Pierwotna postępująca afazja
Diagnostyka i diagnoza
Pierwotna postępująca afazja (PPA) to neurodegeneracyjne schorzenie charakteryzujące się stopniowym upośledzeniem funkcji językowych, które początkowo stanowią jedyny deficyt poznawczy. Diagnostyka PPA opiera się na spełnieniu kryteriów międzynarodowego konsensusu, w tym dominującym i progresywnym zaburzeniu języka przez minimum 2 lata oraz wykluczeniu innych przyczyn, takich jak udar, guz czy infekcje. Wyróżnia się trzy warianty kliniczne: afazję semantyczną (svPPA) z deficytami w nazywaniu i atrofią lewego płata skroniowego, afazję niepłynną/agramatyczną (nfvPPA) z agramatyzmem i atrofią lewego płata czołowego oraz afazję logopeniczną (lvPPA) z trudnościami w powtarzaniu i atrofią obszarów skroniowo-ciemieniowych. Diagnostyka obejmuje szczegółowy wywiad, badanie neurologiczne, kompleksową ocenę mowy i języka (np. Boston Diagnostic Aphasia Examination, Western Aphasia Battery), neuropsychologiczną oraz obrazowanie mózgu (MRI, FDG-PET, SPECT) w celu identyfikacji wzorców atrofii i wykluczenia innych etiologii. Analiza płynu mózgowo-rdzeniowego (Aβ42, t-tau, p-tau) oraz badania genetyczne (mutacje w C9ORF72, GRN) mogą wspomagać diagnostykę różnicową i wskazywać na podłoże patologiczne, np. chorobę Alzheimera.
- Diagnostyka pierwotnej postępującej afazji
- Kompleksowa ocena diagnostyczna
- Wywiad i badanie neurologiczne
- Ocena mowy i języka
- Ocena neuropsychologiczna
- Neuroobrazowanie
- Badania laboratoryjne i analiza płynu mózgowo-rdzeniowego
- Badania genetyczne
- Diagnostyka różnicowa
- Nowe trendy w diagnostyce PPA
- Znaczenie wczesnej i dokładnej diagnostyki
- Wielodyscyplinarne podejście do diagnozy
- Podsumowanie kluczowych aspektów diagnostyki PPA
Diagnostyka pierwotnej postępującej afazji
Pierwotna postępująca afazja (PPA) to stosunkowo rzadkie schorzenie neurologiczne charakteryzujące się postępującym upośledzeniem zdolności językowych. Diagnostyka PPA stanowi złożony proces, który wymaga kompleksowej oceny i wykluczenia innych potencjalnych przyczyn zaburzeń komunikacji 12. W przeciwieństwie do innych form afazji, które mogą wystąpić po udarze czy urazie mózgu, PPA jest związana z neurodegeneracją i stanowi formę otępienia 34.
Kryteria diagnostyczne PPA
Diagnoza pierwotnej postępującej afazji opiera się na spełnieniu określonych kryteriów. Według międzynarodowego konsensusu, PPA jest rozpoznawana, gdy:56
- Występuje stopniowe upośledzenie języka (nie tylko mowy)
- Problem językowy jest początkowo jedynym upośledzeniem
- Podstawową przyczyną jest choroba neurodegeneracyjna
Kryteria te zostały rozszerzone przez Gorno-Tempini i współpracowników, którzy zaproponowali dwustopniowy proces diagnostyczny 78. Pierwsze kryterium wymaga, aby zaburzenia językowe były najbardziej widocznym i dominującym objawem w początkowej fazie choroby (zwykle przez co najmniej pierwsze 2 lata), stanowiąc główne ograniczenie w codziennym funkcjonowaniu 9. Kluczowe jest również wykluczenie innych przyczyn zaburzeń językowych, takich jak udar, guz mózgu, uraz czy infekcja 10.
Warianty PPA
Aktualnie rozróżnia się trzy główne warianty PPA, które różnią się charakterystyką objawów i obszarami mózgu dotkniętymi zanikiem 1112:
- Afazja semantyczna (svPPA) – charakteryzuje się rosnącymi trudnościami w nazywaniu osób, obiektów, faktów i słów
- Afazja niepłynna/agramatyczna (nfvPPA) – pacjenci doświadczają trudności w wymowie słów, mowa jest nieplastyczna, występuje agramatyzm
- Afazja logopeniczna (lvPPA) – trudności w znajdowaniu słów są najbardziej widoczną cechą
Każdy z tych wariantów wiąże się z określonymi wzorcami atrofii mózgu i może sugerować specyficzną patologię 1314.
Kompleksowa ocena diagnostyczna
Diagnostyka PPA wymaga wielokierunkowego podejścia i często angażuje specjalistów z różnych dziedzin medycznych 15.
Wywiad i badanie neurologiczne
Pierwszy etap diagnozy obejmuje szczegółowy wywiad medyczny, zarówno od pacjenta, jak i od członków rodziny, dotyczący początku objawów, ich progresji oraz historii rodzinnej. Neurolog przeprowadza dokładne badanie neurologiczne, wykorzystując informacje dostarczone przez rodzinę oraz wyniki badań laboratoryjnych, aby potwierdzić, że objawy nie są spowodowane innymi przyczynami (np. niedobory witamin, guzy, udar, infekcje) 1617.
Ocena mowy i języka
Kluczowym elementem diagnostyki PPA jest kompleksowa ocena mowy i języka, przeprowadzana przez logopedę lub neuropsychologa 18. Badanie to obejmuje ocenę różnych aspektów funkcji językowych, takich jak:1920
- Zdolność nazywania (np. proszenie pacjenta o nazwanie obiektów)
- Płynność mowy (ocena spontanicznej mowy pod kątem płynności, liczby używanych słów, inicjacji mowy, częstości błędów, wahań)
- Zdolność powtarzania (proszenie pacjenta o powtórzenie złożonego zdania)
- Umiejętność czytania i pisania
- Rozumienie (ocena zdolności pacjenta do wskazywania obiektów nazywanych przez klinicystę, wykonywania poleceń i odpowiadania na pytania)
Do oceny afazji stosuje się wystandaryzowane narzędzia, takie jak Boston Diagnostic Aphasia Examination, Western Aphasia Battery czy Token Test 21. Warto podkreślić, że w wielu krajach istnieją specyficzne testy dostosowane do lokalnego języka i kultury 22.
Ocena neuropsychologiczna
Kompleksowa ocena neuropsychologiczna jest standardową praktyką w ocenie PPA, aby wykluczyć inne diagnozy i zidentyfikować zaburzenia poznawcze, które mogły pojawić się w trakcie progresji choroby 23. Ocena ta może obejmować testy pamięci, rozumowania, umiejętności wzrokowo-przestrzennych i innych funkcji poznawczych 24.
Neuroobrazowanie
Obrazowanie mózgu odgrywa kluczową rolę w diagnostyce PPA, pomagając w wykluczeniu innych neurologicznych przyczyn obserwowanych deficytów oraz dostarczając informacji na temat wzorców atrofii mózgu 25. Najczęściej stosowane techniki neuroobrazowania w diagnostyce PPA to:
- Rezonans magnetyczny (MRI) – może ukazać zanik określonych obszarów mózgu charakterystycznych dla poszczególnych wariantów PPA 2627
- Pozytonowa tomografia emisyjna (PET) – szczególnie z zastosowaniem 18F-fluorodeoksyglukozy (FDG-PET), ukazuje funkcję mózgu i może ujawnić problemy z metabolizmem glukozy w obszarach mózgu związanych z językiem 2829
- Tomografia emisyjna pojedynczego fotonu (SPECT) – może wykazać charakterystyczne dla PPA zmniejszenie aktywności w określonych obszarach mózgu 30
Każdy wariant PPA charakteryzuje się określonym wzorcem zaniku lub hipometabolizmu w mózgu, co może pomóc w różnicowaniu podtypów 3132.
Badania laboratoryjne i analiza płynu mózgowo-rdzeniowego
Badania krwi mogą być pomocne w wykluczeniu infekcji lub innych stanów medycznych, które mogą powodować podobne objawy 33. W niektórych przypadkach przeprowadza się również analizę płynu mózgowo-rdzeniowego (punkcja lędźwiowa) w celu oceny poziomów określonych białek, które mogą wskazywać na patologie leżące u podłoża objawów 3435.
Szczególnie istotne jest badanie płynu mózgowo-rdzeniowego pod kątem 42-aminokwasowej formy peptydu amyloidu-β (Aβ42), całkowitego tau (t-tau) i fosforylowanego tau (p-tau), co może dostarczyć dowodów neuropatologicznych dla choroby Alzheimera, która może leżeć u podłoża niektórych przypadków PPA 36.
Badania genetyczne
Badania genetyczne mogą być dodatkowym narzędziem wspomagającym diagnostykę różnicową PPA 37. W największym badaniu przesiewowym dotyczącym genetyki PPA, 14 z 403 przypadków (3,5%) miało mutacje genowe, głównie w genach C9ORF72 i GRN 38. Chociaż badania genetyczne nie są obecnie standardową procedurą w ocenie PPA, rosnące badania w dziedzinie genetyki PPA wykazały, że mogą one służyć jako komplementarne narzędzie diagnostyczne 39.
Diagnostyka różnicowa
Diagnostyka różnicowa PPA wymaga wykluczenia innych schorzeń, które mogą powodować podobne objawy 40.
Wykluczanie innych przyczyn afazji
Aby postawić diagnozę PPA, konieczne jest wykluczenie innych powoli postępujących etiologii, zwłaszcza guzów wewnątrzczaszkowych i patologii naczyniowych, za pomocą tomografii komputerowej (CT) lub MRI 41. Ważne jest również odróżnienie PPA od afazji spowodowanej udarem, urazem mózgu lub innymi schorzeniami neurologicznymi 42.
Rozróżnienie między wariantami PPA
Po ustaleniu ogólnej diagnozy PPA, kolejnym krokiem jest klasyfikacja według wariantu klinicznego 43. Rozróżnienie między trzema głównymi wariantami PPA opiera się na charakterystyce objawów językowych i wzorcach atrofii mózgu 44:
- svPPA – charakteryzuje się deficytami w rozumieniu pojedynczych słów i obiektów, z atrofią lewego płata skroniowego 4546
- nfvPPA – główne kryteria diagnostyczne obejmują agramatyzm i powolną, pracochłonną mowę, z atrofią obszarów mowy mózgu, szczególnie płata czołowego po lewej stronie 4748
- lvPPA – charakteryzuje się upośledzeniem wyszukiwania słów i powtarzania zdań, z atrofią obszarów położonych bardziej ku tyłowi mózgu niż inne podtypy PPA, szczególnie w miejscu, gdzie spotykają się płaty skroniowy i ciemieniowy 49
Badania wskazują jednak, że nie wszyscy pacjenci z PPA pasują idealnie do jednego z tych trzech wariantów, a niektórzy mogą prezentować mieszane objawy 50. W jednym z badań monocentrycznych obejmujących 84 pacjentów z PPA, 31% nie można było przypisać do żadnego z trzech podtypów 51.
Wyzwania diagnostyczne
Diagnoza PPA może być trudna i czasochłonna z kilku powodów 5253:
- PPA jest stosunkowo rzadką chorobą, niezbyt dobrze znaną wszystkim lekarzom 54
- Początkowe objawy są często przypisywane lękowi lub stresowi, dopóki nie staną się wystarczająco uciążliwe, aby skłonić do dalszej oceny przez specjalistę 55
- Wiele osób z PPA nie otrzymuje jednoznacznej diagnozy, a jeszcze mniej ma informacje na temat konkretnego podtypu 56
- Istnieje opóźnienie między pierwszą konsultacją z powodu problemów poznawczych a pierwszą diagnozą, które jest znacznie dłuższe niż w przypadku choroby Alzheimera 57
Badania pokazują, że diagnoza PPA często ulega zmianie w miarę postępu choroby, gdy izolowane zmiany językowe przechodzą w globalne upośledzenie poznawcze 5859.
Nowe trendy w diagnostyce PPA
W ostatnich latach pojawiły się nowe metody i podejścia, które mogą pomóc w diagnozowaniu PPA 60.
Biomarkery molekularne
Ważnym i rozwijającym się zastosowaniem klinicznym obrazowania PET jest wykorzystanie radioaktywnych znaczników do wykrywania obecności płytek amyloidowych i agregatów splątków neurofibrylarnych tau 61. Te nowe testy mogą pomóc w określeniu prawdopodobnej neuropatologii leżącej u podstaw objawów 62.
Obrazowanie molekularne umożliwia identyfikację in vivo i kwantyfikację metabolizmu mózgu, nieprawidłowego odkładania się β-amyloidu i tau oraz obecności stanu zapalnego mózgu – istotnych biomarkerów neuroobrazowych, które mogą poprawić wczesną diagnostykę i pomóc w ocenie progresji choroby neurodegeneracyjnej 63.
Narzędzia cyfrowe i aplikacje
Pojawiają się nowe narzędzia cyfrowe, które mogą pomóc we wczesnej diagnozie PPA. Przykładem jest aplikacja Sydney Language Battery (SYDBAT), opracowana przez grupę badawczą FRONTIER z Brain and Mind Centre Uniwersytetu w Sydney. Aplikacja ta oferuje cztery oceny języka i mowy, nagrywa mowę, eksportuje dane i automatycznie ocenia wyniki 64.
Wykorzystanie nowych technologii pozwala na bardziej ekologiczne i powtarzalne testy w porównaniu z pewnymi skalami lub testami typu papier-ołówek 65.
Markery graficzne
Interesującym kierunkiem badań są markery graficzne, które mogą pomóc w różnicowaniu PPA od innych form otępienia. Badania sugerują, że ciśnienie pisania w różnych zadaniach językowych i nielingwistycznych może różnicować pacjentów z PPA od pacjentów z chorobą Alzheimera i zdrowych osób 66.
Ponieważ objawy językowe są najwcześniejszymi i najbardziej widocznymi oznakami we wczesnych stadiach choroby, graficzne markery pisania mogą stanowić ekologiczne markery o dużym znaczeniu dla wczesnej diagnozy PPA 67.
Uczenie maszynowe w diagnostyce
Rośnie zainteresowanie zautomatyzowaną diagnostyką opartą na podejściach uczenia maszynowego i identyfikacją cyfrowych markerów, które mogłyby wykrywać PPA we bardzo wczesnym stadium 68.
Znaczenie wczesnej i dokładnej diagnostyki
Wczesna i dokładna diagnoza PPA ma kluczowe znaczenie z kilku powodów 6970:
Korzyści z wczesnej diagnozy
- Umożliwia wczesną interwencję i wdrożenie odpowiednich strategii terapeutycznych 71
- Pozwala specjalistom opieki zdrowotnej na odróżnienie PPA od innych form otępienia, co ułatwia dokładne rokowanie i dostosowane plany opieki 72
- Zwiększa możliwości odpowiednich interwencji klinicznych, skoordynowanych planów opieki, zarządzania objawami i poprawy bezpieczeństwa pacjenta 73
- Może przyczynić się do obniżenia kosztów opieki zdrowotnej i opóźnienia instytucjonalizacji 74
- Rozwiązuje niepewność dla pacjentów i rodzin, umożliwia planowanie przyszłości i odblokowuje dostęp do usług i wsparcia 75
Implikacje dla leczenia
Prawidłowa diagnoza PPA wpływa na postępowanie terapeutyczne. Badania pokazują, że diagnoza PPA jest szczególnie ważna, ponieważ modyfikuje interwencje farmakologiczne i niefarmakologiczne 76.
Chociaż nie ma leku na PPA, odpowiednie leczenie może spowolnić postęp choroby i zwiększyć jakość życia pacjenta 77. Terapia mowy może pomóc w utrzymaniu umiejętności komunikacyjnych tak długo, jak to możliwe 78.
Ponadto, przewidywanie patologii leżącej u podłoża PPA jest potencjalnie istotne dla rokowania i planowania opieki, i będzie coraz częściej wymagane do racjonalnego ukierunkowania terapii modyfikujących przebieg choroby 79.
Wielodyscyplinarne podejście do diagnozy
Najlepszym podejściem do diagnozy PPA jest skierowanie do neurologa lub neuropsychologa z doświadczeniem w leczeniu PPA 80. Diagnoza i klasyfikacja PPA są najczęściej ustalane przez lekarza (zwykle neurologa behawioralnego) na podstawie historii objawów pacjenta i historii rodzinnej, badania neurologicznego, kompleksowej oceny mowy, języka i poznania oraz klinicznego obrazowania mózgu 81.
Zespół diagnostyczny może obejmować 82:
- Neurologów
- Geriatrów
- Psychiatrów
- Neuropsychologów
- Logopedów
- Pielęgniarki i asystentów medycznych
Wykwalifikowani specjaliści w dziedzinie mowy, języka i poznania, w tym logopedzi i neuropsycholodzy, powinni odgrywać integralną rolę w procesie diagnostycznym 83.
Rola logopedy
Logopedzi odgrywają kluczową rolę w diagnozowaniu i leczeniu PPA 84. Podczas oceny osoby z podejrzeniem PPA, ważne jest, aby logopedzi ustalili początek i przebieg problemu komunikacyjnego oraz określili poprzez badanie, czy reprezentuje on ogniskowy deficyt językowy (afazję), czy zaburzenie bardziej zgodne z szerszą konstelacją nieafazycznych deficytów komunikacji poznawczej 85.
Po ustaleniu diagnozy logopedzi mogą zaoferować bogactwo informacji, które pomogą pacjentom, rodzinom i partnerom opieki przystosować się do utraty funkcji poznawczo-komunikacyjnych 86.
Wsparcie dla pacjentów i rodzin
Otrzymanie diagnozy PPA może być skomplikowanym procesem i trudnym doświadczeniem dla pacjentów i ich rodzin 8788. Pacjenci i ich bliscy często czują ulgę, gdy wreszcie otrzymują diagnozę, która dokładnie odzwierciedla ich trudności i pozwala na pewne przewidywania dotyczące przyszłości 89.
Organizacje takie jak National Aphasia Association mogą być doskonałym źródłem informacji i wsparcia dla osób chcących dowiedzieć się więcej o afazji lub znaleźć grupy wsparcia w swojej lokalnej społeczności 90.
Podsumowanie kluczowych aspektów diagnostyki PPA
Diagnostyka pierwotnej postępującej afazji jest złożonym procesem, który wymaga kompleksowego podejścia i współpracy specjalistów z różnych dziedzin 91. Kluczowe aspekty procesu diagnostycznego obejmują:
- Ustalenie, czy zaburzenia językowe są głównym i postępującym objawem
- Wykluczenie innych przyczyn afazji poprzez badania obrazowe i laboratoryjne
- Szczegółową ocenę funkcji językowych i poznawczych
- Identyfikację specyficznego wariantu PPA na podstawie charakterystyki objawów i wyników neuroobrazowania
- Rozważenie badań biomarkerów, takich jak analiza płynu mózgowo-rdzeniowego czy specjalistyczne obrazowanie PET
Wczesna i dokładna diagnoza PPA ma kluczowe znaczenie dla odpowiedniego zarządzania objawami, planowania opieki i maksymalizacji jakości życia pacjenta 92.
Chociaż nie ma obecnie leku na PPA, terapia mowy może pomóc w utrzymaniu umiejętności komunikacyjnych przez dłuższy czas, a właściwe wsparcie może znacząco poprawić jakość życia pacjentów z tą trudną diagnozą 93.
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Materiały źródłowe
- #1https://www.brain.northwestern.edu/dementia/primary-progressive-aphasia/diagnosis.html
Primary progressive aphasia (PPA) is relatively uncommon. There is no one test to diagnose PPA; instead, the diagnosis is made through a process of „ruling in” and „ruling out.” These factors contribute to the long diagnostic journey some experience. […] Components of a PPA evaluation can include obtaining medical history from the individual with language concerns and family, neurological exam and cognitive assessment by a neuropsychologist and laboratory measures (e.g., blood work, cerebrospinal fluid analysis and brain imaging with MRI or PET). […] During the neurological exam, the clinician will use the information provided by the family about symptom onset and the laboratory measures to confirm that symptoms are not due to other causes (e.g., vitamin deficiencies, tumor, stroke, infection).
- #2 Diagnostic Assessment in Primary Progressive Aphasia: An Illustrative Case Examplehttps://pmc.ncbi.nlm.nih.gov/articles/PMC8740567/
Diagnosis and classification of primary progressive aphasia (PPA) requires confirmation of specific speech and language symptoms, highlighting the important role of speech-language pathologists in the evaluation process. […] The diagnosis of primary progressive aphasia (PPA) is made when a patient has a predominant and progressive loss of communication caused by neurodegenerative disease that targets speech and language regions of the brain. […] Diagnosis of PPA requires confirmation of specific speech and language symptoms, highlighting the important contribution of speech-language pathologists (SLPs) during the evaluation process. […] Current consensus criteria delineate clinical, imaging, neuropathological, and genetic features of each of the three variants of PPA. […] Diagnosis by clinical variant has important implications for managing a patient’s symptoms.
- #3 Primary Progressive Aphasia (PPA): Symptoms & Treatmenthttps://my.clevelandclinic.org/health/diseases/17387-primary-progressive-aphasia-ppa
Primary progressive aphasia (PPA) is a neurological condition that causes you to lose language skills. Its a type of dementia and may be a sign of Alzheimers disease. […] Primary progressive aphasia develops when the parts of your brain that control language start breaking down. Your brain tissue shrinks (atrophies), affecting your ability to communicate. […] Your healthcare provider may suspect PPA based on your symptoms. Theyll evaluate your medical history and family history to see if you have an increased risk of primary progressive aphasia. […] You might have specialized cognitive tests and brain scans, such as an MRI or CT scan, to confirm a diagnosis. […] There isnt a cure for primary progressive aphasia and no way to stop its progression. But some treatments may slow the diseases progression or increase your quality of life.
- #4 Six things to know about primary progressive aphasiahttps://www.ualberta.ca/en/folio/2024/06/six-things-to-know-about-primary-progressive-aphasia.html
Actor Bruce Williss diagnosis put a spotlight on a rare condition known as primary progressive aphasia, but the facts are getting muddled. […] The conditions correct name is frontotemporal degeneration. Its one of the underlying disease processes that can cause primary progressive aphasia (PPA), says Esther Kim, a speech-language pathologist, chair of the Department of Communication Sciences and Disorders in the University of Albertas Faculty of Rehabilitation Medicine and member of the Neuroscience and Mental Health Institute. […] The first thing Kim clarifies is that PPA is not a typical form of dementia, which affects memory, but is instead a rare presentation where the brain regions for language are affected first. […] PPA usually presents at an earlier age than Alzheimers or typical dementia does, to people in their 50s and 60s.
- #5 Primary Progressive Aphasia (PPA) | AFTDhttps://www.theaftd.org/what-is-ftd/primary-progressive-aphasia/
PPA is diagnosed when three criteria are met: There is a gradual impairment of language (not just speech). The language problem is initially the only impairment. The underlying cause is a neurodegenerative disease. […] Not all physicians know about FTD, so it is often misdiagnosed or not diagnosed at all. AFTD developed a checklist to help identify red flags for behavioral variant FTD (bvFTD) that you can bring to your doctor. The back of the document is designed to help your physician better understand FTD diagnostic criteria.
- #6 Primary Progressive Aphasias: Diagnosis and Treatmenthttps://www.mdpi.com/2076-3425/15/3/245
Primary Progressive Aphasias (PPAs) are defined as language disorders characterized by an insidious onset and progressive evolution. The term âPrimary Progressive Aphasiaâ applies only when these language impairments are the initial and predominant symptom, remaining isolated for an extended period. While other cognitive deficits may emerge as the disease advances, language difficulties remain the central and most significant feature throughout the progression. […] Gorno-Tempini et al. proposed updated general criteria for the diagnosis of Primary Progressive Aphasia (PPA), building on and expanding Mesulamâs original criteria. These new criteria are less restrictive than those outlined by Mesulam. For instance, Gorno-Tempini introduced the broader concept of a âlanguage complaintâ without specifically requiring the detailed language impairments mentioned by Mesulam, such as anomia or spontaneous language comprehension disorders.
- #7 Diagnostic Assessment in Primary Progressive Aphasia: An Illustrative Case Examplehttps://pmc.ncbi.nlm.nih.gov/articles/PMC8740567/
For each PPA variant, diagnosis can be further supported by imaging if the clinical symptoms are accompanied by evidence of particular patterns of atrophy, hypoperfusion, or glucose hypometabolism in the brain. […] A clinical diagnosis of PPA requires predominant and progressive speech and/or language symptoms during initial stages of the disease, that these deficits are the primary limitation to activities of daily living, and that impairments cannot be better explained by psychiatric, behavioral, or nondegenerative central nervous system disorders. […] The second diagnostic step is classification by PPA clinical variant, when possible. […] The international consensus criteria for PPA define three clinical variants based on core impairments and associated features. […] The etiology of neurodegeneration for each of the three variants of PPA has been linked to different pathological processes.
- #8 Multimodality Imaging in Primary Progressive Aphasia | American Journal of Neuroradiologyhttp://www.ajnr.org/content/early/2022/08/25/ajnr.A7613
While a progressive neurodegenerative disorder characterized by language-predominant impairment has been a recognized entity dating back to the 19th century, inconsistencies regarding its terminology and nomenclature exist in the literature. […] In an effort to improve diagnostic uniformity and the reliability of research results, Gorno-Tempini et al published a 2011 framework for PPA diagnosis and classification using a 2-step process. […] Therefore, an accurate diagnosis of PPA requires a multidisciplinary approach, inclusive of clinical, pathologic, and radiologic evaluation. […] Advances in the understanding of PPA, particularly the recognition of variant-specific patterns of atrophy and/or hypometabolism, have reinforced the role of structural, functional, and molecular neuroimaging in supporting the diagnosis, when clinically suspected.
- #9 Primary progressive aphasia – Wikipediahttps://en.wikipedia.org/wiki/Primary_progressive_aphasia
In neurology, primary progressive aphasia (PPA) is a type of neurological syndrome in which language capabilities slowly and progressively become impaired. […] The following diagnosis criteria were defined by Mesulam: As opposed to having followed trauma to the brain, a patient must show an insidious onset and a gradual progression of aphasia, defined as a disorder of sentence and/or word usage, affecting the production and comprehension of speech. […] Whether or not PPA and other aphasias are the only source of cognitive impairment in a patient is often difficult to assess because: 1) as with other neurologically degenerative diseases, such as Alzheimer’s disease, there are currently no reliable non-invasive diagnostic tests for aphasias, and thus neuropsychological assessments are the only tool physicians have for diagnosing patients; and 2) aphasias often affect other, non-language portions of these neuropsychological tests, such as those specific for memory.
- #10https://www.brain.northwestern.edu/dementia/primary-progressive-aphasia/diagnosis.html
Primary progressive aphasia (PPA) is relatively uncommon. There is no one test to diagnose PPA; instead, the diagnosis is made through a process of „ruling in” and „ruling out.” These factors contribute to the long diagnostic journey some experience. […] Components of a PPA evaluation can include obtaining medical history from the individual with language concerns and family, neurological exam and cognitive assessment by a neuropsychologist and laboratory measures (e.g., blood work, cerebrospinal fluid analysis and brain imaging with MRI or PET). […] During the neurological exam, the clinician will use the information provided by the family about symptom onset and the laboratory measures to confirm that symptoms are not due to other causes (e.g., vitamin deficiencies, tumor, stroke, infection).
- #11 Diagnostic Assessment in Primary Progressive Aphasia: An Illustrative Case Examplehttps://pmc.ncbi.nlm.nih.gov/articles/PMC8740567/
For each PPA variant, diagnosis can be further supported by imaging if the clinical symptoms are accompanied by evidence of particular patterns of atrophy, hypoperfusion, or glucose hypometabolism in the brain. […] A clinical diagnosis of PPA requires predominant and progressive speech and/or language symptoms during initial stages of the disease, that these deficits are the primary limitation to activities of daily living, and that impairments cannot be better explained by psychiatric, behavioral, or nondegenerative central nervous system disorders. […] The second diagnostic step is classification by PPA clinical variant, when possible. […] The international consensus criteria for PPA define three clinical variants based on core impairments and associated features. […] The etiology of neurodegeneration for each of the three variants of PPA has been linked to different pathological processes.
- #12 Primary Progressive Aphasia | Memory and Aging Centerhttps://memory.ucsf.edu/dementia/primary-progressive-aphasia
Primary progressive aphasia (PPA) is a condition that slowly damages the parts of the brain that control speech and language. […] The clinical symptoms of PPA are caused by degeneration in the parts of the brain that control speech and language (typically the left side of the brain in the frontal, temporal, and parietal regions). […] PPA has three variants: Semantic (svPPA): people with svPPA experience increasing trouble naming people, objects, facts and words […] Nonfluent/agrammatic (nfvPPA): people with nfvPPA tend to come to the doctor’s office with complaints of difficulty pronouncing words […] Logopenic (lvPPA): word-finding difficulties are the most prominent feature in patients with logopenic PPA. […] These syndromes result from a variety of underlying diseases, but frontotemporal lobar degeneration (FTLD) (both tau and TDP-43 subtypes) or Alzheimers disease is most often the cause.
- #13https://link.springer.com/article/10.1007/s12149-024-01958-w
The disease progresses in a matter of years while leaving behavioral aspects relatively intact during the early stages. […] Within the diagnostic framework of PPA, the condition is further subdivided into three subtypes: Semantic Dementia (SD), Progressive Non-Fluent Aphasia (PNFA), also known as the agrammatic variant (agPPA), and Logopenic Progressive Aphasia (LPA). […] However, existing diagnostic criteria using only these three variants may be inadequate in encompassing all individuals with PPA. […] An increasing body of literature now supports the likelihood of 56 subtypes instead of the traditionally recognized three. […] Early PPA identification can significantly impacts patient management and prognosis. […] Given that PPA is a neurodegenerative condition leading to a gradual loss of language abilities, early diagnosis allows for timely intervention and the implementation of appropriate therapeutic strategies.
- #14 Primary Progressive Aphasia: Advances in Diagnosis, Prognosis, & Treatment – ASHA Journals Academyhttps://academy.pubs.asha.org/2015/11/primary-progressive-aphasia-advances-in-diagnosis-prognosis-treatment/
So, I mentioned, the main variants reflect the area of the brain most affected by disease and give you a clue to the underlying pathology. The underlying disease often determines what problems the person may develop in the future. […] The three main variants of primary progressive aphasia distinguished by the distinct patterns of language impairments and supportive pattern of atrophy on imaging. All three variants, as I mentioned, have prominent naming impairment. […] So, nonfluent/agrammatic variant primary progressive aphasia is really characterized by effortful halting speech with inconsistent sound errors or apraxia of speech and or agrammatic language production, difficulty in producing complete grammatical sentences. […] The pathology underlying this variant is usually tau pathology.
- #15 Diagnostic Assessment in Primary Progressive Aphasia: An Illustrative Case Examplehttps://pmc.ncbi.nlm.nih.gov/articles/PMC8740567/
In the largest genetic screening study of PPA to date, 14 of 403 cases (or 3.5%) had gene mutations, primarily in C9ORF72 and GRN. […] Genetic testing is not currently standard procedure in the assessment of PPA, emerging research in the genetics of PPA has demonstrated it may serve as a complementary tool for diagnosis. […] In current clinical practice, diagnosis and classification of PPA are determined by a clinician (typically a behavioral neurologist) based on the patient’s history of symptoms and family history, a neurological examination, a comprehensive evaluation of speech, language, and cognition, and clinical brain imaging. […] Skilled professionals in speech, language, and cognition, including SLPs and neuropsychologists, should play an integral role in the diagnostic process.
- #16https://www.brain.northwestern.edu/dementia/primary-progressive-aphasia/diagnosis.html
Primary progressive aphasia (PPA) is relatively uncommon. There is no one test to diagnose PPA; instead, the diagnosis is made through a process of „ruling in” and „ruling out.” These factors contribute to the long diagnostic journey some experience. […] Components of a PPA evaluation can include obtaining medical history from the individual with language concerns and family, neurological exam and cognitive assessment by a neuropsychologist and laboratory measures (e.g., blood work, cerebrospinal fluid analysis and brain imaging with MRI or PET). […] During the neurological exam, the clinician will use the information provided by the family about symptom onset and the laboratory measures to confirm that symptoms are not due to other causes (e.g., vitamin deficiencies, tumor, stroke, infection).
- #17 Primary Progressive Aphasia Causes and Diagnoses | Northwestern Medicinehttps://www.nm.org/conditions-and-care-areas/neurosciences/primary-progressive-aphasia/causes-and-diagnoses
PPA arises when nerve cells in language-related parts of the brain malfunction. […] Specialized positron emission tomography (PET) scans and examination of the spinal fluid may help to resolve the distinction between the two underlying diseases. […] A thorough evaluation of PPA includes the following: Medical History, Neurological Examination, Neuropsychological Examination, Speech and Language Evaluation, Psychosocial Evaluation, Brain Imaging Studies, Psychiatric Evaluation. […] Therefore, some people with PPA often see multiple doctors and receive many different diagnoses before receiving the diagnosis of PPA.
- #18 Diagnostic Assessment in Primary Progressive Aphasia: An Illustrative Case Examplehttps://pmc.ncbi.nlm.nih.gov/articles/PMC8740567/
Diagnosis and classification of primary progressive aphasia (PPA) requires confirmation of specific speech and language symptoms, highlighting the important role of speech-language pathologists in the evaluation process. […] The diagnosis of primary progressive aphasia (PPA) is made when a patient has a predominant and progressive loss of communication caused by neurodegenerative disease that targets speech and language regions of the brain. […] Diagnosis of PPA requires confirmation of specific speech and language symptoms, highlighting the important contribution of speech-language pathologists (SLPs) during the evaluation process. […] Current consensus criteria delineate clinical, imaging, neuropathological, and genetic features of each of the three variants of PPA. […] Diagnosis by clinical variant has important implications for managing a patient’s symptoms.
- #19 Diagnostic Assessment in Primary Progressive Aphasia: An Illustrative Case Examplehttps://pmc.ncbi.nlm.nih.gov/articles/PMC8740567/
The domains examined in a comprehensive speech and language battery are listed in Table 1, along with example tasks for assessing each domain and the expected behavioral pattern for each PPA variant. […] Examination of cognitive abilities is also standard practice in an evaluation of PPA to rule out other diagnoses or to identify cognitive impairments that may have emerged during the progression of the disease. […] MRI and other types of brain imaging may provide context for behavioral findings and can rule out other neurological causes for observed deficits. […] Important and emerging clinical applications of PET imaging involve the use of radioactive tracers to detect the presence of -amyloid plaques and neurofibrillary tau tangle aggregation. […] Additional neuropathological evidence for AD can be acquired by inspecting cerebrospinal fluid (CSF) for the 42 amino acid form of the amyloid- peptide (A42), total tau (t-tau), and phosphorylated tau (p-tau).
- #20 Azthena logo with the word Azthenahttps://www.news-medical.net/health/Aphasia-Diagnosis.aspx
Aphasia is diagnosed based on detailed tests to exclude other communication disorders; neuropsychological testing and brain imaging studies. […] For a clinician to be sure of an aphasia diagnosis, they need to eliminate any possibility that the symptoms are being caused by the motor speech disorder dysarthria or by impaired hearing, vision or motor writing ability. […] Patient assessment in cases of suspected aphasia should include the following: Naming ability is assessed by asking patients to name objects. The clinician will use descriptions of objects to prompt the naming response such as What you use to drive somewhere. […] Spontaneous speech is assessed for fluency, the number of words used, speech initiation, error rate, hesitation and speech style characteristics. […] Repetition ability is tested by asking the patient to repeat a complex sentence.
- #21 Azthena logo with the word Azthenahttps://www.news-medical.net/health/Aphasia-Diagnosis.aspx
Reading and writing ability are tested by asking the patient to read out loud and write something down. The patients ability to spell and write correctly in response to dictation are also assessed. […] Comprehension is tested by assessing the patients ability to point at objects the clinician names, to carry out tasks and to answer both simple and complex questions with a yes or no response. […] A neuropsychologist or speech therapist may perform cognitive testing to check for finer dysfunction. This can give an indication of how likely recovery is, as well as helping to optimise any treatment plans. […] Examples of tests that may be used include the Boston Diagnostic Aphasia Examination, the Token Test, the Western Aphasia Battery and the Action Naming Test.
- #22 Clinical and Linguistic Profiles and Challenges in Diagnosis of Primary Progressive Aphasia in Medan, Indonesia: A Hospital-based Studyhttps://openneurologyjournal.com/VOLUME/18/ELOCATOR/e1874205X305965/
A comprehensive evaluation involving a detailed medical history and cognitive function assessment is crucial for diagnosing and determining the PPA variant. […] The clinical diagnosis of PPA is complex, and there is often a gap of several years between the onset of symptoms and the establishment of a PPA diagnosis. […] The adaptation of language assessment instruments from English poses its challenges. […] The most commonly used instrument for aphasia assessment by clinicians in Indonesia is the Tes Afasia untuk Diagnosis Informasi dan Rehabilitasi (TADIR). […] However, as of now, there are no reported studies on the use of TADIR in PPA assessments. […] The classification of aphasia based on TADIR refers to the Boston Diagnostic Aphasia Examination (BDAE), which reflects vascular aphasia syndromes and their anatomical correlations.
- #23 Diagnostic Assessment in Primary Progressive Aphasia: An Illustrative Case Examplehttps://pmc.ncbi.nlm.nih.gov/articles/PMC8740567/
The domains examined in a comprehensive speech and language battery are listed in Table 1, along with example tasks for assessing each domain and the expected behavioral pattern for each PPA variant. […] Examination of cognitive abilities is also standard practice in an evaluation of PPA to rule out other diagnoses or to identify cognitive impairments that may have emerged during the progression of the disease. […] MRI and other types of brain imaging may provide context for behavioral findings and can rule out other neurological causes for observed deficits. […] Important and emerging clinical applications of PET imaging involve the use of radioactive tracers to detect the presence of -amyloid plaques and neurofibrillary tau tangle aggregation. […] Additional neuropathological evidence for AD can be acquired by inspecting cerebrospinal fluid (CSF) for the 42 amino acid form of the amyloid- peptide (A42), total tau (t-tau), and phosphorylated tau (p-tau).
- #24 Primary Progressive Aphasia Causes and Diagnoses | Northwestern Medicinehttps://www.nm.org/conditions-and-care-areas/neurosciences/primary-progressive-aphasia/causes-and-diagnoses
PPA arises when nerve cells in language-related parts of the brain malfunction. […] Specialized positron emission tomography (PET) scans and examination of the spinal fluid may help to resolve the distinction between the two underlying diseases. […] A thorough evaluation of PPA includes the following: Medical History, Neurological Examination, Neuropsychological Examination, Speech and Language Evaluation, Psychosocial Evaluation, Brain Imaging Studies, Psychiatric Evaluation. […] Therefore, some people with PPA often see multiple doctors and receive many different diagnoses before receiving the diagnosis of PPA.
- #25 Diagnostic Assessment in Primary Progressive Aphasia: An Illustrative Case Examplehttps://pmc.ncbi.nlm.nih.gov/articles/PMC8740567/
The domains examined in a comprehensive speech and language battery are listed in Table 1, along with example tasks for assessing each domain and the expected behavioral pattern for each PPA variant. […] Examination of cognitive abilities is also standard practice in an evaluation of PPA to rule out other diagnoses or to identify cognitive impairments that may have emerged during the progression of the disease. […] MRI and other types of brain imaging may provide context for behavioral findings and can rule out other neurological causes for observed deficits. […] Important and emerging clinical applications of PET imaging involve the use of radioactive tracers to detect the presence of -amyloid plaques and neurofibrillary tau tangle aggregation. […] Additional neuropathological evidence for AD can be acquired by inspecting cerebrospinal fluid (CSF) for the 42 amino acid form of the amyloid- peptide (A42), total tau (t-tau), and phosphorylated tau (p-tau).
- #26 Primary Progressive Aphasia (PPA): Symptoms & Treatmenthttps://my.clevelandclinic.org/health/diseases/17387-primary-progressive-aphasia-ppa
Primary progressive aphasia (PPA) is a neurological condition that causes you to lose language skills. Its a type of dementia and may be a sign of Alzheimers disease. […] Primary progressive aphasia develops when the parts of your brain that control language start breaking down. Your brain tissue shrinks (atrophies), affecting your ability to communicate. […] Your healthcare provider may suspect PPA based on your symptoms. Theyll evaluate your medical history and family history to see if you have an increased risk of primary progressive aphasia. […] You might have specialized cognitive tests and brain scans, such as an MRI or CT scan, to confirm a diagnosis. […] There isnt a cure for primary progressive aphasia and no way to stop its progression. But some treatments may slow the diseases progression or increase your quality of life.
- #27 Primary progressive aphasiahttps://johnsonmemorial.org/jmh-health/disease-conditions/con-20155110
A brain MRI can help diagnose primary progressive aphasia. The test can detect shrinking of specific areas of the brain. MRI scans also can detect strokes, tumors or other conditions that affect brain function. […] You also might get a positron emission tomography (PET) scan, which shows brain function. This test can uncover problems with glucose metabolism in the areas of the brain that are related to language.
- #28 Primary progressive aphasiahttps://johnsonmemorial.org/jmh-health/disease-conditions/con-20155110
A brain MRI can help diagnose primary progressive aphasia. The test can detect shrinking of specific areas of the brain. MRI scans also can detect strokes, tumors or other conditions that affect brain function. […] You also might get a positron emission tomography (PET) scan, which shows brain function. This test can uncover problems with glucose metabolism in the areas of the brain that are related to language.
- #29 FDG-PET in the Diagnosis of Primary Progressive Aphasia | Journal of Nuclear Medicinehttps://jnm.snmjournals.org/content/63/supplement_2/2971
Primary progressive aphasia (PPA) is a disease that is known to affect the frontal and temporal regions of the left hemisphere. Currently, the diagnosis of PPA is based on consensus guidelines such as Gorno-Tempini criteria. […] Neuroimaging studies of patients with PPA showed a promising role of FDG-PET scan as a diagnostic modality especially in differentiating the subtypes of PPA. The value of FDG-PET in the diagnosis of PPA is especially underscored given the challenge of accurate diagnosis and prediction of future development of frontotemporal dementia (FTD). […] Physicians should carefully consider PPA when reporting FDG-PET in patients with language problems. Future FDG-PET studies with larger patient populations and well-designed cohorts of PPA patients can improve our understanding of the disease and its related subclasses.
- #30 Primary Progressive Aphasias: Diagnosis and Treatmenthttps://www.mdpi.com/2076-3425/15/3/245
To make the diagnosis of PPA, it is essential to exclude other slowly progressive etiologies, especially intracranial tumors and vascular pathologies, either by computed tomography (CT) or MRI. In particular, MRI can provide positive clues about this disease: in progressive non-fluent aphasia, an atrophic enlargement of the left Sylvian fissure is typical, whereas for semantic dementia, atrophy of the left temporal lobe is especially typical. In the absence of atrophy, or if the differential diagnosis presents difficulties, the demonstration by nuclear medicine (CBF-SPECT or FDG-PET) of a typical localized and circumscribed decrease in activity can be useful to support the diagnosis of PPA.
- #31https://link.springer.com/article/10.1007/s12149-024-01958-w
Moreover, early identification enables healthcare professionals to differentiate PPA from other forms of dementia, facilitating accurate prognosis and tailored care plans. […] Positron emission tomography (PET) using 2-deoxy-2-[18F]fluoro-D-glucose (FDG) holds a distinct advantage over other neuroimaging modalities due to its ability to provide functional information about glucose metabolism in the brain. […] By visualizing metabolic alterations preceding atrophy, FDG-PET has been extensively applied to PPA with the intention of uncovering underlying metabolic dysfunction prior to structural changes, demonstrating its possible benefit with PPA diagnosis. […] The reviewed studies collectively highlight the utility of FDG-PET in identifying distinct metabolic patterns associated with different PPA subtypes, though they also reveal some discrepancies.
- #32 Multimodality Imaging in Primary Progressive Aphasia | American Journal of Neuroradiologyhttp://www.ajnr.org/content/early/2022/08/25/ajnr.A7613
This review highlights available imaging modalities in the identification of PPA as well as characteristic variant-specific features with which neuroradiologists should be familiar to aid in a prompt and accurate diagnosis. […] The preferred structural imaging technique for the diagnosis of PPA is MR imaging, due to its superior soft-tissue resolution and ability to precisely localize anatomic atrophy. […] The presence of specific regional patterns of atrophy or metabolic impairment is the key neuroimaging diagnostic feature for each of the 3 PPA variants. […] While the presence of left posterior peri-Sylvian or temporoparietal region atrophy is highly suggestive of lvPPA, its absence does not exclude the diagnosis. […] Structural imaging, including CT and MR imaging, can be used to identify these classic patterns of focal atrophy.
- #33 Primary Progressive Aphasia: Symptoms, Diagnosis, Treatment, and Morehttps://www.healthline.com/health/primary-progressive-aphasia
Tests might include: […] Blood tests can look for infections that cause similar symptoms. They can also look for genetic changes that are sometimes linked to neurological conditions, such as primary progressive aphasia. […] A speech-language evaluation can measure your speech and language skills against objective standards. […] Neurological tests will measure your ability to recall facts, name objects, and more. […] A brain scan can show brain atrophy. It can also detect visible signs of other conditions that could cause similar symptoms. The scan might be done with an: […] MRI […] single-photon emission computerized tomography (SPECT) scan […] PET scan. […] There is no cure for primary progressive aphasia, but speech therapy can help slow down the progression of symptoms.
- #34https://www.brain.northwestern.edu/dementia/primary-progressive-aphasia/diagnosis.html
There are newer tests that may assist in determining the likely underlying neuropathology responsible for the symptoms. These include amyloid PET and lumbar puncture (spinal tap) to examine protein levels in cerebrospinal fluid. These procedures may not be covered by insurance and may not be appropriate for everyone. Scientists are working to identify new reliable biomarkers. Our center is actively involved in these studies. […] Receiving a PPA diagnosis can be a complicated process. […] With PPA, problems with language and communication typically deteriorate gradually over time. Initial symptoms are sometimes attributed to anxiety or stress until the ongoing symptoms become disruptive enough to prompt further assessment by a specialist.
- #35 Diagnostic Assessment in Primary Progressive Aphasia: An Illustrative Case Examplehttps://pmc.ncbi.nlm.nih.gov/articles/PMC8740567/
The domains examined in a comprehensive speech and language battery are listed in Table 1, along with example tasks for assessing each domain and the expected behavioral pattern for each PPA variant. […] Examination of cognitive abilities is also standard practice in an evaluation of PPA to rule out other diagnoses or to identify cognitive impairments that may have emerged during the progression of the disease. […] MRI and other types of brain imaging may provide context for behavioral findings and can rule out other neurological causes for observed deficits. […] Important and emerging clinical applications of PET imaging involve the use of radioactive tracers to detect the presence of -amyloid plaques and neurofibrillary tau tangle aggregation. […] Additional neuropathological evidence for AD can be acquired by inspecting cerebrospinal fluid (CSF) for the 42 amino acid form of the amyloid- peptide (A42), total tau (t-tau), and phosphorylated tau (p-tau).
- #36 Diagnostic Assessment in Primary Progressive Aphasia: An Illustrative Case Examplehttps://pmc.ncbi.nlm.nih.gov/articles/PMC8740567/
The domains examined in a comprehensive speech and language battery are listed in Table 1, along with example tasks for assessing each domain and the expected behavioral pattern for each PPA variant. […] Examination of cognitive abilities is also standard practice in an evaluation of PPA to rule out other diagnoses or to identify cognitive impairments that may have emerged during the progression of the disease. […] MRI and other types of brain imaging may provide context for behavioral findings and can rule out other neurological causes for observed deficits. […] Important and emerging clinical applications of PET imaging involve the use of radioactive tracers to detect the presence of -amyloid plaques and neurofibrillary tau tangle aggregation. […] Additional neuropathological evidence for AD can be acquired by inspecting cerebrospinal fluid (CSF) for the 42 amino acid form of the amyloid- peptide (A42), total tau (t-tau), and phosphorylated tau (p-tau).
- #37 Diagnostic Assessment in Primary Progressive Aphasia: An Illustrative Case Examplehttps://pmc.ncbi.nlm.nih.gov/articles/PMC8740567/
Genetic testing is an additional tool that can be used to inform differential diagnosis. […] This case report illustrates a comprehensive and multidisciplinary evaluation conducted with a 49-year-old woman with complaints of progressive word-finding difficulty. […] The first step in the diagnostic process was determining whether DR met diagnostic criteria for PPA. […] Ruling out exclusionary factors for PPA required careful consideration. […] The neuroimaging and CSF results provided additional helpful information to inform DR’s diagnosis. […] Together with findings from the clinical evaluation, the diagnostic team determined that DR’s current presentation of symptoms was consistent with an initial lvPPA syndrome and early-onset AD pathology with predominant language symptoms, followed by the emergence of executive and memory difficulties. […] Advances in clinical research and diagnostic procedures are of paramount importance for detecting PPA as early as possible and for identifying the likely pathological basis, particularly in cases that are behaviorally complex or mixed in their speech-language presentation.
- #38 Diagnostic Assessment in Primary Progressive Aphasia: An Illustrative Case Examplehttps://pmc.ncbi.nlm.nih.gov/articles/PMC8740567/
In the largest genetic screening study of PPA to date, 14 of 403 cases (or 3.5%) had gene mutations, primarily in C9ORF72 and GRN. […] Genetic testing is not currently standard procedure in the assessment of PPA, emerging research in the genetics of PPA has demonstrated it may serve as a complementary tool for diagnosis. […] In current clinical practice, diagnosis and classification of PPA are determined by a clinician (typically a behavioral neurologist) based on the patient’s history of symptoms and family history, a neurological examination, a comprehensive evaluation of speech, language, and cognition, and clinical brain imaging. […] Skilled professionals in speech, language, and cognition, including SLPs and neuropsychologists, should play an integral role in the diagnostic process.
- #39 Diagnostic Assessment in Primary Progressive Aphasia: An Illustrative Case Examplehttps://pmc.ncbi.nlm.nih.gov/articles/PMC8740567/
In the largest genetic screening study of PPA to date, 14 of 403 cases (or 3.5%) had gene mutations, primarily in C9ORF72 and GRN. […] Genetic testing is not currently standard procedure in the assessment of PPA, emerging research in the genetics of PPA has demonstrated it may serve as a complementary tool for diagnosis. […] In current clinical practice, diagnosis and classification of PPA are determined by a clinician (typically a behavioral neurologist) based on the patient’s history of symptoms and family history, a neurological examination, a comprehensive evaluation of speech, language, and cognition, and clinical brain imaging. […] Skilled professionals in speech, language, and cognition, including SLPs and neuropsychologists, should play an integral role in the diagnostic process.
- #40 Primary progressive aphasia – Wikipediahttps://en.wikipedia.org/wiki/Primary_progressive_aphasia
In neurology, primary progressive aphasia (PPA) is a type of neurological syndrome in which language capabilities slowly and progressively become impaired. […] The following diagnosis criteria were defined by Mesulam: As opposed to having followed trauma to the brain, a patient must show an insidious onset and a gradual progression of aphasia, defined as a disorder of sentence and/or word usage, affecting the production and comprehension of speech. […] Whether or not PPA and other aphasias are the only source of cognitive impairment in a patient is often difficult to assess because: 1) as with other neurologically degenerative diseases, such as Alzheimer’s disease, there are currently no reliable non-invasive diagnostic tests for aphasias, and thus neuropsychological assessments are the only tool physicians have for diagnosing patients; and 2) aphasias often affect other, non-language portions of these neuropsychological tests, such as those specific for memory.
- #41 Primary Progressive Aphasias: Diagnosis and Treatmenthttps://www.mdpi.com/2076-3425/15/3/245
To make the diagnosis of PPA, it is essential to exclude other slowly progressive etiologies, especially intracranial tumors and vascular pathologies, either by computed tomography (CT) or MRI. In particular, MRI can provide positive clues about this disease: in progressive non-fluent aphasia, an atrophic enlargement of the left Sylvian fissure is typical, whereas for semantic dementia, atrophy of the left temporal lobe is especially typical. In the absence of atrophy, or if the differential diagnosis presents difficulties, the demonstration by nuclear medicine (CBF-SPECT or FDG-PET) of a typical localized and circumscribed decrease in activity can be useful to support the diagnosis of PPA.
- #42 Primary progressive aphasia: diagnosis and treatment – Terapia Online Presencial Madrid | Mentes Abiertashttps://www.mentesabiertaspsicologia.com/blog-psicologia/primary-progressive-aphasia-diagnosis-and-treatment
The diagnosis of Primary Progressive Aphasia can be complicated, as that the initial symptoms are often confused with other language disorders or cognitive problems. […] Health professionals, such as neurologists, speech therapists, and neuropsychologists, perform in-depth evaluations to determine the underlying cause of the symptoms. […] It is important to differentiate Primary Progressive Aphasia from other language disorders, such as aphasia caused by stroke, traumatic brain injury, or various neurodegenerative diseases. The use of neurological tests, speech and language evaluations, as well as neuroimaging tests, can help confirm the diagnosis of PPA. […] So far, There is no definitive cure for Primary Progressive Aphasia, as it is an irreversible neurodegenerative disorder. However, there are treatment and management strategies that can help improve the quality of life of affected people and reduce the impact of symptoms on their daily lives.
- #43 Diagnostic Assessment in Primary Progressive Aphasia: An Illustrative Case Examplehttps://pmc.ncbi.nlm.nih.gov/articles/PMC8740567/
For each PPA variant, diagnosis can be further supported by imaging if the clinical symptoms are accompanied by evidence of particular patterns of atrophy, hypoperfusion, or glucose hypometabolism in the brain. […] A clinical diagnosis of PPA requires predominant and progressive speech and/or language symptoms during initial stages of the disease, that these deficits are the primary limitation to activities of daily living, and that impairments cannot be better explained by psychiatric, behavioral, or nondegenerative central nervous system disorders. […] The second diagnostic step is classification by PPA clinical variant, when possible. […] The international consensus criteria for PPA define three clinical variants based on core impairments and associated features. […] The etiology of neurodegeneration for each of the three variants of PPA has been linked to different pathological processes.
- #44 Primary progressive aphasia – Wikipediahttps://en.wikipedia.org/wiki/Primary_progressive_aphasia
In 2011, the classification of primary progressive aphasia was updated to include three clinical variants. Patients must first be diagnosed with PPA, and then divided into variants based on speech production features, repetition, single-word and syntax comprehension, confrontation naming, semantic knowledge, and reading/spelling. […] For PNFA, the core criteria for diagnosis include agrammatism and slow and labored speech. […] The second variant, SD, presents with deficits in single-word and object comprehension. […] The logopenic variant involves impairments in word retrieval, sentence repetition, and phonological paraphasias, comparable to conduction aphasia. […] However, these PPA subtypes differ from similar aphasias, as these subtypes do not occur acutely following trauma to the brain, such as following a stroke, due to differing functional and structural neuroanatomical patterns of involvement and the progressive nature of the disease.
- #45 Primary Progressive Aphasias: Diagnosis and Treatmenthttps://www.mdpi.com/2076-3425/15/3/245
Diagnostic Criteria for PPA According to Mesulam. Gorno-Tempini et al. proposed updated general criteria for the diagnosis of Primary Progressive Aphasia (PPA), building on and expanding Mesulamâs original criteria. These new criteria are less restrictive than those outlined by Mesulam. For instance, Gorno-Tempini introduced the broader concept of a âlanguage complaintâ without specifically requiring the detailed language impairments mentioned by Mesulam, such as anomia or spontaneous language comprehension disorders. […] The classification of PPA is based on the correlation between clinical criteria and additional examinations (imaging and biomarkers). Three subtypes of PPA are described: Non-fluent PPA; Semantic PPA; Logopenic PPA. Part of the diagnosis of PPA relies on the fluency criterion. Non-fluent aphasias are characterized by both quantitative and qualitative reductions in speech. Quantitatively, this reduction is reflected in a lower average number of consecutive words produced in a single utterance (typically â¤4 words). Qualitatively, it involves a reduction in or even omission of syntactic structures. In contrast, semantic aphasia is marked by impaired language quality while maintaining a preserved flow, which can sometimes be logorrheic, with a fluency of â¥6 words per utterance. Logopenic aphasias represent an intermediate stage, featuring a slowed verbal flow due to frequent interruptions and hesitationsâprimarily resulting from word retrieval difficultiesâwith fluency typically ranging between five and seven words per utterance.
- #46 Primary Progressive Aphasia (PPA) | Dementia Research Centre – UCL â University College Londonhttps://www.ucl.ac.uk/drc/ftd-support-group/information-about-ftd-and-other-dementias/primary-progressive-aphasia-ppa
There is no single test that allows doctors to make a diagnosis of SD. Usually the diagnosis is made using a combination of clinical assessment, psychology testing and a brain scan. MRI scanning shows loss of brain cells in the temporal lobe, usually more on the left than the right. […] As with SD, there is no single test that allows doctors to make a diagnosis of PNFA. Usually the diagnosis is made using a combination of clinical assessment, psychology testing and a brain scan. MRI scanning shows loss of brain cells in the speech areas of the brain, particularly the frontal lobe on the left side. […] There is no single test that allows doctors to make a diagnosis of LPA. Usually the diagnosis is made using a combination of clinical assessment, psychology testing and a brain scan. Brain scanning shows loss of brain cells in areas further towards the back of the brain than the other PPA subtypes, particularly the area where the temporal and parietal lobes meet.
- #47 Primary Progressive Aphasia | Treatment & Management | Point of Carehttps://www.statpearls.com/point-of-care/82030
PPA characterization and differentiation based on linguistic and nonlinguistic profiles are now easier to accomplish due to extensive research. […] However, despite significant diagnostic advances, a clear consensus on its pathology remains elusive. […] PPA classification and diagnosis can sometimes be controversial. […] Understanding PPA’s neuroanatomical basis is crucial for accurate diagnosis, prognosis estimation, and developing targeted interventions tailored to individual patients’ specific language deficits and affected brain regions. […] The clinical criteria for diagnosing nfvPPA require at least 1 core feature, either agrammatism or effortful speech with inconsistent errors. […] The current diagnostic guidelines identify anomia and single-word comprehension deficits as core features, and both are essential for diagnosis.
- #48 Primary Progressive Aphasia (PPA) | Dementia Research Centre – UCL â University College Londonhttps://www.ucl.ac.uk/drc/ftd-support-group/information-about-ftd-and-other-dementias/primary-progressive-aphasia-ppa
There is no single test that allows doctors to make a diagnosis of SD. Usually the diagnosis is made using a combination of clinical assessment, psychology testing and a brain scan. MRI scanning shows loss of brain cells in the temporal lobe, usually more on the left than the right. […] As with SD, there is no single test that allows doctors to make a diagnosis of PNFA. Usually the diagnosis is made using a combination of clinical assessment, psychology testing and a brain scan. MRI scanning shows loss of brain cells in the speech areas of the brain, particularly the frontal lobe on the left side. […] There is no single test that allows doctors to make a diagnosis of LPA. Usually the diagnosis is made using a combination of clinical assessment, psychology testing and a brain scan. Brain scanning shows loss of brain cells in areas further towards the back of the brain than the other PPA subtypes, particularly the area where the temporal and parietal lobes meet.
- #49 Primary Progressive Aphasia (PPA) | Dementia Research Centre – UCL â University College Londonhttps://www.ucl.ac.uk/drc/ftd-support-group/information-about-ftd-and-other-dementias/primary-progressive-aphasia-ppa
There is no single test that allows doctors to make a diagnosis of SD. Usually the diagnosis is made using a combination of clinical assessment, psychology testing and a brain scan. MRI scanning shows loss of brain cells in the temporal lobe, usually more on the left than the right. […] As with SD, there is no single test that allows doctors to make a diagnosis of PNFA. Usually the diagnosis is made using a combination of clinical assessment, psychology testing and a brain scan. MRI scanning shows loss of brain cells in the speech areas of the brain, particularly the frontal lobe on the left side. […] There is no single test that allows doctors to make a diagnosis of LPA. Usually the diagnosis is made using a combination of clinical assessment, psychology testing and a brain scan. Brain scanning shows loss of brain cells in areas further towards the back of the brain than the other PPA subtypes, particularly the area where the temporal and parietal lobes meet.
- #50 Classification of the primary progressive aphasias: principles and review of progress since 2011 | Alzheimer’s Research & Therapy | Full Texthttps://alzres.biomedcentral.com/articles/10.1186/s13195-016-0185-y
In a monocentric series of 84 PPA patients, 31 % could not be assigned to any of the three subtypes. […] The mixed subtype exhibits word comprehension deficits along with speech apraxia or agrammatism, a combination of positive findings that does not occur in the current classification. […] The clinical utility of biomarkers such as cerebrospinal fluid A42 and amyloid PET in patients with PPA presenting in a memory clinic depends on the difference between pre- and post-test odds of an etiological diagnosis. […] None of the three phenotypic subtypes entirely excludes the possibility of AD as the underlying cause. […] The clinical utility of an etiological diagnosis in PPA has not been proven empirically. […] The observation that a substantial proportion of cases who fulfill the root criterion of PPA do not fall into one of the subtypes is not necessarily a reason for a revision.
- #51 Classification of the primary progressive aphasias: principles and review of progress since 2011 | Alzheimer’s Research & Therapy | Full Texthttps://alzres.biomedcentral.com/articles/10.1186/s13195-016-0185-y
In a monocentric series of 84 PPA patients, 31 % could not be assigned to any of the three subtypes. […] The mixed subtype exhibits word comprehension deficits along with speech apraxia or agrammatism, a combination of positive findings that does not occur in the current classification. […] The clinical utility of biomarkers such as cerebrospinal fluid A42 and amyloid PET in patients with PPA presenting in a memory clinic depends on the difference between pre- and post-test odds of an etiological diagnosis. […] None of the three phenotypic subtypes entirely excludes the possibility of AD as the underlying cause. […] The clinical utility of an etiological diagnosis in PPA has not been proven empirically. […] The observation that a substantial proportion of cases who fulfill the root criterion of PPA do not fall into one of the subtypes is not necessarily a reason for a revision.
- #52 Primary progressive aphasia: diagnosis and treatment – Terapia Online Presencial Madrid | Mentes Abiertashttps://www.mentesabiertaspsicologia.com/blog-psicologia/primary-progressive-aphasia-diagnosis-and-treatment
The diagnosis of Primary Progressive Aphasia can be complicated, as that the initial symptoms are often confused with other language disorders or cognitive problems. […] Health professionals, such as neurologists, speech therapists, and neuropsychologists, perform in-depth evaluations to determine the underlying cause of the symptoms. […] It is important to differentiate Primary Progressive Aphasia from other language disorders, such as aphasia caused by stroke, traumatic brain injury, or various neurodegenerative diseases. The use of neurological tests, speech and language evaluations, as well as neuroimaging tests, can help confirm the diagnosis of PPA. […] So far, There is no definitive cure for Primary Progressive Aphasia, as it is an irreversible neurodegenerative disorder. However, there are treatment and management strategies that can help improve the quality of life of affected people and reduce the impact of symptoms on their daily lives.
- #53 The course of primary progressive aphasia diagnosis: a cross-sectional study | Alzheimer’s Research & Therapy | Full Texthttps://alzres.biomedcentral.com/articles/10.1186/s13195-022-01007-6
The delay between the first consultation for cognitive troubles and the first diagnosis that is significantly longer than in the AD group. […] The number of different diagnoses before the diagnosis of interest that is significantly higher in the PPA group than in the AD group. […] To summarize, despite the evolution of the diagnostic criteria of primary progressive aphasia over time, their diagnostic still does not seem optimal. […] Our study shows also that PPA diagnosis is particularly important because it modifies pharmacological and nonpharmacological interventions. […] It thus appears essential to establish a diagnosis of PPA to set up an adapted medical treatment. […] The importance of early diagnosis between PPA and AD due to differences in therapeutic approaches.
- #54 Primary Progressive Aphasia (PPA) | AFTDhttps://www.theaftd.org/what-is-ftd/primary-progressive-aphasia/
PPA is diagnosed when three criteria are met: There is a gradual impairment of language (not just speech). The language problem is initially the only impairment. The underlying cause is a neurodegenerative disease. […] Not all physicians know about FTD, so it is often misdiagnosed or not diagnosed at all. AFTD developed a checklist to help identify red flags for behavioral variant FTD (bvFTD) that you can bring to your doctor. The back of the document is designed to help your physician better understand FTD diagnostic criteria.
- #55https://www.brain.northwestern.edu/dementia/primary-progressive-aphasia/diagnosis.html
There are newer tests that may assist in determining the likely underlying neuropathology responsible for the symptoms. These include amyloid PET and lumbar puncture (spinal tap) to examine protein levels in cerebrospinal fluid. These procedures may not be covered by insurance and may not be appropriate for everyone. Scientists are working to identify new reliable biomarkers. Our center is actively involved in these studies. […] Receiving a PPA diagnosis can be a complicated process. […] With PPA, problems with language and communication typically deteriorate gradually over time. Initial symptoms are sometimes attributed to anxiety or stress until the ongoing symptoms become disruptive enough to prompt further assessment by a specialist.
- #56 A Diagnosis of Primary Progressive Aphasia? What to Expect⦠– The National Aphasia Associationhttps://aphasia.org/stories/ppa-what-to-expect/
Primary Progressive Aphasia (PPA) is caused by the gradual degeneration of brain tissue in the parts of the brain that control speech and language. […] Many of the individuals with PPA that we have seen have not been given a firm diagnosis and very few have information on the specific subtype. […] The best way to receive a detailed evaluation and prognosis about PPA is a referral to a neurologist or neuropsychologist who has experience with PPA. […] Initially, for a time period that can last a couple of years, a person diagnosed with PPA will exhibit a progressive loss of speech and language capabilities (aphasia). […] Due to the progressive nature of PPA, language abilities will continue to deteriorate over many years. […] With time, PPA may affect other cognitive abilities such as memory, reasoning and visual perceptual skills.
- #57 The course of primary progressive aphasia diagnosis: a cross-sectional study | Alzheimer’s Research & Therapy | Full Texthttps://alzres.biomedcentral.com/articles/10.1186/s13195-022-01007-6
The delay between the first consultation for cognitive troubles and the first diagnosis that is significantly longer than in the AD group. […] The number of different diagnoses before the diagnosis of interest that is significantly higher in the PPA group than in the AD group. […] To summarize, despite the evolution of the diagnostic criteria of primary progressive aphasia over time, their diagnostic still does not seem optimal. […] Our study shows also that PPA diagnosis is particularly important because it modifies pharmacological and nonpharmacological interventions. […] It thus appears essential to establish a diagnosis of PPA to set up an adapted medical treatment. […] The importance of early diagnosis between PPA and AD due to differences in therapeutic approaches.
- #58 The course of primary progressive aphasia diagnosis: a cross-sectional study | Alzheimer’s Research & Therapy | Full Texthttps://alzres.biomedcentral.com/articles/10.1186/s13195-022-01007-6
The primary progressive aphasia (PPA) diagnosis trajectory is debated, as several changes in diagnosis occur during PPA course, due to phenotype evolution from isolated language alterations to global cognitive impairment. […] Proper PPA diagnosis increases the opportunities of providing early appropriate clinical interventions, implementing coordinated care plans, managing symptoms, improving patient safety, cost savings and postponing institutionalization. […] The diagnosis of PPA is a major challenge in clinical practice as this phenotype is complex and constantly evolving. […] Our secondary objectives were to determine if the diagnosis of PPA is more difficult to establish (more changes in diagnosis before the PPA diagnosis) and more delayed, compared to AD diagnosis, and to compare the two syndromes according to the recommended therapeutic approaches.
- #59https://irispublishers.com/ann/fulltext/primary-progressive-aphasia-diagnosis-and-contribution-of-graphic-markers.ID.000633.php
Primary Progressive Aphasia (PPA) is a neurodegenerative disease characterized by a foreground linguistic deficit that extends to other cognitive functions. […] An early differential diagnosis between the different aetiologies seems crucial to set up an appropriate care but remains difficult to make, according to the several diagnosis changes that can occur. […] The PPA diagnosis is made when the three following criteria are met: there is a progressive aphasic disorder that increases distortions of word usage or comprehension; the language impairment constitutes the most salient deficit and causes most of the daily living activities impairment during the initial stages of illness; and the underlying disease is considered neurodegenerative. […] The neurodegenerative diagnosis trajectory is still debated as several diagnosis changes occur during the course of this disease due to phenotype evolution from isolated language alterations to global cognitive impairment with multiple neuropsychiatric symptoms.
- #60 Azthena logo with the word Azthenahttps://www.news-medical.net/news/20250430/Early-detection-of-primary-progressive-aphasia-through-speech-and-hearing-tests.aspx
Difficulty finding words or the habit of substituting them with others that are similar semantically such as knife and cutter or phonologically such as knife and wife are usually the first symptoms of a type of dementia known as primary progressive aphasia (PPA). […] Primary progressive aphasia is a rare and difficult-to-diagnose neurodegenerative disease that begins with language problems that affect communication and progresses to more serious cognitive changes that may resemble Alzheimer’s disease or other types of dementia. […] The neurological diagnosis of this dementia syndrome requires a complete analysis that includes other cognitive functions in addition to language and communication problems. […] Now, a study supported by FAPESP and published in the journal PLOS ONE has shown that this condition can be detected early using a set of speech and hearing tests known as the Montreal-Toulouse Language Assessment Battery (MTL-BR).
- #61 Diagnostic Assessment in Primary Progressive Aphasia: An Illustrative Case Examplehttps://pmc.ncbi.nlm.nih.gov/articles/PMC8740567/
The domains examined in a comprehensive speech and language battery are listed in Table 1, along with example tasks for assessing each domain and the expected behavioral pattern for each PPA variant. […] Examination of cognitive abilities is also standard practice in an evaluation of PPA to rule out other diagnoses or to identify cognitive impairments that may have emerged during the progression of the disease. […] MRI and other types of brain imaging may provide context for behavioral findings and can rule out other neurological causes for observed deficits. […] Important and emerging clinical applications of PET imaging involve the use of radioactive tracers to detect the presence of -amyloid plaques and neurofibrillary tau tangle aggregation. […] Additional neuropathological evidence for AD can be acquired by inspecting cerebrospinal fluid (CSF) for the 42 amino acid form of the amyloid- peptide (A42), total tau (t-tau), and phosphorylated tau (p-tau).
- #62https://www.brain.northwestern.edu/dementia/primary-progressive-aphasia/diagnosis.html
There are newer tests that may assist in determining the likely underlying neuropathology responsible for the symptoms. These include amyloid PET and lumbar puncture (spinal tap) to examine protein levels in cerebrospinal fluid. These procedures may not be covered by insurance and may not be appropriate for everyone. Scientists are working to identify new reliable biomarkers. Our center is actively involved in these studies. […] Receiving a PPA diagnosis can be a complicated process. […] With PPA, problems with language and communication typically deteriorate gradually over time. Initial symptoms are sometimes attributed to anxiety or stress until the ongoing symptoms become disruptive enough to prompt further assessment by a specialist.
- #63 Multimodality Imaging in Primary Progressive Aphasia | American Journal of Neuroradiologyhttp://www.ajnr.org/content/early/2022/08/25/ajnr.A7613
Molecular imaging allows in vivo identification and quantification of cerebral metabolism, abnormal deposition of -amyloid and , and the presence of brain inflammation, important neuroimaging biomarkers that may improve early diagnosis and assist in assessing neurodegenerative disease progression. […] Currently available molecular imaging modalities include SPECT and PET, with a number of investigational radiotracers on the horizon. […] Molecular neuroimaging has been shown to be useful in the diagnosis of PPA. […] A number of structural, functional, and molecular imaging modalities can support an accurate diagnosis, and neuroradiologists should be familiar with the classic imaging features of each PPA subtype, because prompt and accurate diagnosis may allow improved outcomes and intervention, particularly as disease-modifying therapies enter clinical practice.
- #64 SYDBAT App Can Help with Early Primary Progressive Aphasia Diagnosis â Patient Worthyhttps://patientworthy.com/2022/07/18/sydbat-app-can-help-early-ppa-diagnosis/
Primary progressive aphasia (PPA) is a rare neurological disorder and form of frontotemporal dementia which affects oneâs ability to communicate. In many cases, people develop PPA prior to turning 65 years old. Sometimes, in early stages, it can be difficult to diagnose someone with PPA, as the symptoms and presentation are still relatively subtle. However, this can cause issues down the line, particularly in relation to accessing adequate care. […] According to an article from the University of Sydneyâs Brain and Mind Centre, the Centreâs FRONTIER Research Group has developed an application (âappâ) called the Sydney Language Battery (SYDBAT) app to help with early PPA diagnoses. This app, which can be downloaded for free from Apple, offers four language and speech evaluations, records speech, exports data, and scores results automatically. […] Currently, the SYDBAT app is used in Australia to help provide PPA diagnoses. Now that the app is available for download on Apple devices, researchers hope that it will improve diagnoses and outcomes on a more global scale.
- #65 Primary Progressive Aphasia: Use of Graphical Markers for an Early and Differential Diagnosishttps://www.mdpi.com/2076-3425/11/9/1198
Most of the scales available so far for PPA diagnosis are based on language production and comprehension in oral and written modalities. […] The use of new technologies allows more ecological and reproducible tests in comparison to certain scales or paperâpencil tests. […] With language symptoms being the earliest and most prominent signs in the early stages of the disease, graphical writing markers may constitute ecological markers of great interest for the early diagnosis of PPA. […] The aim of this study was to confirm the initial findings of Gros et al. on a larger sample of PPA, concerning the role of writing pressure in differentiating PPA and controls, and to verify if writing pressure is also relevant to distinguish patients with PPA and Alzheimerâs disease. […] In conclusion, graphical markers may allow the performance of an early and differential diagnosis of patients with PPA and patients with AD.
- #66 Primary Progressive Aphasia: Use of Graphical Markers for an Early and Differential Diagnosishttps://www.mdpi.com/2076-3425/11/9/1198
Primary progressive aphasia (PPA) brings together neurodegenerative pathologies whose main characteristic is to start with a progressive language disorder. PPA diagnosis is often delayed in non-specialised clinical settings. […] The objective was to verify if the writing pressure in different linguistic and non-linguistic tasks can differentiate patients with PPA from patients with AD and healthy subjects. […] PPA is diagnosed when three criteria overlap: (1) language is mainly damaged; (2) daily living activities are impaired during the initial stages of illness; and (3) word production and comprehension are impaired due to a progressive aphasic disorder and there is an underlying neurodegenerative disease. […] Early diagnosis of PPA is important in clinical practice because its phenotype is complex, constantly evolving, and is crucial because it increases the possibilities of appropriate clinical interventions.
- #67 Primary Progressive Aphasia: Use of Graphical Markers for an Early and Differential Diagnosishttps://www.mdpi.com/2076-3425/11/9/1198
Most of the scales available so far for PPA diagnosis are based on language production and comprehension in oral and written modalities. […] The use of new technologies allows more ecological and reproducible tests in comparison to certain scales or paperâpencil tests. […] With language symptoms being the earliest and most prominent signs in the early stages of the disease, graphical writing markers may constitute ecological markers of great interest for the early diagnosis of PPA. […] The aim of this study was to confirm the initial findings of Gros et al. on a larger sample of PPA, concerning the role of writing pressure in differentiating PPA and controls, and to verify if writing pressure is also relevant to distinguish patients with PPA and Alzheimerâs disease. […] In conclusion, graphical markers may allow the performance of an early and differential diagnosis of patients with PPA and patients with AD.
- #68https://link.springer.com/article/10.1007/s00415-023-12030-4
Several cognitive instruments have been designed to facilitate the relatively rapid diagnosis of PPA syndromes in the clinic. […] These instruments have yet to be assessed head-to-head (or indeed, in combination) but we would emphasise that some clinical experience of PPA is required to use them optimally. […] There is growing interest in automated diagnosis based on machine learning approaches and the identification of digital markers that might detect very early stage PPA. […] Predicting the underlying proteinopathy in PPA is potentially relevant to prognosis and care planning, and will be increasingly required for the rational targeting of disease-modifying therapies. […] However, this is far from straightforward. […] The strongest and most consistent association links svPPA to TAR-DBA-binding-protein (TDP)-43 type C pathology, in line with the striking clinico-anatomical coherence of this syndrome.
- #69https://irispublishers.com/ann/fulltext/primary-progressive-aphasia-diagnosis-and-contribution-of-graphic-markers.ID.000633.php
Early diagnosis of PPA is therefore crucial because it increases the possibilities of appropriate clinical interventions, coordinated care plans, symptom management, improvement of patient safety, but also the possibility of reducing health costs and the period before the institutionalization of affected persons. […] Nevertheless, the diagnosis of APP is a major challenge in clinical practice because this phenotype is complex and constantly evolving.
- #70https://link.springer.com/article/10.1007/s12149-024-01958-w
The disease progresses in a matter of years while leaving behavioral aspects relatively intact during the early stages. […] Within the diagnostic framework of PPA, the condition is further subdivided into three subtypes: Semantic Dementia (SD), Progressive Non-Fluent Aphasia (PNFA), also known as the agrammatic variant (agPPA), and Logopenic Progressive Aphasia (LPA). […] However, existing diagnostic criteria using only these three variants may be inadequate in encompassing all individuals with PPA. […] An increasing body of literature now supports the likelihood of 56 subtypes instead of the traditionally recognized three. […] Early PPA identification can significantly impacts patient management and prognosis. […] Given that PPA is a neurodegenerative condition leading to a gradual loss of language abilities, early diagnosis allows for timely intervention and the implementation of appropriate therapeutic strategies.
- #71https://link.springer.com/article/10.1007/s12149-024-01958-w
The disease progresses in a matter of years while leaving behavioral aspects relatively intact during the early stages. […] Within the diagnostic framework of PPA, the condition is further subdivided into three subtypes: Semantic Dementia (SD), Progressive Non-Fluent Aphasia (PNFA), also known as the agrammatic variant (agPPA), and Logopenic Progressive Aphasia (LPA). […] However, existing diagnostic criteria using only these three variants may be inadequate in encompassing all individuals with PPA. […] An increasing body of literature now supports the likelihood of 56 subtypes instead of the traditionally recognized three. […] Early PPA identification can significantly impacts patient management and prognosis. […] Given that PPA is a neurodegenerative condition leading to a gradual loss of language abilities, early diagnosis allows for timely intervention and the implementation of appropriate therapeutic strategies.
- #72https://link.springer.com/article/10.1007/s12149-024-01958-w
Moreover, early identification enables healthcare professionals to differentiate PPA from other forms of dementia, facilitating accurate prognosis and tailored care plans. […] Positron emission tomography (PET) using 2-deoxy-2-[18F]fluoro-D-glucose (FDG) holds a distinct advantage over other neuroimaging modalities due to its ability to provide functional information about glucose metabolism in the brain. […] By visualizing metabolic alterations preceding atrophy, FDG-PET has been extensively applied to PPA with the intention of uncovering underlying metabolic dysfunction prior to structural changes, demonstrating its possible benefit with PPA diagnosis. […] The reviewed studies collectively highlight the utility of FDG-PET in identifying distinct metabolic patterns associated with different PPA subtypes, though they also reveal some discrepancies.
- #73https://irispublishers.com/ann/fulltext/primary-progressive-aphasia-diagnosis-and-contribution-of-graphic-markers.ID.000633.php
Early diagnosis of PPA is therefore crucial because it increases the possibilities of appropriate clinical interventions, coordinated care plans, symptom management, improvement of patient safety, but also the possibility of reducing health costs and the period before the institutionalization of affected persons. […] Nevertheless, the diagnosis of APP is a major challenge in clinical practice because this phenotype is complex and constantly evolving.
- #74https://irispublishers.com/ann/fulltext/primary-progressive-aphasia-diagnosis-and-contribution-of-graphic-markers.ID.000633.php
Early diagnosis of PPA is therefore crucial because it increases the possibilities of appropriate clinical interventions, coordinated care plans, symptom management, improvement of patient safety, but also the possibility of reducing health costs and the period before the institutionalization of affected persons. […] Nevertheless, the diagnosis of APP is a major challenge in clinical practice because this phenotype is complex and constantly evolving.
- #75https://link.springer.com/article/10.1007/s00415-023-12030-4
Here, we review recent progress in the diagnosis and management of primary progressive aphasiaâthe language-led dementias. […] How can we diagnose (and track) primary progressive aphasia better? […] Timely diagnosis of PPA resolves uncertainty for patients and families, enables future planning and unlocks access to services and support. […] The dawning of the age of disease-modifying therapies for neurodegenerative disease, with its imperative to diagnose dementias earlier and more accurately, has only amplified this issue. […] However, the clinical diagnosis of PPA is often challenging even for those with extensive experience of the syndromes. […] We present some clinical, cognitive and neuroimaging fingerprints that we have found particularly useful in the early detection of PPA syndromes in Table 1, an approach to bedside syndromic diagnosis in Fig. 1 and an outline of ancillary investigations in Fig. 2.
- #76 The course of primary progressive aphasia diagnosis: a cross-sectional study | Alzheimer’s Research & Therapy | Full Texthttps://alzres.biomedcentral.com/articles/10.1186/s13195-022-01007-6
The delay between the first consultation for cognitive troubles and the first diagnosis that is significantly longer than in the AD group. […] The number of different diagnoses before the diagnosis of interest that is significantly higher in the PPA group than in the AD group. […] To summarize, despite the evolution of the diagnostic criteria of primary progressive aphasia over time, their diagnostic still does not seem optimal. […] Our study shows also that PPA diagnosis is particularly important because it modifies pharmacological and nonpharmacological interventions. […] It thus appears essential to establish a diagnosis of PPA to set up an adapted medical treatment. […] The importance of early diagnosis between PPA and AD due to differences in therapeutic approaches.
- #77 Primary Progressive Aphasia (PPA): Symptoms & Treatmenthttps://my.clevelandclinic.org/health/diseases/17387-primary-progressive-aphasia-ppa
Primary progressive aphasia (PPA) is a neurological condition that causes you to lose language skills. Its a type of dementia and may be a sign of Alzheimers disease. […] Primary progressive aphasia develops when the parts of your brain that control language start breaking down. Your brain tissue shrinks (atrophies), affecting your ability to communicate. […] Your healthcare provider may suspect PPA based on your symptoms. Theyll evaluate your medical history and family history to see if you have an increased risk of primary progressive aphasia. […] You might have specialized cognitive tests and brain scans, such as an MRI or CT scan, to confirm a diagnosis. […] There isnt a cure for primary progressive aphasia and no way to stop its progression. But some treatments may slow the diseases progression or increase your quality of life.
- #78 Primary Progressive Aphasia | Memory and Aging Centerhttps://memory.ucsf.edu/dementia/primary-progressive-aphasia
People with language difficulties may benefit from speech therapy to help them learn alternative ways to supplement and compensate for their lost skills. […] Unlike many people who develop aphasia from head injury or stroke, people with PPA do not typically improve with time, but a therapist may be helpful in maximizing abilities and exploring other ways to communicate. […] Many speech pathologists and occupational therapists have their own practices, while others are available through local hospitals and medical centers.
- #79https://link.springer.com/article/10.1007/s00415-023-12030-4
Several cognitive instruments have been designed to facilitate the relatively rapid diagnosis of PPA syndromes in the clinic. […] These instruments have yet to be assessed head-to-head (or indeed, in combination) but we would emphasise that some clinical experience of PPA is required to use them optimally. […] There is growing interest in automated diagnosis based on machine learning approaches and the identification of digital markers that might detect very early stage PPA. […] Predicting the underlying proteinopathy in PPA is potentially relevant to prognosis and care planning, and will be increasingly required for the rational targeting of disease-modifying therapies. […] However, this is far from straightforward. […] The strongest and most consistent association links svPPA to TAR-DBA-binding-protein (TDP)-43 type C pathology, in line with the striking clinico-anatomical coherence of this syndrome.
- #80 A Diagnosis of Primary Progressive Aphasia? What to Expect⦠– The National Aphasia Associationhttps://aphasia.org/stories/ppa-what-to-expect/
Primary Progressive Aphasia (PPA) is caused by the gradual degeneration of brain tissue in the parts of the brain that control speech and language. […] Many of the individuals with PPA that we have seen have not been given a firm diagnosis and very few have information on the specific subtype. […] The best way to receive a detailed evaluation and prognosis about PPA is a referral to a neurologist or neuropsychologist who has experience with PPA. […] Initially, for a time period that can last a couple of years, a person diagnosed with PPA will exhibit a progressive loss of speech and language capabilities (aphasia). […] Due to the progressive nature of PPA, language abilities will continue to deteriorate over many years. […] With time, PPA may affect other cognitive abilities such as memory, reasoning and visual perceptual skills.
- #81 Diagnostic Assessment in Primary Progressive Aphasia: An Illustrative Case Examplehttps://pmc.ncbi.nlm.nih.gov/articles/PMC8740567/
In the largest genetic screening study of PPA to date, 14 of 403 cases (or 3.5%) had gene mutations, primarily in C9ORF72 and GRN. […] Genetic testing is not currently standard procedure in the assessment of PPA, emerging research in the genetics of PPA has demonstrated it may serve as a complementary tool for diagnosis. […] In current clinical practice, diagnosis and classification of PPA are determined by a clinician (typically a behavioral neurologist) based on the patient’s history of symptoms and family history, a neurological examination, a comprehensive evaluation of speech, language, and cognition, and clinical brain imaging. […] Skilled professionals in speech, language, and cognition, including SLPs and neuropsychologists, should play an integral role in the diagnostic process.
- #82 Get Primary Progressive Aphasia Treatment | Cleveland Clinichttps://my.clevelandclinic.org/services/primary-progressive-aphasia-treatment
Diagnosing Primary Progressive Aphasia (PPA) at Cleveland Clinic […] When aphasia is caused by a neurodegenerative disease like Alzheimers disease or frontotemporal dementia, we call it primary progressive aphasia because it progresses or gets worse over time. […] So, if youre finding it harder to hold conversations or remember words, its time to see a healthcare provider experienced in treating PPA. […] The first thing well do is take some time to get to know you. […] We look at things like language, memory, problem-solving, learning and more. […] CT scans (computed tomography scans), MRIs (magnetic resonance imaging) and PET scans (positron emission tomography) take detailed pictures of your brain so we have a clear idea of whats going on inside. […] Your team may include: Neurologists, Geriatricians, Psychiatrists, Neuropsychologists, Nurse practitioners, Physician assistants, Speech therapists. […] When we build your care plan, we focus on slowing PPAs progression. […] You may start taking medications to help manage the changes that can come with PPA.
- #83 Diagnostic Assessment in Primary Progressive Aphasia: An Illustrative Case Examplehttps://pmc.ncbi.nlm.nih.gov/articles/PMC8740567/
In the largest genetic screening study of PPA to date, 14 of 403 cases (or 3.5%) had gene mutations, primarily in C9ORF72 and GRN. […] Genetic testing is not currently standard procedure in the assessment of PPA, emerging research in the genetics of PPA has demonstrated it may serve as a complementary tool for diagnosis. […] In current clinical practice, diagnosis and classification of PPA are determined by a clinician (typically a behavioral neurologist) based on the patient’s history of symptoms and family history, a neurological examination, a comprehensive evaluation of speech, language, and cognition, and clinical brain imaging. […] Skilled professionals in speech, language, and cognition, including SLPs and neuropsychologists, should play an integral role in the diagnostic process.
- #84 Diagnosis and Treatment of Primary Progressive Aphasia: Where Do SLPs Belong? | Plural Publishinghttps://www.pluralpublishing.com/blog/diagnosis-and-treatment-primary-progressive-aphasia-where-do-slps-belong?srsltid=AfmBOoq08dxIOepBhdaIYJaBor7u062pwrm66ANL3mcEzk87lTS73LMm
When evaluating someone who may have PPA, it is important that speech-language pathologists (SLPs) ascertain the onset and course of the communication problem and determine through examination if it represents a focal language deficit (aphasia) versus a disorder more in keeping with a broader constellation of non-aphasic cognitive communication deficits. […] PPA can be a devastating diagnosis for many reasons, but especially because there are no disease-modifying options at this time. However, patients and their loved ones are often relieved to finally have a diagnosis that accurately captures their difficulty and makes some predictions about the future. Once a diagnosis is established, SLPs can offer a wealth of information to help patients, families, and care partners adapt to the loss of cognitive-communication functions.
- #85 Diagnosis and Treatment of Primary Progressive Aphasia: Where Do SLPs Belong? | Plural Publishinghttps://www.pluralpublishing.com/blog/diagnosis-and-treatment-primary-progressive-aphasia-where-do-slps-belong?srsltid=AfmBOoq08dxIOepBhdaIYJaBor7u062pwrm66ANL3mcEzk87lTS73LMm
When evaluating someone who may have PPA, it is important that speech-language pathologists (SLPs) ascertain the onset and course of the communication problem and determine through examination if it represents a focal language deficit (aphasia) versus a disorder more in keeping with a broader constellation of non-aphasic cognitive communication deficits. […] PPA can be a devastating diagnosis for many reasons, but especially because there are no disease-modifying options at this time. However, patients and their loved ones are often relieved to finally have a diagnosis that accurately captures their difficulty and makes some predictions about the future. Once a diagnosis is established, SLPs can offer a wealth of information to help patients, families, and care partners adapt to the loss of cognitive-communication functions.
- #86 Diagnosis and Treatment of Primary Progressive Aphasia: Where Do SLPs Belong? | Plural Publishinghttps://www.pluralpublishing.com/blog/diagnosis-and-treatment-primary-progressive-aphasia-where-do-slps-belong?srsltid=AfmBOoq08dxIOepBhdaIYJaBor7u062pwrm66ANL3mcEzk87lTS73LMm
When evaluating someone who may have PPA, it is important that speech-language pathologists (SLPs) ascertain the onset and course of the communication problem and determine through examination if it represents a focal language deficit (aphasia) versus a disorder more in keeping with a broader constellation of non-aphasic cognitive communication deficits. […] PPA can be a devastating diagnosis for many reasons, but especially because there are no disease-modifying options at this time. However, patients and their loved ones are often relieved to finally have a diagnosis that accurately captures their difficulty and makes some predictions about the future. Once a diagnosis is established, SLPs can offer a wealth of information to help patients, families, and care partners adapt to the loss of cognitive-communication functions.
- #87https://www.brain.northwestern.edu/dementia/primary-progressive-aphasia/diagnosis.html
There are newer tests that may assist in determining the likely underlying neuropathology responsible for the symptoms. These include amyloid PET and lumbar puncture (spinal tap) to examine protein levels in cerebrospinal fluid. These procedures may not be covered by insurance and may not be appropriate for everyone. Scientists are working to identify new reliable biomarkers. Our center is actively involved in these studies. […] Receiving a PPA diagnosis can be a complicated process. […] With PPA, problems with language and communication typically deteriorate gradually over time. Initial symptoms are sometimes attributed to anxiety or stress until the ongoing symptoms become disruptive enough to prompt further assessment by a specialist.
- #88 Primary Progressive Aphasia Resources | Integrative Reconnective Aphasia Therapyhttps://www.iraphasiatherapy.com/ppa-resources
Primary Progressive Aphasia, also known as PPA, is a degenerative neurological condition in which language capabilities become impaired over time. However, it defers greatly from other forms of Aphasia. […] Receiving a diagnosis of primary progressive aphasia is hard. Sometimes it may only be in hindsight that all of the early signs of difficulty even become apparent. Learn more about what the journey to diagnosis of PPA may look like and how a Speech Language Pathologist plays a role in treatment for communication. […] We want to provide information and resources on both Acquired Aphasia and Primary Progressive Aphasia.
- #89 Diagnosis and Treatment of Primary Progressive Aphasia: Where Do SLPs Belong? | Plural Publishinghttps://www.pluralpublishing.com/blog/diagnosis-and-treatment-primary-progressive-aphasia-where-do-slps-belong?srsltid=AfmBOoq08dxIOepBhdaIYJaBor7u062pwrm66ANL3mcEzk87lTS73LMm
When evaluating someone who may have PPA, it is important that speech-language pathologists (SLPs) ascertain the onset and course of the communication problem and determine through examination if it represents a focal language deficit (aphasia) versus a disorder more in keeping with a broader constellation of non-aphasic cognitive communication deficits. […] PPA can be a devastating diagnosis for many reasons, but especially because there are no disease-modifying options at this time. However, patients and their loved ones are often relieved to finally have a diagnosis that accurately captures their difficulty and makes some predictions about the future. Once a diagnosis is established, SLPs can offer a wealth of information to help patients, families, and care partners adapt to the loss of cognitive-communication functions.
- #90 Bruce Willisâ diagnosis brings aphasia to forefront | College of Applied Health Scienceshttps://ahs.illinois.edu/blog/bruce-willis-diagnosis-brings-aphasia-forefront
It’s a very slow progression of the loss of their communication and along with this kind of dementia too that goes along with it. […] The National Aphasia Association is a great website and a great reference for anybody to learn more about aphasia or just to understand more about what it is, and find support groups, within your local community, Franz said.
- #91 Primary Progressive Aphasia – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/books/NBK563145/
A simplified 2-step process has been developed for standardization and ease of clinical diagnosis. […] Afterward, the diagnostic features crafted by Gorno-Tempini aid in distinguishing between the various types of PPA. […] Following diagnosis, the progressive aphasia severity scale helps clinicians track progression and symptom characterization in people with PPA. […] Brain MRI and FDG-PET scans are the most important neuroimaging studies for PPA. […] The findings in each PPA variant are explained below. […] No medication has proven beneficial for PPA. Proper management of this condition begins with a detailed history and analysis of the patients speech and language components. […] Referral to a speech and language pathologist is essential for properly diagnosing language disorders and planning the appropriate treatment strategy. […] Managing this condition’s complications requires a multidisciplinary approach involving speech-language therapy, cognitive rehabilitation, psychosocial support, and caregiver education to optimize patient care and quality of life.
- #92https://link.springer.com/article/10.1007/s12149-024-01958-w
The disease progresses in a matter of years while leaving behavioral aspects relatively intact during the early stages. […] Within the diagnostic framework of PPA, the condition is further subdivided into three subtypes: Semantic Dementia (SD), Progressive Non-Fluent Aphasia (PNFA), also known as the agrammatic variant (agPPA), and Logopenic Progressive Aphasia (LPA). […] However, existing diagnostic criteria using only these three variants may be inadequate in encompassing all individuals with PPA. […] An increasing body of literature now supports the likelihood of 56 subtypes instead of the traditionally recognized three. […] Early PPA identification can significantly impacts patient management and prognosis. […] Given that PPA is a neurodegenerative condition leading to a gradual loss of language abilities, early diagnosis allows for timely intervention and the implementation of appropriate therapeutic strategies.
- #93 4 Approaches to Primary Progressive Aphasia Treatmenthttps://www.healthline.com/health/primary-progressive-aphasia-treatment
Unlike some other neurodegenerative diseases like Alzheimers or Parkinsons, PPA doesnt have a standardized set of stages that everyone follows. […] In a study that has been preprinted (not yet peer-reviewed), researchers aimed to establish a clear framework for understanding the progression of PPA. […] The progression of primary progressive aphasia can vary widely from person to person. […] However, the rate of progression can be influenced by factors such as the specific variant of PPA, individual differences, and underlying causes. […] The survival rate for PPA varies depending on the specific variant and individual factors. […] While theres no cure, treatment focuses on managing symptoms and improving communication skills through interventions like speech therapy, cognitive training, and supportive strategies. […] These treatments can help you maintain your ability to communicate, engage in daily activities, and enhance your overall quality of life.