A afasia progressiva primária (APP) é um distúrbio neurodegenerativo, caracterizado por uma dissolução primária da linguagem, com relativa preservação de outras faculdades mentais durante pelo menos os primeiros 2 anos de doença. A PPA é reconhecida como a variante de linguagem no espectro de distúrbios da demência frontotemporal (DFT). A PPA pode ser classificada em 3 subtipos com base em características específicas de fala e linguagem: demência semântica (SD), afasia progressiva não fluente (PNFA) e afasia progressiva logopênica (lv-PPA).
Introdução
O que você precisa saber de cara
A afasia primária progressiva é uma condição neurodegenerativa que causa a perda gradual e constante da capacidade de falar e entender a linguagem. Nos primeiros dois anos da doença, outras áreas do pensamento e da memória costumam permanecer relativamente preservadas. A família geralmente percebe que a pessoa tem muita dificuldade em encontrar o nome de palavras comuns ou perde a fluência da comunicação. Ela não possui um Protocolo Clínico e Diretrizes Terapêuticas (PCDT) próprio nem remédios específicos dispensados pelo SUS nos registros oficiais. O acompanhamento deve ser realizado nos serviços neurológicos e multidisciplinares da rede de saúde.
A afasia progressiva primária (APP) é um distúrbio neurodegenerativo, caracterizado por uma dissolução primária da linguagem, com relativa preservação de outras faculdades mentais durante pelo menos os primeiros 2 anos de doença. A PPA é reconhecida como a variante de linguagem no espectro de distúrbios da demência frontotemporal (DFT). A PPA pode ser classificada em 3 subtipos com base em características específicas de fala e linguagem: demência semântica (SD), afasia progressiva não fluente (PNFA) e afasia progressiva logopênica (lv-PPA).
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Entender a doença
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 35 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 61 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Genes associados
7 genes identificados com associação a esta condição. Padrão de herança: Multigenic/multifactorial, Not applicable.
Polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy 2
An autosomal recessive disease characterized by presenile frontal dementia with leukoencephalopathy and basal ganglia calcification. In most cases the disorder first manifests in early adulthood as pain and swelling in ankles and feet, followed by bone fractures. Neurologic symptoms manifest in the fourth decade of life as a frontal lobe syndrome with loss of judgment, euphoria, and disinhibition. Progressive decline in other cognitive domains begins to develop at about the same time. The disorder culminates in a profound dementia and death by age 50 years.
Frontotemporal dementia 2
A form of dementia characterized by pathologic finding of frontotemporal lobar degeneration, presenile dementia with behavioral changes, deterioration of cognitive capacities and loss of memory. Gestural apraxia, parkinsonism, visual loss, and visual hallucinations are present in 25 to 40% of patients.
Frontotemporal dementia and/or amyotrophic lateral sclerosis 7
A neurodegenerative disorder characterized by frontotemporal dementia and/or amyotrophic lateral sclerosis in affected individuals. There is high intrafamilial variation. Frontotemporal dementia (FTD) is characterized by frontal and temporal lobe atrophy associated with neuronal loss, gliosis, and dementia. Patients exhibit progressive changes in social, behavioral, and/or language function. Amyotrophic lateral sclerosis (ALS) is characterized by the death of motor neurons in the brain, brainstem, and spinal cord, resulting in fatal paralysis. FTDALS7 is an autosomal dominant form characterized by onset of ALS or FTD in adulthood. A few patients may have both phenotypes.
Frontotemporal dementia 2
A form of dementia characterized by pathologic finding of frontotemporal lobar degeneration, presenile dementia with behavioral changes, deterioration of cognitive capacities and loss of memory. Gestural apraxia, parkinsonism, visual loss, and visual hallucinations are present in 25 to 40% of patients.
Inclusion body myopathy with early-onset Paget disease with or without frontotemporal dementia 1
An autosomal dominant disease characterized by disabling muscle weakness clinically resembling to limb girdle muscular dystrophy, osteolytic bone lesions consistent with Paget disease, and premature frontotemporal dementia. Clinical features show incomplete penetrance.
Alzheimer disease 3
A familial early-onset form of Alzheimer disease. Alzheimer disease is a neurodegenerative disorder characterized by progressive dementia, loss of cognitive abilities, and deposition of fibrillar amyloid proteins as intraneuronal neurofibrillary tangles, extracellular amyloid plaques and vascular amyloid deposits. The major constituents of these plaques are neurotoxic amyloid-beta protein 40 and amyloid-beta protein 42, that are produced by the proteolysis of the transmembrane APP protein. The cytotoxic C-terminal fragments (CTFs) and the caspase-cleaved products, such as C31, are also implicated in neuronal death.
Variantes genéticas (ClinVar)
259 variantes patogênicas registradas no ClinVar.
Classificação de variantes (ClinVar)
Distribuição de 2 variantes classificadas pelo ClinVar.
Vias biológicas (Reactome)
50 vias biológicas associadas aos genes desta condição.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Afasia primária progressiva
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Outros ensaios clínicos
81 ensaios clínicos encontrados, 34 ativos.
Publicações mais relevantes
Mostrando amostra de 200 publicações de um total de 1.604
Frontotemporal lobar degeneration complexity: atypical presentations and heterogeneous proteinopathies in five cases.
Frontotemporal lobar degeneration (FTLD) encompasses heterogeneous clinical syndrome within the frontotemporal spectrum, where clinicopathological associations may be misleading. This case series illustrates clinicopathological variability and mismatches. A retrospective case series was conducted within the brain donation program at the Golgi Cenci Foundation. Cases presenting at onset with a frontotemporal-spectrum phenotype, longitudinal clinical data, and post-mortem neuropathological characterization were included. Five cases (mean age at onset 65.4 years) were clinically diagnosed with major neurocognitive disorder due to frontotemporal dementia (FTD). Neuropathological examination revealed clinicopathological heterogeneity: two cases showed FTLD-TDP-A associated with GRN mutations, including a classic case and one with posterior (parieto-occipital) involvement; one non-fluent variant primary progressive aphasia (nfvPPA) case demonstrated FTLD-TDP-A with multiple co-pathologies; one semantic-variant-like case was driven by high Alzheimer's disease neuropathological changes; and one behavioral variant FTD-like case corresponded to frontal-variant Alzheimer's disease (fvAD) with extensive mixed pathology, including Lewy body disease, LATE-NC, and vascular pathology. Findings indicate that clinical phenotypes are more influenced by the anatomical distribution of pathology than by the specific molecular substrate. Frequent coexisting proteinopathies and asymmetric involvement contribute to phenotypic variability, reinforcing the role of neuropathological examination of both hemispheres for accurate clinicopathological correlations and definitive etiological diagnosis.
[A case of progressive supranuclear palsy with progressive non-fluent agrammatic variant primary progressive aphasia who could sing fluently].
The patient was a 65-year-old man. He visited the hospital complaining of gait disturbances and difficulty speaking. He was diagnosed with progressive supranuclear palsy because of slow vertical eye movements, frequent falls, and freezing gait; these symptoms were levodopa-resistant. The patient also had aphasia similar to non-fluent agrammatic variant of primary progressive aphasia, stuttering, and palilalia. However, he was able to sing a familiar song fluently. In contrast, for an unfamiliar song, he could not begin singing without the lyrics, and was more accurate at reading aloud than singing. Nonfluent aphasia with preserved singing ability has been reported in patients with stroke and trauma. However, this has rarely been reported for neurodegenerative diseases. This phenomenon may be due to the functional preservation of the right hemisphere and the left temporal lobe. The familiar song may have been stored in long-term memory as a set of lyrics and melody, and he may have produced it as automatic language.
Enhanced diagnostic interpretation of the MoCA using machine learning.
Artificial Intelligence (AI) is increasingly being integrated into clinical practice to optimize diagnosis in neurocognition. By capturing distinct cognitive signatures, this approach may offer a more precise alternative to the traditional interpretation of the Montreal Cognitive Assessment (MoCA) which often relies on a fixed cutoff score (26/30). We aimed to evaluate whether machine learning models, by integrating detailed MoCA subtest scores, demographic variables, and cognitive chart-derived metrics, can improve the detection of cognitive impairment and classification of dementia subtypes. We analyzed 38,746 clinical observations (17,188 unique individuals) from the National Alzheimer's Coordinating Center database. Five supervised learning algorithms, Extreme Gradient Boosting (XGBoost), Random Forest, Support Vector Machine (SVM), Logistic Regression, and k-Nearest Neighbors (KNN), were trained using detailed MoCA subtest scores, demographic variables, and cognitive chart-derived metrics as predictors. To ensure generalizability of results and prevent data leakage, we applied a rigorous nested Repeated Grouped Cross-Validation strategy. Decision thresholds were optimized via the Youden Index on independent calibration sets, and model interpretability was ensured through SHAP value analysis. Machine learning models consistently outperformed conventional approach. For the global detection of cognitive impairment, XGBoost achieved the best performance (Youden Index 0.61 vs. 0.54 for the standard cutoff). Regarding subtype classification, models demonstrated variable discriminative capacity depending on clinical homogeneity: primary progressive aphasia was best classified (Youden ≈ 0.77), followed by Lewy body dementia and Alzheimer's disease, while vascular dementia remained more challenging to isolate. Feature importance analysis highlighted the Cognitive Quotient as a robust universal predictor, while pinpointing disease-specific drivers such as delayed recall for Alzheimer's disease and verbal fluency for primary progressive aphasia. Our findings suggest interpretable machine learning enhances diagnostic utility of the MoCA, yielding superior accuracy compared to a fixed cutoff. By synthesizing individualized subtest profiles within a transparent framework, this approach offers a clinically actionable solution. It transforms the MoCA from a simple screening tool to a precision diagnostic aid, optimizing patient triage in the era of disease-modifying therapies.
White Matter Hyperintensities in Behavioral Variant Frontotemporal Dementia and Semantic Variant Primary Progressive Aphasia.
White matter hyperintensities (WMH) in patients with cerebrovascular risk factors (CVRF), are often linked to cerebral vascular changes, but can be caused by genetic variants selectively targeting white matter. In addition, WMH can be present in neurodegenerative disorders such as frontotemporal lobar degeneration (FTLD) and are linked to some FTLD genetic variants. This study aims to investigate WMH burden in patients with behavioral variant frontotemporal dementia (bvFTD) and semantic variant primary progressive aphasia (svPPA) versus controls and to evaluates the influence of CVRF. This cross-sectional retrospective analysis examined individuals meeting research diagnostic criteria for bvFTD and svPPA with high-quality structural MRI at the UCSF Memory and Aging Center between September 2008 and December 2021. WMH burden and spatial distribution were assessed by disease group compared to age- and sex-matched controls and associations with CVRF evaluated. We included 109 individuals with bvFTD [mean age (SD) 62.9 (8.6), 40% female], 47 with svPPA [mean (SD) age 65.4 (7.5), 51% female], and matched controls. After adjusting for age, apolipoprotein E4 (APOE-ε4) status and intracranial volume (ICV), both disease groups had higher WMH burden compared to controls (bvFTD, R 2 =0.184, p=0.001 and svPPA, R 2 =0.323, p=<0.001). Compared to controls, bvFTD group had more prevalent WMH in the frontal lobe (β=0.403 ; 95% CI 0.27 to 0.54 , p=<0.001), while those with svPPA had more prevalent WMH in the frontal (β=0.462 ; 95% CI 0.26 to 0.66, p <0.001), parietal (β=0.772 ; 95% CI 0.50 to 1.04, p <0.001), temporal (β=0.674 ; 95% CI 0.44 to 0.91, p <0.001), occipital lobes (β=0.364 ; 95% CI 0.14 to 0.59, p=0.002), and corpus callosum (β=0.342 ; 95% CI 0.13 to 0.55, p=0.002). In disease groups, WMH were not significantly associated with CVRF (F=0.468, df=2, p=0.641) suggesting a potential role of non-vascular mechanisms. We did not identify associations between the pathogenic C9orf72 hexanucleotide repeat expansions (HRE) and WMH in bvFTD patients. bvFTD and svPPA are associated with elevated WMH burden independent of CVRF. In bvFTD, WMH are primarily distributed within the frontal lobes, while svPPA shows widespread distribution across lobes. Study limitations include its retrospective, single-center design and limited power for genetic subgroup analyses.
Transcriptomic signature of frontotemporal lobar degeneration with TDP-43 type C pathology.
Semantic variant of primary progressive aphasia is a clinical subtype of frontotemporal lobar degeneration and is marked by TDP-43 subtype C pathology (FTLD-TDP C). It is a sporadic disease, yet has a strikingly homogeneous clinicopathological presentation, suggesting a common pathophysiology. The aim of this study was to discover dysregulated pathways in FTLD-TDP C through transcriptomics of the temporal cortex, its most affected region. Bulk RNA sequencing was conducted on temporal cortices of a post-mortem cohort of 18 FTLD-TDP C patients and 23 sex- and age-matched controls. Differential expression and functional analyses were run to detect differentially expressed genes with FDR<0.05 (DEG) and functionally annotate them. We assessed enrichment of TARDBP's protein interactors and RNA targets in DEG. Our findings were compared to other published RNA sequencing data of tauopathies (Alzheimer's dementia, progressive supranuclear palsy and FTLD with MAPT), FTLD-TDP (subtypes A&B) and available proteomics of this cohort. Furthermore, we performed weighted gene co-expression network analysis (WGCNA). We adjusted for differences in cell type composition between cases and controls using cell deconvolution, and removed genes dysregulated in temporal cortices of other datasets. In DEG of FTLD-TDP we focused on enrichment of synaptic processes using SynGO. We found upregulation of damage response, cell structure, RNA splicing processes and downregulation of synaptic processes in 6322 DEG and five disease-related WGCNA modules. TARDBP-related genes were enriched in DEG. Additionally, transmembrane transport across the neurovascular unit was dysregulated. After cell deconvolution and removal of common tau-genes, postsynaptic processes remained dysregulated, specifically gene ontology terms 'modulation of chemical synaptic transmission' and 'neurotransmitter receptor localisation to postsynaptic specialisation membrane'. We found eleven synaptic FTLD-TDP C-specific genes affected on both RNA- and protein-level in the temporal cortex, which were involved in synaptic adhesion (CADM1, NCAN), signal transmission (COMT, RGS144, SLC1A2, TUBB2B) and synaptic plasticity (BEGAIN, ITPKA, LRFN1, RAB3B, SYNPO). In conclusion, a wide range of processes were dysregulated on RNA-level in the temporal cortex of FTLD-TDP C, including commonly affected processes in neurodegeneration, such as structural cell alterations. Dysregulation of TARDBP-related genes and RNA splicing has also been observed in other TDP-43 proteinopathies. Importantly, we found that postsynaptic processes were downregulated in FTLD-TDP C, after removing tauopathy-related genes and after cell deconvolution. In particular, assembly of receptors at the postsynaptic membrane and synaptic signal transmission were affected, both on RNA and protein level. Future research on these pathways could elucidate distinct pathophysiological mechanisms and guide targeted clinical approaches.
Publicações recentes
Atypical Initial Manifestation of Sporadic Creutzfeldt-Jakob Disease as Progressive Aphasia and Palinopsia: A Case Report.
An acetylated Tau-174 CSF biomarker discriminates between TDP-43 and tau pathology in patients with frontotemporal lobar degeneration.
The Progressive Aphasia Communication Toolkit (PACT): a strengths-based approach to multidomain evaluation for intervention.
Exploring the phenotypic spectrum of frontotemporal lobar degeneration.
Additive effect of patient anosognosia and theory of mind deficit on dementia caregiver distress.
📚 EuropePMC1.226 artigos no totalmostrando 198
Identifying neuropathologic disease in primary progressive aphasia using narrative speech.
Alzheimer's & dementia : the journal of the Alzheimer's AssociationOnline comprehension of verbal time reference in primary progressive aphasia: Evidence from eyetracking.
Journal of neurolinguisticsUnveiling discrepancies in depression detection among persons with dementia: A comparative analysis of caregiver and self-report.
Journal of Alzheimer's disease : JADFrontotemporal lobar degeneration complexity: atypical presentations and heterogeneous proteinopathies in five cases.
Frontiers in neuroscience[A case of progressive supranuclear palsy with progressive non-fluent agrammatic variant primary progressive aphasia who could sing fluently].
Rinsho shinkeigaku = Clinical neurologyImproving aphasia with daily sessions of tDCS: an open-label study coupled to high-density EEG.
Brain stimulationEnhanced diagnostic interpretation of the MoCA using machine learning.
Frontiers in neuroscienceWhite Matter Hyperintensities in Behavioral Variant Frontotemporal Dementia and Semantic Variant Primary Progressive Aphasia.
Neurology open accessEligibility for Anti-Amyloid-β Monoclonal Antibodies in Patients With Primary Progressive Aphasia due to Alzheimer's Disease in Japan.
Psychogeriatrics : the official journal of the Japanese Psychogeriatric SocietyTranscriptomic signature of frontotemporal lobar degeneration with TDP-43 type C pathology.
Brain : a journal of neurology'I Am No Longer Anxious When I Speak': Experiences of People with Primary Progressive Aphasia Taking Part in a Biographic-Narrative Therapy (Cope PPA).
Brain sciencesYour Mileage May Vary: Individuals with Primary Progressive Aphasia Differ Widely in Their Utilization of Congruent Prosodic and Visual Information During Sentence Comprehension.
Brain sciencesNeurostimulation with Naming Therapy for Primary Progressive Aphasia: A Pilot Study Targeting Transcranial Direct Current (tDCS) Stimulation for the Individual.
Brain sciencesIncidence and Survival Rates of Frontotemporal Lobar Degeneration: Population-Based Registry Study.
NeurologyAdvanced neuroimaging assessment of neurodegenerative dementia syndromes: A framework for comprehensive multimodal FDG-PET, MR-perfusion, and MR-diffusion analysis.
NeuroImage. ClinicalMixed primary progressive aphasia and alcohol use disorder: a case of detailed clinical phenotyping outperforming molecular imaging.
NeurocaseMorphometric features enhance phenotype discrimination in frontotemporal lobar degeneration.
Brain communicationsReal-world evaluation of Armstrong's criteria in corticobasal degeneration: Phenotypic overlap and diagnostic challenges.
Parkinsonism & related disordersRepetitive behaviors in syndromes associated with frontotemporal lobar degeneration.
Journal of Alzheimer's disease : JAD"Not That I've Become Exceptional, But I'm Able to Make Myself Understood Better": Impact of Speech and Language Therapy on Everyday Communication in People with Primary Progressive Aphasia and Their Carers.
Neurology and therapyA Single-Item Screening Tool for the Assessment of Hoarding: Preliminary Observations.
The Journal of neuropsychiatry and clinical neurosciencesMultimodal semantic knowledge of emotion concepts in frontotemporal dementia.
medRxiv : the preprint server for health sciencesGamma tACS as a novel treatment for primary progressive aphasia: A pilot case series of four cases.
Brain stimulationMixed Nonfluent/Agrammatic Primary Progressive Aphasia and Behavioral Variant Frontotemporal Dementia: A Case Report From Tanzania.
Alzheimer disease and associated disorders"Doing the Best I Can": Qualitative Outcomes and Participant Feedback From a Combined Communication and Counselling Treatment for Primary Progressive Aphasia.
International journal of language & communication disordersNoninvasive Brain Stimulation in Translational Cognitive Neuroscience-Applications in Aphasia and Beyond: 2025 H. Houston Merritt Award Lecture.
NeurologyTreatment Efficacy of Semantic Feature Analysis in Logopenic and Semantic Variants of Primary Progressive Aphasia.
Healthcare (Basel, Switzerland)Two Languages and One Aphasia: A Systematic Scoping Review of Primary Progressive Aphasia in Chinese Bilingual Speakers, and Implications for Diagnosis and Clinical Care.
Brain sciencesPatterns and Trajectories of Behavioral and Neuropsychiatric Symptoms in Frontotemporal Dementia and Primary Progressive Aphasia.
NeurologyBehavioral and Neuropsychiatric Symptoms in Patients With Frontotemporal Dementia and Primary Progressive Aphasia.
NeurologyPotential role of MRI to optimize clinical trial design for progressive supranuclear palsy and corticobasal degeneration.
The journal of prevention of Alzheimer's diseaseAutomatic Detection of Articulatory-Based Disfluencies in Primary Progressive Aphasia.
IEEE journal of selected topics in signal processingAutomated item-level measures of verbal fluency in semantic and logopenic primary progressive aphasia.
Alzheimer's & dementia : the journal of the Alzheimer's AssociationEffect of Transcranial Direct Current Stimulation on Motor Speech in Nonfluent Primary Progressive Aphasia: A Case Report.
Case reports in neurologyDepression and apathy in frontotemporal dementia: a short assessment of facts and outlook.
Journal of neural transmission (Vienna, Austria : 1996)Understanding the multifaceted nature of quality of life in dementia using a transdiagnostic network analysis approach.
Aging & mental health[What are important goals in the treatment of people with primary progressive aphasia?].
Fortschritte der Neurologie-PsychiatrieLogopenic variant of primary progressive aphasia in a bilingual non-Alzheimer's disease octogenarian.
Dementia & neuropsychologiaSubjective time perception in dementia: a behavioural and neuroanatomical analysis.
Brain communicationsBaseline Functional Connectivity Predicts Who Will Benefit From Neuromodulation: Evidence From Primary Progressive Aphasia.
Neurorehabilitation and neural repairEffectiveness of a teletherapy-based phonological short-term memory training in reducing phonological impairments in the logopenic variant of primary progressive aphasia: a multiple case study.
Frontiers in human neuroscienceApplicability and Correlates of a Symptom-Led Staging System for Primary Progressive Aphasia.
European journal of neurologyRethinking the diagnosis of primary progressive aphasia: current challenges and future directions.
Frontiers in aging neuroscienceThe Progressive Aphasia Communication Toolkit (PACT): A Strengths-Based Approach to Multidomain Evaluation for Intervention.
medRxiv : the preprint server for health sciencesMULTI-OBJECT DATA INTEGRATION IN THE STUDY OF PRIMARY PROGRESSIVE APHASIA.
The annals of applied statisticsCognitive-linguistic skills in production of expository discourse: Insights from longitudinal changes and neural correlates in primary progressive aphasia.
Cortex; a journal devoted to the study of the nervous system and behaviorPeripheral inflammation in a Canadian cohort of neurodegenerative conditions: Occurrence, determinants, and impact.
Journal of Alzheimer's disease : JADPutative mitochondrial components of frontotemporal lobar degeneration: topological correlations between mitochondrial density and atrophy in FTLD/FTD phenotypes.
Journal of neurologyFrontotemporal dementia characterization using neurite orientation dispersion and density imaging.
Brain communicationsComputed tomography-based nnU-Net for region-specific brain structural changes across the alzheimer's continuum and frontotemporal dementia subtypes.
Scientific reportsClassifying and Monitoring Primary Progressive Aphasia in the Greek Population: A "Mini Linguistic State Examination (MLSE)" Tool.
Medicina (Kaunas, Lithuania)Targeting Executive Function and Language Impairments with tACS Combined with Behavioral Intervention in Primary Progressive Aphasia: A Case-Series, Pilot Investigation.
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Brain sciencesSpeaking in Tones: The role of lexical tones in Chinese-speaking Primary Progressive Aphasia.
medRxiv : the preprint server for health sciencesAgentic Generative Artificial Intelligence System for Classification of Pathology-Confirmed Primary Progressive Aphasia Variants.
medRxiv : the preprint server for health sciencesThe influence of bilingualism on the assessment and treatment of an Italian-English speaker with the logopenic variant of PPA.
Research squareFunctional network collapse in neurodegenerative disease.
Nature communicationsPredictors of Spelling and Reading Performance in Logopenic Variant Primary Progressive Aphasia.
Journal of speech, language, and hearing research : JSLHRSocial cognition in the nonfluent and logopenic variants of primary progressive aphasia: A review of variant-specific profiles.
Cortex; a journal devoted to the study of the nervous system and behaviorThe use of discourse particles in oral picture description by individuals with primary progressive aphasia.
Cortex; a journal devoted to the study of the nervous system and behaviorPatterns of cortical atrophy are associated with specific cognitive domains in patients with logopenic primary progressive aphasia.
Journal of Alzheimer's disease : JADAnterior temporal lobe, word comprehension, and physiology of atrophy in semantic primary progressive aphasia.
NeurocaseThe genetic landscape of frontotemporal lobar degeneration: investigation of a diagnostic cohort of 2747 probands.
Brain : a journal of neurologyA multilingual assessment approach in aphasia: Evidence from three Spanish-English speakers with primary progressive aphasia.
Clinical linguistics & phoneticsLanguage and psychiatric symptom overlap in FTD: an SLP perspective.
International review of psychiatry (Abingdon, England)Dysgraphia in Japanese patients with primary progressive aphasia.
Brain and languageDistinguishing Among Variants of Primary Progressive Aphasia with a Brief Multimodal Test of Nouns and Verbs.
Brain sciencesThe emotional journey through the stages of primary progressive aphasia: seven co-produced care pathway recommendations for clinical practice.
Brain impairment : a multidisciplinary journal of the Australian Society for the Study of Brain ImpairmentPsycholinguistic Variables and Spelling Accuracy for People with Logopenic Variant Primary Progressive Aphasia: A Cross-Sectional Study.
AphasiologyFace and Content Validation of the 10-Item Communicative Participation Item Bank General Short Form for Primary Progressive Aphasia: A Cognitive Interviewing Study.
American journal of speech-language pathologyNeuroimaging of vacuolar tauopathy: Response to letter.
Alzheimer's & dementia : the journal of the Alzheimer's AssociationMultidimensional cognitive deficits in the typical and atypical variants of Alzheimer's disease.
Alzheimer's research & therapyClinical Manifestations and Neural Basis of Semantic Dementia: Converging Evidences From Brain Imaging Studies.
Clinical interventions in agingVerbal learning in logopenic variant Primary Progressive Aphasia: An EEG investigation.
Neurobiology of agingTBK1 mutation and its role in frontotemporal dementia and amyotrophic lateral sclerosis in Brazilian families.
Dementia & neuropsychologiaLeveraging language and cognitive data for PPA subtyping: A systematic review of AI-based approaches.
Progress in neuro-psychopharmacology & biological psychiatryRegional CSF volume quantification using deep learning for comparative analysis of brain atrophy in frontotemporal dementia subtypes.
Frontiers in aging neuroscienceAtypical Semantic Variant Primary Progressive Aphasia With Right Temporal Atrophy in an Ambidextrous Older Adult.
Geriatrics & gerontology internationalHigh-definition brain stimulation targeting separate regions leads to differential word retrieval outcomes in patients with primary progressive aphasia: a pilot study.
Frontiers in neurologyAtrophy progression in frontotemporal lobar degeneration-TDP-C with primary progressive aphasia.
Brain : a journal of neurologyDisentangling phonology from phonological short-term memory in Alzheimer's disease phenotypes.
Alzheimer's research & therapyAdaptation of Better Conversations with Primary Progressive Aphasia to Norwegian.
Brain sciencesOptical coherence tomography as a potential biomarker for the logopenic variant of primary progressive aphasia: A cross-sectional prospective study.
Alzheimer's & dementia (Amsterdam, Netherlands)Leveraging Thematic Relationships Between Verbs and Nouns for Noun Retrieval Treatment in Logopenic Primary Progressive Aphasia: A Case Series.
American journal of speech-language pathologyEveryday functioning in young onset dementia: differences between diagnostic groups.
Alzheimer's & dementia : the journal of the Alzheimer's AssociationThe presymptomatic and early manifestations of semantic dementia.
Brain : a journal of neurologyWritten picture descriptions distinguish variants of primary progressive aphasia.
Journal of Alzheimer's disease : JADPrimary Progressive Aphasia Treatment: Current Treatment Options in Neurology Article Topic: Management of Primary Progressive Aphasia.
Current treatment options in neurologyInfluence of Speech and Language Therapy on Quality of Life in People With Primary Progressive Aphasia: A Scoping Review.
International journal of language & communication disordersEffects of Cognitive Demand and Imaginability on Semantic Cognition in Patients with Primary Progressive Aphasia.
Current Alzheimer researchAcoustic Analysis of Apraxia of Speech in a Japanese Patient With Nonfluent/Agrammatic Variant Primary Progressive Aphasia: A Case Study.
CureusNeurodevelopment and neural environment inform Alzheimer's disease age at onset and phenotype.
Alzheimer's & dementia : the journal of the Alzheimer's AssociationSemi-spontaneous language production in Dutch-speaking individuals with primary progressive aphasia.
Cognitive neuropsychologyAcoustically Altered Speech Perception in Frontotemporal Dementia Syndromes.
NeurologyIncidence and Prevalence of Frontotemporal Dementia: A Systematic Review and Meta-Analysis.
JAMA neurologyDistinctive Progression Patterns of Brain Structural Damage Aid Classification of Frontotemporal Dementia Variants.
European journal of neurologyProtocol for a multisite study on the efficacy of transcranial direct current stimulation as an adjuvant to naming and spelling therapy in the treatment of oral and written naming in individuals with primary progressive aphasia.
Frontiers in human neuroscienceBetter Living with Non-memory-led Dementia: study protocol for a randomised controlled trial of a web-based caregiver educational programme (BELIDE trial).
BMJ openA multi-modal approach for the treatment of non-fluent/agrammatic variant of Primary Progressive Aphasia.
Brain communicationsAphasia Leading to the Diagnosis of Myotonic Dystrophy Type 1: A Case Report.
CureusCriminal minds in dementia: A systematic review and quantitative meta-analysis.
Translational psychiatryEfficacy of Transcranial Magnetic Stimulation and Transcranial Direct-Current Stimulation in Primary Progressive Aphasia Treatment: A Review.
Brain sciencesA spouse's perspective on communication breakdowns and supportive strategies for semantic variant primary progressive aphasia.
AphasiologyPicture Description and Functional Communication Rating Correlates in Variants of Primary Progressive Aphasia.
AphasiologyStriatal dopaminergic patterns and clinical features in frontotemporal dementia.
Brain communicationsEfficacy of Transcranial Direct Current Stimulation in Patients with Primary Progressive Aphasia: A Systematic Review and Updated Meta-analysis.
Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical NeurophysiologyParticipatory Development of a Speech-Language Telerehabilitation Intervention Combined With Home-Based Transcranial Direct Current Stimulation for Primary Progressive Aphasia: A Qualitative Study.
American journal of speech-language pathologyCo-morbid seizures in frontotemporal dementia: What do they tell us?
Epilepsy researchLongitudinal evaluation of common and unique brain-networks in variants of primary progressive aphasia.
Alzheimer's research & therapyConnectivity as a universal predictor of tau progression in atypical Alzheimer's disease.
Brain : a journal of neurologyValence as principal dimension of the semantic space in primary progressive aphasia semantic variant.
Brain communicationsApplication of machine learning and temporal response function modeling of EEG data for differential diagnosis in primary progressive aphasia.
Scientific reportsLong-Term Therapy With Transcranial Magnetic Stimulation in Primary Progressive Aphasia: A Randomized Clinical Trial.
JAMA network openHow artificial intelligence is shaping neuropsychology: A focus on cognitive assessment of neurodegenerative disorders.
Journal of neuropsychologyUnderstanding Indirect Speech in Frontotemporal Dementia and Alzheimer's Disease Dementia: Validation of the Hinting Task - Dutch Version (HT-NL).
Journal of the International Neuropsychological Society : JINSSpatial and Temporal Progression of Neurodegeneration in Confirmed and Suspected TDP-43 Type C Pathology.
Imaging neuroscience (Cambridge, Mass.)Neurodevelopmental Vulnerability in Alzheimer's Disease and Frontotemporal Dementia.
European journal of neurologyBeyond language: empathy and emotion recognition deficits in primary progressive aphasias.
NeuroImage. ClinicalFrontal Variant Alzheimer's Disease or Primary Psychiatric Disorder? A Case Report.
Reports (MDPI)Feasibility of Home-Based Transcranial Direct Current Stimulation with Telerehabilitation in Primary Progressive Aphasia-A Case Series.
Brain sciencesCoping With Primary Progressive Aphasia: Factors Predicting Caregiver Psychological Wellbeing and Burden.
International journal of language & communication disordersComprehensive intervention combining group and personalized language therapy in primary progressive aphasia: Quantitative and qualitative findings.
Alzheimer's & dementia (New York, N. Y.)Clinical Translation of Integrated PET-MRI for Neurodegenerative Disease.
Journal of magnetic resonance imaging : JMRIClinicopathological characterization of vacuolar tauopathy associated with VCP D395G.
Alzheimer's & dementia : the journal of the Alzheimer's AssociationModulating language and executive functions in bilingual aphasia with cerebellar tDCS: a case series.
Brain and languageDelayed progressive apraxia of speech: A novel clinical entity distinct from primary progressive aphasia - A descriptive case series.
Journal of the neurological sciencesThe role of structural and functional parameters in designing pathology-specific tDCS protocols for primary progressive aphasia.
Alzheimer's research & therapyNetwork changes associated with right anterior temporal lobe atrophy: insight into unique symptoms.
Brain communicationsCommunication Strategies Used in Primary Progressive Aphasia: A Scoping Review.
Dementia (London, England)Advances in Neuromodulation Techniques for Aphasia Rehabilitation: A Comprehensive Review.
Medical science monitor : international medical journal of experimental and clinical researchMultiparametric MRI-based biomarkers in the non-fluent and semantic variants of primary progressive aphasia.
Journal of neurologyMapping Primary Progressive Aphasia Best Practices to a Person-Centered Video Biopic.
American journal of speech-language pathologySurvival rates in frontotemporal dementia and Alzheimer's disease.
Neurodegenerative disease managementValidation of criteria for frontotemporal dementia with right anterior temporal lobe predominance.
Alzheimer's & dementia : the journal of the Alzheimer's AssociationInterpreting Addenbrooke's Cognitive Examination-III Scores in Dementia: Performance Distributions and Clinically Meaningful Change.
European journal of neurologyA Case Study of a Multilingual Individual with Primary Progressive Aphasia: Diagnostic Considerations and Implications for Language Representation.
Archives of clinical neuropsychology : the official journal of the National Academy of NeuropsychologistsLet's Chat About Spoken Discourse: A Tutorial to Support Use of Spoken Discourse Analysis When Providing Aphasia Clinical Services.
American journal of speech-language pathologyProfiles of Vulnerability to Financial Exploitation in the Degenerative Dementias.
The American journal of geriatric psychiatry : official journal of the American Association for Geriatric PsychiatryLinguistic effects of transcranial Direct Current Stimulation (tDCS) in patients with primary progressive aphasia: A systematic review and meta-analysis of randomised controlled trials.
Neuroscience and biobehavioral reviews"Assessing relationship between blood MAPT methylation levels and clinical expression in Frontotemporal Dementia".
Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical NeurophysiologyDecline in activities of daily living in the rarer dementias.
General psychiatryThe neural basis of frontotemporal dementia (FTD): insights from ALE meta-analyses of four FTD subtypes encompassing 8,057 patients.
medRxiv : the preprint server for health sciencesMultiplex connectomics reveal altered networks in frontotemporal dementia: A multisite study.
Network neuroscience (Cambridge, Mass.)Comparing Two Connected Speech Tasks in Greek Speakers With the Logopenic Variant of Primary Progressive Aphasia and Alzheimer's Disease.
American journal of speech-language pathologyThe Pattern of Phonological, Semantic, and Circumlocution Naming Errors for Nouns and Verbs in Primary Progressive Aphasia.
Aphasiology[Primary progressive aphasia in the neurologist practice].
Zhurnal nevrologii i psikhiatrii imeni S.S. Korsakova[Phenotypes and epidemiology of frontotemporal dementia in Iceland].
Laeknabladid'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 & mental healthSafety and Efficacy of Different Therapeutic Interventions for Primary Progressive Aphasia: A Systematic Review.
Journal of clinical medicineTranscranial direct current stimulation over the temporal-parietal junction yields no lexical-semantic effects in logopenic primary progressive aphasia: a double-blind sham-controlled study.
NeuroImage. ClinicalNavigating the diagnosis and treatment of primary progressive aphasia: Lived experience of a rural patient.
Dementia (London, England)Cognitive stimulation in non-memory led dementias: A scoping review.
Dementia (London, England)ANXA11 Mutations in the FTD Spectrum: A Novel Finding in a Patient With Semantic Variant Primary Progressive Aphasia.
European journal of neurologyKnowledge, attitude, and practice of neurologists toward primary progressive aphasia in Indonesia.
Journal of Alzheimer's disease : JADPotential scalp acupuncture and brain stimulation targets for common neurological disorders: evidence from neuroimaging studies.
Chinese medicineThe cerebellum in frontotemporal dementia: From neglected bystander to potential neuromodulatory target. A narrative review.
Neuroscience and biobehavioral reviewsNation-wide Japanese FTD consortium FTLD-J: Utility of case review meetings.
International psychogeriatricsiSupport for rare dementias: a mixed-methods non-randomised feasibility study of an online self-help programme for carers.
Pilot and feasibility studiesRole of pause duration in primary progressive aphasia.
AphasiologySurvival Differences Between Individuals With Typical and Atypical Phenotypes of Alzheimer Disease.
NeurologyPartial volume effect correction impairs the diagnostic utility of [18F]-THK-5351 PET in nonfluent-agrammatic variant primary progressive aphasia.
NeuroImage. ClinicalDiagnosing neurodegenerative disorders using retina as an external window: A systematic review of OCT-MRI correlations.
Journal of Alzheimer's disease : JADTheory of mind deficits in non-fluent primary progressive aphasia.
Cortex; a journal devoted to the study of the nervous system and behaviorPrimary Tauopathy With Logopenic/Semantic Mixed Progressive Aphasia and Frontotemporal Dementia-like Behavior.
Cognitive and behavioral neurology : official journal of the Society for Behavioral and Cognitive NeurologyProgranulin deficiency in the brain: the interplay between neuronal and non-neuronal cells.
Translational neurodegenerationAddressing Phonological Deficit in Primary Progressive Aphasia With Behavioral Intervention and Transcranial Direct Current Stimulation.
Journal of speech, language, and hearing research : JSLHRThe relevance and challenges of cross-cultural studies in primary progressive aphasia.
Journal of the neurological sciencesConnected Speech Alterations and Progression in Patients With Primary Progressive Aphasia Variants.
NeurologyInsights into primary progressive aphasia.
Journal of neurologyCognitive Phenotyping and Interpretation of Alzheimer Blood Biomarkers.
JAMA neurologyUtilization of resting-state electroencephalography spectral power in convolutional neural networks for classification of primary progressive aphasia.
Neuroimage. ReportsNeuropsychiatric symptoms in sporadic Creutzfeldt-Jakob disease.
Brain : a journal of neurologyPrimary Progressive Aphasias: Diagnosis and Treatment.
Brain sciencesEfficacy of Communication Bridge-2 for primary progressive aphasia: A randomized controlled trial of communication intervention.
Alzheimer's & dementia : the journal of the Alzheimer's AssociationCase report: Behavioral variant FTD confounding a language variant FTD in a case of PSP-CBS.
Frontiers in dementiaThe review of the long-term health risks associated with vasectomy.
International journal of impotence researchCerebellar dysfunction in frontotemporal dementia: intra-cerebellar pathology and cerebellar network degeneration.
Journal of neurologyDistinct cerebral perfusion patterns and linguistic profiles in Alzheimer's disease-related primary progressive aphasia.
Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical NeurophysiologyUnveiling the enigmatic: Primary progressive apraxia of speech - A case report.
Clinical parkinsonism & related disordersComprehensive cross-sectional and longitudinal comparisons of plasma glial fibrillary acidic protein and neurofilament light across FTD spectrum disorders.
Molecular neurodegenerationHemispheric asymmetry in neurodegenerative diseases.
Handbook of clinical neurologyBehavioral and neural effects of temporoparietal high-definition transcranial direct current stimulation in logopenic variant primary progressive aphasia: a preliminary study.
Frontiers in psychologyBrain Perfusion, Atrophy, and Dopaminergic Changes in Amyloid Negative Logopenic Primary Progressive Aphasia.
Scientific reportsCognitive profiles in primary progressive aphasia variants: A cross-cultural Australian and Spanish investigation.
Journal of the neurological sciencesSemantic variant primary progressive aphasia with ANXA11 p.D40G.
Alzheimer's & dementia : the journal of the Alzheimer's AssociationA Regression Framework for Predicting Cognitive Decline in Frontotemporal Dementia using Recurrent Neural Networks.
Annual International Conference of the IEEE Engineering in Medicine and Biology Society. IEEE Engineering in Medicine and Biology Society. Annual International ConferenceArtificial Intelligence Based Hierarchical Classification of Frontotemporal Dementia.
Annual International Conference of the IEEE Engineering in Medicine and Biology Society. IEEE Engineering in Medicine and Biology Society. Annual International ConferenceValidation of the Cognitive-Emotional Perspective Taking test in patients with neurodegeneration.
Journal of Alzheimer's disease : JADFeasibility of home-based transcranial direct current stimulation combined with personalized word retrieval for improving naming in primary progressive aphasia.
Frontiers in neurologyThe Functional Origin of Oral Word Production Deficits in the Logopenic Variant of Primary Progressive Aphasia: A Systematic Review.
Brain sciencesEffects of single-session repetitive transcranial magnetic stimulation to identify the optimal brain target in primary progressive aphasia.
Journal of Alzheimer's disease : JADFrontotemporal dementia subtyping using machine learning, multivariate statistics and neuroimaging.
Brain communicationsThe use of low-density EEG for the classification of PPA and MCI.
Frontiers in human neuroscienceBehavioural changes in frontotemporal dementia and their cognitive and neuroanatomical correlates.
Brain : a journal of neurologyMulti-parametric [18F]PI-2620 tau PET/MRI for the phenotyping of different Alzheimer's disease variants.
European journal of nuclear medicine and molecular imagingPredicting survival rate by plasma biomarkers and clinical variables in syndromes associated with frontotemporal lobar degeneration.
Alzheimer's & dementia : the journal of the Alzheimer's AssociationThe Cortical Asymmetry Index for subtyping dementia patients.
European radiologyaFTLD-U presenting with primary progressive aphasia suggestive of non-fluent type with apraxia of speech.
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Doença com base genética
Um médico geneticista pode ajudar no diagnóstico de Afasia primária progressiva e no aconselhamento genético da família.
Doenças relacionadas
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Perguntas frequentes
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É um distúrbio neurodegenerativo caracterizado pelo declínio primário da linguagem com preservação relativa de outras funções mentais no início. Ela integra o espectro das demências frontotemporais e costuma iniciar na idade adulta.
Referências
Fontes citadas no texto, publicações do grafo e bases de dados usadas neste verbete
10 publicações do grafo RarasNet (PubMed) · 6 bases de dados. Títulos, periódicos e PMIDs vêm direto da fonte, sem intermediação de IA.
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.
Citar este verbete
Raras. (s.d.). Afasia primária progressiva. Em Raras — Enciclopédia de Doenças Raras do Brasil. https://raras.org/doenca/afasia-primaria-progressiva
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Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
Afasia primária progressiva
📋 Origem dos dados
Esta página agrega dados de fontes públicas e oficiais. Dados sobre cobertura no SUS (PCDT, CEAF) são verificados ativamente por agente proativo (ver badge no infobox). Demais dados têm atribuição de fonte + data da última sincronização — clique para abrir o original.
- Doença rara (ontologia)
- fonte: Orphanet
- Identificador unificado
- fonte: MONDO
- CID-11 (futuro)
- fonte: WHO ICD-11
- NIH/GARD
- fonte: GARD (NIH)
- Indexação biomédica
- fonte: MeSH (NLM)
- Dado público estruturado
- fonte: Wikidata
- Ensaios clínicos
- fonte: ClinicalTrials.gov
