Atrofia korowej tylna – Etiologia i przyczyny

Atrofia korowej tylna
Etiologia i przyczyny

Atrofia korowa tylna (PCA) to rzadki zespół neurodegeneracyjny charakteryzujący się postępującą utratą neuronów w tylnych obszarach mózgu, głównie w korze ciemieniowej, potylicznej i skroniowo-potylicznej, co prowadzi do deficytów percepcji wzrokowej i przestrzennej przy względnie zachowanej pamięci we wczesnych stadiach. Ponad 80% przypadków PCA jest spowodowanych atypową postacią choroby Alzheimera (PCA-AD), z obecnością patologicznych złogów amyloidu i tau, wykrywanych w badaniach PET FDG i tau, z charakterystycznym regionalnym zajęciem potylicznym. Inne etiologie obejmują otępienie z ciałami Lewy’ego (PCA-LBD), zwyrodnienie korowo-podstawne (PCA-CBD) oraz choroby prionowe (PCA-prion). Nowe kryteria diagnostyczne z 2017 roku wprowadzają klasyfikację PCA na podtypy w oparciu o prezentację kliniczną i biomarkery, co ma kluczowe znaczenie dla różnicowania i leczenia.

Etiologia atrofii korowej tylnej (Posterior Cortical Atrophy)

Choroba Alzheimera jako główna przyczyna PCA
Inne przyczyny atrofii korowej tylnej
Czynniki genetyczne w etiologii PCA
Wiek zachorowania
Czynniki ryzyka
Mechanizmy patofizjologiczne

Diagnostyka różnicowa

Kolejne rozdziały

Etiologia atrofii korowej tylnej (Posterior Cortical Atrophy)

Atrofia korowej tylna (Posterior Cortical Atrophy, PCA), znana również jako zespół Bensona, jest rzadkim zespołem neurodegeneracyjnym charakteryzującym się postępującą utratą komórek mózgowych w tylnych obszarach mózgu, zwłaszcza w obszarach ciemieniowych, potylicznych i skroniowo-potylicznych.¹² Obszary te odpowiadają za przetwarzanie wzrokowe oraz rozumowanie przestrzenne, co prowadzi do charakterystycznych trudności w percepcji wzrokowej i przestrzennej.³⁴ W przeciwieństwie do typowej choroby Alzheimera, w PCA pamięć pozostaje stosunkowo dobrze zachowana we wczesnych stadiach choroby.⁵

Choroba Alzheimera jako główna przyczyna PCA

W większości przypadków (ponad 80%) atrofia korowej tylna jest spowodowana chorobą Alzheimera, która atypowo manifestuje się w tylnej części mózgu.⁶⁷ PCA jest często określana jako „wzrokowy wariant choroby Alzheimera”.⁸⁹ W przypadkach związanych z chorobą Alzheimera, w tkance mózgowej osób z PCA widoczne są nieprawidłowe złogi białek amyloidu i tau, tworzące charakterystyczne dla choroby Alzheimera blaszki i splątki.¹⁰

W badaniach neuropatologicznych i biomarkerowych, zarówno retrospektywnych jak i prospektywnych, stwierdzono obecność pierwotnej lub współistniejącej patologii choroby Alzheimera w około 75% przypadków PCA.¹¹¹² Chociaż wzorce odkładania się amyloidu w PCA ogólnie przypominają typową chorobę Alzheimera, to badania PET z wykorzystaniem FDG i tau pokazują wyraźne regionalne, szczególnie potyliczne, zajęcie mózgu.¹³

Inne przyczyny atrofii korowej tylnej

Oprócz choroby Alzheimera, atrofia korowej tylna może być spowodowana innymi chorobami neurodegeneracyjnymi:¹⁴¹⁵

Otępienie z ciałami Lewy’ego (DLB) – pacjenci z tą etiologią mogą doświadczać halucynacji wzrokowych oraz sztywności i spowolnienia ruchowego podobnego do objawów choroby Parkinsona¹⁶¹⁷
Zwyrodnienie korowo-podstawne (CBD) – charakteryzuje się trudnościami w używaniu jednej strony ciała lub występowaniem szarpanych, niezgrabnych lub powolnych ruchów¹⁸¹⁹
Choroba prionowa (np. choroba Creutzfeldta-Jakoba, CJD) – bardzo rzadka przyczyna, w której pacjenci doświadczają szybkiego spadku funkcji poznawczych²⁰²¹

W 2017 roku opublikowano nowe kryteria konsensusu dotyczące diagnozy i klasyfikacji PCA. Zaproponowano również ramy klasyfikacyjne, które dzielą PCA na podtypy: PCA-pure, PCA-plus i inne podtypy patologiczne, w zależności od prezentacji klinicznej i dowodów biomarkerowych podstawowej patologii.²² Międzynarodowe ramy klasyfikacji zapewniają opisy na poziomie choroby podstawowych przyczyn zespołu PCA, wyróżniając PCA spowodowane chorobą Alzheimera (PCA-AD) jako najczęstszą patologię, oraz inne przyczyny, w tym chorobę z ciałami Lewy’ego (PCA-LBD), zwyrodnienie korowo-podstawne (PCA-CBD) i chorobę prionową (PCA-prion).²³

Czynniki genetyczne w etiologii PCA

Badania genetyczne wskazują na potencjalny udział czynników genetycznych w rozwoju PCA, szczególnie w przypadkach rodzinnych. Zidentyfikowano szereg mutacji genowych w PCA, w tym:²⁴²⁵

Geny preseniliny 1 i 2 (PSEN1 i PSEN2)²⁶²⁷
Gen białka prionowego (PRNP)²⁸
Gen progranulinowy (GRN)²⁹³⁰
Gen białka tau związanego z mikrotubulami (MAPT)³¹³²
Geny związane z chorobą Alzheimera, takie jak gen białka prekursora amyloidu (APP)³³

Interesujące jest, że w niektórych przypadkach PCA zidentyfikowano wariant genetyczny rs5848 (c.*78C→T) w regionie 3′-UTR genu GRN, który został zgłoszony jako czynnik ryzyka dla otępienia czołowo-skroniowego z dodatnim TDP-43 i innych chorób neurodegeneracyjnych, takich jak choroba Alzheimera.³⁴

Pomimo tych odkryć, PCA jest zasadniczo sporadyczną chorobą, a kliniczne badania genetyczne zwykle nie są wskazane bez przekonującej historii rodzinnej.³⁵ Badacze wciąż pracują nad zrozumieniem, dlaczego u niektórych osób rozwija się PCA, a nie typowa choroba Alzheimera z dominującymi problemami pamięci.³⁶

Wiek zachorowania

PCA ma tendencję do występowania u osób w młodszym wieku niż typowa choroba Alzheimera. Pacjenci często doświadczają pierwszych objawów będąc w wieku 50-65 lat, podczas gdy typowa choroba Alzheimera zwykle pojawia się w późniejszym wieku.³⁷³⁸³⁹ Ta względnie wczesna manifestacja stanowi istotną cechę charakterystyczną PCA i suggeruje możliwe odmienne mechanizmy patogenetyczne w porównaniu do typowej choroby Alzheimera.⁴⁰

Czynniki ryzyka

Nie jest do końca jasne, czy czynniki ryzyka dla choroby Alzheimera są również czynnikami ryzyka dla atrofii korowej tylnej.⁴¹⁴² Naukowcy podejrzewają, że czynniki ryzyka dla PCA mogą być podobne do innych typów demencji i obejmować złożoną mieszankę czynników, takich jak:⁴³⁴⁴

Wiek
Geny odziedziczone od rodziców
Środowisko
Styl życia (w tym nadmierne spożycie alkoholu, brak aktywności fizycznej, palenie tytoniu)
Depresja
Utrata słuchu
Nieodpowiednio kontrolowane nadciśnienie tętnicze
Otyłość
Niekontrolowana cukrzyca

Bardziej szczegółowe zrozumienie czynników ryzyka specyficznych dla PCA wymaga dalszych badań, podobnie jak identyfikacja czynników związanych nie tylko z selektywną wrażliwością tylnych obszarów korowych, ale także z młodym wiekiem wystąpienia PCA.⁴⁵⁴⁶

Mechanizmy patofizjologiczne

W PCA dochodzi do postępującej utraty komórek mózgowych, szczególnie w tylnych obszarach mózgu, co prowadzi do atrofii tych regionów.⁴⁷ Chociaż dokładne mechanizmy, które powodują selektywne uszkodzenie tylnych obszarów mózgu w PCA, nie są w pełni poznane, badania wskazują na kilka procesów patofizjologicznych:⁴⁸⁴⁹

Nieprawidłowe gromadzenie się białek – podobnie jak w chorobie Alzheimera, PCA wiąże się z nieprawidłowym gromadzeniem się białek w mózgu, takich jak blaszki beta-amyloidowe i splątki tau. Te agregaty białkowe mogą zakłócać funkcje mózgu i prowadzić do uszkodzenia neuronów⁵⁰
Selektywna wrażliwość regionów mózgu – konkretne obszary mózgu dotknięte przez PCA, głównie kora potyliczna odpowiedzialna za przetwarzanie wzrokowe, wykazują szczególną wrażliwość na procesy neurodegeneracyjne⁵¹
Proteinopatie neurodegeneracyjne – w niektórych przypadkach PCA może wynikać z określonych proteinopatii neurodegeneracyjnych, w tym tauopatii i synukleinopatii, które prowadzą do gromadzenia się nieprawidłowych białek w mózgu⁵²

Badacze proponują hipotezę, że PCA często współistnieje z patologią choroby Alzheimera, przy czym u części osób występuje charakterystyczny wzorzec atrofii korowej dotykający głównie tylne obszary mózgu.⁵³ Ta obserwacja podkreśla powiązania procesów neurodegeneracyjnych i wskazuje na potrzebę dalszych badań dotyczących podstawowych szlaków molekularnych zaangażowanych w PCA.⁵⁴

Diagnostyka różnicowa

Diagnoza atrofii korowej tylnej często jest opóźniona, a pacjenci mogą być błędnie diagnozowani jako mający chorobę oczu lub zaburzenia psychologiczne.⁵⁵ PCA jest często niedostatecznie rozpoznawana lub niewłaściwie diagnozowana, ponieważ pacjenci i lekarze mogą mylić spadek funkcji poznawczych związanych ze wzrokiem (problem z mózgiem) z upośledzeniem ostrości widzenia (problem z oczami).⁵⁶

W diagnostyce różnicowej PCA należy uwzględnić:⁵⁷

Chorobę Alzheimera jako najczęściej związaną neuropatologię
Chorobę z ciałami Lewy’ego
Zwyrodnienie korowo-podstawne
Choroby prionowe, takie jak choroba Creutzfeldta-Jakoba

Z wprowadzeniem terapii modyfikujących przebieg choroby, ukierunkowanych na konkretne czynniki etiologiczne, ustalenie obecności tych czynników będzie istotne dla leczenia i poradnictwa pacjentów z PCA.⁵⁸

Podsumowując, atrofia korowej tylnej jest zespołem, a nie jedną chorobą. Oznacza to, że jest to zespół objawów, który może być spowodowany różnymi chorobami. Jak wspomniano, najczęstszą chorobą powodującą PCA jest choroba Alzheimera (około 75% przypadków), a u niektórych osób z chorobą Alzheimera powodującą PCA mogą również występować ciała Lewy’ego przyczyniające się do ich zespołu PCA (około 10%). Pozostałe 10% przypadków jest spowodowane zwyrodnieniem korowo-podstawnym, chorobą prionową lub kombinacją chorób.⁵⁹

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Materiały źródłowe

#1 Posterior cortical atrophy | Radiology Reference Article | Radiopaedia.org
https://radiopaedia.org/articles/posterior-cortical-atrophy?lang=us
Posterior cortical atrophy, also known as Benson syndrome, is an uncommon neurodegenerative disease typically affecting individuals in the sixth and seventh decades of life. It is characterized by dysfunction of the parietal, posterior temporal, and occipital lobes which results in progressive apraxias, problems with visuospatial and visuoperceptual perception as well as literacy deficiencies. […] Although generally thought of as a variant of Alzheimer disease (and thus sometimes referred to as a visual variant of Alzheimer disease), it is now recognized as sometimes being caused by other pathologies. As such, posterior cortical atrophy should be considered to be a clinical syndrome with variable disease etiology. […] It should be noted, that in some individuals who present with posterior cortical atrophy, other underlying etiologies are identified including dementia with Lewy bodies, corticobasal degeneration and even prion disease. […] Furthermore, a number of gene mutations have been identified in posterior cortical atrophy including presenilin 1 and 2 genes (PSEN1 and PSEN2), prion protein gene (PRNP), progranulin gene (GRN) and microtubule-associated protein tau gene (MAPT).
#2 Posterior Cortical Atrophy – StatPearls – NCBI Bookshelf
https://www.ncbi.nlm.nih.gov/books/NBK580553/
Alzheimer disease (AD) is the commonest underlying pathology, and hence it is frequently labeled as a „visual variant” of AD. […] Lewy body disease, corticobasal degeneration, and the Heidenham variant of Creutzfeldt-Jakob disease (CJD) have also been described as less common alternative pathologies to posterior cortical atrophy. […] A new consensus criterion for the diagnosis and classification of PCA was published in 2017. A classification framework has also been proposed that subtypes PCA into PCA-pure, PCA-plus, and other pathologic subtypes depending on the presentation and biomarker evidence of underlying pathology.
#3 Posterior cortical atrophy – Symptoms and causes – Mayo Clinic
https://www.mayoclinic.org/diseases-conditions/posterior-cortical-atrophy/symptoms-causes/syc-20376560
Posterior cortical atrophy is a brain and nervous system syndrome that causes brain cells to die over time. […] Posterior cortical atrophy causes the loss of brain cells in back of the brain. This is the region responsible for visual processing and spatial reasoning. […] In more than 80% of cases, posterior cortical atrophy is due to Alzheimer’s disease. However, it can be due to other neurological conditions such as Lewy body dementia or corticobasal degeneration. […] The most common cause of posterior cortical atrophy is a form of Alzheimer’s disease that’s not typical. It affects the back of the brain. Other less common causes include corticobasal degeneration, Lewy body dementia and Creutzfeldt-Jakob disease. Researchers are looking at potential gene variations that may be related to the condition. […] Further study is needed to determine whether the risk factors for Alzheimer’s disease may play a role in posterior cortical atrophy.
#4 Posterior cortical atrophy | UM Health-Sparrow
https://www.uofmhealthsparrow.org/departments-conditions/conditions/posterior-cortical-atrophy
Posterior cortical atrophy causes the loss of brain cells in back of the brain. This is the region responsible for visual processing and spatial reasoning. This changes a person’s ability to process visual and spatial information. […] In more than 80% of cases, posterior cortical atrophy is due to Alzheimer’s disease. However, it can be due to other neurological conditions such as Lewy body dementia or corticobasal degeneration. […] The most common cause of posterior cortical atrophy is a form of Alzheimer’s disease that’s not typical. It affects the back of the brain. Other less common causes include corticobasal degeneration, Lewy body dementia and Creutzfeldt-Jakob disease. Researchers are looking at potential gene variations that may be related to the condition.
#5 SSA – POMS: DI 23022.643 – Posterior Cortical Atrophy – 08/10/2022
https://secure.ssa.gov/apps10/poms.nsf/lnx/0423022643
Posterior Cortical Atrophy (PCA) is a rare neurologic disease characterized by impairment of higher visual processing skills and other posterior cortical functions without any evidence of ocular abnormalities, relatively intact memory and language in the early stages. PCA causes atrophy of the posterior part of the cerebral cortex, resulting in the progressive disruption of complex visual processing. […] The cause of PCA is unknown, and there are no fully accepted diagnostic criteria for the disease. […] PCA usually affects people at an earlier age than typical cases of Alzheimer’s disease, with initial symptoms often experienced in people in their mid-50s or early 60s. […] Specific and accepted treatment for PCA has yet to be discovered; this may be due to the rarity and variations of the disease.
#6 Posterior cortical atrophy – Symptoms and causes – Mayo Clinic
https://www.mayoclinic.org/diseases-conditions/posterior-cortical-atrophy/symptoms-causes/syc-20376560
Posterior cortical atrophy is a brain and nervous system syndrome that causes brain cells to die over time. […] Posterior cortical atrophy causes the loss of brain cells in back of the brain. This is the region responsible for visual processing and spatial reasoning. […] In more than 80% of cases, posterior cortical atrophy is due to Alzheimer’s disease. However, it can be due to other neurological conditions such as Lewy body dementia or corticobasal degeneration. […] The most common cause of posterior cortical atrophy is a form of Alzheimer’s disease that’s not typical. It affects the back of the brain. Other less common causes include corticobasal degeneration, Lewy body dementia and Creutzfeldt-Jakob disease. Researchers are looking at potential gene variations that may be related to the condition. […] Further study is needed to determine whether the risk factors for Alzheimer’s disease may play a role in posterior cortical atrophy.
#7 Posterior cortical atrophy | Beacon Health System
https://www.beaconhealthsystem.org/library/diseases-and-conditions/posterior-cortical-atrophy/
This rare neurological syndrome that’s often caused by Alzheimer’s disease affects vision and coordination. […] Posterior cortical atrophy causes the loss of brain cells in back of the brain. This is the region responsible for visual processing and spatial reasoning. This changes a person’s ability to process visual and spatial information. […] In more than 80% of cases, posterior cortical atrophy is due to Alzheimer’s disease. However, it can be due to other neurological conditions such as Lewy body dementia or corticobasal degeneration. […] The most common cause of posterior cortical atrophy is a form of Alzheimer’s disease that’s not typical. It affects the back of the brain. Other less common causes include corticobasal degeneration, Lewy body dementia and Creutzfeldt-Jakob disease. Researchers are looking at potential gene variations that may be related to the condition.
#8 Posterior Cortical Atrophy – StatPearls – NCBI Bookshelf
https://www.ncbi.nlm.nih.gov/books/NBK580553/
Alzheimer disease (AD) is the commonest underlying pathology, and hence it is frequently labeled as a „visual variant” of AD. […] Lewy body disease, corticobasal degeneration, and the Heidenham variant of Creutzfeldt-Jakob disease (CJD) have also been described as less common alternative pathologies to posterior cortical atrophy. […] A new consensus criterion for the diagnosis and classification of PCA was published in 2017. A classification framework has also been proposed that subtypes PCA into PCA-pure, PCA-plus, and other pathologic subtypes depending on the presentation and biomarker evidence of underlying pathology.
#9 Posterior Cortical Atrophy | Memory and Aging Center
https://memory.ucsf.edu/dementia/posterior-cortical-atrophy
Posterior cortical atrophy (PCA), also called Bensons syndrome, is a rare, visual variant of Alzheimers disease. […] In the vast majority of PCA cases, the underlying cause is Alzheimers disease, and the brain tissue at autopsy shows an abnormal accumulation of the proteins amyloid and tau that form the plaques and tangles seen in Alzheimers disease. […] Although PCA is almost always caused by Alzheimers disease, it can result from other diseases including dementia with Lewy bodies and Creutzfeldt-Jakob disease. […] PCA tends to affect people at an earlier age than typical Alzheimers disease, with individuals often being in their mid-fifties or early sixties when they experience the initial symptoms.
#10 Posterior Cortical Atrophy | Memory and Aging Center
https://memory.ucsf.edu/dementia/posterior-cortical-atrophy
Posterior cortical atrophy (PCA), also called Bensons syndrome, is a rare, visual variant of Alzheimers disease. […] In the vast majority of PCA cases, the underlying cause is Alzheimers disease, and the brain tissue at autopsy shows an abnormal accumulation of the proteins amyloid and tau that form the plaques and tangles seen in Alzheimers disease. […] Although PCA is almost always caused by Alzheimers disease, it can result from other diseases including dementia with Lewy bodies and Creutzfeldt-Jakob disease. […] PCA tends to affect people at an earlier age than typical Alzheimers disease, with individuals often being in their mid-fifties or early sixties when they experience the initial symptoms.
#11
https://link.springer.com/article/10.1007/s11940-022-00745-0
Posterior cortical atrophy (PCA) is a clinico-radiological syndrome characterised by the progressive loss of higher order visuospatial, visuoperceptual and other posterior cortical functions consistent with occipito-parietal and occipito-temporal atrophy. […] Retrospective and prospective neuropathological and biomarker studies have reported evidence of primary or co-existing AD pathology in 75% of cases. […] PCA is essentially sporadic, and clinical genetic testing is not usually indicated without a compelling family history. […] Despite evidence of increased occipital amyloid in PCA, amyloid PET deposition in PCA broadly resembles typical AD, whilst FDG and tau PET scans emphasise marked regional, particularly occipital, involvement. […] PCA diagnosis is frequently delayed and patients may be misdiagnosed with an ocular or psychological illness.
#12
https://medschool.cuanschutz.edu/pca/what-is-pca/what-is-pca
PCA is a condition of the brain that progressively worsens over time and affects areas in the brain that process visual information. […] Although PCA is not caused by one disease, for the majority of patients with PCA the cause is Alzheimers disease. […] An autopsy can be performed at the time of death to determine the cause of PCA. […] In these instances, where we can confirm the cause or causes of PCA by autopsy, the most common cause is Alzheimers disease (approximately 75% of autopsies for PCA). […] In summary, PCA is a syndrome and not a disease. This means that it is a constellation of symptoms that can be caused by different diseases. As noted, the most common disease to cause PCA is Alzheimers disease (approximately 75%), and some people with Alzheimers disease-causing PCA can also have Lewy Bodies contributing to their PCA syndrome (about 10%). […] The remainder of the 10% is due to corticobasal degeneration, prion disease, or a combination of diseases.
#13
https://link.springer.com/article/10.1007/s11940-022-00745-0
Posterior cortical atrophy (PCA) is a clinico-radiological syndrome characterised by the progressive loss of higher order visuospatial, visuoperceptual and other posterior cortical functions consistent with occipito-parietal and occipito-temporal atrophy. […] Retrospective and prospective neuropathological and biomarker studies have reported evidence of primary or co-existing AD pathology in 75% of cases. […] PCA is essentially sporadic, and clinical genetic testing is not usually indicated without a compelling family history. […] Despite evidence of increased occipital amyloid in PCA, amyloid PET deposition in PCA broadly resembles typical AD, whilst FDG and tau PET scans emphasise marked regional, particularly occipital, involvement. […] PCA diagnosis is frequently delayed and patients may be misdiagnosed with an ocular or psychological illness.
#14 Posterior Cortical Atrophy – StatPearls – NCBI Bookshelf
https://www.ncbi.nlm.nih.gov/books/NBK580553/
Alzheimer disease (AD) is the commonest underlying pathology, and hence it is frequently labeled as a „visual variant” of AD. […] Lewy body disease, corticobasal degeneration, and the Heidenham variant of Creutzfeldt-Jakob disease (CJD) have also been described as less common alternative pathologies to posterior cortical atrophy. […] A new consensus criterion for the diagnosis and classification of PCA was published in 2017. A classification framework has also been proposed that subtypes PCA into PCA-pure, PCA-plus, and other pathologic subtypes depending on the presentation and biomarker evidence of underlying pathology.
#15 Understanding PCA – Rare Dementia Support
https://www.raredementiasupport.org/posterior-cortical-atrophy/understanding-pca/
Posterior cortical atrophy (PCA) means back of the brain shrinkage and it refers to the progressive loss of brain cells, particularly in brain regions that process visual and sensory information, such as the occipital and parietal lobes. The changes in the brain that cause PCA can be caused by different disease processes. In the majority of cases PCA is caused by changes to brain cells similar to those that occur in Alzheimers disease. […] There are a small number of underlying conditions, as well as Alzheimers disease, that can cause PCA: […] These include: […] Lewy body dementia (LBD) people may experience visual hallucinations, and may become stiffer and slower in their movements, in a similar way to people living with Parkinsons disease. […] Corticobasal Syndrome (CBS) people may have difficulty using one side of their body or experience jerky, awkward or slow movements. […] Prion diseases (e.g. Creutzfeldt-Jakob disease (CJD)) very rare conditions in which people may experience a very rapid decline in their cognition. […] Very little is known about why PCA affects some people and not others, nor how many people there are living with PCA.
#16 Understanding PCA – Rare Dementia Support
https://www.raredementiasupport.org/posterior-cortical-atrophy/understanding-pca/
Posterior cortical atrophy (PCA) means back of the brain shrinkage and it refers to the progressive loss of brain cells, particularly in brain regions that process visual and sensory information, such as the occipital and parietal lobes. The changes in the brain that cause PCA can be caused by different disease processes. In the majority of cases PCA is caused by changes to brain cells similar to those that occur in Alzheimers disease. […] There are a small number of underlying conditions, as well as Alzheimers disease, that can cause PCA: […] These include: […] Lewy body dementia (LBD) people may experience visual hallucinations, and may become stiffer and slower in their movements, in a similar way to people living with Parkinsons disease. […] Corticobasal Syndrome (CBS) people may have difficulty using one side of their body or experience jerky, awkward or slow movements. […] Prion diseases (e.g. Creutzfeldt-Jakob disease (CJD)) very rare conditions in which people may experience a very rapid decline in their cognition. […] Very little is known about why PCA affects some people and not others, nor how many people there are living with PCA.
#17 Posterior cortical atrophy | talkdementia.uk
https://www.talkdementia.uk/posterior-cortical-atrophy
Posterior cortical atrophy (PCA) is a form of dementia that mainly affects the parts of the brain that process visual and spatial information. […] Causes […] Posterior cortical atrophy (PCA) means back of the brain shrinkage and it refers to the progressive loss of brain cells, particularly in brain regions that process visual and sensory information, such as the occipital and parietal lobes. The changes in the brain that cause PCA can be caused by different disease processes. In the majority of cases PCA is caused by changes to brain cells similar to those that occur in Alzheimers disease. […] There are a small number of underlying conditions, as well as Alzheimers disease, that can cause PCA: […] Lewy body dementia (LBD) people may experience visual hallucinations, and may become stiffer and slower in their movements, in a similar way to people living with Parkinsons disease.
#18 Understanding PCA – Rare Dementia Support
https://www.raredementiasupport.org/posterior-cortical-atrophy/understanding-pca/
Posterior cortical atrophy (PCA) means back of the brain shrinkage and it refers to the progressive loss of brain cells, particularly in brain regions that process visual and sensory information, such as the occipital and parietal lobes. The changes in the brain that cause PCA can be caused by different disease processes. In the majority of cases PCA is caused by changes to brain cells similar to those that occur in Alzheimers disease. […] There are a small number of underlying conditions, as well as Alzheimers disease, that can cause PCA: […] These include: […] Lewy body dementia (LBD) people may experience visual hallucinations, and may become stiffer and slower in their movements, in a similar way to people living with Parkinsons disease. […] Corticobasal Syndrome (CBS) people may have difficulty using one side of their body or experience jerky, awkward or slow movements. […] Prion diseases (e.g. Creutzfeldt-Jakob disease (CJD)) very rare conditions in which people may experience a very rapid decline in their cognition. […] Very little is known about why PCA affects some people and not others, nor how many people there are living with PCA.
#19 Posterior cortical atrophy | talkdementia.uk
https://www.talkdementia.uk/posterior-cortical-atrophy
Corticobasal Syndrome (CBS) people may have difficulty using one side of their body or experience jerky, awkward or slow movements. […] Prion diseases (e.g. Creutzfeldt-Jakob disease (CJD)) very rare conditions in which people may experience a very rapid decline in their cognition. […] Very little is known about why PCA affects some people and not others, nor how many people there are living with PCA.
#20 Understanding PCA – Rare Dementia Support
https://www.raredementiasupport.org/posterior-cortical-atrophy/understanding-pca/
Posterior cortical atrophy (PCA) means back of the brain shrinkage and it refers to the progressive loss of brain cells, particularly in brain regions that process visual and sensory information, such as the occipital and parietal lobes. The changes in the brain that cause PCA can be caused by different disease processes. In the majority of cases PCA is caused by changes to brain cells similar to those that occur in Alzheimers disease. […] There are a small number of underlying conditions, as well as Alzheimers disease, that can cause PCA: […] These include: […] Lewy body dementia (LBD) people may experience visual hallucinations, and may become stiffer and slower in their movements, in a similar way to people living with Parkinsons disease. […] Corticobasal Syndrome (CBS) people may have difficulty using one side of their body or experience jerky, awkward or slow movements. […] Prion diseases (e.g. Creutzfeldt-Jakob disease (CJD)) very rare conditions in which people may experience a very rapid decline in their cognition. […] Very little is known about why PCA affects some people and not others, nor how many people there are living with PCA.
#21 Posterior cortical atrophy | talkdementia.uk
https://www.talkdementia.uk/posterior-cortical-atrophy
Corticobasal Syndrome (CBS) people may have difficulty using one side of their body or experience jerky, awkward or slow movements. […] Prion diseases (e.g. Creutzfeldt-Jakob disease (CJD)) very rare conditions in which people may experience a very rapid decline in their cognition. […] Very little is known about why PCA affects some people and not others, nor how many people there are living with PCA.
#22 Posterior Cortical Atrophy – StatPearls – NCBI Bookshelf
https://www.ncbi.nlm.nih.gov/books/NBK580553/
Alzheimer disease (AD) is the commonest underlying pathology, and hence it is frequently labeled as a „visual variant” of AD. […] Lewy body disease, corticobasal degeneration, and the Heidenham variant of Creutzfeldt-Jakob disease (CJD) have also been described as less common alternative pathologies to posterior cortical atrophy. […] A new consensus criterion for the diagnosis and classification of PCA was published in 2017. A classification framework has also been proposed that subtypes PCA into PCA-pure, PCA-plus, and other pathologic subtypes depending on the presentation and biomarker evidence of underlying pathology.
#23 Orphanet: Posterior cortical atrophy
https://www.orpha.net/en/disease/detail/54247
A rare neurologic disease characterized by impairment of higher visual processing skills and other posterior cortical functions without any evidence of ocular abnormalities, relatively intact memory and language in the early stages, and atrophy of posterior brain regions. […] A third level of the international classification framework provides disease-level descriptions of the underlying causes of the PCA syndrome, based on available pathophysiological biomarker evidence. This distinguishes between Alzheimer’s disease as the most common underlying pathology (PCA-AD), and other causes including Lewy Body disease (PCA-LBD), corticobasal degeneration (PCA-CBD) and prion disease (PCA-prion). […] Differential diagnosis includes the most commonly associated neuropathology Alzheimer disease, but may also include Lewy body disease, cortico-basal degeneration, and prion diseases such as Creutzfeldt-Jakob disease.
#24 Posterior cortical atrophy | Radiology Reference Article | Radiopaedia.org
https://radiopaedia.org/articles/posterior-cortical-atrophy?lang=us
Posterior cortical atrophy, also known as Benson syndrome, is an uncommon neurodegenerative disease typically affecting individuals in the sixth and seventh decades of life. It is characterized by dysfunction of the parietal, posterior temporal, and occipital lobes which results in progressive apraxias, problems with visuospatial and visuoperceptual perception as well as literacy deficiencies. […] Although generally thought of as a variant of Alzheimer disease (and thus sometimes referred to as a visual variant of Alzheimer disease), it is now recognized as sometimes being caused by other pathologies. As such, posterior cortical atrophy should be considered to be a clinical syndrome with variable disease etiology. […] It should be noted, that in some individuals who present with posterior cortical atrophy, other underlying etiologies are identified including dementia with Lewy bodies, corticobasal degeneration and even prion disease. […] Furthermore, a number of gene mutations have been identified in posterior cortical atrophy including presenilin 1 and 2 genes (PSEN1 and PSEN2), prion protein gene (PRNP), progranulin gene (GRN) and microtubule-associated protein tau gene (MAPT).
#25 Mysteries Of Posterior Cortical Atrophy: Symptoms, Causes, And Management | The Lifesciences Magazine
https://thelifesciencesmagazine.com/unraveling-posterior-cortical-atrophy/
One prominent hypothesis suggests that PCA often coexists with Alzheimers disease pathology, with a subset of individuals exhibiting a distinct pattern of cortical atrophy predominantly affecting the posterior brain regions. […] In addition to Alzheimers disease, PCA has been linked to other neurodegenerative proteinopathies characterized by the abnormal accumulation of specific proteins in the brain. […] Genetic factors also play a role in the development of PCA, particularly in familial forms of the disease. Mutations in genes associated with Alzheimers disease, such as the amyloid precursor protein (APP), presenilin 1 (PSEN1), and presenilin 2 (PSEN2) genes, have been implicated in familial PCA cases. Additionally, mutations in other genes, such as GRN (progranulin) and MAPT (microtubule-associated protein tau), have been linked to familial frontotemporal dementia (FTD) syndromes, which may present with clinical features overlapping with PCA. […] In summary, the underlying causes of Posterior Cortical Atrophy are multifaceted, involving a complex interplay of genetic, molecular, and environmental factors.
#26 Posterior cortical atrophy | Radiology Reference Article | Radiopaedia.org
https://radiopaedia.org/articles/posterior-cortical-atrophy?lang=us
Posterior cortical atrophy, also known as Benson syndrome, is an uncommon neurodegenerative disease typically affecting individuals in the sixth and seventh decades of life. It is characterized by dysfunction of the parietal, posterior temporal, and occipital lobes which results in progressive apraxias, problems with visuospatial and visuoperceptual perception as well as literacy deficiencies. […] Although generally thought of as a variant of Alzheimer disease (and thus sometimes referred to as a visual variant of Alzheimer disease), it is now recognized as sometimes being caused by other pathologies. As such, posterior cortical atrophy should be considered to be a clinical syndrome with variable disease etiology. […] It should be noted, that in some individuals who present with posterior cortical atrophy, other underlying etiologies are identified including dementia with Lewy bodies, corticobasal degeneration and even prion disease. […] Furthermore, a number of gene mutations have been identified in posterior cortical atrophy including presenilin 1 and 2 genes (PSEN1 and PSEN2), prion protein gene (PRNP), progranulin gene (GRN) and microtubule-associated protein tau gene (MAPT).
#27 Mysteries Of Posterior Cortical Atrophy: Symptoms, Causes, And Management | The Lifesciences Magazine
https://thelifesciencesmagazine.com/unraveling-posterior-cortical-atrophy/
One prominent hypothesis suggests that PCA often coexists with Alzheimers disease pathology, with a subset of individuals exhibiting a distinct pattern of cortical atrophy predominantly affecting the posterior brain regions. […] In addition to Alzheimers disease, PCA has been linked to other neurodegenerative proteinopathies characterized by the abnormal accumulation of specific proteins in the brain. […] Genetic factors also play a role in the development of PCA, particularly in familial forms of the disease. Mutations in genes associated with Alzheimers disease, such as the amyloid precursor protein (APP), presenilin 1 (PSEN1), and presenilin 2 (PSEN2) genes, have been implicated in familial PCA cases. Additionally, mutations in other genes, such as GRN (progranulin) and MAPT (microtubule-associated protein tau), have been linked to familial frontotemporal dementia (FTD) syndromes, which may present with clinical features overlapping with PCA. […] In summary, the underlying causes of Posterior Cortical Atrophy are multifaceted, involving a complex interplay of genetic, molecular, and environmental factors.
#28 Posterior cortical atrophy | Radiology Reference Article | Radiopaedia.org
https://radiopaedia.org/articles/posterior-cortical-atrophy?lang=us
Posterior cortical atrophy, also known as Benson syndrome, is an uncommon neurodegenerative disease typically affecting individuals in the sixth and seventh decades of life. It is characterized by dysfunction of the parietal, posterior temporal, and occipital lobes which results in progressive apraxias, problems with visuospatial and visuoperceptual perception as well as literacy deficiencies. […] Although generally thought of as a variant of Alzheimer disease (and thus sometimes referred to as a visual variant of Alzheimer disease), it is now recognized as sometimes being caused by other pathologies. As such, posterior cortical atrophy should be considered to be a clinical syndrome with variable disease etiology. […] It should be noted, that in some individuals who present with posterior cortical atrophy, other underlying etiologies are identified including dementia with Lewy bodies, corticobasal degeneration and even prion disease. […] Furthermore, a number of gene mutations have been identified in posterior cortical atrophy including presenilin 1 and 2 genes (PSEN1 and PSEN2), prion protein gene (PRNP), progranulin gene (GRN) and microtubule-associated protein tau gene (MAPT).
#29 Posterior cortical atrophy | Radiology Reference Article | Radiopaedia.org
https://radiopaedia.org/articles/posterior-cortical-atrophy?lang=us
Posterior cortical atrophy, also known as Benson syndrome, is an uncommon neurodegenerative disease typically affecting individuals in the sixth and seventh decades of life. It is characterized by dysfunction of the parietal, posterior temporal, and occipital lobes which results in progressive apraxias, problems with visuospatial and visuoperceptual perception as well as literacy deficiencies. […] Although generally thought of as a variant of Alzheimer disease (and thus sometimes referred to as a visual variant of Alzheimer disease), it is now recognized as sometimes being caused by other pathologies. As such, posterior cortical atrophy should be considered to be a clinical syndrome with variable disease etiology. […] It should be noted, that in some individuals who present with posterior cortical atrophy, other underlying etiologies are identified including dementia with Lewy bodies, corticobasal degeneration and even prion disease. […] Furthermore, a number of gene mutations have been identified in posterior cortical atrophy including presenilin 1 and 2 genes (PSEN1 and PSEN2), prion protein gene (PRNP), progranulin gene (GRN) and microtubule-associated protein tau gene (MAPT).
#30 Mysteries Of Posterior Cortical Atrophy: Symptoms, Causes, And Management | The Lifesciences Magazine
https://thelifesciencesmagazine.com/unraveling-posterior-cortical-atrophy/
One prominent hypothesis suggests that PCA often coexists with Alzheimers disease pathology, with a subset of individuals exhibiting a distinct pattern of cortical atrophy predominantly affecting the posterior brain regions. […] In addition to Alzheimers disease, PCA has been linked to other neurodegenerative proteinopathies characterized by the abnormal accumulation of specific proteins in the brain. […] Genetic factors also play a role in the development of PCA, particularly in familial forms of the disease. Mutations in genes associated with Alzheimers disease, such as the amyloid precursor protein (APP), presenilin 1 (PSEN1), and presenilin 2 (PSEN2) genes, have been implicated in familial PCA cases. Additionally, mutations in other genes, such as GRN (progranulin) and MAPT (microtubule-associated protein tau), have been linked to familial frontotemporal dementia (FTD) syndromes, which may present with clinical features overlapping with PCA. […] In summary, the underlying causes of Posterior Cortical Atrophy are multifaceted, involving a complex interplay of genetic, molecular, and environmental factors.
#31 Posterior cortical atrophy | Radiology Reference Article | Radiopaedia.org
https://radiopaedia.org/articles/posterior-cortical-atrophy?lang=us
Posterior cortical atrophy, also known as Benson syndrome, is an uncommon neurodegenerative disease typically affecting individuals in the sixth and seventh decades of life. It is characterized by dysfunction of the parietal, posterior temporal, and occipital lobes which results in progressive apraxias, problems with visuospatial and visuoperceptual perception as well as literacy deficiencies. […] Although generally thought of as a variant of Alzheimer disease (and thus sometimes referred to as a visual variant of Alzheimer disease), it is now recognized as sometimes being caused by other pathologies. As such, posterior cortical atrophy should be considered to be a clinical syndrome with variable disease etiology. […] It should be noted, that in some individuals who present with posterior cortical atrophy, other underlying etiologies are identified including dementia with Lewy bodies, corticobasal degeneration and even prion disease. […] Furthermore, a number of gene mutations have been identified in posterior cortical atrophy including presenilin 1 and 2 genes (PSEN1 and PSEN2), prion protein gene (PRNP), progranulin gene (GRN) and microtubule-associated protein tau gene (MAPT).
#32 Mysteries Of Posterior Cortical Atrophy: Symptoms, Causes, And Management | The Lifesciences Magazine
https://thelifesciencesmagazine.com/unraveling-posterior-cortical-atrophy/
One prominent hypothesis suggests that PCA often coexists with Alzheimers disease pathology, with a subset of individuals exhibiting a distinct pattern of cortical atrophy predominantly affecting the posterior brain regions. […] In addition to Alzheimers disease, PCA has been linked to other neurodegenerative proteinopathies characterized by the abnormal accumulation of specific proteins in the brain. […] Genetic factors also play a role in the development of PCA, particularly in familial forms of the disease. Mutations in genes associated with Alzheimers disease, such as the amyloid precursor protein (APP), presenilin 1 (PSEN1), and presenilin 2 (PSEN2) genes, have been implicated in familial PCA cases. Additionally, mutations in other genes, such as GRN (progranulin) and MAPT (microtubule-associated protein tau), have been linked to familial frontotemporal dementia (FTD) syndromes, which may present with clinical features overlapping with PCA. […] In summary, the underlying causes of Posterior Cortical Atrophy are multifaceted, involving a complex interplay of genetic, molecular, and environmental factors.
#33 Mysteries Of Posterior Cortical Atrophy: Symptoms, Causes, And Management | The Lifesciences Magazine
https://thelifesciencesmagazine.com/unraveling-posterior-cortical-atrophy/
One prominent hypothesis suggests that PCA often coexists with Alzheimers disease pathology, with a subset of individuals exhibiting a distinct pattern of cortical atrophy predominantly affecting the posterior brain regions. […] In addition to Alzheimers disease, PCA has been linked to other neurodegenerative proteinopathies characterized by the abnormal accumulation of specific proteins in the brain. […] Genetic factors also play a role in the development of PCA, particularly in familial forms of the disease. Mutations in genes associated with Alzheimers disease, such as the amyloid precursor protein (APP), presenilin 1 (PSEN1), and presenilin 2 (PSEN2) genes, have been implicated in familial PCA cases. Additionally, mutations in other genes, such as GRN (progranulin) and MAPT (microtubule-associated protein tau), have been linked to familial frontotemporal dementia (FTD) syndromes, which may present with clinical features overlapping with PCA. […] In summary, the underlying causes of Posterior Cortical Atrophy are multifaceted, involving a complex interplay of genetic, molecular, and environmental factors.
#34 Posterior cortical atrophy as a primary clinical phenotype of corticobasal syndrome with a progranulin gene rs5848 TT genotype | Orphanet Journal of Rare Diseases | Full Text
https://ojrd.biomedcentral.com/articles/10.1186/s13023-016-0396-0
In addition, genetic tests identified no reported pathogenic genetic mutations for early-onset AD, and an APOE E3/E3 genotype. FTLD-associated gene testing revealed a genetic variant, rs5848 (c.*78CT) in the 3-UTR of the GRN gene, which has been reported as a risk factor for TDP-43-positive frontotemporal dementia and other neurodegenerative diseases such as AD. […] This study provides evidence for CBS belonging to the GRN genetic variant (rs5848) clinical spectrum, and demonstrates that in rare cases, CBS may initially present with symptoms of PCA. Longitudinal follow-up is required to ascertain the most likely etiology and determine the clinical-genetic-pathological mechanism of the T allele of the rs5848 polymorphism in CBS diagnosis.
#35
https://link.springer.com/article/10.1007/s11940-022-00745-0
Posterior cortical atrophy (PCA) is a clinico-radiological syndrome characterised by the progressive loss of higher order visuospatial, visuoperceptual and other posterior cortical functions consistent with occipito-parietal and occipito-temporal atrophy. […] Retrospective and prospective neuropathological and biomarker studies have reported evidence of primary or co-existing AD pathology in 75% of cases. […] PCA is essentially sporadic, and clinical genetic testing is not usually indicated without a compelling family history. […] Despite evidence of increased occipital amyloid in PCA, amyloid PET deposition in PCA broadly resembles typical AD, whilst FDG and tau PET scans emphasise marked regional, particularly occipital, involvement. […] PCA diagnosis is frequently delayed and patients may be misdiagnosed with an ocular or psychological illness.
#36 Posterior cortical atrophy risk factors – Alzheimer’s Research UK
https://www.alzheimersresearchuk.org/dementia-information/types-of-dementia/posterior-cortical-atrophy/risk-factors/
The symptoms of PCA are caused by changes in the brain cells that process visual information from our eyes. […] Researchers have found that the symptoms of PCA are caused by changes in the brain cells that process visual information from our eyes. […] In PCA caused by Alzheimerâs disease, it is not clear why the disease affects this part of the brain more so than the areas affected by typical Alzheimerâs, which generally are involved in memory. It is thought that what makes up a personâs risk of developing PCA is similar to other types of dementia. This is a complex mix of factors including: our age, the genes we inherit from our parents, our environment, our lifestyle. Scientists are working hard to find out more, as well as exploring specific genetic or lifestyle risk factors for the condition.
#37 Posterior Cortical Atrophy | Memory and Aging Center
https://memory.ucsf.edu/dementia/posterior-cortical-atrophy
Posterior cortical atrophy (PCA), also called Bensons syndrome, is a rare, visual variant of Alzheimers disease. […] In the vast majority of PCA cases, the underlying cause is Alzheimers disease, and the brain tissue at autopsy shows an abnormal accumulation of the proteins amyloid and tau that form the plaques and tangles seen in Alzheimers disease. […] Although PCA is almost always caused by Alzheimers disease, it can result from other diseases including dementia with Lewy bodies and Creutzfeldt-Jakob disease. […] PCA tends to affect people at an earlier age than typical Alzheimers disease, with individuals often being in their mid-fifties or early sixties when they experience the initial symptoms.
#38 Rarer causes of dementia | DPT
https://www.dpt.nhs.uk/our-services/older-people/devon-memory-service/what-is-dementia/rarer-causes-of-dementia
Posterior cortical atrophy (PCA), also known as Benson’s syndrome, is a rare degenerative condition in which damage occurs at the back (posterior region) of the brain. In the vast majority of people, the cause of PCA is Alzheimer’s disease. […] The first symptoms of PCA tend to occur when people are in their mid-50s or early 60s. However, the first signs are often subtle and so it may be some time before a formal diagnosis is made. […] Initially, people with PCA tend to have a relatively well-preserved memory but experience problems with their vision, such as difficulty recognising faces and objects in pictures. […] As damage in the brain spreads and the disease progresses, people develop the more typical symptoms of Alzheimer’s disease, such as memory loss and confusion.
#39 What is posterior cortical atrophy (PCA)? – Dementia UK
https://www.dementiauk.org/information-and-support/types-of-dementia/posterior-cortical-atrophy-pca/
Posterior cortical atrophy (PCA), also known as Bensonâs Syndrome, is the degeneration of the cells at the back or posterior of the brain. The changes in the brain resemble those in someone with Alzheimerâs disease but the effects are very different. […] PCA is a rare form of dementia that people often develop between the ages of 50 and 65.
#40 Posterior cortical atrophy – PubMed
https://pubmed.ncbi.nlm.nih.gov/22265212/
Posterior cortical atrophy (PCA) is a neurodegenerative syndrome that is characterised by progressive decline in visuospatial, visuoperceptual, literacy, and praxic skills. The progressive neurodegeneration affecting parietal, occipital, and occipitotemporal cortices that underlies PCA is attributable to Alzheimer’s disease in most patients. […] However, alternative underlying causes, including dementia with Lewy bodies, corticobasal degeneration, and prion disease, have also been identified, and not all patients with PCA have atrophy on clinical imaging. […] Important challenges remain, including the identification of factors associated not only with the selective vulnerability of posterior cortical regions but also with the young age of onset of PCA.
#41 Posterior Cortical Atrophy (PCA) | Symptoms & Treatments | alz.org
https://www.alz.org/alzheimers-dementia/what-is-dementia/types-of-dementia/posterior-cortical-atrophy
Posterior cortical atrophy (PCA) refers to gradual and progressive degeneration of the outer layer of the brain (the cortex) in the part of the brain located in the back of the head (posterior). […] It is not known whether posterior cortical atrophy is a unique disease or a possible variant form of Alzheimers disease. […] Similar to Alzheimers disease, the causes of posterior cortical atrophy are unknown, and no obvious genetic mutations have been shown to be linked to the condition. […] It is also not known if the risk factors for Alzheimers disease are also risk factors for posterior cortical atrophy.
#42 Posterior cortical atrophy – Symptoms and causes – Mayo Clinic
https://www.mayoclinic.org/diseases-conditions/posterior-cortical-atrophy/symptoms-causes/syc-20376560
Posterior cortical atrophy is a brain and nervous system syndrome that causes brain cells to die over time. […] Posterior cortical atrophy causes the loss of brain cells in back of the brain. This is the region responsible for visual processing and spatial reasoning. […] In more than 80% of cases, posterior cortical atrophy is due to Alzheimer’s disease. However, it can be due to other neurological conditions such as Lewy body dementia or corticobasal degeneration. […] The most common cause of posterior cortical atrophy is a form of Alzheimer’s disease that’s not typical. It affects the back of the brain. Other less common causes include corticobasal degeneration, Lewy body dementia and Creutzfeldt-Jakob disease. Researchers are looking at potential gene variations that may be related to the condition. […] Further study is needed to determine whether the risk factors for Alzheimer’s disease may play a role in posterior cortical atrophy.
#43 Posterior cortical atrophy risk factors – Alzheimer’s Research UK
https://www.alzheimersresearchuk.org/dementia-information/types-of-dementia/posterior-cortical-atrophy/risk-factors/
The symptoms of PCA are caused by changes in the brain cells that process visual information from our eyes. […] Researchers have found that the symptoms of PCA are caused by changes in the brain cells that process visual information from our eyes. […] In PCA caused by Alzheimerâs disease, it is not clear why the disease affects this part of the brain more so than the areas affected by typical Alzheimerâs, which generally are involved in memory. It is thought that what makes up a personâs risk of developing PCA is similar to other types of dementia. This is a complex mix of factors including: our age, the genes we inherit from our parents, our environment, our lifestyle. Scientists are working hard to find out more, as well as exploring specific genetic or lifestyle risk factors for the condition.
#44 What to Know About Cortical Dementia | Senior Lifestyle
https://www.seniorlifestyle.com/resources/blog/cortical-dementia-what-to-know/
Cortical dementia, or posterior cortical atrophy, is the gradual, progressive degeneration of the cortex. Primarily, this occurs in the part of the brain in the back of the head, or the posterior. The outer layers of the brain play an important role in such functions such as language and memory. […] Doctors are uncertain whether posterior cortical atrophy is a condition separate from Alzheimers disease or whether it is a variant. […] Many people with posterior cortical atrophy have conditions similar to Alzheimers disease, such as amyloid plaques and neurofibrillary tangles, but in a different part of the brain. The damage is similar to those with Lewy body disease. […] Doctors are aware of a number of risk factors for dementia, including: aging, depression, excessive alcohol consumption, family history of dementia, hearing loss, inadequately controlled high blood pressure, obesity, physical inactivity, smoking, uncontrolled diabetes.
#45 Posterior cortical atrophy – PubMed
https://pubmed.ncbi.nlm.nih.gov/22265212/
Posterior cortical atrophy (PCA) is a neurodegenerative syndrome that is characterised by progressive decline in visuospatial, visuoperceptual, literacy, and praxic skills. The progressive neurodegeneration affecting parietal, occipital, and occipitotemporal cortices that underlies PCA is attributable to Alzheimer’s disease in most patients. […] However, alternative underlying causes, including dementia with Lewy bodies, corticobasal degeneration, and prion disease, have also been identified, and not all patients with PCA have atrophy on clinical imaging. […] Important challenges remain, including the identification of factors associated not only with the selective vulnerability of posterior cortical regions but also with the young age of onset of PCA.
#46
https://discovery.ucl.ac.uk/1337217/
Posterior cortical atrophy (PCA) is a neurodegenerative syndrome that is characterised by progressive decline in visuospatial, visuoperceptual, literacy, and praxic skills. […] The progressive neurodegeneration affecting parietal, occipital, and occipitotemporal cortices that underlies PCA is attributable to Alzheimer’s disease in most patients. However, alternative underlying causes, including dementia with Lewy bodies, corticobasal degeneration, and prion disease, have also been identified, and not all patients with PCA have atrophy on clinical imaging. […] Important challenges remain, including the identification of factors associated not only with the selective vulnerability of posterior cortical regions but also with the young age of onset of PCA.
#47 Posterior cortical atrophy (PCA) | Dementia Australia
https://www.dementia.org.au/about-dementia/posterior-cortical-atrophy-pca
If you have posterior cortical atrophy, tissue in your brain atrophies (shrinks) as cells are lost. […] Posterior cortical atrophy (PCA) involves the loss and dysfunction of brain cells, particularly at the posterior (back) of the brain. […] In most cases, the underlying cause of posterior cortical atrophy is Alzheimers disease. But other conditions can show similar symptoms. These include Lewy body disease, corticobasal degeneration and Creutzfeldt-Jakob disease. […] Many experts believe posterior cortical atrophy is a form of Alzheimers disease. This is because the brain changes observed in both types of dementia can be similar. However, the symptoms for each type are different. […] Alzheimers disease affects most areas of the brain and is commonly associated with deterioration in memory, language and perception.
#48 Mysteries Of Posterior Cortical Atrophy: Symptoms, Causes, And Management | The Lifesciences Magazine
https://thelifesciencesmagazine.com/unraveling-posterior-cortical-atrophy/
Posterior Cortical Atrophy, often referred to as PCA, is a neurodegenerative condition characterized by progressive degeneration of the posterior regions of the brain, particularly the occipital, parietal, and temporal lobes. […] The exact cause of Posterior Cortical Atrophy remains elusive, although it is often associated with underlying neurodegenerative conditions such as Alzheimers disease or dementia with Lewy bodies. In some cases, PCA may result from specific neurodegenerative proteinopathies, including tauopathies and synucleinopathies, which lead to the accumulation of abnormal proteins in the brain. Genetic factors may also play a role, as certain gene mutations have been implicated in familial forms of PCA. […] While the exact cause of PCA remains elusive, researchers have made significant strides in understanding the diverse mechanisms that contribute to the development and progression of this neurodegenerative disorder.
#49 Posterior Cortical Atrophy | Stellar Care
https://stellarcaresd.com/posterior-cortical-atrophy/
The exact cause of Posterior Cortical Atrophy (PCA) is not well understood, and it remains a subject of ongoing research. These factors may contribute to its development: […] Like Alzheimers disease, PCA is associated with the abnormal accumulation of proteins in the brain, such as beta-amyloid plaques and tau tangles. These protein aggregates can disrupt brain function and lead to neuronal damage. […] Some cases of PCA are believed to have a genetic component. Mutations in certain genes, such as PSEN1, PSEN2, or MAPT, have been identified in a subset of people with PCA. These genetic mutations can increase the risk of developing the condition. […] In some cases, PCA may co-occur with other neurodegenerative conditions, like Alzheimers disease or Lewy body dementia. These overlapping pathologies can contribute to the cognitive and visual symptoms observed in PCA.
#50 Posterior Cortical Atrophy | Stellar Care
https://stellarcaresd.com/posterior-cortical-atrophy/
The exact cause of Posterior Cortical Atrophy (PCA) is not well understood, and it remains a subject of ongoing research. These factors may contribute to its development: […] Like Alzheimers disease, PCA is associated with the abnormal accumulation of proteins in the brain, such as beta-amyloid plaques and tau tangles. These protein aggregates can disrupt brain function and lead to neuronal damage. […] Some cases of PCA are believed to have a genetic component. Mutations in certain genes, such as PSEN1, PSEN2, or MAPT, have been identified in a subset of people with PCA. These genetic mutations can increase the risk of developing the condition. […] In some cases, PCA may co-occur with other neurodegenerative conditions, like Alzheimers disease or Lewy body dementia. These overlapping pathologies can contribute to the cognitive and visual symptoms observed in PCA.
#51 Posterior Cortical Atrophy | Stellar Care
https://stellarcaresd.com/posterior-cortical-atrophy/
The specific brain regions affected by PCA, primarily the posterior cortex responsible for visual processing, are an important part of this condition. Structural changes or atrophy in these brain regions are believed to be central to the development of PCA. […] PCA typically affects individuals later in life, often between the ages of 50 and 65. Advanced age is a significant risk factor for many neurodegenerative disorders, including PCA. […] While there isn’t a single gene responsible for PCA, some genetic factors may increase a person’s susceptibility to neurodegenerative diseases in general. These genetic factors can interact with other environmental and lifestyle factors to influence an individual’s risk. […] In some cases, a family history of neurodegenerative diseases may suggest a genetic predisposition, but this doesn’t guarantee that someone will develop PCA.
#52 Mysteries Of Posterior Cortical Atrophy: Symptoms, Causes, And Management | The Lifesciences Magazine
https://thelifesciencesmagazine.com/unraveling-posterior-cortical-atrophy/
Posterior Cortical Atrophy, often referred to as PCA, is a neurodegenerative condition characterized by progressive degeneration of the posterior regions of the brain, particularly the occipital, parietal, and temporal lobes. […] The exact cause of Posterior Cortical Atrophy remains elusive, although it is often associated with underlying neurodegenerative conditions such as Alzheimers disease or dementia with Lewy bodies. In some cases, PCA may result from specific neurodegenerative proteinopathies, including tauopathies and synucleinopathies, which lead to the accumulation of abnormal proteins in the brain. Genetic factors may also play a role, as certain gene mutations have been implicated in familial forms of PCA. […] While the exact cause of PCA remains elusive, researchers have made significant strides in understanding the diverse mechanisms that contribute to the development and progression of this neurodegenerative disorder.
#53 Mysteries Of Posterior Cortical Atrophy: Symptoms, Causes, And Management | The Lifesciences Magazine
https://thelifesciencesmagazine.com/unraveling-posterior-cortical-atrophy/
One prominent hypothesis suggests that PCA often coexists with Alzheimers disease pathology, with a subset of individuals exhibiting a distinct pattern of cortical atrophy predominantly affecting the posterior brain regions. […] In addition to Alzheimers disease, PCA has been linked to other neurodegenerative proteinopathies characterized by the abnormal accumulation of specific proteins in the brain. […] Genetic factors also play a role in the development of PCA, particularly in familial forms of the disease. Mutations in genes associated with Alzheimers disease, such as the amyloid precursor protein (APP), presenilin 1 (PSEN1), and presenilin 2 (PSEN2) genes, have been implicated in familial PCA cases. Additionally, mutations in other genes, such as GRN (progranulin) and MAPT (microtubule-associated protein tau), have been linked to familial frontotemporal dementia (FTD) syndromes, which may present with clinical features overlapping with PCA. […] In summary, the underlying causes of Posterior Cortical Atrophy are multifaceted, involving a complex interplay of genetic, molecular, and environmental factors.
#54 Mysteries Of Posterior Cortical Atrophy: Symptoms, Causes, And Management | The Lifesciences Magazine
https://thelifesciencesmagazine.com/unraveling-posterior-cortical-atrophy/
Posterior Cortical Atrophy, often referred to as PCA, is a neurodegenerative condition characterized by progressive degeneration of the posterior regions of the brain, particularly the occipital, parietal, and temporal lobes. […] The exact cause of Posterior Cortical Atrophy remains elusive, although it is often associated with underlying neurodegenerative conditions such as Alzheimers disease or dementia with Lewy bodies. In some cases, PCA may result from specific neurodegenerative proteinopathies, including tauopathies and synucleinopathies, which lead to the accumulation of abnormal proteins in the brain. Genetic factors may also play a role, as certain gene mutations have been implicated in familial forms of PCA. […] While the exact cause of PCA remains elusive, researchers have made significant strides in understanding the diverse mechanisms that contribute to the development and progression of this neurodegenerative disorder.
#55
https://link.springer.com/article/10.1007/s11940-022-00745-0
Posterior cortical atrophy (PCA) is a clinico-radiological syndrome characterised by the progressive loss of higher order visuospatial, visuoperceptual and other posterior cortical functions consistent with occipito-parietal and occipito-temporal atrophy. […] Retrospective and prospective neuropathological and biomarker studies have reported evidence of primary or co-existing AD pathology in 75% of cases. […] PCA is essentially sporadic, and clinical genetic testing is not usually indicated without a compelling family history. […] Despite evidence of increased occipital amyloid in PCA, amyloid PET deposition in PCA broadly resembles typical AD, whilst FDG and tau PET scans emphasise marked regional, particularly occipital, involvement. […] PCA diagnosis is frequently delayed and patients may be misdiagnosed with an ocular or psychological illness.
#56 Posterior Cortical Atrophy (PCA)
https://ftdboston.org/diagnosis/posterior-cortical-atrophy/
Posterior cortical atrophy (PCA) is a progressive neurodegenerative condition that affects areas in the brain responsible for visual processing and spatial perception. Over time, other thinking skills such as memory, executive functions, and word retrieval may become affected. […] In the majority of PCA cases, the underlying cause is Alzheimers disease. Lumbar punctures conducted in the clinic as well as specialized positron emission tomography (PET) scans can show abnormal accumulation of amyloid and tau proteins that form the plaques and tangles seen in Alzheimers disease, thus confirming the diagnosis. Although PCA is usually caused by Alzheimers disease, it can also result from other diseases including Lewy body disease and corticobasal degeneration, and the clinical features may be different in these cases. […] PCA is often under-recognized or misdiagnosed since patients and doctors can mistake visual cognitive decline (a problem with the brain) for a visual acuity impairment (a problem with the eyes).
#57 Orphanet: Posterior cortical atrophy
https://www.orpha.net/en/disease/detail/54247
A rare neurologic disease characterized by impairment of higher visual processing skills and other posterior cortical functions without any evidence of ocular abnormalities, relatively intact memory and language in the early stages, and atrophy of posterior brain regions. […] A third level of the international classification framework provides disease-level descriptions of the underlying causes of the PCA syndrome, based on available pathophysiological biomarker evidence. This distinguishes between Alzheimer’s disease as the most common underlying pathology (PCA-AD), and other causes including Lewy Body disease (PCA-LBD), corticobasal degeneration (PCA-CBD) and prion disease (PCA-prion). […] Differential diagnosis includes the most commonly associated neuropathology Alzheimer disease, but may also include Lewy body disease, cortico-basal degeneration, and prion diseases such as Creutzfeldt-Jakob disease.
#58 Posterior Cortical Atrophy in Alzheimer Disease
https://decisionpoint.medscape.com/neurology/viewarticle/985528
Posterior cortical atrophy is a clinical syndrome, which is commonly associated with Alzheimer’s disease. […] However, separating the etiologic cause from the clinical presentation is important, as there are other potential causes of the posterior cortical atrophy clinical syndrome beyond Alzheimer’s disease. This includes prion diseases such as sporadic Creutzfeldt-Jakob disease. […] However, the most common etiologic association of posterior cortical atrophy syndrome is Alzheimer’s disease, but given this broad differential, a positive diagnosis should be sought with biomarker data that can be in the form of cerebrospinal fluid testing for amyloid beta in tau species or positron emission tomography with amyloid and TauPET imaging. […] With the advent of etiologic-specific disease-modifying therapies, establishing the presence of these etiologic factors will be important for management and counseling.
#59
https://medschool.cuanschutz.edu/pca/what-is-pca/what-is-pca
PCA is a condition of the brain that progressively worsens over time and affects areas in the brain that process visual information. […] Although PCA is not caused by one disease, for the majority of patients with PCA the cause is Alzheimers disease. […] An autopsy can be performed at the time of death to determine the cause of PCA. […] In these instances, where we can confirm the cause or causes of PCA by autopsy, the most common cause is Alzheimers disease (approximately 75% of autopsies for PCA). […] In summary, PCA is a syndrome and not a disease. This means that it is a constellation of symptoms that can be caused by different diseases. As noted, the most common disease to cause PCA is Alzheimers disease (approximately 75%), and some people with Alzheimers disease-causing PCA can also have Lewy Bodies contributing to their PCA syndrome (about 10%). […] The remainder of the 10% is due to corticobasal degeneration, prion disease, or a combination of diseases.