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Atrofia cortical posterior
ORPHA:54247CID-10 · G31.1CID-11 · 8A21.0DOENÇA RARA

A Atrofia Cortical Posterior (ACP) é uma condição rara do cérebro que vai piorando com o tempo, e que geralmente começa entre os 50 e 65 anos de idade. Ela é caracterizada por dificuldades crescentes em processar e interpretar o que se vê, além de afetar outras funções da parte de trás do cérebro (córtex posterior), sem que haja problemas nos olhos que expliquem essa dificuldade.

Mantido por Agente Raras·Colaborar como especialista →

Introdução

O que você precisa saber de cara

📋

A Atrofia Cortical Posterior (ACP) é uma condição rara do cérebro que vai piorando com o tempo, e que geralmente começa entre os 50 e 65 anos de idade. Ela é caracterizada por dificuldades crescentes em processar e interpretar o que se vê, além de afetar outras funções da parte de trás do cérebro (córtex posterior), sem que haja problemas nos olhos que expliquem essa dificuldade.

Pesquisas ativas
6 ensaios
13 total registrados no ClinicalTrials.gov
Publicações científicas
700 artigos
Último publicado: 2026 Apr 13

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
Unknown
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.0
Worldwide
Início
Adult
🏥
SUS: Cobertura mínimaScore: 15%
CID-10: G31.1
🇧🇷Dados SUS / DATASUS
PROCEDIMENTOS SIGTAP (2)
0202010694
Sequenciamento completo do exoma (WES)genetic_test
0301070040
Atendimento em reabilitação — doenças rarasrehabilitation
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Entender a doença

Do básico ao detalhe, leia no seu ritmo

Preparando trilha educativa...

Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Partes do corpo afetadas

🧠
Neurológico
3 sintomas
👁️
Olhos
3 sintomas
🦴
Ossos e articulações
2 sintomas

+ 13 sintomas em outras categorias

Características mais comuns

90%prev.
Discalculia
Muito frequente (99-80%)
90%prev.
Agnosia digital
Muito frequente (99-80%)
90%prev.
Agnosia
Muito frequente (99-80%)
90%prev.
Ataxia
Muito frequente (99-80%)
90%prev.
Apraxia oculomotora
Muito frequente (99-80%)
90%prev.
Confusão
Muito frequente (99-80%)
21sintomas
Muito frequente (10)
Frequente (5)
Ocasional (6)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 21 características clínicas mais associadas, ordenadas por frequência.

DiscalculiaDyscalculia
Muito frequente (99-80%)90%
Agnosia digitalFinger agnosia
Muito frequente (99-80%)90%
Agnosia
Muito frequente (99-80%)90%
Ataxia
Muito frequente (99-80%)90%
Apraxia oculomotoraOculomotor apraxia
Muito frequente (99-80%)90%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico700PubMed
Últimos 10 anos200publicações
Pico202555 papers
Linha do tempo
2026Hoje · 2026🧪 2009Primeiro ensaio clínico📈 2025Ano de pico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Genética e causas

O que está alterado no DNA e como passa nas famílias

🧬

Nenhum gene associado encontrado

Os dados genéticos desta condição ainda estão sendo catalogados.

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

Carregando...

Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
1Fase 11
·Pré-clínico10
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 11 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

🇧🇷 Atendimento SUS — Atrofia cortical posterior

🗺️

Selecione um estado ou use sua localização para ver resultados.

Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.

Pesquisa ativa

Ensaios clínicos abertos e novidades científicas recentes

🟢 Recrutando agora

5 pesquisas recrutando participantes. Converse com seu médico sobre a possibilidade de participar.

Outros ensaios clínicos

13 ensaios clínicos encontrados, 6 ativos.

Distribuição por fase
Ver todos no ClinicalTrials.gov
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Publicações mais relevantes

Timeline de publicações
470 papers (10 anos)
#1

Disrupted brain topological network and its association with clinical features in posterior cortical atrophy.

Brain communications2026

The characteristics of structural brain topological network alterations and their correlation with clinical features in posterior cortical atrophy (PCA) remain elusive. This study aims to explore the structural topological network alterations and their correlation with clinical features in PCA. Thirty-four patients with PCA and 34 healthy controls were enrolled in this cross-sectional study and underwent diffusion tensor imaging, structural MRI and neuropsychological assessment. The graph theory method was applied to capture the individual structural properties of the network. Partial correlation analysis was conducted to investigate the clinical relevance of the network properties. The global metrics of the structural and topological network are altered in the PCA group. The nodal metrics were changed in the occipital, parietal, temporal and frontal lobes. Global network metrics are associated with cognition and disease severity. For the node associated with apraxia, finger agnosia, left-right disorientation and agnosia for colour, face and object, we observed a similar but not identical nodal distribution, which was mainly distributed in the parietal cortex. Notably, we observed frontal nodes, including the orbital gyrus, contribute to the left-right disorientation and colour agnosia, and the rectus contributes to the object agnosia. This study helps us understand the underlying mechanism of the symptom network in PCA and provides a promising biomarker for PCA.

#2

Gray matter atrophy and structural connectivity in Posterior Cortical Atrophy: A voxel-based meta-analysis.

Neuroscience and biobehavioral reviews2026 Apr

Posterior Cortical Atrophy (PCA) is a neurodegenerative syndrome most commonly associated with Alzheimer's disease, characterized by progressive visuospatial and visuoperceptual decline. Although voxel-based morphometry studies have described gray matter loss in PCA, a comprehensive and updated coordinate-based meta-analysis is still missing, and associated structural connectivity alterations remain unclear. We conducted a systematic review and meta-analysis of whole-brain voxel-based morphometry studies comparing patients with PCA and healthy controls (PROSPERO ID: CRD420251010673). Analyses were performed using Seed-based d Mapping with Permutation of Subject Images (SDM-PSI) with family-wise error correction, and meta-regressions assessed the impact of demographic and clinical variables. To investigate structural connectivity, deterministic tractography was carried out on a normative diffusion MRI template, using meta-analytic gray matter clusters as seeds. Eighteen studies were included (339 PCA; 577 healthy controls). The meta-analysis revealed consistent bilateral gray matter atrophy in the lateral occipital cortex, inferior parietal lobule, precuneus, and ventral occipitotemporal regions. Meta-regression highlighted an interaction between age and disease duration, associated with atrophy in the left superior temporal gyrus and right thalamus. Tractography demonstrated that affected clusters were embedded within major long-range pathways, including the superior and inferior longitudinal fasciculi, vertical occipital fasciculi, and parietal aslant tract. Regression-derived clusters additionally mapped onto the arcuate fasciculus, frontal aslant tract, and superior thalamic radiations. This is the first systematic review and voxel-based meta-analysis of PCA conducted after the establishment of consensus diagnostic criteria, providing a statistically robust characterization of gray and white matter alterations and identifying potential imaging biomarkers for diagnosis and treatment.

#3

Detecting Dementia Using Lexical Analysis: Terry Pratchett's Discworld Tells a More Personal Story.

Brain sciences2026 Jan 16

Background/Objectives: Dementia, characterised by cognitive decline, significantly impacts language abilities. While the risk of dementia increases with age, it often manifests years before clinical diagnosis. Identifying early warning signs is crucial for timely intervention. Previous research has demonstrated that changes in language, such as reduced vocabulary diversity and simpler sentence structures, may be observed in individuals with dementia. This study investigates the potential of linguistic analysis to detect early signs of cognitive decline by examining the writing of Sir Terry Pratchett, a renowned author diagnosed with Posterior Cortical Atrophy (PCA), typically a form of dementia caused by Alzheimer's disease. Methods: This study analysed 33 Discworld novels by Terry Pratchett, comparing linguistic features before and after a potential turning point identified through analysis of adjective type-token ratios (TTR). Results: A significant decrease in lexical diversity (TTR) was observed for nouns and adjectives in later works. Total wordcount increased, while lexical diversity decreased, suggesting a shift towards simpler language. This shift coincided with a decrease in adjective TTR below a defined threshold, occurring approximately ten years before Pratchett's formal diagnosis. Conclusions: These findings suggest that subtle changes in linguistic patterns, such as decreased lexical diversity, may precede clinical diagnosis of dementia by a considerable margin. This research highlights the potential of linguistic analysis as a valuable tool for early detection of cognitive decline. Further research is needed to validate these findings in larger cohorts and explore the specific linguistic markers associated with different types of dementia.

#4

Alzheimer's Disease Continuum: Evaluating the Relationship between Fluid Biomarkers and Patients' Phenotype and Profile.

Aging and disease2026 Jan 22

The measurement of fluid biomarkers such as phosphorylated tau at threonine 181 (pTAU181), amyloid-β 40 (Aβ40), and Aβ42 is a routine medical examination that contributes to achieving an accurate diagnosis within the Alzheimer's disease (AD) clinical continuum. The aim of this study is to compare the concentration of these biomarkers in CSF and plasma and determine their relationship with the patients' clinical variant and profile. Patients were diagnosed following the NIA-AA criteria. Plasma and CSF were obtained from healthy controls (HC), mild cognitive impairment (MCI non-AD), MCI displaying positive AD markers (MCI-AD), and AD patients. Biomarker levels were assessed using the LUMIPULSE® G600II instrument (Fujirebio, Japan). Data showed a significant increase in pTAU181 concentration, specifically in the progression from MCI non-AD to AD conditions. The opposite trend was observed for Aβ42 and Aβ42/Aβ40. Furthermore, these biomarker trends appeared to change consistently with variations in the MMSE score, highlighting the relevance of plasma in detecting changes in patients' cognitive function. Considering the clinical variant, atypical patients displayed the highest pTAU181 and lowest Aβ42 levels, consistent with their lower MMSE scores. Lastly, the posterior cortical atrophy (PCA) profile showed higher pTAU181 and lower Aβ42 levels when compared to other profiles. Nevertheless, these last results are to be cautiously interpreted given the limited number of samples included in the analysis. Hence, further analyses on larger cohorts are needed to better define the role of these biomarkers in distinguishing between patients' clinical variants.

#5

How reproducible are data-driven subtypes of Alzheimer's disease atrophy?

Journal of Alzheimer's disease : JAD2026 Mar

BackgroundAlzheimer's disease (AD) exhibits substantial clinical and biological heterogeneity, complicating efforts in treatment and intervention development. While new computational methods offer insights into AD subtyping and disease staging, the reproducibility of these subtypes across datasets remains understudied, particularly concerning the robustness of subtype definitions when validated on diverse databases.ObjectiveThis study evaluates the robustness of the AD progression subtypes identified by the Subtype and Stage Inference (SuStaIn) algorithm on a larger and more diverse cohort.MethodsWe extracted T1-weighted MRI data for 5444 subjects from ANMerge, OASIS, and ADNI datasets, forming four independent cohorts. Each cohort was analyzed with SuStaIn under two conditions: one using the full cohort, including cognitively normal controls, and another excluding controls to test subtype robustness.ResultsResults confirm the three primary atrophy subtypes identified in earlier studies: Typical, Cortical, and Subcortical, as well as the emergence of rare and atypical AD variants such as posterior cortical atrophy. Notably, each subtype displayed varying robustness to the inclusion of controls, with certain subtypes, like the Subcortical subtype, more influenced by cohort composition.ConclusionsThis investigation underscores SuStaIn's reliability for defining stable AD subtypes and suggests its utility in clinical stratification for trials and diagnosis. However, our findings also highlight the need for improved dataset ethnic and demographic diversity, particularly in terms of ethnic representation, to enhance generalizability and support broader clinical application.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC433 artigos no totalmostrando 195

2026

Disrupted brain topological network and its association with clinical features in posterior cortical atrophy.

Brain communications
2026

Alzheimer's Disease Continuum: Evaluating the Relationship between Fluid Biomarkers and Patients' Phenotype and Profile.

Aging and disease
2026

Gray matter atrophy and structural connectivity in Posterior Cortical Atrophy: A voxel-based meta-analysis.

Neuroscience and biobehavioral reviews
2026

How reproducible are data-driven subtypes of Alzheimer's disease atrophy?

Journal of Alzheimer's disease : JAD
2026

Detecting Dementia Using Lexical Analysis: Terry Pratchett's Discworld Tells a More Personal Story.

Brain sciences
2025

Eye movement abnormalities in Alzheimer's disease and other neurodegenerative dementias: insights from current evidence and priorities for future research.

Frontiers in ophthalmology
2025

Posterior Cortical Atrophy Masked by a Pituitary Macroadenoma: Diagnostic Challenges in the Setting of Dual Visual Pathology.

Cureus
2025

Posterior Cortical Atrophy: Altered Language Processing System Connectivity and Its Implications on Language Comprehension and Production.

Brain sciences
2025

The Heterogeneity of Reading and Spelling Deficits in Posterior Cortical Atrophy.

Brain sciences
2025

Lecanemab for posterior cortical atrophy: Two contrasting cases.

The Clinical neuropsychologist
2025

A Cerebral Origin for Retinal Degeneration in the Visual Form of Alzheimer's Disease?

The European journal of neuroscience
2025

Digital dementia and testing of cognitive intervention for degenerating neural networks.

NPJ systems biology and applications
2025

Posterior cortical atrophy: reorganization of the dorsal attention network and its implications on volume loss and clinical performance.

Journal of neurology
2025

Posterior cortical atrophy misdiagnosed as typical Alzheimer's disease: Clinical and neuropsychological evolution over eight years. A case report.

Journal of Alzheimer's disease reports
2026

Visual modulation of vestibular-evoked balance response disturbed by posterior cortical atrophy.

The Journal of physiology
2025

Multidimensional cognitive deficits in the typical and atypical variants of Alzheimer's disease.

Alzheimer's research &amp; therapy
2025

Symptomatic Epilepsy and Aspiration Pneumonia in a Female Patient With Posterior Cortical Atrophy.

Geriatrics &amp; gerontology international
2025

Spatial Patterns of Cerebral Blood Flow in Alzheimer's Disease Identified by the Subtype and Stage Inference Algorithm.

Dementia and geriatric cognitive disorders extra
2025

The First Shall Be First: Letter-Position Coding and Spatial Invariance in Two Cases of Attentional Dyslexia.

Brain sciences
2025

Everyday functioning in young onset dementia: differences between diagnostic groups.

Alzheimer's &amp; dementia : the journal of the Alzheimer's Association
2025

Investigating the Impact of Presentation Format on Reading Ability in Posterior Cortical Atrophy: A Case Study.

Reports (MDPI)
2025

Take Caution of Normal Visual Acuity in Posterior Cortical Atrophy!

Neuro-ophthalmology (Aeolus Press)
2025

Neurodevelopment and neural environment inform Alzheimer's disease age at onset and phenotype.

Alzheimer's &amp; dementia : the journal of the Alzheimer's Association
2025

Computerized Open-Source Navon Test (COSNaT): Normative data for the assessment of global processing abilities and simultanagnosia in the Italian population.

Behavior research methods
2025

Alterations of the brain functional organization in young-onset Alzheimer's disease: Is it a matter of "time"?

Journal of Alzheimer's disease : JAD
2025

Better Living with Non-memory-led Dementia: study protocol for a randomised controlled trial of a web-based caregiver educational programme (BELIDE trial).

BMJ open
2025

Ishihara color plates utilized as an assessment for simultanagnosia in posterior cortical atrophy: A case report.

World journal of clinical cases
2025

Neuroimaging in posterior cortical atrophy: an integrative review for clinicians and radiologists.

Frontiers in neurology
2025

Connectivity as a universal predictor of tau progression in atypical Alzheimer's disease.

Brain : a journal of neurology
2025

Visuospatial impairment and resting-state network correlations in Alzheimer's disease: A systematic review.

Behavioural brain research
2025

Visual field defects in dementia: A scoping review.

Ophthalmic &amp; physiological optics : the journal of the British College of Ophthalmic Opticians (Optometrists)
2025

An FDG-PET-Based Machine Learning Framework to Support Neurologic Decision-Making in Alzheimer Disease and Related Disorders.

Neurology
2025

Posterior cortical atrophy in the age of anti-amyloid treatments: An 11-year retrospective study of eligible patients from the Leenaards Memory Center.

Journal of Alzheimer's disease : JAD
2025

Decline in activities of daily living in the rarer dementias.

General psychiatry
2025

An uncommon variant of Alzheimer's disease: Posterior cortical atrophy.

The South African journal of psychiatry : SAJP : the journal of the Society of Psychiatrists of South Africa
2025

Neural mechanisms of disease pathology and cognition in young-onset Alzheimer's disease variants.

Journal of Alzheimer's disease : JAD
2025

[Posterior cortical atrophy presenting with agraphia for kanji and statokinetic dissociation (Riddoch phenomenon): a case report].

Rinsho shinkeigaku = Clinical neurology
2025

Parkinsonism Associated with Posterior Cortical Atrophy: Atypical Alzheimer's Disease.

Movement disorders clinical practice
2025

'Dementia has got two faces': grief as an experience of holding on and letting go for people living with primary progressive aphasia and posterior cortical atrophy.

Aging &amp; mental health
2025

Cognitive stimulation in non-memory led dementias: A scoping review.

Dementia (London, England)
2025

iSupport for rare dementias: a mixed-methods non-randomised feasibility study of an online self-help programme for carers.

Pilot and feasibility studies
2025

Survival Differences Between Individuals With Typical and Atypical Phenotypes of Alzheimer Disease.

Neurology
2025

Longitudinal Evolution of Posterior Cortical Atrophy: Diagnostic Delays, Overlapping Phenotypes, and Clinical Outcomes.

Neurology
2025

Cognitive Phenotyping and Interpretation of Alzheimer Blood Biomarkers.

JAMA neurology
2025

Pathological Characterisation of Posterior Cortical Atrophy in Comparison With Amnestic Alzheimer's Disease.

Neuropathology and applied neurobiology
2025

White matter hyperintensities in dementia with lewy bodies and posterior cortical atrophy.

Neurobiology of aging
2025

Phenotype-specific metabolic patterns in Posterior cortical atrophy and early-onset typical Alzheimer's disease.

Annals of nuclear medicine
2025

Towards realistic simulation of disease progression in the visual cortex with CNNs.

Scientific reports
2025

Autopsy-proven patient with corticobasal degeneration presenting with visuo-constructive disorders as initial symptoms: How advanced MRI sequences can help clinical practice.

Journal of Alzheimer's disease : JAD
2025

Multi-parametric [18F]PI-2620 tau PET/MRI for the phenotyping of different Alzheimer's disease variants.

European journal of nuclear medicine and molecular imaging
2025

[The experience of diagnosing Alzheimer's disease based on the study of cerebrospinal fluid biomarkers].

Zhurnal nevrologii i psikhiatrii imeni S.S. Korsakova
2025

Detection of Alzheimer Neuropathology in Alzheimer and Non-Alzheimer Clinical Syndromes With Blood-Based Biomarkers.

JAMA neurology
2025

Improvement in gait and functional abilities in an adult with posterior cortical atrophy after translingual neuromodulation with neurorehabilitation physical therapy: a case report.

Physiotherapy theory and practice
2025

Heterogeneous clinical phenotypes of sporadic early-onset Alzheimer's disease: a neuropsychological data-driven approach.

Alzheimer's research &amp; therapy
2024

Effects of Route Complexity and Lighting on Route Following in Alzheimer's Disease and Posterior Cortical Atrophy.

Brain sciences
2024

A Case of Posterior Cortical Atrophy in Alzheimer's Disease.

Cureus
2025

Differences in baseline cognitive performance between participants with early-onset and late-onset Alzheimer's disease: Comparison of LEADS and ADNI.

Alzheimer's &amp; dementia : the journal of the Alzheimer's Association
2024

Greater baseline cortical atrophy in the dorsal attention network predicts faster clinical decline in Posterior Cortical Atrophy.

Alzheimer's research &amp; therapy
2025

Posterior Cortical Atrophy Due to Alzheimer Disease in a Person With Down Syndrome: A Case Report.

Neurology
2024

Atypical Presentations of Alzheimer Disease.

Continuum (Minneapolis, Minn.)
2025

Cognitive rehabilitation in posterior cortical atrophy.

Practical neurology
2025

Late-onset diplopia: an unusual presentation of posterior cortical atrophy.

Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology
2024

Alterations of the pulvinar in posterior cortical atrophy: A multimodal MRI study.

Cortex; a journal devoted to the study of the nervous system and behavior
2024

Preserved musical working memory and absolute pitch in posterior cortical atrophy.

Cortex; a journal devoted to the study of the nervous system and behavior
2025

Default mode network tau predicts future clinical decline in atypical early Alzheimer's disease.

Brain : a journal of neurology
2024

Alzheimer's disease clinical variants show distinct neuroinflammatory profiles with neuropathology.

Neuropathology and applied neurobiology
2024

Pathologically Confirmed Alzheimer's Disease Presenting as Clinical Parkinson's Disease, A Case Report.

Movement disorders clinical practice
2023

Consensus recommendations for clinical assessment tools for the diagnosis of posterior cortical atrophy syndrome from the Atypical AD PIA of ISTAART.

Alzheimer's &amp; dementia (Amsterdam, Netherlands)
2024

Baseline multimodal imaging to predict longitudinal clinical decline in atypical Alzheimer's disease.

Cortex; a journal devoted to the study of the nervous system and behavior
2024

Global Processing Deficit in Amnestic Mild Cognitive Impairment.

Journal of Alzheimer's disease : JAD
2024

The Role of Population Receptive Field Sizes in Higher-Order Visual Dysfunction.

Current neurology and neuroscience reports
2024

Association of outer retinal and choroidal alterations with neuroimaging and clinical features in posterior cortical atrophy.

Alzheimer's research &amp; therapy
2024

[A case of left posterior cortical atrophy presenting with kana-predominant reading impairment].

Rinsho shinkeigaku = Clinical neurology
2024

Neuropsychological Diagnosis and Assessment of Alexia: A Mixed-Methods Study.

Brain sciences
2024

Graded Multidimensional Clinical and Radiologic Variation in Patients With Alzheimer Disease and Posterior Cortical Atrophy.

Neurology
2025

Dementia With Lewy Bodies Phenocopy in a Case of RT-QuIC-Negative Creutzfeldt-Jakob Disease: Implications of Functional Anatomy.

The Journal of neuropsychiatry and clinical neurosciences
2024

Rehabilitation Services for Young-Onset Dementia: Examples from High- and Low-Middle-Income Countries.

International journal of environmental research and public health
2024

The Graded Incomplete Letters Test (GILT): a rapid test to detect cortical visual loss, with UK Biobank implementation.

Behavior research methods
2024

Exploring experiential differences in everyday activities - A focused ethnographic study in the homes of people living with memory-led Alzheimer's disease and posterior cortical atrophy.

Journal of aging studies
2024

Case report: pre-symptomatic clinical and metabolic profile in posterior cortical atrophy and dementia with Lewy bodies.

Neurocase
2023

Visual imagery deficits in posterior cortical atrophy.

Cognitive neuropsychology
2024

Peer Support for Caregivers of People Living with Posterior Cortical Atrophy in Melbourne, Australia: A Feasibility Study.

International journal of environmental research and public health
2024

"I Want to Do Something" - Exploring What Makes Activities Meaningful for Community-Dwelling People Living With Dementia: A Focused Ethnographic Study.

Qualitative health research
2024

Age-specific prevalence of the different clinical presentations of AD and FTD in young-onset dementia.

Journal of neurology
2024

Secondary language impairment in posterior cortical atrophy: insights from sentence repetition.

Frontiers in neuroscience
2024

Greater white matter degeneration and lower structural connectivity in non-amnestic vs. amnestic Alzheimer's disease.

Frontiers in neuroscience
2024

Atypical Alzheimer's disease: new insights into an overlapping spectrum between the language and visual variants.

Journal of neurology
2024

Retinal and Cortical Visual Processing Dysfunction in a Case of Mild Cognitive Impairment with Lewy Bodies: A Case Report.

Journal of Alzheimer's disease reports
2024

Cognitive phenotype and neurodegeneration associated with Tau in Huntington's disease.

Annals of clinical and translational neurology
2024

Potential ocular indicators to distinguish posterior cortical atrophy and typical Alzheimer's disease: a cross-section study using optical coherence tomography angiography.

Alzheimer's research &amp; therapy
2024

Atypical clinical variants of Alzheimer's disease: are they really atypical?

Frontiers in neuroscience
2024

Longitudinal default mode sub-networks in the language and visual variants of Alzheimer's disease.

Brain communications
2024

Altered structural and functional connectivity in Posterior Cortical Atrophy and Dementia with Lewy bodies.

NeuroImage
2024

Prosopagnosia: face blindness and its association with neurological disorders.

Brain communications
2024

Language uncovers visuospatial dysfunction in posterior cortical atrophy: a natural language processing approach.

Frontiers in neuroscience
2024

Ventral Variant Posterior Cortical Atrophy with Occipito-temporal Accumulation of Tau Proteins/Astrocyte Gliosis.

Internal medicine (Tokyo, Japan)
2024

Neurophysiological Alterations of the Visual Pathway in Posterior Cortical Atrophy: Systematic Review and a Case Series.

Journal of Alzheimer's disease : JAD
2024

A Case of Posterior Cortical Atrophy Presenting with Mood and Psychotic Symptoms.

Case reports in psychiatry
2024

Visual Agnosia Mimicking Memory Impairment: A Case Report of Posterior Cortical Atrophy.

Neuro-ophthalmology (Aeolus Press)
2024

Diagnostic performance of molecular imaging methods in predicting the progression from mild cognitive impairment to dementia: an updated systematic review.

European journal of nuclear medicine and molecular imaging
2024

'Communication is difficult': Speech, language and communication needs of people with young onset or rarer forms of non-language led dementia.

International journal of language &amp; communication disorders
2024

Auditory naming is impaired in posterior cortical atrophy and early-onset Alzheimer's disease.

Frontiers in neuroscience
2024

Demographic, clinical, biomarker, and neuropathological correlates of posterior cortical atrophy: an international cohort study and individual participant data meta-analysis.

The Lancet. Neurology
2024

Posterior cortical atrophy: new insights into treatments and biomarkers for Alzheimer's disease.

The Lancet. Neurology
2024

Lobar Microbleeds in the Posterior Cortical Atrophy Syndrome: A Comparison to Typical Alzheimer's Disease.

Current neurology and neuroscience reports
2024

Effects of a Hybrid Model of Occupational Therapy on Visuospatial Perception and Functional Skills for Cases With Posterior Cortical Atrophy: A Five-Case Report.

American journal of Alzheimer's disease and other dementias
2024

Behavioral and Neuropsychiatric Differences Across Two Atypical Alzheimer's Disease Variants: Logopenic Progressive Aphasia and Posterior Cortical Atrophy.

Journal of Alzheimer's disease : JAD
2024

Social cognition and behavioral changes in patients with posterior cortical atrophy.

Journal of neurology
2023

Pareidolias are a function of visuoperceptual impairment.

PloS one
2023

Abnormal network connections to early visual cortex in posterior cortical atrophy.

Journal of the neurological sciences
2024

E-mail as an early indicator of posterior cortical atrophy.

Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology
2023

"Better Living with Non-memory-led Dementia": protocol for a feasibility randomised controlled trial of a web-based caregiver educational programme.

Pilot and feasibility studies
2023

Medial Temporal Lobe Tau Aggregation Relates to Divergent Cognitive and Emotional Empathy Abilities in Alzheimer's Disease.

Journal of Alzheimer's disease : JAD
2023

Increased Resolution of Structural MRI at 3T Improves Estimation of Regional Cortical Degeneration in Individual Dementia Patients Using Surface Thickness Maps.

Journal of Alzheimer's disease : JAD
2023

A Case of Corticobasal Syndrome and Posterior Cortical Atrophy With Biomarkers of Alzheimer Disease.

Alzheimer disease and associated disorders
2023

Is Shadowing Behavior Caused by Body Representation Disorders and Apraxia?

Journal of Alzheimer's disease : JAD
2023

How Many Alzheimer-Perusini's Atypical Forms Do We Still Have to Discover?

Biomedicines
2023

Longitudinal rates of atrophy and tau accumulation differ between the visual and language variants of atypical Alzheimer's disease.

Alzheimer's &amp; dementia : the journal of the Alzheimer's Association
2023

Shadowing Behavior May Be Associated with an Inability to Recognize the External World: A Case Report of Shadowing in a Patient with Posterior Cortical Atrophy.

Journal of Alzheimer's disease : JAD
2023

Altered within- and between-network functional connectivity in atypical Alzheimer's disease.

Brain communications
2023

"I felt like I had been put on the shelf and forgotten about" - lasting lessons about the impact of COVID-19 on people affected by rarer dementias.

BMC geriatrics
2023

Atypical forms of Alzheimer's disease: patients not to forget.

Current opinion in neurology
2023

A Novel PSEN1 Variant Leading to Posterior Cortical Atrophy: A Case Report.

Journal of Alzheimer's disease reports
2023

Diffusivity Changes in Posterior Cortical Atrophy and Logopenic Progressive Aphasia: A Longitudinal Diffusion Tensor Imaging Study.

Journal of Alzheimer's disease : JAD
2023

Association between pseudoexfoliation and Alzheimer's disease-related brain atrophy.

PloS one
2023

Composite Indices of the Color-Picture Version of Boston Naming Test Have Better Discriminatory Power: Reliability and Validity in a Chinese Sample with Diverse Neurodegenerative Diseases.

Journal of Alzheimer's disease : JAD
2023

Expanding MAPT p.V363I Mutation Phenotype: An Overlapping of PSP-CBS and Posterior Cortical Atrophy.

Movement disorders clinical practice
2023

APOE ε4 influences within and between network functional connectivity in posterior cortical atrophy and logopenic progressive aphasia.

Alzheimer's &amp; dementia : the journal of the Alzheimer's Association
2023

Suitability of memory aids and strategies for people with posterior cortical atrophy: protocol for a scoping review.

Systematic reviews
2023

Sensory Integration Deficits in Neurodegenerative Diseases: Implications for Apraxia.

Archives of clinical neuropsychology : the official journal of the National Academy of Neuropsychologists
2023

Neurofunctional Correlates of Activities of Daily Living in Patients with Posterior Cortical Atrophy.

Journal of Alzheimer's disease : JAD
2023

A Selective Hand Posture Apraxia in an Individual With Posterior Cortical Atrophy and Probable Corticobasal Syndrome.

Cognitive and behavioral neurology : official journal of the Society for Behavioral and Cognitive Neurology
2023

Antiphospholipid syndrome mimicking posterior cortical atrophy and the "railroad track" sign on brain fluorodeoxyglucose-positron emission tomography.

Journal of the neurological sciences
2023

Effects of the visual environment on object localization in posterior cortical atrophy and typical Alzheimer's disease.

Frontiers in medicine
2023

Posterior cortical atrophy: clinical, neuroimaging, and neuropathological features.

Expert review of neurotherapeutics
2023

Diagnosis and Management of Posterior Cortical Atrophy.

Current treatment options in neurology
2023

Posterior white matter integrity and self-reported posterior cortical symptoms using the Colorado Posterior Cortical Questionnaire.

Frontiers in neurology
2023

Interest of eye movement study in early diagnosis of posterior cortical atrophy: A case-report.

Revue neurologique
2023

Recent research advances in young-onset dementia.

Current opinion in psychiatry
2023

[Rare forms of dementia-Atypical variants of Alzheimer's dementia].

Innere Medizin (Heidelberg, Germany)
2022

Meaningless imitation in neurodegenerative diseases: Effects of body part, bimanual imitation, asymmetry, and body midline crossing.

Cognitive neuropsychology
2022

The first report of a known 4A syndrome patient with suspected manifestations of COVID-19, what was the final outcome?

Heliyon
2022

Visual Dysfunction in Posterior Cortical Atrophy: A Masquerade.

Cureus
2022

Allochiria for spatial landmarks as the presenting feature of posterior cortical atrophy.

Cortex; a journal devoted to the study of the nervous system and behavior
2022

Research on Alzheimer's syndromes is critical to improve diagnosis, patient management and non-pharmacological treatments, but is under-pursued.

Frontiers in aging neuroscience
2022

Perspectives and a Systematic Scoping Review on Longitudinal Profiles of Posterior Cortical Atrophy Syndrome.

Current neurology and neuroscience reports
2023

Anterior dorsal attention network tau drives visual attention deficits in posterior cortical atrophy.

Brain : a journal of neurology
2022

Missing apolipoprotein E ε4 allele associated with nonamnestic Alzheimer's disease in a Tunisian population.

Journal of genetics
2022

Temporal Cortical Thickness and Cognitive Associations among Typical and Atypical Phenotypes of Alzheimer's Disease.

Journal of Alzheimer's disease reports
2022

Better conversations: a language and communication intervention for aphasia in posterior cortical atrophy.

Neurocase
2022

CSF Aβ40 and P-Tau181 Might Differentiate Atypical from Typical AD Phenotypes: Preliminary Evidence.

Neuro-degenerative diseases
2023

Dementia in Convolutional Neural Networks: Using Deep Learning Models to Simulate Neurodegeneration of the Visual System.

Neuroinformatics
2022

Neural correlates of episodic memory in adults with Down syndrome and Alzheimer's disease.

Alzheimer's research &amp; therapy
2022

Clinical Reasoning: An 80-Year-Old Woman With a Homonymous Hemianopsia.

Neurology
2022

Distinct brain iron profiles associated with logopenic progressive aphasia and posterior cortical atrophy.

NeuroImage. Clinical
2022

Atypical Alzheimer's disease phenotypes with normal or borderline PET biomarker profiles.

Journal of neurology
2023

Posterior cortical atrophy.

Journal of osteopathic medicine
2023

A Case of Atypical Alzheimer's Disease With Clinical Manifestation That Straddled the Boundary Between Primary Progressive Aphasia and Posterior Cortical Atrophy.

The neurologist
2022

Regional white matter hyperintensities in posterior cortical atrophy and logopenic progressive aphasia.

Neurobiology of aging
2023

Seeing the Woods for the Trees? Applying Diagnostic Criteria for Dementia with Lewy Bodies to Patients Presenting with Posterior Cortical Atrophy.

Alzheimer disease and associated disorders
2022

Clinical and metabolic imaging features of late-onset and early-onset posterior cortical atrophy.

European journal of neurology
2022

Development of the Colorado posterior cortical questionnaire within an Alzheimer's disease study cohort.

Journal of clinical and experimental neuropsychology
2022

Verbal Encoding Deficits Impact Recognition Memory in Atypical "Non-Amnestic" Alzheimer's Disease.

Brain sciences
2022

The Development of Videoconference-Based Support for People Living With Rare Dementias and Their Carers: Protocol for a 3-Phase Support Group Evaluation.

JMIR research protocols
2023

APOE ε4 influences medial temporal atrophy and tau deposition in atypical Alzheimer's disease.

Alzheimer's &amp; dementia : the journal of the Alzheimer's Association
2022

Atypical Alzheimer Disease Variants.

Continuum (Minneapolis, Minn.)
2022

Is the pathology of posterior cortical atrophy clinically predictable?

Reviews in the neurosciences
2022

Clinical Screening for Posterior Cortical Atrophy.

Cognitive and behavioral neurology : official journal of the Society for Behavioral and Cognitive Neurology
2022

Primary and Secondary Progressive Aphasia in Posterior Cortical Atrophy.

Life (Basel, Switzerland)
2022

Characterizing Differences in Functional Connectivity Between Posterior Cortical Atrophy and Semantic Dementia by Seed-Based Approach.

Frontiers in aging neuroscience
2022

Religious conversion in an older male with longstanding epilepsy.

Epilepsy &amp; behavior reports
2022

Investigating Heterogeneity and Neuroanatomic Correlates of Longitudinal Clinical Decline in Atypical Alzheimer Disease.

Neurology
2022

"Occipital Pole" Sign on 18 F-FDG PET for Dementia With Lewy Bodies and Posterior Cortical Atrophy : Evidence From the Treviso Dementia (TREDEM) Registry.

Clinical nuclear medicine
2022

Tau and the fractionated default mode network in atypical Alzheimer's disease.

Brain communications
2022

The clinical approach to the identification of higher-order visual dysfunction in neurodegenerative disease.

Current neurology and neuroscience reports
2022

Posterior cortical atrophy: Primary occipital variant.

European journal of neurology
2022

Causal Evidence for the Multiple Demand Network in Change Detection: Auditory Mismatch Magnetoencephalography across Focal Neurodegenerative Diseases.

The Journal of neuroscience : the official journal of the Society for Neuroscience
2022

Imaging Clinical Subtypes and Associated Brain Networks in Alzheimer's Disease.

Brain sciences
2022

Tracking the clinical progression of posterior cortical atrophy: implications for post-diagnostic and therapeutic interventions.

Journal of neurology, neurosurgery, and psychiatry
2022

Medial Temporal Atrophy in Posterior Cortical Atrophy and Its Relationship to the Cingulate Island Sign.

Journal of Alzheimer's disease : JAD
2022

MRI findings suggestive of Alzheimer's disease in patients with primary open angle glaucoma - a single sequence analysis using rapid 3D T1 spoiled gradient echo.

The British journal of radiology
2021

Recent Insights and Multifactorial Applications of Carbon Nanotubes.

Micromachines
2021

What's "up"? Impaired Spatial Preposition Processing in Posterior Cortical Atrophy.

Frontiers in human neuroscience
2021

Modeling Neurodegeneration in silico With Deep Learning.

Frontiers in neuroinformatics
2022

Altered visual and haptic verticality perception in posterior cortical atrophy and Alzheimer's disease.

The Journal of physiology
2022

Homonymous hemianopia as the presenting sign of posterior cortical atrophy.

Practical neurology
2021

Posterior cortical atrophy phenotypic heterogeneity revealed by decoding 18F-FDG-PET.

Brain communications
2022

Brain structural and functional anomalies associated with simultanagnosia in patients with posterior cortical atrophy.

Brain imaging and behavior
2022

Case Study 1: A 55-Year-Old Woman With Progressive Cognitive, Perceptual, and Motor Impairments.

The Journal of neuropsychiatry and clinical neurosciences
2021

Breakdown of specific functional brain networks in clinical variants of Alzheimer's disease.

Ageing research reviews
2021

Relationship of APOE, age at onset, amyloid and clinical phenotype in Alzheimer disease.

Neurobiology of aging
2022

Rates of longitudinal change in 18 F-flortaucipir PET vary by brain region, cognitive impairment, and age in atypical Alzheimer's disease.

Alzheimer's &amp; dementia : the journal of the Alzheimer's Association
2021

When it is not primary progressive aphasia: A scoping review of spoken language impairment in other neurodegenerative dementias.

Alzheimer's &amp; dementia (New York, N. Y.)
2021

Neuropsychological deficits in Posterior Cortical Atrophy and typical Alzheimer's disease: A meta-analytic review.

Cortex; a journal devoted to the study of the nervous system and behavior
2021

Retinal phenotyping of variants of Alzheimer's disease using ultra-widefield retinal images.

Alzheimer's &amp; dementia (Amsterdam, Netherlands)
Ver todos os 433 no EuropePMC

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Doenças relacionadas

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Referências e fontes

Bases de dados externas citadas neste artigo

Publicações científicas

Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.

  1. Disrupted brain topological network and its association with clinical features in posterior cortical atrophy.
    Brain communications· 2026· PMID 41809438mais citado
  2. Gray matter atrophy and structural connectivity in Posterior Cortical Atrophy: A voxel-based meta-analysis.
    Neuroscience and biobehavioral reviews· 2026· PMID 41616931mais citado
  3. Detecting Dementia Using Lexical Analysis: Terry Pratchett's Discworld Tells a More Personal Story.
    Brain sciences· 2026· PMID 41594815mais citado
  4. Alzheimer's Disease Continuum: Evaluating the Relationship between Fluid Biomarkers and Patients' Phenotype and Profile.
    Aging and disease· 2026· PMID 41619269mais citado
  5. How reproducible are data-driven subtypes of Alzheimer's disease atrophy?
    Journal of Alzheimer's disease : JAD· 2026· PMID 41603392mais citado
  6. Posterior Cortical Atrophy Presenting with Psychotic Delirium.
    Can J Neurol Sci· 2026· PMID 41969246recente
  7. Single-nucleus transcriptomics identifies a shared vulnerable excitatory neuronal population across typical and atypical Alzheimer's disease.
    bioRxiv· 2026· PMID 41959086recente
  8. Factors Associated With the Use of Digital Health and Well-Being Resources in Non-Memory-Led Dementias: Quantitative Survey Study.
    JMIR Aging· 2026· PMID 41911434recente
  9. Posterior Cortical Atrophy in the Asia-Pacific: A Report From the PCA Asian Workgroup.
    Ann Clin Transl Neurol· 2026· PMID 41885336recente
  10. Homonymous hemianopia in posterior cortical atrophy: right-left asymmetry, progression over time and relationship to the classical neuropsychological deficits.
    Front Neurol· 2026· PMID 41884339recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:54247(Orphanet)
  2. MONDO:0018899(MONDO)
  3. GARD:18846(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. Artigo Wikipedia(Wikipedia)
  6. Q2869816(Wikidata)

Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Atrofia cortical posterior
Compêndio · Raras BR

Atrofia cortical posterior

ORPHA:54247 · MONDO:0018899
Prevalência
Unknown
Herança
Unknown
CID-10
G31.1 · Degeneração cerebral senil, não classificadas em outra parte
CID-11
Ensaios
6 ativos
Início
Adult
Prevalência
0.0 (Worldwide)
MedGen
UMLS
C4275079
EuropePMC
Wikidata
Wikipedia
Papers 10a
DiscussaoAtiva

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