjenta – jest to kluczowe, ponieważ osoby z FTD często nie są świadome zmian w swoim zachowaniu

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Specjalista (najczęściej neurolog lub psychiatra geriatryczny) może podejrzewać FTD po rozmowie z pacjentem i osobą, która dobrze go zna. Lekarz zbiera szczegółowy wywiad dotyczący objawów pacjenta i zadaje pytania, aby lepiej zrozumieć jego zachowanie i zdolności.11

Testy diagnostyczne w demencji czołowo-skroniowej

Badania neuropsychologiczne

Standardowe testy oceniające zdolności umysłowe, które głównie skupiają się na utracie pamięci, mogą być mniej pomocne w diagnostyce FTD. Bardziej wyspecjalizowane testy oceniające świadomość społeczną lub zachowanie mogą być konieczne do dokładnej diagnozy. Kompleksowa ocena neuropsychologiczna może obejmować:1213

  • Ocenę funkcji wykonawczych – większość osób z FTD wykazuje słabe wyniki w testach neuropsychiatrycznych funkcji wykonawczych
  • Ocenę fluencji słownej – szczególnie istotna w wariancie językowym FTD
  • Testy rozumienia słów – kluczowe przy podejrzeniu wariantu semantycznego
  • Inwentarz Zachowań Czołowych (Frontal Behavioral Inventory) – uwzględnia elementy wspierające kryteria Neary’ego i jest przydatny w diagnostyce różnicowej
  • Ocenę zdolności poznawczych – porównanie wyników pacjenta z normami dla populacji ogólnej w tym samym wieku i o podobnym poziomie wykształcenia

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Klasyczny wzorzec testowania dla FTD obejmuje deficyty w funkcji wykonawczej, upośledzenie pamięci roboczej/koncentracji, a także trudności z kodowaniem i odtwarzaniem informacji. Ocena zdolności poznania społecznego, zwłaszcza rozpoznawania emocji, jest pomocna w różnicowaniu FTD od innych form demencji.1718

Badania obrazowe

Badania obrazowe mózgu są niezbędne w diagnostyce FTD, aby wykluczyć inne możliwe przyczyny objawów i zidentyfikować charakterystyczne wzorce zaniku mózgu. Do najczęściej stosowanych badań obrazowych należą:1920

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W badaniach obrazowych strukturalnych (MRI, CT) charakterystyczne dla FTD jest występowanie zaniku płatów czołowych i/lub przednich części płatów skroniowych. Zanik może być obustronny lub asymetryczny. We wczesnych przypadkach obraz może wyglądać prawidłowo, dlatego ważne jest porównanie obrazów wykonanych w różnych punktach czasowych, np. w odstępie jednego roku.2425

FDG-PET może być szczególnie przydatny we wczesnych stadiach choroby i został zatwierdzony przez FDA do rozróżniania między demencją czołowo-skroniową a chorobą Alzheimera. W FTD klasycznie obserwuje się hipometabolizm czołowy i/lub przedni skroniowy, w przeciwieństwie do choroby Alzheimera, gdzie w badaniu PET klasycznie widoczny jest hipometabolizm obupółkulowy.2627

Badania laboratoryjne

Badania laboratoryjne są wykonywane głównie w celu wykluczenia innych potencjalnych przyczyn objawów poznawczych i behawioralnych. Podstawowe badania krwi powinny obejmować:2829

  • Morfologię krwi (CBC)
  • Panel metaboliczny
  • Badania tarczycy (TSH, wolna T4)
  • Poziom witaminy B12
  • Badania serologiczne w kierunku kiły (RPR)
  • OB (ESR)

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Nie istnieją charakterystyczne wyniki laboratoryjne dla FTD. Obecnie nie ma znanych biomarkerów krwi, które mogłyby wiarygodnie potwierdzić diagnozę demencji czołowo-skroniowej.3233

Badanie płynu mózgowo-rdzeniowego

Punkcja lędźwiowa jest badaniem, które może być wykorzystane do wykluczenia chorób naśladujących FTD (infekcji, etiologii immunologicznych, zespołów nowotworowych i paraneoplastycznych). Badanie płynu mózgowo-rdzeniowego, który otacza mózg i rdzeń kręgowy, może dostarczyć ważnych informacji o problemach związanych z mózgiem.3435

Badania płynu mózgowo-rdzeniowego mogą wiarygodnie odróżnić demencję czołowo-skroniową od innych demencji, takich jak choroba Alzheimera. Badacze sugerują, że stosowanie biomarkerów płynu mózgowo-rdzeniowego patologicznych blaszek amyloidowych, splotów i neurodegeneracji, zbiorczo nazywanych ATN, może być przydatne w diagnostyce FTD.3637

Potencjalne biomarkery płynu mózgowo-rdzeniowego obecnie badane w kontekście FTD obejmują łańcuch lekki neurofilamentu i białka powtórzeń dipeptydowych.38

Kryteria diagnostyczne demencji czołowo-skroniowej

Kryteria dla wariantu behawioralnego FTD (bvFTD)

Międzynarodowe konsorcjum opracowało zrewidowane wytyczne dotyczące diagnozy behawioralnego wariantu FTD (bvFTD). Kryteria te zapewniają trzy poziomy pewności diagnostycznej:3940

  • Możliwe bvFTD wymaga postępującego pogorszenia zachowania oraz trzech z sześciu głównych cech:
    • Odhamowanie
    • Apatia/inercja
    • Utrata empatii/współczucia
    • Zachowania perseweracyjne/kompulsywne
    • Hiperoralność
    • Zaburzony profil neuropsychologiczny (deficyty wykonawcze)
  • Prawdopodobne bvFTD wymaga spełnienia kryteriów możliwego bvFTD oraz:
    • Znaczące pogorszenie funkcjonowania
    • Obrazowanie zgodne z bvFTD (zanik czołowo-skroniowy w MRI lub zmniejszony metabolizm w PET)
  • Pewne bvFTD wymaga obecności zespołu klinicznego z genetycznym lub patologicznym potwierdzeniem zwyrodnienia płata czołowo-skroniowego (FTLD)

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Kryteria dla wariantów językowych FTD

Rozpoznanie kliniczne semantycznego wariantu afazji pierwotnie postępującej (svPPA) wymaga zarówno zaburzeń nazywania konfrontacyjnego, jak i pojedynczego rozumienia słów, z co najmniej 3 z 4 dodatkowych głównych cech.44

Rozpoznanie wspierane badaniami obrazowymi niepłynnego wariantu afazji pierwotnie postępującej (nfvPPA) wymaga obrazowania zgodnego z tym zespołem i musi wykazywać albo przeważający zanik lewej tylnej okolicy czołowo-wyspowej w MRI, albo przeważające zmniejszenie perfuzji lub hipometabolizm w tej okolicy w badaniu SPECT lub PET, albo oba.4546

Genetyczne i patologiczne potwierdzenie diagnozy

Specjalista może zalecić przeprowadzenie testu genetycznego u osoby z objawami FTD. Może to pokazać, czy stan osoby jest spowodowany określonym wadliwym genem. W przypadku pozytywnego wywiadu rodzinnego w kierunku FTD można zaoferować poradnictwo genetyczne i testy jako część diagnozy.4748

Badania genetyczne mogą wykryć mutacje znane jako powodujące FTD i mogą potwierdzić diagnozę. Najczęściej badane geny to C9ORF72, MAPT i GRN.4950

Definitywna diagnoza FTD może zostać postawiona jedynie post-mortem poprzez autopsję mózgu lub przy potwierdzeniu genetycznym FTLD. Po śmierci pacjenta możliwe jest postawienie bardziej pewnej diagnozy FTD, ponieważ mózg może być bezpośrednio badany w badaniach pośmiertnych.5152

Różnicowanie demencji czołowo-skroniowej

Demencja czołowo-skroniowa musi być różnicowana z innymi schorzeniami wywołującymi podobne objawy. Do najważniejszych schorzeń w diagnostyce różnicowej należą:5354

  • Choroba Alzheimera – w przeciwieństwie do FTD, w chorobie Alzheimera przeważają zaburzenia pamięci
  • Zaburzenia psychiatryczne – depresja, schizofrenia, zaburzenia osobowości
  • Otępienie naczyniowe – związane z chorobą naczyń mózgowych
  • Choroba Parkinsona lub otępienie z ciałami Lewy’ego
  • Obturacyjny bezdech senny – dysfunkcje wykonawcze i zmiany zachowania są częste w OSA

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Kluczowe różnice między FTD a chorobą Alzheimera obejmują:5758

  • Wiek wystąpienia – FTD zwykle rozpoczyna się wcześniej (40-65 lat)
  • Zachowanie pamięci i zdolności przestrzenno-wzrokowych w FTD, szczególnie we wczesnych stadiach
  • Wczesne i wyraźne zmiany osobowości i zachowania w bvFTD
  • Zaburzenia językowe jako główny objaw w wariantach językowych FTD
  • Różnice w obrazowaniu – w FTD zanik dotyczy głównie płatów czołowych i skroniowych, podczas gdy w chorobie Alzheimera zanik jest bardziej rozsiany
  • W badaniu FDG-PET w FTD widoczny jest hipometabolizm czołowy i skroniowy, podczas gdy w chorobie Alzheimera – ciemieniowy

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Nowe kierunki w diagnostyce demencji czołowo-skroniowej

Badacze aktualnie pracują nad metodami wcześniejszego i dokładniejszego diagnozowania FTD oraz odróżniania tego schorzenia od innych typów demencji. Badania koncentrują się na:6162

  • Biomarkerach – substancjach we krwi lub płynie mózgowo-rdzeniowym, które mogą pomóc w diagnozowaniu FTD i pomiarze progresji choroby
  • Nowych technikach obrazowania – takich jak spektroskopia rezonansu magnetycznego, obrazowanie funkcjonalne i pomiary grubości kory mózgowej
  • Badaniach nad białkami charakterystycznymi dla FTD – takimi jak tau, TDP-43 i FUS

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Istnieje duże zainteresowanie nowymi biomarkerami i metodami obrazowania do pomiaru progresji lub typu FTD, które mogą w najbliższych latach pozwolić na bardziej precyzyjną diagnozę podstawowej formy FTD, szczególnie w rozróżnianiu między formami związanymi z tau a formami związanymi z TDP-43.65

Badacze opracowali również nowatorski wskaźnik MRI, nazwany wskaźnikiem demencji czołowo-skroniowej (FTDI), który jest stosunkiem między ważoną sumą wskaźników wolumetrycznych w strukturach dominujących w FTD a wartością uzyskaną ze struktur dominujących w chorobie Alzheimera. FTDI wykazał doskonałą dokładność w identyfikacji FTD w odróżnieniu od choroby Alzheimera (AUC=0,90; SEN=89%, SPE=75% przy wartości progowej=1,08).6667

Diagnostyka demencji czołowo-skroniowej – praktyczne podejście

Diagnostyka demencji czołowo-skroniowej powinna obejmować kompleksowe podejście składające się z następujących elementów:6869

  • Dokładny wywiad – uzyskany zarówno od pacjenta, jak i opiekuna/osoby bliskiej
  • Badanie neurologiczne – oceniające odruchy, siłę mięśniową, napięcie mięśniowe, czucie dotykowe i wzrokowe, koordynację i równowagę
  • Ocena stanu neuropsychologicznego – pamięć, zdolność rozwiązywania problemów, rozpiętość uwagi, umiejętności liczenia i zdolności językowe
  • Standaryzowane kwestionariusze – takie jak Inwentarz Zachowań Czołowych
  • Badania laboratoryjne – wykluczające inne przyczyny objawów
  • Badania obrazowe – MRI, CT, PET lub SPECT

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Z uwagi na złożoność diagnozy FTD, kluczowe znaczenie ma konsultacja z neurologiem specjalizującym się w zaburzeniach poznawczych lub psychiatrą geriatrycznym. W niektórych przypadkach diagnoza może być odroczona do czasu uzyskania większej ilości informacji z badań krwi lub obrazowania mózgu.737475

Warto podkreślić, że diagnoza FTD jest procesem i czasami dopiero po kilku wizytach lekarskich i dłuższej obserwacji możliwe jest postawienie jednoznacznej diagnozy.7677

Wyzwania i perspektywy w diagnostyce FTD

Pomimo postępów w diagnostyce FTD, nadal istnieją znaczące wyzwania:787980

  • Brak powszechnej świadomości wczesnych objawów FTD
  • Mylenie objawów FTD z problemami związanymi z pracą, stresem lub problemami w relacjach
  • Błędne przekonanie, że demencja występuje tylko u osób starszych i zazwyczaj obejmuje problemy z pamięcią
  • Niechęć pacjenta do szukania porady medycznej – może to wynikać z braku świadomości zmian u siebie lub strachu przed diagnozą demencji

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Obecnie nie ma leczenia modyfikującego przebieg choroby zatwierdzonego przez FDA dla FTD, choć prowadzone są intensywne badania. Terapie skierowane na redukcję beta-amyloidu, które wydają się obiecujące w chorobie Alzheimera, nie są oczekiwane jako skuteczne w FTD, ponieważ patologia FTD zwykle składa się z agregatów tau lub TDP-43.8384

Wczesna i dokładna diagnoza FTD jest kluczowa, ponieważ pozwala na odpowiednie decyzje dotyczące leczenia, a także adekwatne poradnictwo dla rodzin i opiekunów. Potrzebne są dalsze badania, aby lepiej zdefiniować objawy behawioralne FTD i poprawić metody diagnostyczne.8586

Wsparcie dla pacjentów i rodzin

Po otrzymaniu diagnozy FTD kluczowe jest zapewnienie odpowiedniego wsparcia pacjentom i ich rodzinom:878889

  • Edukacja na temat choroby i jej przebiegu
  • Gruppy wsparcia – zarówno lokalne, jak i online
  • Terapia mowy – szczególnie w przypadku wariantów językowych FTD
  • Konsultacje z geriatrycznym psychiatrą w ramach zespołu terapeutycznego
  • Strategie kompensacyjne pomagające w radzeniu sobie z objawami
  • Informacje o dostępnych badaniach klinicznych

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Ważne jest, aby pacjenci z progresywnym zespołem otępiennym, takim jak FTD, nadal dobrze się odżywiali, regularnie ćwiczyli i utrzymywali kontakty z ludźmi. Te działania nie są lekami, nie leczą choroby, ale mogą pomóc mózgowi pracować jak najlepiej.9293

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  1. 09.04.2026
  2. www.leksykon.com.pl

Materiały źródłowe

  • #1 Frontotemporal dementia – Diagnosis and treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/frontotemporal-dementia/diagnosis-treatment/drc-20354741
    There’s no single test for frontotemporal dementia. Healthcare professionals consider your symptoms and exclude other possible causes of your symptoms. FTD can be hard to diagnose early because symptoms of frontotemporal dementia often overlap with those of other conditions. Healthcare professionals may order the following tests. […] Healthcare professionals may test your reasoning and memory skills. This type of testing is especially helpful to learn which type of dementia you may have at an early stage. It also can help distinguish FTD from other causes of dementia. […] There’s hope that diagnosing frontotemporal dementia may become easier in the future. Researchers are studying potential biomarkers of FTD. Biomarkers are substances that can be measured to help diagnose a disease. […] In addition to a physical exam, your healthcare professional checks your neurological health. This is done by testing things such as your balance, muscle tone and strength. You also may have a brief mental status evaluation to check your memory and thinking skills.
  • #2 Diagnosing FTD | AFTD
    https://www.theaftd.org/for-health-professionals/diagnosing-ftd/
    FTD is frequently misdiagnosed as Alzheimer’s, depression, Parkinson’s disease, or a psychiatric condition. On average, it currently takes 3.6 years to get an accurate diagnosis. […] With the exception of occasional genetic causes, today there is no single test that can diagnose FTD with certainty. The diagnosis of FTD requires a thorough history, verified by a caregiver, and a neurological examination. […] When the diagnosis is uncertain, referral to a neurologist with an interest in cognition and behavior and/or a geriatric neuropsychologist is indicated. […] All patients should be screened for obstructive sleep apnea (OSA), as executive dysfunction and behavior changes are common in OSA. […] Blood work should be done to exclude alternative causes of cognitive symptoms, including a basic metabolic panel, CBC, RPR, ESR, B12 level and thyroid studies.
  • #3 Diagnosing FTD | AFTD
    https://www.theaftd.org/for-health-professionals/diagnosing-ftd/
    FTD is frequently misdiagnosed as Alzheimer’s, depression, Parkinson’s disease, or a psychiatric condition. On average, it currently takes 3.6 years to get an accurate diagnosis. […] With the exception of occasional genetic causes, today there is no single test that can diagnose FTD with certainty. The diagnosis of FTD requires a thorough history, verified by a caregiver, and a neurological examination. […] When the diagnosis is uncertain, referral to a neurologist with an interest in cognition and behavior and/or a geriatric neuropsychologist is indicated. […] All patients should be screened for obstructive sleep apnea (OSA), as executive dysfunction and behavior changes are common in OSA. […] Blood work should be done to exclude alternative causes of cognitive symptoms, including a basic metabolic panel, CBC, RPR, ESR, B12 level and thyroid studies.
  • #4 Getting a diagnosis of frontotemporal dementia | Alzheimer’s Society
    https://www.alzheimers.org.uk/about-dementia/types-dementia/frontotemporal-dementia-diagnosis
    Frontotemporal dementia (FTD) can be hard to diagnose, because it is an uncommon type of dementia and does not cause memory problems at first. However, there are some ways to diagnose FTD including scans and genetic testing. […] Frontotemporal dementia is much less common than other types of dementia and often has different early symptoms. This means FTD can be hard for doctors to diagnose as they may not recognise its symptoms as dementia. […] If a person is showing some of the symptoms mentioned in this factsheet, they may want to ask their doctor to explore FTD as a possible diagnosis especially if they have family members with the condition. […] A specialist normally an old age psychiatrist or neurologist may think a person has FTD after talking to them and to someone who knows them well. The specialist will take a detailed history of the persons symptoms and ask questions to understand the persons behaviour and abilities better.
  • #5 FTD Misdiagnosis | Memory and Aging Center
    https://memory.ucsf.edu/ftd-misdiagnosis
    People with frontotemporal dementia (FTD) are often misdiagnosed with Alzheimers disease (AD), psychiatric disorders, vascular dementia or Parkinsons disease. The early symptoms and the brain image are often the most helpful tools to reach the right diagnosis. […] Once other likely explanations have been ruled out, an FTD diagnosis is made by looking at the data from a neurological exam and personal history (which may come from the patient, family, or another caregiver); neuropsychological tests that help quantify memory, language, and other cognitive skills; and a brain image usually an MRI (magnetic resonance imaging) scan but perhaps a functional scan like PET (positron emission tomography), which can show increased or lowered brain activity in the frontal and anterior temporal areas.
  • #6 Frontotemporal dementia (FTD) | Alzheimer’s Society
    https://www.alzheimers.org.uk/about-dementia/types-dementia/frontotemporal-dementia
    FTD is mostly diagnosed in people under 65. […] Getting a diagnosis of frontotemporal dementia can be challenging, as many people with behavioural variant FTD are not fully aware of their symptoms. […] This can cause long delays in getting an accurate diagnosis. […] The other main type of FTD is called primary progressive aphasia (PPA). […] Problems with other aspects of thinking, mood and behaviour tend to be less noticeable at first. […] As FTD progresses, the differences between the various types become less obvious. […] Many people with the behavioural variant develop language problems and may eventually lose all speech. […] Similarly, many people with language-led dementia develop clear changes in their behaviour and personality. […] At this late stage, they are also likely to need full-time care to meet their daily needs, such as eating, drinking, washing, dressing and going to the toilet.
  • #7 Diagnosing FTD | AFTD
    https://www.theaftd.org/for-health-professionals/diagnosing-ftd/
    FTD is frequently misdiagnosed as Alzheimer’s, depression, Parkinson’s disease, or a psychiatric condition. On average, it currently takes 3.6 years to get an accurate diagnosis. […] With the exception of occasional genetic causes, today there is no single test that can diagnose FTD with certainty. The diagnosis of FTD requires a thorough history, verified by a caregiver, and a neurological examination. […] When the diagnosis is uncertain, referral to a neurologist with an interest in cognition and behavior and/or a geriatric neuropsychologist is indicated. […] All patients should be screened for obstructive sleep apnea (OSA), as executive dysfunction and behavior changes are common in OSA. […] Blood work should be done to exclude alternative causes of cognitive symptoms, including a basic metabolic panel, CBC, RPR, ESR, B12 level and thyroid studies.
  • #8 Getting a diagnosis of frontotemporal dementia | Alzheimer’s Society
    https://www.alzheimers.org.uk/about-dementia/types-dementia/frontotemporal-dementia-diagnosis
    Frontotemporal dementia (FTD) can be hard to diagnose, because it is an uncommon type of dementia and does not cause memory problems at first. However, there are some ways to diagnose FTD including scans and genetic testing. […] Frontotemporal dementia is much less common than other types of dementia and often has different early symptoms. This means FTD can be hard for doctors to diagnose as they may not recognise its symptoms as dementia. […] If a person is showing some of the symptoms mentioned in this factsheet, they may want to ask their doctor to explore FTD as a possible diagnosis especially if they have family members with the condition. […] A specialist normally an old age psychiatrist or neurologist may think a person has FTD after talking to them and to someone who knows them well. The specialist will take a detailed history of the persons symptoms and ask questions to understand the persons behaviour and abilities better.
  • #9 Getting a diagnosis of frontotemporal dementia | Alzheimer’s Society
    https://www.alzheimers.org.uk/about-dementia/types-dementia/frontotemporal-dementia-diagnosis
    Frontotemporal dementia (FTD) can be hard to diagnose, because it is an uncommon type of dementia and does not cause memory problems at first. However, there are some ways to diagnose FTD including scans and genetic testing. […] Frontotemporal dementia is much less common than other types of dementia and often has different early symptoms. This means FTD can be hard for doctors to diagnose as they may not recognise its symptoms as dementia. […] If a person is showing some of the symptoms mentioned in this factsheet, they may want to ask their doctor to explore FTD as a possible diagnosis especially if they have family members with the condition. […] A specialist normally an old age psychiatrist or neurologist may think a person has FTD after talking to them and to someone who knows them well. The specialist will take a detailed history of the persons symptoms and ask questions to understand the persons behaviour and abilities better.
  • #10
    https://www.nhs.uk/conditions/frontotemporal-dementia/
    Frontotemporal dementia is an uncommon type of dementia that causes problems with behaviour and language. […] Dementia is the name for problems with mental abilities caused by gradual changes and damage in the brain. […] Frontotemporal dementia affects the front and sides of the brain (the frontal and temporal lobes). […] Most cases are diagnosed in people aged 45-65, although it can also affect younger or older people. […] The GP can do some simple checks to try to find the cause of your symptoms, and they can refer you to a memory clinic or another specialist for further tests if needed. […] There’s no single test for frontotemporal dementia. […] The following may be needed to make a diagnosis: an assessment of symptoms it’s normally helpful to have somebody who knows the person well to give an account of their symptoms, especially as someone with frontotemporal dementia may not be aware of changes in their behaviour; an assessment of mental abilities this will usually involve a number of tasks and questions; blood tests to rule out conditions with similar symptoms; brain scans such as an MRI scan, a CT scan or a PET scan; these can detect signs of dementia and help identify which parts of the brain are most affected, or help rule out other problems with the brain; lumbar puncture to test the spinal fluid (fluid that surrounds and supports the brain and spine); this may be useful to rule out Alzheimer’s disease as the cause of symptoms. […] Read more about the tests used to diagnose dementia.
  • #11 Getting a diagnosis of frontotemporal dementia | Alzheimer’s Society
    https://www.alzheimers.org.uk/about-dementia/types-dementia/frontotemporal-dementia-diagnosis
    Frontotemporal dementia (FTD) can be hard to diagnose, because it is an uncommon type of dementia and does not cause memory problems at first. However, there are some ways to diagnose FTD including scans and genetic testing. […] Frontotemporal dementia is much less common than other types of dementia and often has different early symptoms. This means FTD can be hard for doctors to diagnose as they may not recognise its symptoms as dementia. […] If a person is showing some of the symptoms mentioned in this factsheet, they may want to ask their doctor to explore FTD as a possible diagnosis especially if they have family members with the condition. […] A specialist normally an old age psychiatrist or neurologist may think a person has FTD after talking to them and to someone who knows them well. The specialist will take a detailed history of the persons symptoms and ask questions to understand the persons behaviour and abilities better.
  • #12 Getting a diagnosis of frontotemporal dementia | Alzheimer’s Society
    https://www.alzheimers.org.uk/about-dementia/types-dementia/frontotemporal-dementia-diagnosis
    Standard tests of mental abilities, which mostly focus on memory loss, can be less helpful in diagnosing FTD. More specialised tests of social awareness or behaviour may be needed. […] CT (computerised tomography) and MRI (magnetic resonance imaging) scans are used to see what parts of the brain are most damaged. They can also rule out other possible causes of a persons symptoms, such as a stroke or tumour. […] A specialist may recommend that a person with FTD symptoms has a genetic test. This can show if the persons condition is caused by a specific faulty gene. […] After a person dies, it is possible to make a more certain diagnosis of FTD. This is because the brain can be seen directly in post-mortem examinations.
  • #13 Frontotemporal Dementia: A Review for Primary Care Physicians | AAFP
    https://www.aafp.org/pubs/afp/issues/2010/1201/p1372.html
    The usefulness of standard dementia screening tests, such as the Mini-Mental State Examination or Addenbrooke’s Cognitive Examination, is limited to confirming the low language performance versus the relatively preserved memory and visuospatial ability in patients with FTD compared with patients who have Alzheimer disease; patients with Alzheimer disease predominantly have memory loss. […] The Frontal Behavioral Inventory incorporates supporting items from the Neary criteria, and is useful in the differential diagnosis.
  • #14 Frontotemporal Dementia: A Review for Primary Care Physicians | AAFP
    https://www.aafp.org/pubs/afp/issues/2010/1201/p1372.html
    The usefulness of standard dementia screening tests, such as the Mini-Mental State Examination or Addenbrooke’s Cognitive Examination, is limited to confirming the low language performance versus the relatively preserved memory and visuospatial ability in patients with FTD compared with patients who have Alzheimer disease; patients with Alzheimer disease predominantly have memory loss. […] The Frontal Behavioral Inventory incorporates supporting items from the Neary criteria, and is useful in the differential diagnosis.
  • #15 Differential Diagnosis of Frontotemporal Dementia (FTD) | alz.org
    https://www.alz.org/professionals/health-systems-medical-professionals/dementia-diagnosis/differential-diagnosis/differential_diagnosis_of_frontotemporal_dementia
    Frontotemporal dementia (FTD) is a group of disorders in which neurodegeneration chiefly affects brain areas called the frontal and temporal lobes. No single underlying pathological process is known. […] FTD, once considered rare, is now thought to account for up to 10 to 15 percent of all dementia cases. […] Most individuals with FTD perform poorly on neuropsychiatric tests of executive function. […] Brain imaging often shows damage to the anterior temporal and frontal lobes. If present, this atrophy may be diagnostic. Distinguishing FTD from Alzheimer’s is the only indication for which Medicare may cover a PET scan for evaluation of dementia.
  • #16 Frontotemporal Dementias
    https://practicalneurology.com/articles/2019-june/frontotemporal-dementias
    Reliance on collateral history, and subtle changes on neuroimaging can make diagnosis of bvFTD challenging. […] Patient insight is often limited, and so taking a history from a reliable informant who knows the patient well is essential. Standardized questionnaires may be used, such as the Frontal Behavioral Inventory. A comprehensive neurological examination should include assessment of vertical saccades, cortical sensation, praxis, primitive reflexes, and the presence of axial and appendicular parkinsonism. Brain imaging with CT or preferably MRI is essential to rule out alternative structural or vascular causes and to assess for characteristic patterns of atrophy, although detection of atrophy may be challenging, even for neuroradiologists. […] There are no validated biomarkers that reliably distinguish FTD from controls or other nonAD dementias; however, candidate CSF biomarkers are currently under investigation, including neurofilament light chain and dipeptide repeat proteins.
  • #17
    https://link.springer.com/article/10.1007/s13670-021-00360-y
    Obtaining clinical history from family verifying the gradual and progressive behavioral change is the most essential step toward making a diagnosis of bvFTD. […] Neuropsychological testing may be helpful in distinguishing bvFTD symptoms from other dementia syndromes. […] Neurological examination is an important component in suspected bvFTD. […] There are no characteristic laboratory findings for bvFTD. […] Neuroimaging may further support a diagnosis of bvFTD. […] When the diagnosis remains elusive, nuclear imaging such as fluorodeoxyglucose (FDG) – positron emission tomography (PET) or single-photon emission tomography (SPECT) may be used to elucidate abnormal patterns of brain function. […] Every patient should receive a cognitive performance screening test. […] The classic testing pattern for bvFTD includes deficits in executive function, impaired working memory/concentration, as well as difficulty with encoding and retrieval.
  • #18
    https://link.springer.com/article/10.1007/s13670-021-00360-y
    Assessment of social cognitive abilities, specifically emotion recognition, is helpful in differentiating bvFTD from other forms of dementia. […] The diagnosis of bvFTD is challenging as the initial presentation often overlaps with more common psychiatric syndromes and neurodegenerative diseases. […] A careful clinical history and thorough diagnostic evaluation, including neurological examination, laboratory studies, neuropsychological testing, and neuroimaging are indicated.
  • #19 Diagnosing FTD | AFTD
    https://www.theaftd.org/for-health-professionals/diagnosing-ftd/
    When a family history is positive, genetic testing of the diagnosed patient can be undertaken. […] A full neuropsychological testing evaluation should be used to better assess the pattern of cognitive loss in an individual suspected of having FTD and to help rule out psychiatric etiologies for an individual’s symptoms. […] Brain imaging is indicated in all individuals with symptoms of FTD to rule out structural causes. […] The MRI is more sensitive for assessing vascular changes and subtle patterns of atrophy, but it requires an individual to lie still for 15 to 30 minutes. […] Lumbar puncture is another test that can be used to rule out mimicking conditions (infection, immune etiologies, carcinomatous and paraneoplastic syndromes). […] Many primary care physicians are uncomfortable making the diagnosis of FTD. […] A definitive diagnosis of FTD can only be made post-mortem via autopsy of the brain. […] FTD disorders are identified by their clinical symptoms which present as behavioral/cognitive changes, or language deficits, or decline in motor function.
  • #20 Getting a diagnosis of frontotemporal dementia | Alzheimer’s Society
    https://www.alzheimers.org.uk/about-dementia/types-dementia/frontotemporal-dementia-diagnosis
    Standard tests of mental abilities, which mostly focus on memory loss, can be less helpful in diagnosing FTD. More specialised tests of social awareness or behaviour may be needed. […] CT (computerised tomography) and MRI (magnetic resonance imaging) scans are used to see what parts of the brain are most damaged. They can also rule out other possible causes of a persons symptoms, such as a stroke or tumour. […] A specialist may recommend that a person with FTD symptoms has a genetic test. This can show if the persons condition is caused by a specific faulty gene. […] After a person dies, it is possible to make a more certain diagnosis of FTD. This is because the brain can be seen directly in post-mortem examinations.
  • #21 Frontotemporal Dementia: What It Is, Symptoms & Treatment
    https://my.clevelandclinic.org/health/diseases/21075-frontotemporal-dementia
    Frontotemporal dementia (FTD) is a progressive brain disease. […] Frontotemporal dementia refers to a group of diseases that involve the deterioration of your brain’s frontal and temporal lobes. […] A healthcare provider, usually a neurologist, can diagnose FTD based on your medical history and a physical and neurological examination. […] The most likely tests for FTD include: Computerized tomography (CT) scans, Magnetic resonance imaging (MRI) scans, Positron emission tomography (PET) scan, Lumbar puncture (spinal tap), Blood tests, Electroencephalography (less common). […] FTD is not curable, and there’s no way to treat it directly. […] FTD isn’t a condition you can diagnose on your own. […] FTD happens unpredictably, so there’s no way to prevent it. […] The average life expectancy for a person after diagnosis with FTD is 7.5 years.
  • #22 Frontotemporal dementia – Wikipedia
    https://en.wikipedia.org/wiki/Frontotemporal_dementia
    Frontotemporal dementia (FTD) is traditionally difficult to diagnose owing to the diverse nature of the associated symptoms. Signs and symptoms are classified into three groups based on the affected functions of the frontal and temporal lobes: These are behavioural variant frontotemporal dementia, semantic dementia, and progressive nonfluent aphasia. An overlap between symptoms can occur as the disease progresses and spreads through the brain regions. […] Structural MRI scans often reveal frontal lobe and/or anterior temporal lobe atrophy, but in early cases the scan may seem normal. Atrophy can be either bilateral or asymmetric. Registration of images at different points of time (e.g., one year apart) can show evidence of atrophy that otherwise at individual time points may be reported as normal. Many research groups have begun using techniques such as magnetic resonance spectroscopy, functional imaging, and cortical thickness measurements in an attempt to offer an earlier diagnosis to the FTD patient. Fluorine-18-fluorodeoxyglucose positron emission tomography scans classically show frontal and/or anterior temporal hypometabolism, which helps differentiate the disease from Alzheimer’s disease, as the PET scan in Alzheimer’s disease classically shows biparietal hypometabolism.
  • #23 Frontotemporal dementia | MedLink Neurology
    https://www.medlink.com/articles/frontotemporal-dementia
    Clinically, structural magnetic resonance imaging (MRI) is best for reviewing findings. Atrophy is often asymmetric and in the early-middle stages, confined to the medial frontal and anterior temporal lobes. […] FDG-PET can be used to reveal a specific pattern of hypometabolism in the frontal and temporal lobes consistent with the synaptic dysfunction present in that region. It has been FDA approved to distinguish between frontotemporal dementia and Alzheimer disease and is particularly beneficial in the early stages of disease.
  • #24 Frontotemporal dementia – Wikipedia
    https://en.wikipedia.org/wiki/Frontotemporal_dementia
    Frontotemporal dementia (FTD) is traditionally difficult to diagnose owing to the diverse nature of the associated symptoms. Signs and symptoms are classified into three groups based on the affected functions of the frontal and temporal lobes: These are behavioural variant frontotemporal dementia, semantic dementia, and progressive nonfluent aphasia. An overlap between symptoms can occur as the disease progresses and spreads through the brain regions. […] Structural MRI scans often reveal frontal lobe and/or anterior temporal lobe atrophy, but in early cases the scan may seem normal. Atrophy can be either bilateral or asymmetric. Registration of images at different points of time (e.g., one year apart) can show evidence of atrophy that otherwise at individual time points may be reported as normal. Many research groups have begun using techniques such as magnetic resonance spectroscopy, functional imaging, and cortical thickness measurements in an attempt to offer an earlier diagnosis to the FTD patient. Fluorine-18-fluorodeoxyglucose positron emission tomography scans classically show frontal and/or anterior temporal hypometabolism, which helps differentiate the disease from Alzheimer’s disease, as the PET scan in Alzheimer’s disease classically shows biparietal hypometabolism.
  • #25 Frontotemporal Dementia and Frontotemporal Lobar Degeneration: Overview, Etiology, Genetic Distribution and Variation
    https://emedicine.medscape.com/article/1135164-overview
    The mode of presentation in PPA suggests a focal lesion of the left hemisphere language cortex, but a focal lesion, other than evidence of focal atrophy, is usually not found. […] Routine brain imaging with computed tomography (CT) scanning or MRI is usually remarkable only for cerebral atrophy. Some patients show impressive localized atrophy in the frontal or temporal lobe on 1 or both sides. […] Patients with frontal lobe neurobehavioral disorders (behavioral variant frontotemporal lobe dementia) often have bilateral frontal atrophy, especially involving the medial frontal cortex, sometimes with anterior temporal lobe atrophy as well.
  • #26 Frontotemporal dementia | MedLink Neurology
    https://www.medlink.com/articles/frontotemporal-dementia
    Clinically, structural magnetic resonance imaging (MRI) is best for reviewing findings. Atrophy is often asymmetric and in the early-middle stages, confined to the medial frontal and anterior temporal lobes. […] FDG-PET can be used to reveal a specific pattern of hypometabolism in the frontal and temporal lobes consistent with the synaptic dysfunction present in that region. It has been FDA approved to distinguish between frontotemporal dementia and Alzheimer disease and is particularly beneficial in the early stages of disease.
  • #27 Frontotemporal dementia – Wikipedia
    https://en.wikipedia.org/wiki/Frontotemporal_dementia
    Frontotemporal dementia (FTD) is traditionally difficult to diagnose owing to the diverse nature of the associated symptoms. Signs and symptoms are classified into three groups based on the affected functions of the frontal and temporal lobes: These are behavioural variant frontotemporal dementia, semantic dementia, and progressive nonfluent aphasia. An overlap between symptoms can occur as the disease progresses and spreads through the brain regions. […] Structural MRI scans often reveal frontal lobe and/or anterior temporal lobe atrophy, but in early cases the scan may seem normal. Atrophy can be either bilateral or asymmetric. Registration of images at different points of time (e.g., one year apart) can show evidence of atrophy that otherwise at individual time points may be reported as normal. Many research groups have begun using techniques such as magnetic resonance spectroscopy, functional imaging, and cortical thickness measurements in an attempt to offer an earlier diagnosis to the FTD patient. Fluorine-18-fluorodeoxyglucose positron emission tomography scans classically show frontal and/or anterior temporal hypometabolism, which helps differentiate the disease from Alzheimer’s disease, as the PET scan in Alzheimer’s disease classically shows biparietal hypometabolism.
  • #28 Diagnosing FTD | AFTD
    https://www.theaftd.org/for-health-professionals/diagnosing-ftd/
    FTD is frequently misdiagnosed as Alzheimer’s, depression, Parkinson’s disease, or a psychiatric condition. On average, it currently takes 3.6 years to get an accurate diagnosis. […] With the exception of occasional genetic causes, today there is no single test that can diagnose FTD with certainty. The diagnosis of FTD requires a thorough history, verified by a caregiver, and a neurological examination. […] When the diagnosis is uncertain, referral to a neurologist with an interest in cognition and behavior and/or a geriatric neuropsychologist is indicated. […] All patients should be screened for obstructive sleep apnea (OSA), as executive dysfunction and behavior changes are common in OSA. […] Blood work should be done to exclude alternative causes of cognitive symptoms, including a basic metabolic panel, CBC, RPR, ESR, B12 level and thyroid studies.
  • #29
    https://www.nhs.uk/conditions/frontotemporal-dementia/
    Frontotemporal dementia is an uncommon type of dementia that causes problems with behaviour and language. […] Dementia is the name for problems with mental abilities caused by gradual changes and damage in the brain. […] Frontotemporal dementia affects the front and sides of the brain (the frontal and temporal lobes). […] Most cases are diagnosed in people aged 45-65, although it can also affect younger or older people. […] The GP can do some simple checks to try to find the cause of your symptoms, and they can refer you to a memory clinic or another specialist for further tests if needed. […] There’s no single test for frontotemporal dementia. […] The following may be needed to make a diagnosis: an assessment of symptoms it’s normally helpful to have somebody who knows the person well to give an account of their symptoms, especially as someone with frontotemporal dementia may not be aware of changes in their behaviour; an assessment of mental abilities this will usually involve a number of tasks and questions; blood tests to rule out conditions with similar symptoms; brain scans such as an MRI scan, a CT scan or a PET scan; these can detect signs of dementia and help identify which parts of the brain are most affected, or help rule out other problems with the brain; lumbar puncture to test the spinal fluid (fluid that surrounds and supports the brain and spine); this may be useful to rule out Alzheimer’s disease as the cause of symptoms. […] Read more about the tests used to diagnose dementia.
  • #30 Diagnosing FTD | AFTD
    https://www.theaftd.org/for-health-professionals/diagnosing-ftd/
    FTD is frequently misdiagnosed as Alzheimer’s, depression, Parkinson’s disease, or a psychiatric condition. On average, it currently takes 3.6 years to get an accurate diagnosis. […] With the exception of occasional genetic causes, today there is no single test that can diagnose FTD with certainty. The diagnosis of FTD requires a thorough history, verified by a caregiver, and a neurological examination. […] When the diagnosis is uncertain, referral to a neurologist with an interest in cognition and behavior and/or a geriatric neuropsychologist is indicated. […] All patients should be screened for obstructive sleep apnea (OSA), as executive dysfunction and behavior changes are common in OSA. […] Blood work should be done to exclude alternative causes of cognitive symptoms, including a basic metabolic panel, CBC, RPR, ESR, B12 level and thyroid studies.
  • #31 Frontotemporal dementia – Symptoms, diagnosis and treatment | BMJ Best Practice
    https://bestpractice.bmj.com/topics/en-gb/968?g=w_bmj_bp
    1st investigations to order include formal cognitive testing, brain MRI, brain CT, FBC, serum CRP, serum thyroid-stimulating hormone (TSH), free thyroxine (T4), metabolic panel, serum urea, serum creatinine, LFTs, serum vitamin B12 levels, serum folate levels, syphilis serology, HIV testing, and serum enzyme-linked immunosorbent assay. […] Investigations to consider include brain fluorodeoxyglucose (FDG)-PET, brain single-photon emission computed tomography (SPECT), brain biopsy, genetic testing, connective tissue panel, and serum erythrocyte sedimentation rate. […] Emerging tests include cerebrospinal fluid analysis.
  • #32
    https://link.springer.com/article/10.1007/s13670-021-00360-y
    Obtaining clinical history from family verifying the gradual and progressive behavioral change is the most essential step toward making a diagnosis of bvFTD. […] Neuropsychological testing may be helpful in distinguishing bvFTD symptoms from other dementia syndromes. […] Neurological examination is an important component in suspected bvFTD. […] There are no characteristic laboratory findings for bvFTD. […] Neuroimaging may further support a diagnosis of bvFTD. […] When the diagnosis remains elusive, nuclear imaging such as fluorodeoxyglucose (FDG) – positron emission tomography (PET) or single-photon emission tomography (SPECT) may be used to elucidate abnormal patterns of brain function. […] Every patient should receive a cognitive performance screening test. […] The classic testing pattern for bvFTD includes deficits in executive function, impaired working memory/concentration, as well as difficulty with encoding and retrieval.
  • #33 Diagnosis of Frontotemporal Dementia | PortalCLÍNIC
    https://www.clinicbarcelona.org/en/assistance/diseases/frontotemporal-dementia/diagnosis
    Diagnosis of frontotemporal dementia is clinical, which means the medical professional makes the diagnosis based on interviewing the patient (anamnesis) and neurological examination. […] Moreover, tests are carried out to support the diagnosis and rule out other diseases, but these must always be interpreted through the lens of the patient’s symptoms. […] Neuropsychological tests. These tests objectively assess the patient’s cognitive abilities (memory, language, reasoning, etc.). The patients score is compared to the general population of the same age and educational level, as both of these factors affect the test results. […] Blood analysis. General health parameters that may affect brain function are assessed. Currently, no blood marker can reliably confirm the diagnosis of frontotemporal dementia.
  • #34 Diagnosing FTD | AFTD
    https://www.theaftd.org/for-health-professionals/diagnosing-ftd/
    When a family history is positive, genetic testing of the diagnosed patient can be undertaken. […] A full neuropsychological testing evaluation should be used to better assess the pattern of cognitive loss in an individual suspected of having FTD and to help rule out psychiatric etiologies for an individual’s symptoms. […] Brain imaging is indicated in all individuals with symptoms of FTD to rule out structural causes. […] The MRI is more sensitive for assessing vascular changes and subtle patterns of atrophy, but it requires an individual to lie still for 15 to 30 minutes. […] Lumbar puncture is another test that can be used to rule out mimicking conditions (infection, immune etiologies, carcinomatous and paraneoplastic syndromes). […] Many primary care physicians are uncomfortable making the diagnosis of FTD. […] A definitive diagnosis of FTD can only be made post-mortem via autopsy of the brain. […] FTD disorders are identified by their clinical symptoms which present as behavioral/cognitive changes, or language deficits, or decline in motor function.
  • #35 Diagnosis of Frontotemporal Dementia | PortalCLÍNIC
    https://www.clinicbarcelona.org/en/assistance/diseases/frontotemporal-dementia/diagnosis
    Structural neuroimaging. Radiological neuroimaging techniques such as computed tomography (CT) and magnetic resonance imaging (MRI) provide a structural image of the brain, which allows the pattern of brain atrophy to be assessed. […] Functional neuroimaging. Other nuclear medicine techniques, such as positron emission tomography, use substances with a small dose of radioactivity to provide an image of how the brain’s metabolism works (fluorodeoxyglucose PET and DAT-SPECT). […] Lumbar puncture. This test allows a sample of cerebrospinal fluid (the fluid surrounding the brain and spinal cord) to be obtained. Because it is in contact with the brain, it provides us with important information about problems involving this organ. Cerebrospinal fluid studies can reliably distinguish frontotemporal dementia from other dementias such as Alzheimer’s disease. […] Genetic testing. In cases of familial frontotemporal dementia, tests can be conducted to study potential genetic mutations. These genetic studies can be carried out using a blood test.
  • #36 Diagnosis of Frontotemporal Dementia | PortalCLÍNIC
    https://www.clinicbarcelona.org/en/assistance/diseases/frontotemporal-dementia/diagnosis
    Structural neuroimaging. Radiological neuroimaging techniques such as computed tomography (CT) and magnetic resonance imaging (MRI) provide a structural image of the brain, which allows the pattern of brain atrophy to be assessed. […] Functional neuroimaging. Other nuclear medicine techniques, such as positron emission tomography, use substances with a small dose of radioactivity to provide an image of how the brain’s metabolism works (fluorodeoxyglucose PET and DAT-SPECT). […] Lumbar puncture. This test allows a sample of cerebrospinal fluid (the fluid surrounding the brain and spinal cord) to be obtained. Because it is in contact with the brain, it provides us with important information about problems involving this organ. Cerebrospinal fluid studies can reliably distinguish frontotemporal dementia from other dementias such as Alzheimer’s disease. […] Genetic testing. In cases of familial frontotemporal dementia, tests can be conducted to study potential genetic mutations. These genetic studies can be carried out using a blood test.
  • #37 Frontotemporal dementia – Wikipedia
    https://en.wikipedia.org/wiki/Frontotemporal_dementia
    Recent studies over several years have developed new criteria for the diagnosis of behavioral variant frontotemporal dementia (bvFTD). The confirmatory diagnosis is made by brain biopsy, but other tests can be used to help, such as MRI, EEG, CT, and physical examination and history. Of the six features, three must be present in a patient to diagnose one with possible bvFTD. Similar to standard FTD, the primary diagnosis stems from clinical trials that identify the associated symptoms, instead of imaging studies. […] A 2021 study determined that using cerebrospinal fluid (CSF) biomarkers of pathologic amyloid plaques, tangles, and neurodegeneration, collectively called ATN, can be useful in diagnosing FTD.
  • #38 Frontotemporal Dementias
    https://practicalneurology.com/articles/2019-june/frontotemporal-dementias
    Reliance on collateral history, and subtle changes on neuroimaging can make diagnosis of bvFTD challenging. […] Patient insight is often limited, and so taking a history from a reliable informant who knows the patient well is essential. Standardized questionnaires may be used, such as the Frontal Behavioral Inventory. A comprehensive neurological examination should include assessment of vertical saccades, cortical sensation, praxis, primitive reflexes, and the presence of axial and appendicular parkinsonism. Brain imaging with CT or preferably MRI is essential to rule out alternative structural or vascular causes and to assess for characteristic patterns of atrophy, although detection of atrophy may be challenging, even for neuroradiologists. […] There are no validated biomarkers that reliably distinguish FTD from controls or other nonAD dementias; however, candidate CSF biomarkers are currently under investigation, including neurofilament light chain and dipeptide repeat proteins.
  • #39 Frontotemporal dementia: diagnosis, deficits and management
    https://pmc.ncbi.nlm.nih.gov/articles/PMC4824317/
    Frontotemporal dementia (FTD) is a progressive neurologic syndrome with diverse clinical presentations and attendant underlying pathologies. […] Comprehensive clinical assessment in combination with evaluation of language, socio-emotional functioning, cognition and neuroimaging aid in accurate and early diagnosis and treatment planning. […] This review underscores the importance of identification of these deficits to aid in early and accurate clinical diagnosis and management. […] The average survival time from diagnosis to death can be as brief as 3 years for bvFTD patients with motor neuron disease and up to 12 years in patients with svPPA. […] The ability to predict the underlying pathology in FTD syndromes in vivo has been a major focus of research. […] The international consortium proposed new criteria from 2011 provide three levels of diagnostic certainty for a diagnosis of bvFTD: possible, probable and definite.
  • #40 Frontotemporal dementia: diagnosis, deficits and management
    https://pmc.ncbi.nlm.nih.gov/articles/PMC4824317/
    A summary of these criteria can be found in Box 1. Possible bvFTD requires a patient to have a progressive deterioration of behavior accompanied by three out of six core features. […] Definite bvFTD requires presence of the clinical syndrome with genetic or pathological confirmation of FTLD. […] Clinical diagnosis of svPPA requires both impaired confrontation naming, and single-word comprehension, with at least 3 out of 4 additional core features. […] An imaging-supported diagnosis of nfvPPA requires neuroimaging consistent with this syndrome, and must show either predominant left posterior fronto-insular atrophy on MRI, or predominant left posterior fronto-insular hypoperfusion or hypometabolism on SPECT or PET, or both. […] To date, no disease-modifying treatments exist that change the course of this neurodegenerative disease, and as such, the focus of treatment across subtypes is necessarily symptomatic. […] The combination of comprehensive history, neuropsychological testing, measures of social cognition, as well as advances in neuroimaging modalities, all aid in earlier and more accurate diagnosis of FTD.
  • #41 Frontotemporal Dementias
    https://practicalneurology.com/articles/2019-june/frontotemporal-dementias
    Careful clinical, neuropsychologic, and imaging investigations make differential diagnosis of variants possible. […] After Alzheimers disease (AD), FTD is the second most common neurodegenerative dementia with onset before age 65 (ie, early-onset dementia). There is general consensus that the prevalence of FTD is underestimated because of misdiagnosis or lack of recognition, primarily by nonneurologists. […] An international consortium developed revised guidelines for diagnosis of bvFTD. The validation process retrospectively reviewed clinical records and compared the sensitivity of proposed and earlier criteria in a multisite sample of patients with pathologically verified FTLD. According to the revised criteria, possible bvFTD requires 3 of 6 clinically discriminating features (disinhibition, apathy/inertia, loss of sympathy/empathy, perseverative/compulsive behaviours, hyperorality, and dysexecutive neuropsychologic profile).
  • #42 Frontotemporal Dementia (FTD) – PsychDB
    https://www.psychdb.com/geri/dementia/frontotemporal
    Frontotemporal dementia (FTD), also known as frontotemporal lobar degeneration (FTLD), or less commonly, Pick’s disease, is one of the most common causes of dementia in adults younger than 60 years. […] The criteria are met for major or mild neurocognitive disorder. […] The disturbance has insidious onset and gradual progression. […] Probable frontotemporal neurocognitive disorder is diagnosed if either of the following is present; otherwise, possible frontotemporal neurocognitive disorder should be diagnosed: Evidence of a causative frontotemporal neurocognitive disorder genetic mutation, from either family history or genetic testing. […] Evidence of disproportionate frontal and/or temporal lobe involvement from neuroimaging. […] Possible frontotemporal neurocognitive disorder is diagnosed if there is no evidence of a genetic mutation, and neuroimaging has not been performed.
  • #43 Frontotemporal Dementia (FTD) – PsychDB
    https://www.psychdb.com/geri/dementia/frontotemporal
    The International Behavioural Variant FTD Criteria Consortium (FTDC) developed revised guidelines for the diagnosis of bvFTD in 2011 that are more comprehensive than the DSM-5. […] The following symptom must be present to meet criteria for bvFTD: Shows progressive deterioration of behaviour and/or cognition by observation or history (as provided by a knowledgeable informant). […] 3 of the following behavioural/cognitive symptoms (AF) must be present to meet criteria. Ascertainment requires that symptoms be persistent or recurrent, rather than single or rare events. […] All of the following symptoms (AC) must be present to meet criteria. […] Imaging results consistent with bvFTD [1 of the following (C1C2) must be present]: Frontal and/or anterior temporal atrophy on MRI or CT. […] There are no approved treatments for FTD. All current treatments are thus off-label and used to manage symptoms of the disease, primarily behavioural symptoms. […] Evidence suggests cholinesterase inhibitors may actually exacerbate behavioural symptoms.
  • #44 Frontotemporal dementia: diagnosis, deficits and management
    https://pmc.ncbi.nlm.nih.gov/articles/PMC4824317/
    A summary of these criteria can be found in Box 1. Possible bvFTD requires a patient to have a progressive deterioration of behavior accompanied by three out of six core features. […] Definite bvFTD requires presence of the clinical syndrome with genetic or pathological confirmation of FTLD. […] Clinical diagnosis of svPPA requires both impaired confrontation naming, and single-word comprehension, with at least 3 out of 4 additional core features. […] An imaging-supported diagnosis of nfvPPA requires neuroimaging consistent with this syndrome, and must show either predominant left posterior fronto-insular atrophy on MRI, or predominant left posterior fronto-insular hypoperfusion or hypometabolism on SPECT or PET, or both. […] To date, no disease-modifying treatments exist that change the course of this neurodegenerative disease, and as such, the focus of treatment across subtypes is necessarily symptomatic. […] The combination of comprehensive history, neuropsychological testing, measures of social cognition, as well as advances in neuroimaging modalities, all aid in earlier and more accurate diagnosis of FTD.
  • #45 Frontotemporal dementia: diagnosis, deficits and management
    https://pmc.ncbi.nlm.nih.gov/articles/PMC4824317/
    A summary of these criteria can be found in Box 1. Possible bvFTD requires a patient to have a progressive deterioration of behavior accompanied by three out of six core features. […] Definite bvFTD requires presence of the clinical syndrome with genetic or pathological confirmation of FTLD. […] Clinical diagnosis of svPPA requires both impaired confrontation naming, and single-word comprehension, with at least 3 out of 4 additional core features. […] An imaging-supported diagnosis of nfvPPA requires neuroimaging consistent with this syndrome, and must show either predominant left posterior fronto-insular atrophy on MRI, or predominant left posterior fronto-insular hypoperfusion or hypometabolism on SPECT or PET, or both. […] To date, no disease-modifying treatments exist that change the course of this neurodegenerative disease, and as such, the focus of treatment across subtypes is necessarily symptomatic. […] The combination of comprehensive history, neuropsychological testing, measures of social cognition, as well as advances in neuroimaging modalities, all aid in earlier and more accurate diagnosis of FTD.
  • #46 Frontotemporal dementia | MedLink Neurology
    https://www.medlink.com/articles/frontotemporal-dementia
    Similar to bvFTD, the criteria for diagnosis of nfvPPA and svPPA have been significantly revised to facilitate scientific exchange across centers. In the new International Consensus Criteria outlined for primary progressive aphasia, establishing a diagnosis is based on a 2-step process. First, a patient must meet criteria for Mesulam’s guidelines for primary progressive aphasia. If the patient meets criteria for Step 1, then his or her language disturbance is further parcellated into one of three variants: nonfluent variant primary progressive aphasia, semantic variant primary progressive aphasia, and logopenic variant primary progressive aphasia. […] A comprehensive diagnostic evaluation for frontotemporal dementia should include a clinical interview, neurologic exam, neuropsychological assessment, and review of neuroimaging.
  • #47 Getting a diagnosis of frontotemporal dementia | Alzheimer’s Society
    https://www.alzheimers.org.uk/about-dementia/types-dementia/frontotemporal-dementia-diagnosis
    Standard tests of mental abilities, which mostly focus on memory loss, can be less helpful in diagnosing FTD. More specialised tests of social awareness or behaviour may be needed. […] CT (computerised tomography) and MRI (magnetic resonance imaging) scans are used to see what parts of the brain are most damaged. They can also rule out other possible causes of a persons symptoms, such as a stroke or tumour. […] A specialist may recommend that a person with FTD symptoms has a genetic test. This can show if the persons condition is caused by a specific faulty gene. […] After a person dies, it is possible to make a more certain diagnosis of FTD. This is because the brain can be seen directly in post-mortem examinations.
  • #48 Frontotemporal dementia diagnosis – Alzheimer’s Research UK
    https://www.alzheimersresearchuk.org/dementia-information/types-of-dementia/frontotemporal-dementia/diagnosis/
    If your doctor suspects dementia, they may refer you to a memory clinic or another specialist doctor. Here you may have another physical check-up and complete some more memory and thinking tests. FTD is an uncommon form of dementia, not often seen by doctors. It can take longer than usual to get the right diagnosis. […] In situations where there is a strong family history of FTD, you may be offered genetic counselling and testing as part of your diagnosis. This is to identify whether you have a faulty gene inherited by a parent who also had FTD. […] FTD is an uncommon form of dementia, not often seen by doctors. Therefore it can take longer than usual to get the right diagnosis.
  • #49 Athena Diagnostics
    https://www.athenadiagnostics.com/view-full-catalog/frontotemporal-dementia-ftd-evaluation1
    Test code: 281 Type of disorder: Dementia Disease(s) tested for: Frontotemporal Dementia Genes Included: C9ORF72, MAPT, GRN, Tests included: C9ORF72 (FTD) DNA TestGRN DNA Sequencing TestMAPT DNA Sequencing Test Informed Consent Required: This test requires physician attestation that patient consent has been received […] Clinical Significance: Detects expansions in the C9ORF72 gene. Detects mutations in the MAPT and GRN genes associated with frontotemporal dementia Typical Presentation: Typical presentation includes behavioral variant and primary progressive aphasia. Behavioral variant FTD includes inappropriate social behavior, lack of empathy, changes in appetite, agitation, blunted emotions, neglect of personal hygiene, compulsive behavior, and lack of energy and motivation. Primary progressive aphasia features symptoms of language disturbance, such as difficulty making or understanding speech. Indications for testing: Onset of symptoms before 65 years of age, positive family history of neurodegenerative conditions, atypical Alzheimer’s symptoms such as prominent neurobehavior features and/or absence of significant amnesia […] Methodology: Next Generation Sequencing, Repeat Expansion Detection by PCR Reference Range: No sequencing variants detected for MAPT and GRN genes. For C9ORF72: Normal
  • #50 Frontotemporal dementia: MedlinePlus Medical EncyclopediaLock
    https://medlineplus.gov/ency/article/000744.htm
    Frontotemporal dementia (FTD) is a rare form of dementia that is similar to Alzheimer disease, except that it tends to affect only certain areas of the brain. […] FTD is diagnosed based on symptoms and results of tests, including: […] Tests may be ordered to check for other causes of dementia, including dementia due to metabolic causes. […] Genetic tests can find mutations known to cause FTD and can support the diagnosis. A brain biopsy can also confirm the diagnosis, although it’s usually never recommended. […] Newer methods that test brain metabolism or protein deposits may better allow for more accurate diagnosis in the future.
  • #51 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 are a common cause of early-onset dementia. […] There is currently no cure for FTD or related disorders, and no treatments to slow or stop the progression of the disease, but there are ways to help manage the symptoms. […] This progress is opening doors to better diagnosis, improved care, and eventually, new treatments. […] Physicians and psychologists diagnose the different forms of FTD based on a persons symptoms as well as the results of brain scans and genetic tests. […] FTD can be hard to diagnose because the symptoms resemble those of other conditions. […] A diagnosis of FTD can only be confirmed by genetic tests (in familial cases) or a brain autopsy after death. […] Researchers are studying ways to diagnose FTD earlier and more accurately, and to distinguish these conditions from other types of dementia. […] One area of research involves biomarkers, such as proteins or other substances in the blood or cerebrospinal fluid, which can be used to help diagnose FTD and measure disease progression.
  • #52 Getting a diagnosis of frontotemporal dementia | Alzheimer’s Society
    https://www.alzheimers.org.uk/about-dementia/types-dementia/frontotemporal-dementia-diagnosis
    Standard tests of mental abilities, which mostly focus on memory loss, can be less helpful in diagnosing FTD. More specialised tests of social awareness or behaviour may be needed. […] CT (computerised tomography) and MRI (magnetic resonance imaging) scans are used to see what parts of the brain are most damaged. They can also rule out other possible causes of a persons symptoms, such as a stroke or tumour. […] A specialist may recommend that a person with FTD symptoms has a genetic test. This can show if the persons condition is caused by a specific faulty gene. […] After a person dies, it is possible to make a more certain diagnosis of FTD. This is because the brain can be seen directly in post-mortem examinations.
  • #53 FTD Misdiagnosis | Memory and Aging Center
    https://memory.ucsf.edu/ftd-misdiagnosis
    People with frontotemporal dementia (FTD) are often misdiagnosed with Alzheimers disease (AD), psychiatric disorders, vascular dementia or Parkinsons disease. The early symptoms and the brain image are often the most helpful tools to reach the right diagnosis. […] Once other likely explanations have been ruled out, an FTD diagnosis is made by looking at the data from a neurological exam and personal history (which may come from the patient, family, or another caregiver); neuropsychological tests that help quantify memory, language, and other cognitive skills; and a brain image usually an MRI (magnetic resonance imaging) scan but perhaps a functional scan like PET (positron emission tomography), which can show increased or lowered brain activity in the frontal and anterior temporal areas.
  • #54 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. […] Diagnosing FTD. The Association for Frontotemporal Degeneration. […] Frontotemporal dementia: Treatment. […] What are frontotemporal disorders? Causes, symptoms and treatment.
  • #55 Diagnosing FTD | AFTD
    https://www.theaftd.org/for-health-professionals/diagnosing-ftd/
    FTD is frequently misdiagnosed as Alzheimer’s, depression, Parkinson’s disease, or a psychiatric condition. On average, it currently takes 3.6 years to get an accurate diagnosis. […] With the exception of occasional genetic causes, today there is no single test that can diagnose FTD with certainty. The diagnosis of FTD requires a thorough history, verified by a caregiver, and a neurological examination. […] When the diagnosis is uncertain, referral to a neurologist with an interest in cognition and behavior and/or a geriatric neuropsychologist is indicated. […] All patients should be screened for obstructive sleep apnea (OSA), as executive dysfunction and behavior changes are common in OSA. […] Blood work should be done to exclude alternative causes of cognitive symptoms, including a basic metabolic panel, CBC, RPR, ESR, B12 level and thyroid studies.
  • #56
    https://www.cbsnews.com/news/frontotemporal-dementia-bruce-willis-diagnosis-explained/
    Wendy Williams has been diagnosed with frontotemporal dementia, or FTD, which is used to describe a group of brain disorders that affect the brain’s frontal and temporal lobes. These parts of the brain are associated with personality, behavior and language. […] It can be hard to diagnose. […] „Oftentimes patients can just present with behavioral problems, their personality can change. But as opposed to something like Alzheimer’s, there’s no clear test to definitively make the diagnosis,” Dr. Gayatri Devi, a clinical professor of neurology at Northwell Health who specializes in dementia, explained on „CBS Mornings.” […] „Frontotemporal dementia is when you have loss of nerve cells in the brain that causes cognitive issues much like other types of dementias including Alzheimer’s [or] Lewy body, frontotemporal is a type of dementia,” Devi said.
  • #57 Frontotemporal Dementia: A Review for Primary Care Physicians | AAFP
    https://www.aafp.org/pubs/afp/issues/2010/1201/p1372.html
    Frontotemporal dementia (FTD) is one of the most common forms of dementia in persons younger than 65 years. […] A careful history and physical examination, and judicious use of magnetic resonance imaging, can help distinguish FTD from other common forms of dementia, including Alzheimer disease, dementia with Lewy bodies, and vascular dementia. […] The diagnosis of FTD requires a thorough history, especially family history of dementia, and physical examination, with a focus on the timing and rate of progression of symptoms, and behavioral and personality changes over the previous months or years. […] Clinical consensus criteria for diagnosing FTD, also referred to as the Neary criteria, were published in 1998. […] Neuroimaging (magnetic resonance imaging) is helpful, even essential, in the diagnosis of FTD, and usually demonstrates focal atrophy in addition to excluding other etiologies.
  • #58 Frontotemporal Dementia and Frontotemporal Lobar Degeneration: Overview, Etiology, Genetic Distribution and Variation
    https://emedicine.medscape.com/article/1135164-overview
    To date, most efforts have concentrated on diagnosing FTD and understanding its pathogenesis. […] All current pharmacologic treatments are unproved, but selective serotonin reuptake inhibitor (SSRI) antidepressants and trazodone are widely recommended. […] The findings in electroencephalography (EEG) are commonly abnormal in FTD, often showing focal slowing of electrical activity over 1 or both frontal or temporal lobes. […] Other than brain imaging studies, the most specific tests for evaluating frontotemporal lobe dementia (FTD) are evaluation with standardized language batteries and neuropsychological testing. […] Preservation of many of these functions distinguishes FTD and primary progressive aphasia (PPA) syndromes from Alzheimer disease. […] In distinguishing FTD from Alzheimer disease, the involvement of specific cognitive functions is the most important differentiating factor.
  • #59 Frontotemporal dementia – Wikipedia
    https://en.wikipedia.org/wiki/Frontotemporal_dementia
    Frontotemporal dementia (FTD) is traditionally difficult to diagnose owing to the diverse nature of the associated symptoms. Signs and symptoms are classified into three groups based on the affected functions of the frontal and temporal lobes: These are behavioural variant frontotemporal dementia, semantic dementia, and progressive nonfluent aphasia. An overlap between symptoms can occur as the disease progresses and spreads through the brain regions. […] Structural MRI scans often reveal frontal lobe and/or anterior temporal lobe atrophy, but in early cases the scan may seem normal. Atrophy can be either bilateral or asymmetric. Registration of images at different points of time (e.g., one year apart) can show evidence of atrophy that otherwise at individual time points may be reported as normal. Many research groups have begun using techniques such as magnetic resonance spectroscopy, functional imaging, and cortical thickness measurements in an attempt to offer an earlier diagnosis to the FTD patient. Fluorine-18-fluorodeoxyglucose positron emission tomography scans classically show frontal and/or anterior temporal hypometabolism, which helps differentiate the disease from Alzheimer’s disease, as the PET scan in Alzheimer’s disease classically shows biparietal hypometabolism.
  • #60 Frontotemporal Dementia (FTD) – Neurologic Disorders – Merck Manual Professional Edition
    https://www.merckmanuals.com/professional/neurologic-disorders/delirium-and-dementia/frontotemporal-dementia-ftd
    CT and MRI are done to determine location and extent of brain atrophy and to exclude other possible causes (eg, brain tumors, abscesses, stroke). […] FTDs are characterized by severely atrophic, sometimes paper-thin gyri in the temporal and frontal lobes. […] However, MRI or CT may not show these changes until late in FTD. […] Thus, FTDs and Alzheimer disease can usually be differentiated more easily by clinical criteria. […] For example, primary progressive aphasia differs from Alzheimer disease in that memory and visuospatial function are preserved and syntax and fluency are impaired. […] Positron emission tomography (PET) with fluorine-18 (18F)labeled deoxyglucose (fluorodeoxyglucose, or FDG) may help differentiate Alzheimer disease from FTD by showing differences in location of hypometabolic areas.
  • #61 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 are a common cause of early-onset dementia. […] There is currently no cure for FTD or related disorders, and no treatments to slow or stop the progression of the disease, but there are ways to help manage the symptoms. […] This progress is opening doors to better diagnosis, improved care, and eventually, new treatments. […] Physicians and psychologists diagnose the different forms of FTD based on a persons symptoms as well as the results of brain scans and genetic tests. […] FTD can be hard to diagnose because the symptoms resemble those of other conditions. […] A diagnosis of FTD can only be confirmed by genetic tests (in familial cases) or a brain autopsy after death. […] Researchers are studying ways to diagnose FTD earlier and more accurately, and to distinguish these conditions from other types of dementia. […] One area of research involves biomarkers, such as proteins or other substances in the blood or cerebrospinal fluid, which can be used to help diagnose FTD and measure disease progression.
  • #62 Frontotemporal dementia – Diagnosis and treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/frontotemporal-dementia/diagnosis-treatment/drc-20354741
    There’s no single test for frontotemporal dementia. Healthcare professionals consider your symptoms and exclude other possible causes of your symptoms. FTD can be hard to diagnose early because symptoms of frontotemporal dementia often overlap with those of other conditions. Healthcare professionals may order the following tests. […] Healthcare professionals may test your reasoning and memory skills. This type of testing is especially helpful to learn which type of dementia you may have at an early stage. It also can help distinguish FTD from other causes of dementia. […] There’s hope that diagnosing frontotemporal dementia may become easier in the future. Researchers are studying potential biomarkers of FTD. Biomarkers are substances that can be measured to help diagnose a disease. […] In addition to a physical exam, your healthcare professional checks your neurological health. This is done by testing things such as your balance, muscle tone and strength. You also may have a brief mental status evaluation to check your memory and thinking skills.
  • #63 Frontotemporal Dementia | Emory University | Atlanta GA
    https://alzheimers.emory.edu/healthy_aging/articles/frontotemporal-dementia.html
    Frontotemporal dementia (FTD) is the second most common dementing illness in those under the age of 65. […] FTD is an umbrella term. Your neurologist may give a more specific diagnosis based on your medical history and symptoms. Possible FTD diagnosis include: […] A number of unique neuropathological changes have been identified in FTD. Scientists at Emory and elsewhere are working on how to tell these changes apart through clinical examination, Magnetic Resonance Imaging (MRI), testing of blood or spinal fluid, or a combination of these tests. […] Some patients show changes in a protein called TDP-43. These cases are referred to as FTLD-TDP. […] Some patients have changes associated with an abnormal form of the protein Tau. Neurologists and pathologists refer to these cases collectively as FTLD-Tau or tauopathy, although they may refine their diagnosis with more specific terms such as Picks disease, Corticobasal Degeneration (CBD), Progressive Supranuclear palsy (PSP), etc. […] A small group of patients have changes in a protein called FUS, and this group is known to have FTLD-FUS.
  • #64 Advancing frontotemporal dementia diagnosis through biomarkers and imaging | VJDementia
    https://www.vjdementia.com/video/fxb3hntsonu-advancing-frontotemporal-dementia-diagnosis-through-biomarkers-and-imaging/
    Bradford Dickerson highlights the potential for new biomarkers and imaging methods to improve diagnosis and understanding of FTD, which is currently challenging due to its variability and difficulty in distinguishing between tau-related and TDP-43-related forms. […] There’s also a tremendous amount of excitement about new biomarkers and imaging methods to measure the progression or type of frontotemporal dementia that hopefully will be in a position in the next few years to be more similar to Alzheimer’s disease and some of the emerging biomarkers in Lewy body disease where we can have a more precise diagnosis of the underlying form of FTD. […] Because it’s difficult to know right now whether it’s tau-related or TDP43-related and hopefully in the next few years we’ll see more imaging or fluid biomarkers that can make that distinction more obvious.
  • #65 Advancing frontotemporal dementia diagnosis through biomarkers and imaging | VJDementia
    https://www.vjdementia.com/video/fxb3hntsonu-advancing-frontotemporal-dementia-diagnosis-through-biomarkers-and-imaging/
    Bradford Dickerson highlights the potential for new biomarkers and imaging methods to improve diagnosis and understanding of FTD, which is currently challenging due to its variability and difficulty in distinguishing between tau-related and TDP-43-related forms. […] There’s also a tremendous amount of excitement about new biomarkers and imaging methods to measure the progression or type of frontotemporal dementia that hopefully will be in a position in the next few years to be more similar to Alzheimer’s disease and some of the emerging biomarkers in Lewy body disease where we can have a more precise diagnosis of the underlying form of FTD. […] Because it’s difficult to know right now whether it’s tau-related or TDP43-related and hopefully in the next few years we’ll see more imaging or fluid biomarkers that can make that distinction more obvious.
  • #66 An MRI-based strategy for differentiation of frontotemporal dementia and Alzheimer’s disease | Alzheimer’s Research & Therapy | Full Text
    https://alzres.biomedcentral.com/articles/10.1186/s13195-020-00757-5
    The differential diagnosis of frontotemporal dementia (FTD) and Alzheimers disease (AD) is difficult due to the overlaps of clinical symptoms. […] In this study, we aim at deriving a novel integrated index by leveraging the volumetric measures in brain regions with significant difference between AD and FTD and developing an MRI-based strategy for the differentiation of FTD and AD. […] A novel MRI index, named the frontotemporal dementia index (FTDI), was derived as the ratio between the weighted sum of the volumetric indexes in FTD dominant structures over that obtained from AD dominant structures. […] The differentiation performance of FTDI was validated using independent data from ADNI and FTLDNI databases. […] FTDI showed excellent accuracy in identifying FTD from AD (AUC=0.90; SEN=89%, SPE=75% with threshold value=1.08).
  • #67 An MRI-based strategy for differentiation of frontotemporal dementia and Alzheimer’s disease | Alzheimer’s Research & Therapy | Full Text
    https://alzres.biomedcentral.com/articles/10.1186/s13195-020-00757-5
    By combining AD-RAI and FTDI, an MRI-based decision strategy was further proposed as a promising solution for the differential diagnosis of AD and FTD in clinical practice. […] The primary objective of this study is to investigate the MRI volumetric and atrophic patterns among FTD, AD, and normal controls (NC) collected from multicenter data. […] In addition to AD-RAI, in this study, a novel MRI-based biomarker named FTDI was derived based on the volumetric results of brain regions with significant volume difference between AD and FTD using the NACC data. […] The proposed strategy is more accurate, objective, and easier to implement.
  • #68 Frontotemporal dementia | MedLink Neurology
    https://www.medlink.com/articles/frontotemporal-dementia
    Similar to bvFTD, the criteria for diagnosis of nfvPPA and svPPA have been significantly revised to facilitate scientific exchange across centers. In the new International Consensus Criteria outlined for primary progressive aphasia, establishing a diagnosis is based on a 2-step process. First, a patient must meet criteria for Mesulam’s guidelines for primary progressive aphasia. If the patient meets criteria for Step 1, then his or her language disturbance is further parcellated into one of three variants: nonfluent variant primary progressive aphasia, semantic variant primary progressive aphasia, and logopenic variant primary progressive aphasia. […] A comprehensive diagnostic evaluation for frontotemporal dementia should include a clinical interview, neurologic exam, neuropsychological assessment, and review of neuroimaging.
  • #69 Frontotemporal Dementia: Diagnosis | Stanford Health Care
    https://stanfordhealthcare.org/medical-conditions/brain-and-nerves/frontotemporal-dementia/diagnosis.html
    Family members are often the first to notice subtle changes in a persons behavior or language skills. Its important for a person to see a doctor as early as possible to discuss: […] No single test can diagnose frontotemporal dementia. Typically, doctors will order routine blood tests and perform physical exams to rule out other conditions that cause similar symptoms. If they suspect dementia, they may: […] Evaluate the persons neurological healthreflexes, muscle strength, muscle tone, sense of touch and sight, coordination, and balance […] Assess the persons neuropsychological statusmemory, problem-solving ability, attention span and counting skills–and language abilities […] Order magnetic resonance imaging, computed tomography or PET scans of the brain […] Genetic testing.
  • #70 Frontotemporal Dementias
    https://practicalneurology.com/articles/2019-june/frontotemporal-dementias
    Reliance on collateral history, and subtle changes on neuroimaging can make diagnosis of bvFTD challenging. […] Patient insight is often limited, and so taking a history from a reliable informant who knows the patient well is essential. Standardized questionnaires may be used, such as the Frontal Behavioral Inventory. A comprehensive neurological examination should include assessment of vertical saccades, cortical sensation, praxis, primitive reflexes, and the presence of axial and appendicular parkinsonism. Brain imaging with CT or preferably MRI is essential to rule out alternative structural or vascular causes and to assess for characteristic patterns of atrophy, although detection of atrophy may be challenging, even for neuroradiologists. […] There are no validated biomarkers that reliably distinguish FTD from controls or other nonAD dementias; however, candidate CSF biomarkers are currently under investigation, including neurofilament light chain and dipeptide repeat proteins.
  • #71 What is frontotemporal dementia? Here’s what to know about Bruce Willis’ diagnosis
    https://www.wjcl.com/article/ftd-frontotemporal-dementia-bruce-willis-diagnosis/42945484
    Because his symptoms began with difficulty speaking, Bruce Willis would be classified as having a type of FTD called primary progressive aphasia, said Dr. Henry Paulson, a professor of neurology and director of the Michigan Alzheimer’s Disease Center at the University of Michigan. […] „Because his diagnosis is frontotemporal dementia, Mr. Willis clearly has a progressive, neurodegenerative disease as opposed to a stroke or a tumor or some other lesion on the brain,” he added. […] To diagnose FTD disorders, a neurologist will conduct a careful clinical examination, paired with psychological testing designed to evaluate cognitive skills, Paulson said. […] „A brain MRI can tell us if certain parts of the brain are shrinking or showing signs of atrophy. We will do some blood tests to make sure we’re not missing some treatable causes of cognitive impairment like thyroid disease or vitamin B12 deficiency as well,” he said.
  • #72 What is frontotemporal dementia? Here’s what to know about Bruce Willis’ diagnosis
    https://www.wjcl.com/article/ftd-frontotemporal-dementia-bruce-willis-diagnosis/42945484
    „And oftentimes, we also will do brain metabolism imaging,” Paulson said. „It’s Positron Emission Tomography or PET imaging, and that can tell us which parts of the frontal lobes or the temporal lobes are involved.” […] Unlike Alzheimer’s disease, there are no current therapies to slow down the progression of FTD. […] A speech-language pathologist can help determine the best strategies and tools for an FTD patient struggling with language skills. […] „It is really important for people who have a progressive dementing syndrome like FTD to continue to eat well, exercise regularly, and stay connected with people. Those activities are not medications, they’re not curing the disease, but they can help your brain work as well as possible,” Paulson said.
  • #73 Diagnosing FTD | AFTD
    https://www.theaftd.org/for-health-professionals/diagnosing-ftd/
    FTD is frequently misdiagnosed as Alzheimer’s, depression, Parkinson’s disease, or a psychiatric condition. On average, it currently takes 3.6 years to get an accurate diagnosis. […] With the exception of occasional genetic causes, today there is no single test that can diagnose FTD with certainty. The diagnosis of FTD requires a thorough history, verified by a caregiver, and a neurological examination. […] When the diagnosis is uncertain, referral to a neurologist with an interest in cognition and behavior and/or a geriatric neuropsychologist is indicated. […] All patients should be screened for obstructive sleep apnea (OSA), as executive dysfunction and behavior changes are common in OSA. […] Blood work should be done to exclude alternative causes of cognitive symptoms, including a basic metabolic panel, CBC, RPR, ESR, B12 level and thyroid studies.
  • #74 Frontotemporal Dementia | Conditions | UCSF Health
    https://www.ucsfhealth.org/conditions/frontotemporal-dementia
    At the UCSF Memory and Aging Center, patients undergo an extensive neurological, neurophysicial and nursing assessment, usually taking about three hours. […] After the evaluation, the medical team meets to discuss the diagnosis and potential treatments and then discusses its findings with the patient and the family. […] In some cases, a diagnosis will be deferred until more information from blood tests or brain imaging is collected.
  • #75 Factsheet 11: How is FTD diagnosed? – FTD Talk
    https://www.ftdtalk.org/factsheets/how-is-ftd-diagnosed/
    Frontotemporal dementia or FTD is a progressive disorder of the brain. Diagnosis is usually made by a specialist and involves a clinical assessment and a series of tests. The initial part of the diagnostic process is an assessment by a specialist. They will go through the symptoms that have been happening and ask about problems that run in the family. Blood tests may be performed at this appointment to exclude some rare causes of dementia. It is possible to test for problems in these genes at specialist centres by taking a blood test. Brain imaging should be performed to help make a diagnosis of FTD. This may be a CT scan but more commonly is a magnetic resonance imaging or MRI scan. This is sometimes performed when trying to diagnose FTD and can be helpful to exclude other diagnoses. Apart from a genetic test there is no single test that diagnoses FTD. A specialist will gather all of the information from the different tests together to help them make a diagnosis. […] Sometimes the diagnosis is not completely clear at the first couple of appointments and it is only over time that the diagnosis becomes clear.
  • #76 Factsheet 11: How is FTD diagnosed? – FTD Talk
    https://www.ftdtalk.org/factsheets/how-is-ftd-diagnosed/
    Frontotemporal dementia or FTD is a progressive disorder of the brain. Diagnosis is usually made by a specialist and involves a clinical assessment and a series of tests. The initial part of the diagnostic process is an assessment by a specialist. They will go through the symptoms that have been happening and ask about problems that run in the family. Blood tests may be performed at this appointment to exclude some rare causes of dementia. It is possible to test for problems in these genes at specialist centres by taking a blood test. Brain imaging should be performed to help make a diagnosis of FTD. This may be a CT scan but more commonly is a magnetic resonance imaging or MRI scan. This is sometimes performed when trying to diagnose FTD and can be helpful to exclude other diagnoses. Apart from a genetic test there is no single test that diagnoses FTD. A specialist will gather all of the information from the different tests together to help them make a diagnosis. […] Sometimes the diagnosis is not completely clear at the first couple of appointments and it is only over time that the diagnosis becomes clear.
  • #77 FTD Toolkit — Eastern Cognitive Disorders Clinic
    https://ecdc.org.au/ftd-toolkit
    Frontotemporal dementia (FTD) is an unfamiliar condition to many health service providers and members of the public. […] FTD is often thought to be a relatively rare form of dementia, though this perception may be due in part to the fact that it is not yet widely understood and may therefore be under-diagnosed. For this reason, many people with FTD and their families will experience a long and difficult journey before receiving a diagnosis and access to appropriate advice and support. […] Diagnosing FTD can be a complex process which is facilitated by the input of a variety of healthcare specialists. The most important diagnostic information is a clear history of the nature, onset, and progression of symptoms. […] A thorough cognitive assessment and neuroimaging are also critical to the diagnostic process. It is important to use cognitive and behavioural screening tools which are sensitive to the specific changes displayed by individuals with FTD, as many tools may only detect changes in other forms of dementia.
  • #78 FTD Toolkit — Eastern Cognitive Disorders Clinic
    https://ecdc.org.au/ftd-toolkit
    Frontotemporal dementia (FTD) is an unfamiliar condition to many health service providers and members of the public. […] FTD is often thought to be a relatively rare form of dementia, though this perception may be due in part to the fact that it is not yet widely understood and may therefore be under-diagnosed. For this reason, many people with FTD and their families will experience a long and difficult journey before receiving a diagnosis and access to appropriate advice and support. […] Diagnosing FTD can be a complex process which is facilitated by the input of a variety of healthcare specialists. The most important diagnostic information is a clear history of the nature, onset, and progression of symptoms. […] A thorough cognitive assessment and neuroimaging are also critical to the diagnostic process. It is important to use cognitive and behavioural screening tools which are sensitive to the specific changes displayed by individuals with FTD, as many tools may only detect changes in other forms of dementia.
  • #79 Frontotemporal Dementia and Aphasia: Celebrity Diagnoses and Misconceptions – Neurology Advisor
    https://www.neurologyadvisor.com/features/frontotemporal-dementia-and-aphasia-celebrity-diagnoses-misconceptions/
    Frontotemporal dementia, also referred to as Pick Disease, presents with neuronal damage to the brains frontal and temporal lobes. […] Aphasia is a hallmark symptom of frontotemporal dementia and is identifiable by the immense impact it has on a patients behavioral and emotional capabilities. […] In his interview, Dr Paulson shares insights into common misconceptions of frontotemporal dementia, as well as challenges that physicians face in the field. He also explains differential diagnosis, etiologies, and rare presentations of frontotemporal dementia, as well as recent work conducted at his laboratory. […] A common misconception I see in the clinic is when people say, Doctor, the good news is I dont have AD, I just have dementia. Well, some dementias are not as good as AD. […] I think sometimes physicians are more comfortable telling a patient that they have aphasia, rather than saying, you have dementia.
  • #80 Frontotemporal Dementia and Aphasia: Celebrity Diagnoses and Misconceptions – Neurology Advisor
    https://www.neurologyadvisor.com/features/frontotemporal-dementia-and-aphasia-celebrity-diagnoses-misconceptions/
    Frontotemporal dementia can have a lot of mimics. […] There are different forms of primary progressive aphasia. […] I think it can be challenging even for the expert to diagnose because a condition like frontotemporal dementia, which has different clinical phenotypes, has to start with something; and that something can be a fairly subtle symptom. […] For a couple of years at minimum, the pathology of any dementia is occurring before any symptoms manifest. […] In the case of frontotemporal dementia, the genetic load is even greater. […] In those families that have a clearly inherited form of frontotemporal dementia, it can even be a little bit earlier. […] The disease tends to occur earlier. Instead of being diagnosed at age 68 or 75, its more likely to be diagnosed at age 58 or 60.
  • #81 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. […] Unlike other types of dementia, memory loss and concentration problems are less common in the early stages. […] FTD is a rare form of dementia affecting around one in 20 people with a dementia diagnosis. […] Getting a diagnosis of FTD can be difficult for reasons including: lack of awareness of the early signs, mistaking signs for other conditions such as depression, work stress and relationship problems, the misconception that dementia only occurs in older people and usually involves memory problems, the person’s reluctance to seek medical advice – this may be because they lack awareness of the changes in themselves, or are afraid of a dementia diagnosis.
  • #82 Alzheimer’s Disease and Related Dementias | LA County Department of Public Health
    http://publichealth.lacounty.gov/healthybrainla/AlzheimersDementias/frontotemporaldementia.htm
    Frontotemporal Dementia (FTD) is a group of related disorders that damage the frontal and temporal lobes of the brain. FTD is typically diagnosed in people in their 40s and early 60s. Signs and symptoms related to FTD are progressive and can worsen over time. […] There is no specific test to diagnose FTD. Doctors can perform a variety of tests including blood tests, sleep studies, and brain scans to help determine a diagnosis. Since FTD is hard to diagnose due to signs and symptoms sharing similarities with other conditions, family members or those close to the person often play an important role in sharing any early changes in behavior or language with doctors. Getting an early diagnosis can help people and their families find the resources and support they need to live with frontotemporal dementia.
  • #83 Frontotemporal dementia: Treatment – UpToDate
    https://www.uptodate.com/contents/frontotemporal-dementia-treatment
    Frontotemporal dementia (FTD) is a neuropathologically and clinically heterogeneous disorder characterized by focal degeneration of the frontal and/or temporal lobes. Age of onset is typically in the late 50s or early 60s. The primary initial clinical manifestations include changes in personality and social behavior or language, progressing over time to a more global dementia affecting other cognitive domains. A subset of patients may also exhibit symptoms of extrapyramidal or motor neuron involvement during the disease course. […] There are no current disease-modifying treatments for FTD approved by the US Food and Drug Administration (FDA), although active investigations are underway. Since the underlying pathology of FTD usually consists of tau or TAR DNA binding protein (TDP-43) aggregates, treatments geared toward beta-amyloid reduction that appear promising in Alzheimer disease (AD) are not expected to be effective for FTD.
  • #84 Frontotemporal dementia: Treatment – UpToDate
    https://www.uptodate.com/contents/frontotemporal-dementia-treatment
    Symptomatic relief can be provided by a variety of agents. However, these medications are not FDA approved for FTD and have limited evidence of efficacy. These medications should be considered in conjunction with nonpharmacologic interventions, such as thoughtful evaluation of the patient’s living environment. […] This topic discusses treatment issues specific to patients with FTD. Epidemiology, pathology, clinical features, and diagnosis of FTD, as well as other aspects of the management of dementia, are discussed separately.
  • #85
    https://link.springer.com/article/10.1007/s13670-021-00360-y
    The diagnosis and treatment of behavioral variant frontotemporal dementia is challenging and often delayed because of overlapping symptoms with more common dementia syndromes or primary psychiatric illnesses. […] Early and accurate diagnosis of bvFTD is crucial, as it allows for appropriate treatment decisions as well as adequate counseling of families and caregivers. Diagnosis is made based on presentation, course, family history, neuroimaging, and cognitive testing. […] Definitive diagnosis of bvFTD requires histopathological confirmation at autopsy. […] The first step is the clinical recognition of progressive deterioration in behavior and/or cognition. […] The International Behavioral Variant FTD Criteria Consortium guidelines provide diagnostic criterion for bvFTD. […] To meet criteria for probable bvFTD, a patient must exhibit functional disability with characteristic neuroimaging findings, in addition to the features noted for possible bvFTD.
  • #86 Frontotemporal dementia: From the clinic to the differential diagnosis – International Journal of Case Reports and Images (IJCRI)
    https://www.ijcasereportsandimages.com/archive/article-full-text/101377Z01DP2023
    Therefore, this report is relevant because it correlates a patient with neurological examination and tests with a good indication of FTD; however, the diagnosis can be confirmed with greater accuracy through brain perfusion scintigraphy. It is still possible to observe that although there has been a significant increase in the literature on FTD, its variants and its clinic still need further studies regarding their possible differential diagnoses, mainly related to psychiatric disorders and the behavioral variant of FTD. Therefore, more research is needed to define these behavioral occurrences of FTD better.
  • #87 Frontotemporal Dementia: Diagnosis, Trials & Treatment
    https://www.brightfocus.org/resource/frontotemporal-dementia-diagnosis-trials-treatment/
    The average tends to be 7 to 12 years or so. […] The vast majority of cases of FTD are what we call sporadic, meaning they dont seem to be associated with any known abnormalities of genes. […] So these three genes are really not that common, but theyre very much targets for trying to understand the underlying problems in FTD and what we can do to try to treat it. […] The goal is to develop essentially the largest worldwide placebo group of people that are essentially living with the illness and being tracked very carefully over time with the idea that new treatments hopefully will improve what the course of the illness is compared to people that are just being followed for the so-called natural history of the condition. […] The way I think about treatment is, I want to find out what are the most important symptoms that people are experiencing that are compromising their quality of life?
  • #88 Frontotemporal Dementia: Diagnosis, Trials & Treatment
    https://www.brightfocus.org/resource/frontotemporal-dementia-diagnosis-trials-treatment/
    Its not yet possible to stop in their tracks. […] Its more like youre helping them develop strategies to compensate for the difficulty. […] And then there are some types of symptoms that respond to medication therapies like some patients with FTD have compulsive types of behaviors where they do the same thing over and over again. […] But finding out what the local community resources are or the resources that may be accessible online, that can provide education and support is so critical because I think many people feel very isolated when theyre living with one of these rare conditions.
  • #89 Frontotemporal dementia | Dementia Australia
    https://www.dementia.org.au/about-dementia/frontotemporal-dementia
    Neuropsychological assessments, particularly for the language onset ones, semantic, non-fluent, really helpful. […] The diagnostic delays are the biggest reasons why, and then the unique issues because of the behavioural problems. […] It’s really essential to emphasise there are support groups available right throughout Australia. […] The mainstay of treatment is still support. Support for you if you have FTD, your loved ones, and for all family members, and I think that that’s really essential. […] We know that the prognosis, how things are going to change, is really variable. […] There are more than 300 studies listed on what is the biggest worldwide register clinicaltrials.gov, and there’s over a hundred that are actually trials of treatments. […] The toolkit that we have on the ECDC website has a list of some of the genetic FDD trials that are running in Australia and in our clinic, and also in FTD trials, so take a look at that if you need to.
  • #90 Frontotemporal dementia – Penn Memory Center
    https://pennmemorycenter.org/education-and-support-resources/understanding-my-diagnosis/ed-frontotemporal-dementia/
    Frontotemporal disorders are a form of dementia caused by a family of brain diseases known as frontotemporal lobar degeneration (FTLD). Frontotemporal dementia can be grouped into four types, defined by the earliest symptoms physicians identify when they examine patients. Currently there are no curative treatments available for frontotemporal degeneration (FTD). The current goal of therapy in treatment of FTD is symptomatic relief. […] You may find it helpful to have a geriatric psychiatrist on your treatment team.
  • #91 FTD Toolkit — Eastern Cognitive Disorders Clinic
    https://ecdc.org.au/ftd-toolkit
    Just as many screening tools for dementia can’t diagnose the specific disabilities of FTD, management strategies which were developed for people with Alzheimers and other types of dementia, may not always be suitable for people with FTD. […] FTD presents unique management challenges to carers and health professionals. Currently, the evidence base for effective management techniques is limited. While pharmacological approaches are sometimes utilised, non-pharmacological strategies are the preferred management approach for most of the behavioural and psychological symptoms of dementia. A person-centred, problem-solving approach helps to ensure that such strategies are employed effectively for each individual.
  • #92 What is frontotemporal dementia? Here’s what to know about Bruce Willis’ diagnosis
    https://www.wjcl.com/article/ftd-frontotemporal-dementia-bruce-willis-diagnosis/42945484
    „And oftentimes, we also will do brain metabolism imaging,” Paulson said. „It’s Positron Emission Tomography or PET imaging, and that can tell us which parts of the frontal lobes or the temporal lobes are involved.” […] Unlike Alzheimer’s disease, there are no current therapies to slow down the progression of FTD. […] A speech-language pathologist can help determine the best strategies and tools for an FTD patient struggling with language skills. […] „It is really important for people who have a progressive dementing syndrome like FTD to continue to eat well, exercise regularly, and stay connected with people. Those activities are not medications, they’re not curing the disease, but they can help your brain work as well as possible,” Paulson said.
  • #93 What is frontotemporal dementia? Here’s what to know about Bruce Willis’ diagnosis
    https://www.wtae.com/article/ftd-frontotemporal-dementia-bruce-willis-diagnosis/42945484
    „It is really important for people who have a progressive dementing syndrome like FTD to continue to eat well, exercise regularly, and stay connected with people. Those activities are not medications, they’re not curing the disease, but they can help your brain work as well as possible,” Paulson said.