A demência semântica (DS) é uma forma de demência frontotemporal (DFT), caracterizada pela perda progressiva, amodal e profunda do conhecimento semântico (combinação de agnosia visual associativa, anomia, dislexia superficial ou disgrafia e compreensão perturbada do significado das palavras) e anormalidades comportamentais, atribuíveis à degeneração dos lobos temporais anteriores.
Introdução
O que você precisa saber de cara
A demência semântica (DS) é uma forma de demência frontotemporal (DFT), caracterizada pela perda progressiva, amodal e profunda do conhecimento semântico (combinação de agnosia visual associativa, anomia, dislexia superficial ou disgrafia e compreensão perturbada do significado das palavras) e anormalidades comportamentais, atribuíveis à degeneração dos lobos temporais anteriores.
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 19 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 29 características clínicas mais associadas, ordenadas por frequência.
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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.
Probable core component of the endosomal sorting required for transport complex III (ESCRT-III) which is involved in multivesicular bodies (MVBs) formation and sorting of endosomal cargo proteins into MVBs. MVBs contain intraluminal vesicles (ILVs) that are generated by invagination and scission from the limiting membrane of the endosome and mostly are delivered to lysosomes enabling degradation of membrane proteins, such as stimulated growth factor receptors, lysosomal enzymes and lipids. The M
Cytoplasm, cytosolLate endosome membrane
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.
Promotes microtubule assembly and stability, and might be involved in the establishment and maintenance of neuronal polarity (PubMed:21985311). The C-terminus binds axonal microtubules while the N-terminus binds neural plasma membrane components, suggesting that tau functions as a linker protein between both (PubMed:21985311, PubMed:32961270). Axonal polarity is predetermined by TAU/MAPT localization (in the neuronal cell) in the domain of the cell body defined by the centrosome. The short isofo
Cytoplasm, cytosolCell membraneCytoplasm, cytoskeletonCell projection, axonCell projection, dendriteSecreted
Catalytic subunit of the gamma-secretase complex, an endoprotease complex that catalyzes the intramembrane cleavage of integral membrane proteins such as Notch receptors and APP (amyloid-beta precursor protein) (PubMed:10206644, PubMed:10545183, PubMed:10593990, PubMed:10811883, PubMed:10899933, PubMed:12679784, PubMed:12740439, PubMed:15274632, PubMed:20460383, PubMed:25043039, PubMed:26280335, PubMed:28269784, PubMed:30598546, PubMed:30630874). Requires the presence of the other members of the
Endoplasmic reticulumEndoplasmic reticulum membraneGolgi apparatus membraneCytoplasmic granuleCell membraneCell projection, growth coneEarly endosomeEarly endosome membraneCell projection, neuron projectionCell projection, axonSynapse
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.
Secreted protein that acts as a key regulator of lysosomal function and as a growth factor involved in inflammation, wound healing and cell proliferation (PubMed:12526812, PubMed:18378771, PubMed:28073925, PubMed:28453791, PubMed:28541286). Regulates protein trafficking to lysosomes, and also the activity of lysosomal enzymes (PubMed:28453791, PubMed:28541286). Also facilitates the acidification of lysosomes, causing degradation of mature CTSD by CTSB (PubMed:28073925). In addition, functions as
SecretedLysosome
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.
Acts as a guanine-nucleotide releasing factor (GEF) for Rab GTPases by promoting the conversion of inactive RAB-GDP to the active form RAB-GTP (PubMed:27103069, PubMed:27193190, PubMed:27617292, PubMed:28195531, PubMed:37821429). Acts as a GEF for RAB39A which enables HOPS-mediated autophagosome-lysosome membrane tethering and fusion in mammalian autophagy (PubMed:37821429). Component of the C9orf72-SMCR8 complex where both subunits display GEF activity and that regulates autophagy (PubMed:27103
CytoplasmNucleusCytoplasm, P-bodyCytoplasm, Stress granuleEndosomeLysosomeCytoplasmic vesicle, autophagosomeAutolysosomeSecretedCell projection, axonCell projection, growth conePerikaryonCell projection, dendritePresynapsePostsynapseNucleus membrane
Frontotemporal dementia and/or amyotrophic lateral sclerosis 1
An autosomal dominant neurodegenerative disorder characterized by adult onset of frontotemporal dementia and/or amyotrophic lateral sclerosis in an affected individual. There is high intrafamilial variation. Frontotemporal dementia 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 is characterized by the death of motor neurons in the brain, brainstem, and spinal cord, resulting in fatal paralysis.
Forms a receptor signaling complex with TYROBP which mediates signaling and cell activation following ligand binding (PubMed:10799849). Acts as a receptor for amyloid-beta protein 42, a cleavage product of the amyloid-beta precursor protein APP, and mediates its uptake and degradation by microglia (PubMed:27477018, PubMed:29518356). Binding to amyloid-beta 42 mediates microglial activation, proliferation, migration, apoptosis and expression of pro-inflammatory cytokines, such as IL6R and CCL3, a
Cell membraneSecreted
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.
In neurons, involved in the transport of late endosomes/lysosomes (PubMed:25066864). May be involved in dendrite morphogenesis and maintenance by regulating lysosomal trafficking (PubMed:25066864). May act as a molecular brake for retrograde transport of late endosomes/lysosomes, possibly via its interaction with MAP6 (By similarity). In motoneurons, may mediate the axonal transport of lysosomes and axonal sorting at the initial segment (By similarity). It remains unclear whether TMEM106B affect
Late endosome membraneLysosome membraneCell membrane
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.
Medicamentos e terapias
Mecanismo: Myeloperoxidase inhibitor
Variantes genéticas (ClinVar)
164 variantes patogênicas registradas no ClinVar.
Classificação de variantes (ClinVar)
Distribuição de 1 variantes classificadas pelo ClinVar.
Vias biológicas (Reactome)
31 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, progressiva primária, tipo fluente
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Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.
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Outros ensaios clínicos
419 ensaios clínicos encontrados, 3 ativos.
Publicações mais relevantes
Behavioural rigidity as a transdiagnostic marker of nucleus accumbens dysfunction in dementia.
Behavioural rigidity, the tendency to persist with inflexible patterns of thoughts or actions, is increasingly recognised as a transdiagnostic symptom across psychiatric, neurodevelopmental, and neurodegenerative disorders. Empirical studies exploring the prevalence and underlying neural mechanisms of behavioural rigidity in dementia, however, are lacking. This cross-sectional study sought to delineate the structural and functional neural correlates of behavioural rigidity using a transdiagnostic approach looking across the frontotemporal lobar degeneration (FTLD) spectrum and Alzheimer's disease. A total of 204 participants were recruited, including 110 frontotemporal dementia (FTD), 53 Alzheimer's disease (AD), and 41 healthy older control participants. Within the FTD group, 66 cases were diagnosed with clinically probable behavioural variant FTD (bvFTD), 26 presented with semantic dementia (SD), and 18 cases had progressive non-fluent aphasia (PNFA). Behavioural rigidity was assessed using the Stereotypical and Motor Behaviour subscale of the Cambridge Behavioural Inventory-Revised. Voxel-based morphometry (VBM) was performed to identify grey matter regions associated with behavioural rigidity transdiagnostically, the results of which informed subsequent seed-based voxel-wise functional connectivity analyses. All imaging analyses were adjusted for relevant demographic and technical covariates. Statistical thresholds were set at voxel-level p < 0.001 (uncorrected) and cluster-level p < 0.05 (FDR-corrected). Our main finding was that behavioural rigidity is pervasive across dementia subtypes, ranging from most pronounced in bvFTD to milder/relatively absent in PNFA, relative to Controls. Whole-brain VBM across the entire patient sample revealed a significant negative association between behavioural rigidity and grey matter intensity exclusively in the bilateral nucleus accumbens. Using the bilateral nucleus accumbens as seeds, resting-state functional connectivity analysis showed that higher levels of behavioural rigidity were associated with stronger connectivity between the left nucleus accumbens and the left supplementary motor area, paracentral lobule, and precuneus. This is the first study, to our knowledge, to examine the neural substrates of behavioural rigidity across FTLD syndromes transdiagnostically using structural and functional neuroimaging approaches. Our findings reveal a gradation of rigid and repetitive behaviours, most apparent in bvFTD, which in turn reflects pathological disruption of the nucleus accumbens. Taken together, our findings highlight the need to consider repetitive and rigid behaviours as a transdiagnostic feature in neurodegenerative disorders, and one which indexes underlying nucleus accumbens pathology. More broadly, this study underscores the importance of screening for rigid and repetitive behaviours in the clinic and identifies the nucleus accumbens as a promising neural target to ameliorate these symptoms.
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.
Fixel-based analysis reveals detailed white matter changes in semantic dementia.
Accurately characterizing white matter (WM) microstructure is critical for understanding neurodegenerative diseases such as semantic dementia (SD). Regionally constrained techniques like tract-based spatial statistics (TBSS) rely on diffusion-tensor imaging (DTI) and assume a single fiber population per voxel, limiting their sensitivity to complex architecture. Fixel-based analysis (FBA) overcomes these constraints by resolving multiple fiber populations (fixels) within a single voxel, enabling more anatomically specific assessment of WM organization. Multi-shell diffusion MRI from 16 semantic-variant PPA (svPPA) and 15 semantic-behavioral fronto-temporal dementia (sbvFTD) cases—with imaging-confirmed left- and right-predominant temporal atrophy, respectively—and 44 neurologically healthy controls were analyzed using both TBSS-DTI and whole-brain FBA. Fiber-specific metrics of fiber density and cross-section were evaluated alongside conventional DTI measures. Both methods confirmed damage to the anterior temporal lobe (ATL) connected tracts—the uncinate fasciculus, inferior longitudinal fasciculus, inferior fronto-occipital fasciculus, and temporal projections of the arcuate fasciculus. FBA further detected involvement of juxtacortical and other pathways that were not captured by TBSS, including the tapetum and anterior commissure, projections to the parahippocampal gyrus and amygdala, and longer-range parietal connections. By capturing multiple fiber populations within each voxel, FBA provides greater anatomical precision and sensitivity to micro- and macro-structural changes than TBSS. This fiber-specific framework enables a more comprehensive mapping of WM degeneration in SD and helps delineate early alterations in commissural and mesial-temporal pathways that may underlie disease spread and cognitive decline. The online version contains supplementary material available at 10.1007/s00429-025-03064-7.
Modeling contributions of cognition and apathy to functional impairment in younger-onset dementia.
Overlaps in symptom presentation limits the capacity to predict functional impairment and future care needs in younger-onset dementia syndromes. A general additive model (GAM) was applied to cross-sectional retrospective data from 375 participants with younger-onset dementia; 152 behavioral-variant frontotemporal dementia (bvFTD), 118 Alzheimer's disease (AD), 66 semantic dementia, and 39 progressive nonfluent aphasia (PNFA). This GAM aimed to explore the dynamic interrelationships between established measures of global cognition, apathy, and functional impairment. Our GAM significantly predicted functional impairment in all syndromes with a high explained variance (59.5%). Cognition and apathy emerged as significant predictors of functional impairment in each syndrome (p-values < .015). These relationships were consistently linear in AD, non-linear in SD, and mixed in bvFTD and PNFA (i.e., cognition linear and apathy non-linear). Our study shows the potential prognostic utility of GAMs for identifying syndrome-specific transition periods across group-level staging's of functional impairment. First study to apply a general additive model to functional impairment in younger-onset dementia.Studied 375 individuals with younger-onset Alzheimer's disease or frontotemporal dementia.Apathy and cognition were significant predictors of functional impairment in all syndromes.This modeling has significant implications for syndrome-specific prognosis and management.
Atrophy progression in frontotemporal lobar degeneration-TDP-C with primary progressive aphasia.
Clinicopathologic correlations in neurodegenerative diseases have led to novel insights on the neurobiology of selective vulnerability and the anatomy of cognitive networks. The neuropathologic entity of frontotemporal lobar degeneration with abnormal precipitates of the transactive response DNA binding protein TDP-43 (FTLD-TDP) of type C (TDP-C) is one of the most distinctive examples. TDP-C invariably starts with neurodegeneration (atrophy) confined to the temporopolar regions (TPR). The process is usually asymmetric, causing behavioral abnormalities and associative agnosia when predominantly right-sided, semantic Primary Progressive Aphasia (PPA) when left-sided, and semantic dementia when bilateral. In this study, we investigated the relationship between atrophy progression and the progressive dissolution of word comprehension in TDP-C patients with asymmetric left TPR degeneration. For the sake of homogeneity, and in order to use a common yardstick of functional progression, we focused on patients with leftward asymmetry and initially isolated verbal impairment. Using data from 24 visits with structural MRI scans and specialized tests of naming, word definition, and word-to-picture matching, we stratified the anatomy of peak degeneration sites according to three increasingly more advanced stages of impaired noun representations. According to this pattern of progression, an initial stage of relatively isolated anomia is followed by additional intra-category blurring of word meaning and, at still more advanced stages, inter-category blurring. Voxel Based Morphometry (VBM) maps of gray matter volume were binarized to identify regions most frequently atrophied at each of these stages. Results illustrate that the progressive dissolution of word meaning is associated with caudal progression of atrophy from the left temporopolar cortex at initial stages to more posterior fusiform, lateral temporal and temporooccipital regions in later stages. The stratification of progressive atrophy by precisely characterized stages of word comprehension impairment, rather than by estimated time of symptom onset, offers a functional rather than just chronological anatomy of progression for TDP-C and a clearer view of the relationship between TPR components and the cognitive mapping of word representations.
Publicações recentes
Eating behaviour profiles across the frontotemporal dementia spectrum.
Behavioural rigidity as a transdiagnostic marker of nucleus accumbens dysfunction in dementia.
Transcriptomic signature of frontotemporal lobar degeneration with TDP-43 type C pathology.
Modeling contributions of cognition and apathy to functional impairment in younger-onset dementia.
Fixel-based analysis reveals detailed white matter changes in semantic dementia.
📚 EuropePMC479 artigos no totalmostrando 195
Behavioural rigidity as a transdiagnostic marker of nucleus accumbens dysfunction in dementia.
Brain : a journal of neurologyTranscriptomic signature of frontotemporal lobar degeneration with TDP-43 type C pathology.
Brain : a journal of neurologyModeling contributions of cognition and apathy to functional impairment in younger-onset dementia.
Alzheimer's & dementia (Amsterdam, Netherlands)Fixel-based analysis reveals detailed white matter changes in semantic dementia.
Brain structure & function[51-Year-old Patient With Word Finding Difficulties].
PraxisSudoku Preoccupation in Semantic Dementia.
Internal medicine (Tokyo, Japan)Clinical Manifestations and Neural Basis of Semantic Dementia: Converging Evidences From Brain Imaging Studies.
Clinical interventions in aging[Classification and Clinical Characteristics of Dementia].
No shinkei geka. Neurological surgeryAtrophy progression in frontotemporal lobar degeneration-TDP-C with primary progressive aphasia.
Brain : a journal of neurologyA Bayesian analysis of diagnostic timelines across Alzheimer's disease, frontotemporal dementia, and other neurodegenerative conditions.
Alzheimer's & dementia (Amsterdam, Netherlands)The presymptomatic and early manifestations of semantic dementia.
Brain : a journal of neurologyCognitive and neuroanatomical assessment of alexia and agraphia in Japanese: implications for the European languages.
Frontiers in human neuroscienceEffects of Cognitive Demand and Imaginability on Semantic Cognition in Patients with Primary Progressive Aphasia.
Current Alzheimer researchA Case of Late-Onset Semantic Dementia With Right-Predominant Temporal Lobe Atrophy due to Primary Tauopathy.
Psychogeriatrics : the official journal of the Japanese Psychogeriatric SocietyUnderstanding the mind-brain relationship through focal neurodegenerative pathology.
Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical NeurophysiologyThe effect of congruency on visual exploration and episodic memory performance in Alzheimer's disease and semantic dementia.
NeuropsychologiaUnderstanding Theory of Mind-pragmatics relationship through the spectrum of neurodegenerative diseases.
Philosophical transactions of the Royal Society of London. Series B, Biological sciencesMeaning for reading: the neurocognitive basis of semantic reading impairment after stroke.
Brain : a journal of neurologyProteomics of the temporal cortex in semantic dementia reveals brain-region specific molecular pathology and regulation of the TDP-43-ANXA11 interactome.
Acta neuropathologica communicationsVCP p.Arg191Gln mutation in a patient with semantic dementia: a case report.
NeurocaseLeft inferior temporal hemorrhage due to cerebral amyloid angiopathy mimicking semantic dementia.
Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical NeurophysiologySurvival 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 AssociationSelf- versus caregiver-reported apathy across neurological disorders.
Brain communicationsThe 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 sciencesThe role of disconnection mechanisms in category-specific semantic disorders for living beings. A critical review.
Cortex; a journal devoted to the study of the nervous system and behaviorLower water-soluble vitamins and higher homocysteine are associated with neurodegenerative diseases.
Scientific reportsDefective Awareness of Person-Recognition Disorders Through Face, Voice and Name in Right and Left Variants of Semantic Dementia: A Pilot Study.
Brain sciencesNation-wide Japanese FTD consortium FTLD-J: Utility of case review meetings.
International psychogeriatricsRegional cerebral blood flow correlated with aphasia in dementia of the Alzheimer's type and frontotemporal lobar degeneration.
Acta neurobiologiae experimentalisPrimary Progressive Aphasias: Diagnosis and Treatment.
Brain sciencesBehavioural and cognitive profiles in frontotemporal dementia and Alzheimer's disease: a longitudinal study.
Journal of neurologyThe causal association between resting state intrinsic functional networks and neurodegeneration.
Brain communicationsDouble dissociation between lexical decision and comprehension in a Japanese patient with semantic dementia: based on the characteristic processing of Kanji.
NeurocaseSemantic and pre-semantic defects of person recognition in semantic dementia. A commentary to the Belliard & Merck's paper: "Is semantic dementia an outdated entity? ".
Cortex; a journal devoted to the study of the nervous system and behaviorBehavioural changes in frontotemporal dementia and their cognitive and neuroanatomical correlates.
Brain : a journal of neurologyLong-Term Multimodal Exercise Intervention for Patients with Frontotemporal Lobar Degeneration: Feasibility and Preliminary Outcomes.
Dementia and geriatric cognitive disorders extraImpaired semantic control in the logopenic variant of primary progressive aphasia.
Brain communicationsSemantic knowledge of social norms in frontotemporal dementia patients with either predominant frontal or temporal lobe atrophy.
Applied neuropsychology. AdultLinking structural and functional changes during healthy aging and semantic dementia using multilayer brain network analysis.
Cortex; a journal devoted to the study of the nervous system and behaviorSemantic behavioral variant frontotemporal dementia and semantic dementia associated with TARDBP mutations.
Amyotrophic lateral sclerosis & frontotemporal degenerationLongitudinal changes in functional capacity in frontotemporal dementia and Alzheimer's disease.
Alzheimer's & dementia (Amsterdam, Netherlands)Resolving the problem of surface dyslexia in Italian through inflection of irregular verbs.
Journal of neuropsychologySocial-semantic knowledge in frontotemporal dementia and after anterior temporal lobe resection.
Brain communicationsPerceptual and semantic deficits in face recognition in semantic dementia.
NeuropsychologiaFrontotemporal Dementia Differential Diagnosis in Clinical Practice: A Single-Center Retrospective Review of Frontal Behavioral Referrals.
Neurology. Clinical practiceRelationship between hearing impairment and dementia and cognitive function: a Mendelian randomization study.
Alzheimer's research & therapyIs semantic dementia an outdated entity?
Cortex; a journal devoted to the study of the nervous system and behaviorDrawing as a means to characterize memory and cognition.
Memory & cognitionEstablishing and evaluating the gradient of item naming difficulty in post-stroke aphasia and semantic dementia.
Cortex; a journal devoted to the study of the nervous system and behaviorThe impact of bilateral versus unilateral anterior temporal lobe damage on face recognition, person knowledge and semantic memory.
Cerebral cortex (New York, N.Y. : 1991)Clinicopathologic and Neuroimaging Correlations of Nonverbal Oral Apraxia in Patients With Neurodegenerative Disease.
NeurologyFDG-PET in the diagnosis of primary progressive aphasia: a systematic review.
Annals of nuclear medicineDysphagia in primary progressive aphasia: Clinical predictors and neuroanatomical basis.
European journal of neurologyClinical recognition of frontotemporal dementia with right anterior temporal predominance: A multicenter retrospective cohort study.
Alzheimer's & dementia : the journal of the Alzheimer's AssociationA Critical Review of Noninvasive Brain Stimulation Technologies in Alzheimer's Dementia and Primary Progressive Aphasia.
Journal of Alzheimer's disease : JADAdvances in semantic dementia: Neuropsychology, pathology & neuroimaging.
Ageing research reviewsDrawing from name in semantic dementia reveals graded object knowledge representations in anterior temporal lobe.
Memory & cognitionProfiles of motivational impairment and their relationship to functional decline in frontotemporal dementia.
Journal of neurologySymptomatic progression of frontotemporal dementia with the TARDBP I383V variant.
NeurocaseHemispheric asymmetries in hippocampal volume related to memory in left and right temporal variants of frontotemporal degeneration.
Frontiers in neurologyRight versus left temporal lobe semiology in dementia: Lessons from two cases with focal frontotemporal dementia syndromes.
Journal of neuropsychologyReversal of the concreteness effect can be detected in the natural speech of older adults with amnestic, but not non-amnestic, mild cognitive impairment.
Alzheimer's & dementia (Amsterdam, Netherlands)Data-driven subtypes of mixed semantic-logopenic primary progressive aphasia: Linguistic features, biomarker profiles and brain metabolic patterns.
Journal of the neurological sciencesExamining the propensity and nature of criminal risk behaviours in frontotemporal dementia syndromes and Alzheimer's disease.
Alzheimer's & dementia (Amsterdam, Netherlands)Volumetric analysis of hippocampal subregions and subfields in left and right semantic dementia.
Brain communicationsWhite matter network underlying semantic processing: evidence from stroke patients.
Brain communications(What) can patients with semantic dementia learn?
NeuropsychologiaProsopagnosia: face blindness and its association with neurological disorders.
Brain communicationsA neuroanatomical and cognitive model of impaired social behaviour in frontotemporal dementia.
Brain : a journal of neurologyLongitudinal volumetric changes in amygdala subregions in frontotemporal dementia.
Journal of neurologyExploring graded profiles of hippocampal atrophy along the anterior-posterior axis in semantic dementia and Alzheimer's disease.
Neurobiology of agingStructural connectivity of cytoarchitectonically distinct human left temporal pole subregions: a diffusion MRI tractography study.
Frontiers in neuroanatomyTranscranial direct current stimulation in semantic variant of primary progressive aphasia: a state-of-the-art review.
Frontiers in human neuroscienceBreakdown of category-specific word representations in a brain-constrained neurocomputational model of semantic dementia.
Scientific reportsEstablishing the link between motivational disturbances and behavioural rigidity in frontotemporal dementia.
European journal of neurologySemantic dementia in Arabic: An assessment of Arabic word reading within sentences.
Applied neuropsychology. AdultPrimary progressive aphasia: six questions in search of an answer.
Journal of neurologyDiminished baseline autonomic outflow in semantic dementia relates to left-lateralized insula atrophy.
NeuroImage. ClinicalBehavioral Treatment for Speech and Language in Primary Progressive Aphasia and Primary Progressive Apraxia of Speech: A Systematic Review.
Neuropsychology reviewA heterozygous splicing variant IVS9-7A > T in intron 9 of the MAPT gene in a patient with right-temporal variant frontotemporal dementia with atypical 4 repeat tauopathy.
Acta neuropathologica communicationsSymptom-led staging for semantic and non-fluent/agrammatic variants of primary progressive aphasia.
Alzheimer's & dementia : the journal of the Alzheimer's AssociationGenetic and Neuroimaging Analysis of SIGMAR1 for Frontotemporal Dementia.
Journal of Alzheimer's disease : JADGlobular glial tauopathy presenting clinically as atypical parkinsonism with dementia: A clinicopathological case report.
Clinical parkinsonism & related disordersExpressive Prosody in Patients With Focal Anterior Temporal Neurodegeneration.
NeurologyHemispheric contributions toward interoception and emotion recognition in left-vs right-semantic dementia.
NeuropsychologiaConcrete and Abstract Concepts in Primary Progressive Aphasia and Alzheimer's Disease: A Scoping Review.
Brain sciencesFunctional and structural network changes related with cognition in semantic dementia longitudinally.
Human brain mappingIncreased oligomeric TDP-43 in the plasma of Korean frontotemporal dementia patients with semantic dementia.
Alzheimer's & dementia : the journal of the Alzheimer's AssociationFeasibility and Benefits of Intermittent Multiple tDCS Courses With Concomitant Language Training in Semantic Dementia: A Case Report.
Indian journal of psychological medicineDistinguishing Semantic Variant Primary Progressive Aphasia from Alzheimer's Disease.
Journal of Alzheimer's disease reportsWord production and comprehension in frontotemporal degeneration: A neurocognitive computational Pickian account.
Cortex; a journal devoted to the study of the nervous system and behaviorError profiles of facial emotion recognition in frontotemporal dementia and Alzheimer's disease.
International psychogeriatricsCommunication-related experiences of individuals in the early phase of semantic dementia and their families: an interview study.
Psychogeriatrics : the official journal of the Japanese Psychogeriatric SocietyThe architecture of abnormal reward behaviour in dementia: multimodal hedonic phenotypes and brain substrate.
Brain communicationsSemantic cognition in healthy ageing: Neural signatures of representation and control mechanisms in naming typical and atypical objects.
NeuropsychologiaAnalysis of positron emission tomography hypometabolic patterns and neuropsychiatric symptoms in patients with dementia syndromes.
CNS neuroscience & therapeuticsThe cognitive and behavioral correlates of functional status in patients with frontotemporal dementia: A pilot study.
Frontiers in human neuroscienceAccounting for word production, comprehension, and repetition in semantic dementia, Alzheimer's dementia, and mild cognitive impairment.
Brain and languageNeurofilament light oligomers in neurodegenerative diseases: quantification by homogeneous immunoassay in cerebrospinal fluid.
BMJ neurology openCognitive interventions for memory and psychological well-being in aging and dementias.
Frontiers in psychologyWhat semantic dementia tells us about the ability to infer others' communicative intentions.
The Behavioral and brain sciencesAnatomical MRI staging of frontotemporal dementia variants.
Alzheimer's & dementia : the journal of the Alzheimer's AssociationSemantic dementia: a complex and culturally influenced presentation.
BJPsych bulletinThe problematic syndrome of right temporal lobe atrophy: Unweaving the phenotypic rainbow.
Frontiers in neurologyMapping behavioural, cognitive and affective transdiagnostic dimensions in frontotemporal dementia.
Brain communicationsMore extensive hypometabolism and higher mortality risk in patients with right- than left-predominant neurodegeneration of the anterior temporal lobe.
Alzheimer's research & therapyMeaningless imitation in neurodegenerative diseases: Effects of body part, bimanual imitation, asymmetry, and body midline crossing.
Cognitive neuropsychologyAmyotrophic lateral sclerosis with primary progressive aphasia: a case report and literature review.
Neuro endocrinology lettersProteomics of the dentate gyrus reveals semantic dementia specific molecular pathology.
Acta neuropathologica communicationsThe Assessment of the Socioemotional Disorder in Neurodegenerative Diseases with the Revised Self-Monitoring Scale (RSMS).
Journal of clinical medicineCommentary on 'Somewhere out there in a place no one knows: Yoko Ogawa's The Memory Police and the literature of forgetting' by John Henning.
Medical humanities'Like going into a chocolate shop, blindfolded': What do people with primary progressive aphasia want from speech and language therapy?
International journal of language & communication disordersRemote versus face-to-face neuropsychological testing for dementia research: a comparative study in people with Alzheimer's disease, frontotemporal dementia and healthy older individuals.
BMJ openAltered reward processing underpins emotional apathy in dementia.
Cognitive, affective & behavioral neuroscienceClinicopathological diversity of semantic dementia: Comparisons of patients with early-onset versus late-onset, left-sided versus right-sided temporal atrophy, and TDP-type A versus type C pathology.
Neuropathology : official journal of the Japanese Society of NeuropathologyTemporopolar regions of the human brain.
Brain : a journal of neurologyChanging perspectives on frontotemporal dementia: A review.
Journal of neuropsychologyIndividual differences in theory of mind correlate with the occurrence of false memory: A study with the DRM task.
Quarterly journal of experimental psychology (2006)Damage to temporoparietal cortex is sufficient for impaired semantic control.
Cortex; a journal devoted to the study of the nervous system and behaviorPrimary progressive aphasia and motor neuron disease: A review.
Frontiers in aging neuroscienceAn examination of retrieval practice and production training in the treatment of lexical-semantic comprehension deficits in aphasia.
NeuropsychologyComparing Medial Temporal Atrophy Between Early-Onset Semantic Dementia and Early-Onset Alzheimer's Disease Using Voxel-Based Morphometry: A Multicenter MRI Study.
Current Alzheimer researchFrontotemporal dementia: A unique window on the functional role of the temporal lobes.
Handbook of clinical neurologyThe neural correlates of abstract and concrete words.
Handbook of clinical neurologyMusic appreciation phenotypes in patients with frontotemporal dementia: A pilot study.
International journal of geriatric psychiatryEnhanced positive emotional reactivity in frontotemporal dementia reflects left-lateralized atrophy in the temporal and frontal lobes.
Cortex; a journal devoted to the study of the nervous system and behavior"Split-day syndrome", a patient with frontotemporal dementia who lives two days in the span of one: a case report and review of articles.
NeurocaseRight Temporal Lobe Variant of Frontotemporal Dementia: Systematic Review.
Alzheimer disease and associated disordersOlfactory Bulb Integrity in Frontotemporal Dementia and Alzheimer's Disease.
Journal of Alzheimer's disease : JADRight temporal degeneration and socioemotional semantics: semantic behavioural variant frontotemporal dementia.
Brain : a journal of neurologyPlasma Oxytocin Is Not Associated with Social Cognition or Behavior in Frontotemporal Dementia and Alzheimer's Disease Syndromes.
Dementia and geriatric cognitive disordersPhonemic restoration in Alzheimer's disease and semantic dementia: a preliminary investigation.
Brain communicationsCharacterizing Differences in Functional Connectivity Between Posterior Cortical Atrophy and Semantic Dementia by Seed-Based Approach.
Frontiers in aging neurosciencePhysical understanding in neurodegenerative diseases.
Cognitive neuropsychologyNeuropathological fingerprints of survival, atrophy and language in primary progressive aphasia.
Brain : a journal of neurologyDopamine Transporter Imaging for Frontotemporal Lobar Degeneration With Motor Neuron Disease.
Frontiers in neuroscienceSpeech and Language Abilities of Persons with Frontotemporal Dementia.
Psychiatria DanubinaA presumptive association between obsessive compulsions and asymmetric temporal lobe atrophy: a case report.
Journal of medical case reportsCognitive and Neural Mechanisms of Social Communication Dysfunction in Primary Progressive Aphasia.
Brain sciences"Hard to Say, Hard to Understand, Hard to Live": Possible Associations between Neurologic Language Impairments and Suicide Risk.
Brain sciencesSemantic Variant Primary Progressive Aphasia: Practical Recommendations for Treatment from 20 Years of Behavioural Research.
Brain sciencesThe Free Association Task: Proposal of a Clinical Tool for Detecting Differential Profiles of Semantic Impairment in Semantic Dementia and Alzheimer's Disease.
Medicina (Kaunas, Lithuania)Two Rare Variants in PLAU and BACE1 Genes-Do They Contribute to Semantic Dementia Clinical Phenotype?
GenesNo negative impact of word retraining on vocabulary use or clarity of communication in semantic dementia.
Neuropsychological rehabilitationCharacteristics of behavioral symptoms in right-sided predominant semantic dementia and their impact on caregiver burden: a cross-sectional study.
Alzheimer's research & therapyImpaired emotion perception and categorization in semantic aphasia.
NeuropsychologiaConsidering Hemispheric Specialization in Emotional Face Processing: An Eye Tracking Study in Left- and Right-Lateralised Semantic Dementia.
Brain sciencesThe Role of Oxytocin in Social Circuits and Social Behavior in Dementia.
Methods in molecular biology (Clifton, N.J.)Anhedonia in Semantic Dementia-Exploring Right Hemispheric Contributions to the Loss of Pleasure.
Brain sciencesSupporting behaviour change in younger-onset dementia: mapping the needs of family carers in the community.
Aging & mental healthOn-Reading (Chinese-Style Pronunciation) Predominance Over Kun-Reading (Native Japanese Pronunciation) in Japanese Semantic Dementia.
Frontiers in human neuroscienceThe role of semantic memory in prospective memory and episodic future thinking: new insights from a case of semantic dementia.
Memory (Hove, England)Content Word Production during Discourse in Aphasia: Deficits in Word Quantity, Not Lexical-Semantic Complexity.
Journal of cognitive neuroscienceExamining the episodic-semantic interaction during future thinking - A reanalysis of external details.
Memory & cognitionNeuroimaging in Frontotemporal Dementia: Heterogeneity and Relationships with Underlying Neuropathology.
Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeuticsEpisodic-semantic interactions in spontaneous thought.
Memory & cognitionRight temporal variant frontotemporal dementia is pathologically heterogeneous: a case-series and a systematic review.
Acta neuropathologica communicationsPutative Alcohol-Related Dementia as an Early Manifestation of Right Temporal Variant of Frontotemporal Dementia.
Journal of Alzheimer's disease : JADHistory of Magnetic Resonance Imaging: A Trip Down Memory Lane.
NeuroscienceNeural dynamics of semantic categorization in semantic variant of primary progressive aphasia.
eLifeDifferent types of abstract concepts: evidence from two neurodegenerative patients.
NeurocaseEmotional Comparison Between Semantic Dementia and Alzheimer's Disease.
Frontiers in psychiatry"More than words" - Longitudinal linguistic changes in the works of a writer diagnosed with semantic dementia.
NeurocaseSemantic memory impairment in dementia: A cross-cultural adaptation study.
Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical NeurophysiologyUsing concept typicality to explore semantic representation and control in healthy ageing.
Cognitive processingUncovering the prevalence and neural substrates of anhedonia in frontotemporal dementia.
Brain : a journal of neurologyBeyond language impairment: Profiles of apathy in primary progressive aphasia.
Cortex; a journal devoted to the study of the nervous system and behaviorNeuroinflammation is highest in areas of disease progression in semantic dementia.
Brain : a journal of neurologyNeurobehavioral Characteristics of FDG-PET Defined Right-Dominant Semantic Dementia: A Longitudinal Study.
Dementia and geriatric cognitive disordersChanges in local brain function in mild cognitive impairment due to semantic dementia.
CNS neuroscience & therapeuticsGene Expression Imputation Across Multiple Tissue Types Provides Insight Into the Genetic Architecture of Frontotemporal Dementia and Its Clinical Subtypes.
Biological psychiatryDistinctive Oculomotor Behaviors in Alzheimer's Disease and Frontotemporal Dementia.
Frontiers in aging neuroscienceClinical features of behavioral symptoms in patients with semantic dementia: Does semantic dementia cause autistic traits?
PloS onePrimary Progressive Aphasia: Toward a Pathophysiological Synthesis.
Current neurology and neuroscience reportsFrontotemporal Dementia: A Window to Alexithymia.
The Journal of neuropsychiatry and clinical neurosciences"Semantic variant primary progressive aphasia" due to comorbidity of Lewy body disease and a previous cerebral venous infarction in the left anterior temporal lobe: A case report.
eNeurologicalSciSocial Cognition Deficits Are Pervasive across Both Classical and Overlap Frontotemporal Dementia Syndromes.
Dementia and geriatric cognitive disorders extraAltered functional connectivity of cortical networks in semantic variant Primary Progressive Aphasia.
NeuroImage. ClinicalExamining prefrontal contributions to past- and future-oriented memory disturbances in daily life in dementia.
Cortex; a journal devoted to the study of the nervous system and behaviorAssessing natural metalinguistic skills in people with Alzheimer's disease and frontotemporal dementia.
Journal of communication disordersErrorless Learning Therapy in Semantic Variant of Primary Progressive Aphasia.
Journal of Alzheimer's disease : JADDaily life activities in patients with Alzheimer's disease or semantic dementia: Multitasking assessment.
NeuropsychologiaWhen affect overlaps with concept: emotion recognition in semantic variant of primary progressive aphasia.
Brain : a journal of neurologySomatic TARDBP variants as a cause of semantic dementia.
Brain : a journal of neurologyUsing a second-person approach to identify disease-specific profiles of social behavior in frontotemporal dementia and Alzheimer's disease.
Cortex; a journal devoted to the study of the nervous system and behaviorDistinct performance profiles on the Brixton test in frontotemporal dementia.
Journal of neuropsychologyVerbal Semantics and the Left Dorsolateral Anterior Temporal Lobe: A Longitudinal Case of Bilateral Temporal Degeneration.
AphasiologyThe neurophysiological architecture of semantic dementia: spectral dynamic causal modelling of a neurodegenerative proteinopathy.
Scientific reportsGraded, multidimensional intra- and intergroup variations in primary progressive aphasia and post-stroke aphasia.
Brain : a journal of neurologyRegional and hemispheric susceptibility of the temporal lobe to FTLD-TDP type C pathology.
NeuroImage. ClinicalDifferential Atrophy in the Hippocampal Subfield Volumes in Four Types of Mild Dementia.
Frontiers in neuroscienceMultilingualism and semantic dementia - A case report.
Asian journal of psychiatryFunctional Connectivity Alterations of the Temporal Lobe and Hippocampus in Semantic Dementia and Alzheimer's Disease.
Journal of Alzheimer's disease : JADPSP-FTD Complex: A Possible Variant of PSP.
American journal of Alzheimer's disease and other dementiasAssociações
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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.
- Behavioural rigidity as a transdiagnostic marker of nucleus accumbens dysfunction in dementia.
- Transcriptomic signature of frontotemporal lobar degeneration with TDP-43 type C pathology.
- Fixel-based analysis reveals detailed white matter changes in semantic dementia.
- Modeling contributions of cognition and apathy to functional impairment in younger-onset dementia.
- Atrophy progression in frontotemporal lobar degeneration-TDP-C with primary progressive aphasia.
- Eating behaviour profiles across the frontotemporal dementia spectrum.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:100069(Orphanet)
- OMIM OMIM:600274(OMIM)
- MONDO:0010857(MONDO)
- GARD:10792(GARD (NIH))
- Variantes catalogadas(ClinVar)
- Busca completa no PubMed(PubMed)
- Q18587(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
