A demência frontotemporal variante comportamental (bv-FTD) é uma forma de demência frontotemporal (FTD) que se caracteriza por alterações progressivas no comportamento e uma diminuição na capacidade de planejar, organizar e tomar decisões. Isso ocorre com um encolhimento (atrofia) que afeta principalmente a parte da frente do cérebro.
Introdução
O que você precisa saber de cara
A demência frontotemporal variante comportamental (bv-FTD) é uma forma de demência frontotemporal (FTD) que se caracteriza por alterações progressivas no comportamento e uma diminuição na capacidade de planejar, organizar e tomar decisões. Isso ocorre com um encolhimento (atrofia) que afeta principalmente a parte da frente do cérebro.
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 39 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 71 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
Triagem neonatal (Teste do Pezinho)
A triagem neonatal permite diagnóstico precoce e início imediato do tratamento.
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Genes associados
9 genes identificados com associação a esta condição. Padrão de herança: Autosomal dominant.
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.
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
Necessary for the fragmentation of Golgi stacks during mitosis and for their reassembly after mitosis. Involved in the formation of the transitional endoplasmic reticulum (tER). The transfer of membranes from the endoplasmic reticulum to the Golgi apparatus occurs via 50-70 nm transition vesicles which derive from part-rough, part-smooth transitional elements of the endoplasmic reticulum (tER). Vesicle budding from the tER is an ATP-dependent process. The ternary complex containing UFD1, VCP and
Cytoplasm, cytosolEndoplasmic reticulumNucleusCytoplasm, Stress granule
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.
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.
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.
Molecular adapter required for selective macroautophagy (aggrephagy) by acting as a bridge between polyubiquitinated proteins and autophagosomes (PubMed:15340068, PubMed:15953362, PubMed:16286508, PubMed:17580304, PubMed:20168092, PubMed:22017874, PubMed:22622177, PubMed:24128730, PubMed:28404643, PubMed:29343546, PubMed:29507397, PubMed:31857589, PubMed:33509017, PubMed:34471133, PubMed:34893540, PubMed:35831301, PubMed:37306101, PubMed:37802024). Promotes the recruitment of ubiquitinated cargo
Cytoplasmic vesicle, autophagosomePreautophagosomal structureCytoplasm, cytosolNucleus, PML bodyLate endosomeLysosomeNucleusEndoplasmic reticulumCytoplasm, myofibril, sarcomere
Paget disease of bone 3
A disorder of bone remodeling characterized by increased bone turnover affecting one or more sites throughout the skeleton, primarily the axial skeleton. Osteoclastic overactivity followed by compensatory osteoblastic activity leads to a structurally disorganized mosaic of bone (woven bone), which is mechanically weaker, larger, less compact, more vascular, and more susceptible to fracture than normal adult lamellar bone.
Variantes genéticas (ClinVar)
154 variantes patogênicas registradas no ClinVar.
Classificação de variantes (ClinVar)
Distribuição de 1 variantes classificadas pelo ClinVar.
Vias biológicas (Reactome)
58 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 — Variante comportamental da demência frontotemporal
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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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Publicações mais relevantes
Multimodal semantic knowledge of emotion concepts in frontotemporal dementia.
Recent work has delineated the semantic behavioral variant of frontotemporal dementia (sbvFTD; or right temporal variant of FTD, which is thought to preferentially impair semantic knowledge for emotional concepts. However, this proposed core feature has not yet been empirically validated, and no clinical tool exists to assess it. Establishing reliable markers is essential to clinically differentiate sbvFTD from behavioral variant FTD (bvFTD), which is critical given their overlapping behavioral symptoms but divergent underlying pathologies. Furthermore, contrasting sbvFTD with semantic variant primary progressive aphasia (svPPA) can advance our understanding of semantic memory, revealing how the right and left anterior temporal lobes (ATLs) support emotion- versus tool-related knowledge, highlighting the graded, lateralized organization of the semantic system. We studied 15 patients with sbvFTD, 15 with svPPA, 18 with bvFTD, and 37 healthy controls. A novel multimodal semantic battery, the Fear and Spider Test (FST), which assesses tool- and emotion-related concepts across word-based semantic associations, picture-based semantic associations, and sound-to-picture matching, was administered. Stimuli were matched on psycholinguistic and perceptual features, and emotional items were drawn from multicultural facial expressions validated with the Facial Action Coding System. Neural correlates of semantic performance were investigated using voxel-based morphometry. As expected, patients with both sbvFTD and svPPA showed greater deficits in all semantic tasks compared to controls and bvFTD, and bilateral anterior temporal lobe (ATL) volumes were broadly associated with performance across all semantic tasks. Interactions between modality and categories were necessary for the emergence of differences between sbvFTD and svPPA and right and left ATL atrophy: performance on the Words-Tools condition was more impaired in svPPA and correlated with left ATL volume, while performance on the Pictures-Emotions condition was more impaired in sbvFTD and correlated with right ATL volume. The FST provides the first clear dissociation of sbvFTD from bvFTD, a distinction of critical clinical importance given their divergent pathological substrates and the absence of frontotemporal lobar degeneration specific biomarkers. The study also refines our understanding of semantic memory: contrasting sbvFTD with svPPA reveals complementary roles of the right and left ATLs in supporting emotion and tool knowledge, underscoring the graded, lateralized organization of the semantic system.
CSF1R mutations in an Italian population of early-onset dementia: a case series.
The diagnostic approach to subjects with early-onset dementia (EOD) is often challenging due to the broader range of possible etiologies as compared to late-onset dementia cases. Pathogenic variants in CSF1R gene have been increasingly reported in subjects with EOD, mostly clinically mimicking behavioral variant of frontotemporal dementia (bvFTD). Here we screened for variants in CSF1R gene in a large cohort of dementia patients consecutively referred for genetic analysis to an Italian tertiary center between 2005 and 2024 (n = 2163). Sequence of CSF1R gene was determined with next-generation sequencing through either a dedicated panel or whole-exome sequencing. Pathogenic variants or variants of uncertain significance with higher evidence of pathogenicity were found in four participants (one female); three of these were not previously reported. Clinical data were collected, including brain magnetic resonance imaging and neuropsychological assessment. Cerebrospinal fluid (CSF) levels of neurofilament light chain (NfL) protein were measured. A family history of dementia was present in one subject. Mean age at onset was 51.5. Seizures were the presenting symptom in two cases and later appeared in other two. Two subjects presented with behavioral disturbances, resembling early bvFTD. Neuropsychological assessment revealed executive and language impairment in most cases. Anterior-predominant atrophy, symmetric white-matter involvement, and serpentine calcifications were the most common imaging abnormalities. High CSF NfL levels were found in all cases, with two of them showing markedly elevated values. CSF1R-related disease should be considered in EOD subjects, especially those presenting with executive/language deficits, seizures, white matter involvement, and markedly elevated CSF NfL levels.
Exploring neuropsychiatric and behavioral symptoms, and social cognitive deficits in the differential diagnosis of behavioral variant of frontotemporal dementia and primary psychiatric disorders: A systematic literature review.
The clinical overlap between behavioral variant frontotemporal dementia (bvFTD) and primary psychiatric disorders (PPD) often results in delayed and incorrect diagnoses. We performed a literature review of 16 studies to evaluate how neuropsychiatric, behavioral, and social cognitive symptoms can aid in distinguishing bvFTD from PPD during early clinical assessment. Our study revealed differences in symptom profile and trajectory in the two diseases. Apathy, disinhibition, and compulsions were more likely to progress in bvFTD, while often remaining stable or improving in PPD. Changes in eating behavior and inappropriate social conduct, and emotion recognition, socioemotional sensitivity, and theory-of-mind impairments were more severe in bvFTD than in PPD. Although differential diagnosis can be challenging, studying the profile and progression of neuropsychiatric, behavioral, and social cognitive symptoms in bvFTD and PDD offers valuable diagnostic clues. Implementing structured tools and targeted assessments may improve differential diagnosis, ultimately guiding more appropriate treatment strategies.
Repetitive behaviors in syndromes associated with frontotemporal lobar degeneration.
BackgroundRepetitive behaviors (RB) are purposeless movements, speech, or routines performed without self-awareness or conscious intent.ObjectiveThe present study aims to investigate the prevalence and longitudinal changes of RB and to assess these symptoms in a large cohort of patients with frontotemporal lobar degeneration (FTLD)-associated syndromes using a newly developed caregiver-based questionnaire.MethodsThis was a longitudinal cohort study conducted in tertiary frontotemporal dementia research clinics. A total of 210 FTLD patients were included, 68 of whom had follow-up evaluation. RB were assessed through structured caregiver interviews. Compulsive/impulsive behaviors, stereotypies, and ritualistic behaviors were recorded. Univariate and multiple generalized linear models and generalized linear mixed models were used to estimate predictors and longitudinal changes associated with RB.ResultsRB were reported in 71% of patients, showing a progressive increase from the prodromal to moderate dementia stages. Notably, 30% of patients presented RB since the disease onset phase, especially in the form of compulsive/impulsive behaviors. Predictors included male gender, the behavioral variant of frontotemporal dementia and the semantic variant of primary progressive aphasia phenotypes, and higher scores on Frontal Behavioral Inventory scale, part B. A significant increase in total RB was observed in patients reassessed at 8 to 22 months follow-up from baseline (p = 0.0001), especially in the form of stereotypies and ritualistic behaviors.ConclusionsThe questionnaire developed in this study effectively captures the prevalence and progression of RB. It could contribute to the standardization of the behavioral assessment in FTLD clinical trials and, consequently, to a deeper understanding of these syndromes.
A Psychosocial Intervention for Managing Disinhibition in People With the Behavioral Variant of Frontotemporal Dementia: A Matter of Focusing.
Management of disinhibited behavior in people with the behavioral variant of frontotemporal dementia is challenging. To support health care professionals in long-term care, we developed "Focusing," a psychosocial intervention based on theory of automatic behavior, stimulus processing, and resident-staff interactions. The intervention was evaluated for feasibility and limited efficacy with a replicated single-case A-B observation study in 6 cases, and using questionnaires and interviews with health care professionals. The intervention was feasible in all cases and a decrease in disinhibited behavior was observed following the introduction of the intervention in 4 cases. Professionals deemed the intervention highly relevant, as they perceive disinhibition as a complex problem. These results form a foundation for larger-scale evaluation of effectiveness and show promise of the intervention for further implementation in clinical practice.
Publicações recentes
Dysphagia in behavioural variant of frontotemporal dementia- a systematic review.
Exploring neuropsychiatric and behavioral symptoms, and social cognitive deficits in the differential diagnosis of behavioral variant of frontotemporal dementia and primary psychiatric disorders: A systematic literature review.
Repetitive behaviors in syndromes associated with frontotemporal lobar degeneration.
Multimodal semantic knowledge of emotion concepts in frontotemporal dementia.
CSF1R mutations in an Italian population of early-onset dementia: a case series.
📚 EuropePMC81 artigos no totalmostrando 198
Exploring neuropsychiatric and behavioral symptoms, and social cognitive deficits in the differential diagnosis of behavioral variant of frontotemporal dementia and primary psychiatric disorders: A systematic literature review.
Alzheimer's & dementia (Amsterdam, Netherlands)Repetitive behaviors in syndromes associated with frontotemporal lobar degeneration.
Journal of Alzheimer's disease : JADMultimodal semantic knowledge of emotion concepts in frontotemporal dementia.
medRxiv : the preprint server for health sciencesCSF1R mutations in an Italian population of early-onset dementia: a case series.
Journal of neurologyA Psychosocial Intervention for Managing Disinhibition in People With the Behavioral Variant of Frontotemporal Dementia: A Matter of Focusing.
Journal of the American Medical Directors Association[Cognitive profiles in dementia].
Vertex (Buenos Aires, Argentina)The Evolving Role of FDG-PET in Behavioral Variant Frontotemporal Dementia: Current Applications and Future Opportunities.
International journal of molecular sciencesCross-country variance in facial emotion recognition in presymptomatic and symptomatic behavioral variant frontotemporal dementia: Insights from the GENFI and ReDLat consortia.
Alzheimer's & dementia : the journal of the Alzheimer's Association[Classification and Clinical Characteristics of Dementia].
No shinkei geka. Neurological surgeryCriminal Behavior in Frontotemporal Dementia: A Multimodal MRI Study.
Human brain mappingLongitudinal changes in MEG-based brain network topology of ALS patients with cognitive/behavioral impairment-An exploratory study.
Network neuroscience (Cambridge, Mass.)The Diagnostic Landscape of Behavioral Variant Frontotemporal Dementia.
Rhode Island medical journal (2013)Sex differences in clinical phenotypes of behavioral variant frontotemporal dementia.
Alzheimer's & dementia : the journal of the Alzheimer's AssociationA computational ontology framework for the synthesis of multi-level pathology reports from brain MRI scans.
Journal of Alzheimer's disease : JADA Novel CHMP2B Splicing Variant in Atypical Presentation of Familial Frontotemporal Lobar Degeneration.
Annals of clinical and translational neurologyRegional cerebral blood flow correlated with aphasia in dementia of the Alzheimer's type and frontotemporal lobar degeneration.
Acta neurobiologiae experimentalisProgranulin deficiency in the brain: the interplay between neuronal and non-neuronal cells.
Translational neurodegenerationDifferential miRNA expression in neural-enriched extracellular vesicles as potential biomarker for frontotemporal dementia and bipolar disorder.
Neurobiology of diseaseContrastive self-supervised learning for neurodegenerative disorder classification.
Frontiers in neuroinformaticsaFTLD-U presenting with primary progressive aphasia suggestive of non-fluent type with apraxia of speech.
NeurocaseSemantic knowledge of social norms in frontotemporal dementia patients with either predominant frontal or temporal lobe atrophy.
Applied neuropsychology. Adult[Behavioral variant of frontotemporal dementia with stereotypic speech: a case report].
Rinsho shinkeigaku = Clinical neurologyStructural and functional correlates of olfactory reward processing in behavioral variant frontotemporal dementia.
Cortex; a journal devoted to the study of the nervous system and behaviorClinical and imaging correlates of hyperorality in syndromes associated with frontotemporal lobar degeneration.
Psychiatry and clinical neurosciencesFrontotemporal lobar degeneration changes neuronal beta-frequency dynamics during the mismatch negativity response.
NeuroImage. ClinicalBehavioral variant of frontotemporal dementia in carriers of biallelic TREM2 variants: cases study.
Folia neuropathologicaThe emerging role of the cerebellum in the affective theory of mind in the behavioral variant of frontotemporal dementia.
Journal of neurologyCase report: Assessing criminal responsibility and recidivism risk in the behavioral variant of frontotemporal dementia.
Frontiers in psychiatryThe relative brain signal variability increases in the behavioral variant of frontotemporal dementia and Alzheimer's disease but not in schizophrenia.
Journal of cerebral blood flow and metabolism : official journal of the International Society of Cerebral Blood Flow and MetabolismA systematic review of cognitive and behavioral tools to differentiate behavioral variant frontotemporal dementia from other conditions.
PCN reports : psychiatry and clinical neurosciencesLong-term, home-based transcranial direct current stimulation coupled with computerized cognitive training in frontotemporal dementia: A case report.
Journal of central nervous system diseaseDelusions of love and passion in the behavioral variant of frontotemporal dementia.
NeurocaseClinical Accuracy of Serum Neurofilament Light to Differentiate Frontotemporal Dementia from Primary Psychiatric Disorders is Age-Dependent.
The American journal of geriatric psychiatry : official journal of the American Association for Geriatric PsychiatryClinical and neuroanatomical characterization of the semantic behavioral variant of frontotemporal dementia in a multicenter Italian cohort.
Journal of neurologyThe use of synaptic biomarkers in cerebrospinal fluid to differentiate behavioral variant of frontotemporal dementia from primary psychiatric disorders and Alzheimer's disease.
Alzheimer's research & therapyDiagnostic Performance of Socio-Emotional Informant-Based Questionnaires for the Clinical Detection of the Behavioral Variant of Frontotemporal Dementia.
Journal of Alzheimer's disease : JAD[Behavioral Variant of Frontotemporal Dementia: A Case Report of a 54-Year-Old Female Patient].
Neuropsychopharmacologia Hungarica : a Magyar Pszichofarmakologiai Egyesulet lapja = official journal of the Hungarian Association of PsychopharmacologyRationale and Design of the "DIagnostic and Prognostic Precision Algorithm for behavioral variant Frontotemporal Dementia" (DIPPA-FTD) Study: A Study Aiming to Distinguish Early Stage Sporadic FTD from Late-Onset Primary Psychiatric Disorders.
Journal of Alzheimer's disease : JADCorrelates of Functional Impairment in Patients with the Behavioral Variant of Frontotemporal Dementia: A PRISMA-Compliant Systematic Review.
International journal of molecular sciencesApplication of the mild behavioral impairment checklist in Chinese patients with the behavioral variant of frontotemporal dementia.
Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical NeurophysiologyA Five-Year Clinical Course of Potential Phenocopy Syndrome of Behavioral Variant Frontotemporal Dementia: A Case Report and Literature Review.
CureusCerebral atrophy as a cause of aphasia: From Pick to the modern era.
Cortex; a journal devoted to the study of the nervous system and behaviorDiagnostic Instability Over Time in the Late-Onset Frontal Lobe Syndrome: When Can We Say it's FTD?
The American journal of geriatric psychiatry : official journal of the American Association for Geriatric PsychiatryAssociation of cortical and subcortical microstructure with disease severity: impact on cognitive decline and language impairments in frontotemporal lobar degeneration.
Alzheimer's research & therapyThe Effects of SARS-CoV-2 Infection on the Cognitive Functioning of Patients with Pre-Existing Dementia.
Journal of Alzheimer's disease reportsIncidence of Syndromes Associated With Frontotemporal Lobar Degeneration in 9 European Countries.
JAMA neurologyBipolar spectrum disorders in neurologic disorders.
Frontiers in psychiatryNeuropsychological Assessment in the Distinction Between Biomarker Defined Frontal-Variant of Alzheimer's Disease and Behavioral-Variant of Frontotemporal Dementia.
Journal of Alzheimer's disease : JADSporadic Creutzfeldt-Jakob disease is associated with reorganization of metabolic connectivity in a pathological brain network.
European journal of neurologyProsopagnosia, Other Specific Cognitive Deficits, and Behavioral Symptoms: Comparison between Right Temporal and Behavioral Variant of Frontotemporal Dementia.
Vision (Basel, Switzerland)The Cerebellum Is a Key Structure in the Neural Network for Mentalizing: An MRI Study in the Behavioral Variant of Frontotemporal Dementia.
BiomedicinesGenuine high-order interactions in brain networks and neurodegeneration.
Neurobiology of diseaseDisease specific and nonspecific metabolic brain networks in behavioral variant of frontotemporal dementia.
Human brain mappingRelationship Between Sporadic Behavioral Variant Frontotemporal Dementia and Primary Psychiatric Disorders: A Study in Families.
The Journal of clinical psychiatryVery early-onset behavioral variant frontotemporal dementia in a patient with a variant of uncertain significance of a FUS gene mutation.
NeurocaseBehind closed doors. A case study exploring the lived experiences of a family of a person with the behavioral variant of frontotemporal dementia.
Dementia (London, England)Behavioral and psychological symptoms in neurodegenerative dementias: harbinger, follower, or constant collateral?
The Egyptian journal of neurology, psychiatry and neurosurgeryAn Indonesian elderly with primary progressive aphasia and behavioral variant of frontotemporal dementia: A case report and review article.
Annals of medicine and surgery (2012)Hyperorality in Frontotemporal Dementia: Cognitive and Psychiatric Symptom Profiles in Early-Stage Disease.
Journal of Alzheimer's disease : JADTREM2 Gene Compound Heterozygosity in Neurodegenerative Disorders.
Journal of Alzheimer's disease : JADSodium selenate as a therapeutic for tauopathies: A hypothesis paper.
Frontiers in aging neuroscienceGenetic Spectrum and Clinical Heterogeneity of Chinese Frontotemporal Dementia Patients: Data from PUMCH Dementia Cohort.
Journal of Alzheimer's disease : JADEarly Identification of Different Behavioral Phenotypes in the Behavioral Variant of Frontotemporal Dementia with the Aid of the Mini-Frontal Behavioral Inventory (mini-FBI).
Journal of Alzheimer's disease : JADIdentification and validation of Alzheimer's disease-related metabolic brain pattern in biomarker confirmed Alzheimer's dementia patients.
Scientific reportsCase Report: Novel CSF1R Variant in a Patient With Behavioral Variant Frontotemporal Dementia Syndrome With Prodromal Repetitive Scratching Behavior.
Frontiers in neurologyNeuropsychiatric or Behavioral and Psychological Symptoms of Dementia (BPSD): Focus on Prevalence and Natural History in Alzheimer's Disease and Frontotemporal Dementia.
Frontiers in neurology12-item version of Boston Naming Test: usefulness in the diagnosis of primary progressive aphasia, frontotemporal dementia, and Alzheimer's disease.
Dementia & neuropsychologiaA temporal classification method based on behavior time series data in patients with behavioral variant of frontotemporal dementia and apathy.
Journal of neuroscience methodsNeurosyphilis Mimicking Behavioral Variant of Frontotemporal Dementia in a 59-Year-Old Man.
Cognitive and behavioral neurology : official journal of the Society for Behavioral and Cognitive NeurologyResting state functional brain networks associated with emotion processing in frontotemporal lobar degeneration.
Molecular psychiatry[Clinical features of mental disorders in amyotrophic lateral sclerosis].
Zhurnal nevrologii i psikhiatrii imeni S.S. KorsakovaSPECT analysis and language profile in Greek speaking patients with subtypes of frontotemporal dementia.
Hellenic journal of nuclear medicineFrontotemporal Brain Sagging Syndrome as a Treatable Cause Mimicking Frontotemporal Dementia: A Case Report.
Case reports in neurologyThe Role of Hub and Spoke Regions in Theory of Mind in Early Alzheimer's Disease and Frontotemporal Dementia.
BiomedicinesSustained Long-term Improvement in Neuropsychiatric Symptoms of an Individual With Initial bvFTD Diagnosis: A Case Report.
Cognitive and behavioral neurology : official journal of the Society for Behavioral and Cognitive NeurologyEye-gaze Strategies During Facial Emotion Recognition in Neurodegenerative Diseases and Links With Neuropsychiatric Disorders.
Cognitive and behavioral neurology : official journal of the Society for Behavioral and Cognitive NeurologyApathy, Executive Function, and Emotion Recognition Are the Main Drivers of Functional Impairment in Behavioral Variant of Frontotemporal Dementia.
Frontiers in neurologyKnowledge and Attitudes for the Management of Behavioral Variant of Frontotemporal Dementia.
Frontiers in neurologySubjective clustering in patients with fronto-temporal dementia.
Applied neuropsychology. AdultPredicting disease progression in behavioral variant frontotemporal dementia.
Alzheimer's & dementia (Amsterdam, Netherlands)Brain network modulation in Alzheimer's and frontotemporal dementia with transcranial electrical stimulation.
Neurobiology of agingFrontotemporal Dementia and Late-Onset Bipolar Disorder: The Many Directions of a Busy Road.
Frontiers in psychiatryAmyotrophic Lateral Sclerosis-Frontotemporal Dementia: Shared and Divergent Neural Correlates Across the Clinical Spectrum.
NeurologyHonorific Speech Impairment: A Characteristic Sign of Frontotemporal Dementia.
Cognitive and behavioral neurology : official journal of the Society for Behavioral and Cognitive Neurology[Theory of mind, empathy and eye gaze strategies during an artwork observation in neurodegenerative pathologies].
Geriatrie et psychologie neuropsychiatrie du vieillissementTreatment of the behavioral variant of frontotemporal dementia: a narrative review.
Dementia & neuropsychologiaSocial Cognition and Behavioral Assessments Improve the Diagnosis of Behavioral Variant of Frontotemporal Dementia in Older Peruvians With Low Educational Levels.
Frontiers in neurologyExploring Signatures of Neurodegeneration in Early-Onset Older-Age Bipolar Disorder and Behavioral Variant Frontotemporal Dementia.
Frontiers in neurologyMolecular Pathology of ALS: What We Currently Know and What Important Information Is Still Missing.
Diagnostics (Basel, Switzerland)In vivo exploration of synaptic projections in frontotemporal dementia.
Scientific reportsA Spanish Neuropsychological Battery Discriminates Between the Behavioral Variant of Frontotemporal Dementia and Primary Progressive Aphasia in a Colombian Sample.
Frontiers in neurologyImpact of Confinement on the Burden of Caregivers of Patients with the Behavioral Variant of Frontotemporal Dementia and Alzheimer Disease during the COVID-19 Crisis in France.
Dementia and geriatric cognitive disorders extraRecognition of musical emotions in the behavioral variant of frontotemporal dementia.
Revista Colombiana de psiquiatriaTREM2 variants as a possible cause of frontotemporal dementia with distinct neuroimaging features.
European journal of neurologyCannabidiol in the management of bruxism in behavioral variant of frontotemporal degeneration.
NeurocaseNeurofilaments as Emerging Biomarkers of Neuroaxonal Damage to Differentiate Behavioral Frontotemporal Dementia from Primary Psychiatric Disorders: A Systematic Review.
Diagnostics (Basel, Switzerland)Underlying pathology identified after 20 years of disease course in two cases of slowly progressive frontotemporal dementia syndromes.
NeurocaseA case of developmental pedophilia unmasked by frontotemporal dementia.
NeurocaseFrontotemporal dementia subtypes based on behavioral inhibition deficits.
Alzheimer's & dementia (Amsterdam, Netherlands)Use of Alzheimer's Disease Cerebrospinal Fluid Biomarkers in A Tertiary Care Memory Clinic.
The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiquesFrontal hypometabolism in neurocognitive disorder with behavioral disturbance.
Journal of nuclear medicine : official publication, Society of Nuclear MedicineBehavioral Variant of Frontotemporal Dementia and Homicide in a Historical Case.
The journal of the American Academy of Psychiatry and the LawDiagnostic Utility of Measuring Cerebral Atrophy in the Behavioral Variant of Frontotemporal Dementia and Association With Clinical Deterioration.
JAMA network openDiagnostic Accuracy of Affective Social Tasks in the Clinical Classification Between the Behavioral Variant of Frontotemporal Dementia and Other Neurodegenerative Disease.
Journal of Alzheimer's disease : JADDistinctive Oculomotor Behaviors in Alzheimer's Disease and Frontotemporal Dementia.
Frontiers in aging neuroscienceSex differences in the behavioral variant of frontotemporal dementia: A new window to executive and behavioral reserve.
Alzheimer's & dementia : the journal of the Alzheimer's AssociationA Comparative Study of the Behavioral Profile of the Behavioral Variant of Frontotemporal Dementia and Parkinson's Disease Dementia.
Dementia and geriatric cognitive disorders extraBrain Structural Covariance Networks in Behavioral Variant of Frontotemporal Dementia.
Brain sciencesPast, Present, and Future of Non-invasive Brain Stimulation Approaches to Treat Cognitive Impairment in Neurodegenerative Diseases: Time for a Comprehensive Critical Review.
Frontiers in aging neuroscienceShape Profile of Corpus Callosum As a Signature to Phenotype Different Dementia.
Journal of neurosciences in rural practiceDistinguishing Behavioral Variant Frontotemporal Dementia From Primary Psychiatric Disorders: A Review of Recently Published Consensus Recommendations From the Neuropsychiatric International Consortium for Frontotemporal Dementia.
The Journal of neuropsychiatry and clinical neurosciencesThe Behavioral/Dysexecutive Variant of Alzheimer's Disease: A Case Series with Clinical, Neuropsychological, and FDG-PET Characterization.
Dementia and geriatric cognitive disordersInvestigating the clinico-anatomical dissociation in the behavioral variant of Alzheimer disease.
Alzheimer's research & therapyConfabulations in Cases of Dementia: Atypical Early Sign of Alzheimer's Disease or Misleading Feature in Dementia Diagnosis?
Frontiers in psychologyAlterations of Cerebral Blood Flow Network in Behavioral Variant Frontotemporal Dementia patients with and without Apathy.
Psychiatry research. NeuroimagingEvaluation of Discriminative Detection Abilities of Social Cognition Measures for the Diagnosis of the Behavioral Variant of Frontotemporal Dementia: a Systematic Review.
Neuropsychology reviewSocial Cognition Tests Can Discriminate Behavioral Variant Frontotemporal Dementia From Alzheimer's Disease Independently of Executive Functioning.
Archives of clinical neuropsychology : the official journal of the National Academy of NeuropsychologistsThe applause sign in elderly patients with idiopathic normal pressure hydrocephalus.
Applied neuropsychology. AdultRecall and Recognition in Alzheimer's Disease and Frontotemporal Dementia.
Journal of Alzheimer's disease : JADCognitive and behavioral profile of Perry syndrome in two families.
Parkinsonism & related disorders[Symptomatology of Behavioral Variant of Frontotemporal Dementia].
Brain and nerve = Shinkei kenkyu no shinpoSalience Network Atrophy Links Neuron Type-Specific Pathobiology to Loss of Empathy in Frontotemporal Dementia.
Cerebral cortex (New York, N.Y. : 1991)Clinical and Molecular Characterization of a Novel Progranulin Deletion Associated with Different Phenotypes.
Journal of Alzheimer's disease : JADDifferential Diagnosis of Behavioral Variant and Semantic Variant of Frontotemporal Dementia Using Visual Rating Scales.
Current medical imagingEvaluating Semantic Knowledge Through a Semantic Association Task in Individuals With Dementia.
American journal of Alzheimer's disease and other dementiasFirst case of an UBQLN2 gene mutation causing frontotemporal dementia preceded by adult onset psychiatric symptoms.
Amyotrophic lateral sclerosis & frontotemporal degenerationEpilepsy in Neurodegenerative Dementias: A Clinical, Epidemiological, and EEG Study.
Journal of Alzheimer's disease : JADCerebrospinal Fluid and Plasma Biomarkers do not Differ in the Presenile and Late-Onset Behavioral Variants of Frontotemporal Dementia.
Journal of Alzheimer's disease : JADNormative data for Emotion Hexagon test and frequency of impairment in behavioral variant frontotemporal dementia, Alzheimer's disease and Huntington's disease.
Applied neuropsychology. AdultA proposed synergistic effect of CSF1R and NMUR2 variants contributes to binge eating in hereditary diffuse leukoencephalopathy with spheroids.
Annals of translational medicineClinical and MRI Predictors of Conversion From Mild Behavioural Impairment to Dementia.
The American journal of geriatric psychiatry : official journal of the American Association for Geriatric PsychiatryThe relationship between theory of mind and cognition in Alzheimer's disease: A systematic review.
Journal of clinical and experimental neuropsychologyThe Behavioral Dyscontrol Scale in the differential diagnosis of behavioral variant of frontotemporal dementia and Alzheimer disease.
The Clinical neuropsychologist[Current approaches to the treatment of fronto-temporal dementia].
Zhurnal nevrologii i psikhiatrii imeni S.S. KorsakovaBrain metabolic signatures across the Alzheimer's disease spectrum.
European journal of nuclear medicine and molecular imagingBehavioral variant of frontotemporal dementia or frontal variant of Alzheimer's disease? A case study.
Dementia & neuropsychologiaBehavioral variant frontotemporal dementia in patients with previous severe mental illness: a systematic and critical review.
Arquivos de neuro-psiquiatriaExploration Deficits Under Ecological Conditions as a Marker of Apathy in Frontotemporal Dementia.
Frontiers in neurologyNeuropathologic basis of frontotemporal dementia in progressive supranuclear palsy.
Movement disorders : official journal of the Movement Disorder SocietyProdromal and Early bvFTD: Evaluating Clinical Features and Current Biomarkers.
Frontiers in neuroscienceMoral Emotions in Frontotemporal Dementia.
Journal of Alzheimer's disease : JADEarly pathological gambling in co-occurrence with semantic variant primary progressive aphasia: a case report.
Clinical interventions in agingIncidence of frontotemporal lobar degeneration in Italy: The Salento-Brescia Registry study.
NeurologyPhenocopy syndrome of behavioral variant frontotemporal dementia: a systematic review.
Alzheimer's research & therapyIndividual Prediction of Behavioral Variant Frontotemporal Dementia Development Using Multivariate Pattern Analysis of Magnetic Resonance Imaging Data.
Journal of Alzheimer's disease : JAD18F-THK5351 PET Imaging in the Behavioral Variant of Frontotemporal Dementia.
Dementia and neurocognitive disordersA clinical TRIAD for early suspicion of autoimmune encephalitis as a possibility in patients presenting with progressive cognitive decline.
Asian journal of psychiatryHeterogeneous brain FDG-PET metabolic patterns in patients with C9orf72 mutation.
Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical NeurophysiologyMoral judgment in patients with behavioral variant of frontotemporal dementia and amyotrophic lateral sclerosis: no impairment of the moral position, but rather its execution.
Amyotrophic lateral sclerosis & frontotemporal degenerationBifrontal Hypermetabolism on Brain FDG-PET in a Case of C9orf72-Related Behavioral Variant of Frontotemporal Dementia.
The Journal of neuropsychiatry and clinical neurosciencesResting parasympathetic dysfunction predicts prosocial helping deficits in behavioral variant frontotemporal dementia.
Cortex; a journal devoted to the study of the nervous system and behavior18F-FIBT may expand PET for β-amyloid imaging in neurodegenerative diseases.
Molecular psychiatryPerfusion alterations converge with patterns of pathological spread in transactive response DNA-binding protein 43 proteinopathies.
Neurobiology of agingThe Accuracy of INECO Frontal Screening in the Diagnosis of Executive Dysfunction in Frontotemporal Dementia and Alzheimer Disease.
Alzheimer disease and associated disordersAuto-antibodies against P/Q- and N-type voltage-dependent calcium channels mimicking frontotemporal dementia.
SAGE open medical case reportsLongitudinal Diffusion Tensor Imaging Resembles Patterns of Pathology Progression in Behavioral Variant Frontotemporal Dementia (bvFTD).
Frontiers in aging neuroscienceFDG-PET and CSF biomarker accuracy in prediction of conversion to different dementias in a large multicentre MCI cohort.
NeuroImage. Clinical[Emotional, affective and behavioral disorders in a behavioral variant of frontotemporal dementia].
Zhurnal nevrologii i psikhiatrii imeni S.S. KorsakovaCombined Socio-Behavioral Evaluation Improves the Differential Diagnosis Between the Behavioral Variant of Frontotemporal Dementia and Alzheimer's Disease: In Search of Neuropsychological Markers.
Journal of Alzheimer's disease : JAD[Social cognition in Parkinson's disease dementia and behavioral variant of frontotemporal dementia].
Revista de neurologiaMental States in Moving Shapes: Distinct Cortical and Subcortical Contributions to Theory of Mind Impairments in Dementia.
Journal of Alzheimer's disease : JADMultimodal MRI quantification of the common neurostructural bases within the FTD-ALS continuum.
Neurobiology of agingMistakes, Too Few to Mention? Impaired Self-conscious Emotional Processing of Errors in the Behavioral Variant of Frontotemporal Dementia.
Frontiers in behavioral neuroscienceBehavioral and affective features of amyotrophic lateral sclerosis patients.
Journal of the neurological sciencesThe Pitfall of Behavioral Variant Frontotemporal Dementia Mimics Despite Multidisciplinary Application of the FTDC Criteria.
Journal of Alzheimer's disease : JADPick's disease with neuronal four-repeat tau accumulation in the basal ganglia, brain stem nuclei and cerebellum.
Neuropathology : official journal of the Japanese Society of NeuropathologyCSF sAPPβ, YKL-40, and neurofilament light in frontotemporal lobar degeneration.
NeurologyPsychosis in behavioral variant frontotemporal dementia.
Neuropsychiatric disease and treatmentData-driven regions of interest for longitudinal change in three variants of frontotemporal lobar degeneration.
Brain and behaviorDiagnosing the frontal variant of Alzheimer's disease: a clinician's yellow brick road.
Journal of clinical movement disorders[Mental simulation in frontotemporal dementia].
Vertex (Buenos Aires, Argentina)Cortical function in Alzheimer's disease and frontotemporal dementia.
Translational neuroscienceSpatio-temporal and kinematic gait analysis in patients with Frontotemporal dementia and Alzheimer's disease through 3D motion capture.
Gait & posture[Pathomechanisms and clinical aspects of frontotemporal lobar degeneration].
Der NervenarztDiagnostic utility of ASL-MRI and FDG-PET in the behavioral variant of FTD and AD.
Annals of clinical and translational neurologyAn autopsy case of dementia with Lewy bodies clinically diagnosed to have a behavioral variant of frontotemporal dementia.
Clinical neuropathologyMapping temporo-parietal and temporo-occipital cortico-cortical connections of the human middle longitudinal fascicle in subject-specific, probabilistic, and stereotaxic Talairach spaces.
Brain imaging and behaviorNeuropsychiatric Symptoms in a Cohort of Patients with Frontotemporal Dementia: Our Experience.
Indian journal of psychological medicineDifferent FDG-PET metabolic patterns at single-subject level in the behavioral variant of fronto-temporal dementia.
Cortex; a journal devoted to the study of the nervous system and behaviorTau-Centric Targets and Drugs in Clinical Development for the Treatment of Alzheimer's Disease.
BioMed research internationalC9ORF72 G4C2-repeat expansion and frontotemporal dementia first reported case in Argentina.
NeurocaseUncovering the Neural Bases of Cognitive and Affective Empathy Deficits in Alzheimer's Disease and the Behavioral-Variant of Frontotemporal Dementia.
Journal of Alzheimer's disease : JADSchizophrenia as a mimic of behavioral variant frontotemporal dementia.
NeurocaseNeurochemical biomarkers in the diagnosis of frontotemporal lobar degeneration: an update.
Journal of neurochemistryDifferent functional connectivity and network topology in behavioral variant of frontotemporal dementia and Alzheimer's disease: an EEG study.
Neurobiology of agingThe behavioral variant of frontotemporal dementia: An analysis of the literature and a case report.
International journal of law and psychiatryDominant hemisphere lateralization of cortical parasympathetic control as revealed by frontotemporal dementia.
Proceedings of the National Academy of Sciences of the United States of AmericaChorea as a clinical feature of the basophilic inclusion body disease subtype of fused-in-sarcoma-associated frontotemporal lobar degeneration.
Acta neuropathologica communicationsBehavioral and neural impairments of frontotemporal dementia: Potential implications for criminal responsibility and sentencing.
International journal of law and psychiatryPortuguese family with the co-occurrence of frontotemporal lobar degeneration and neuronal ceroid lipofuscinosis phenotypes due to progranulin gene mutation.
Neurobiology of agingBehavioral variant of frontotemporal dementia: Fundamental clinical issues associated with prediction of pathological bases.
Neuropathology : official journal of the Japanese Society of NeuropathologyDAPHNE: A New Tool for the Assessment of the Behavioral Variant of Frontotemporal Dementia.
Dementia and geriatric cognitive disorders extraLongitudinal Memory Profiles in Behavioral-Variant Frontotemporal Dementia and Alzheimer's Disease.
Journal of Alzheimer's disease : JADMolecular Pathways Bridging Frontotemporal Lobar Degeneration and Psychiatric Disorders.
Frontiers in aging neuroscienceDifferential Impairment of Cognitive and Affective Mentalizing Abilities in Neurodegenerative Dementias: Evidence from Behavioral Variant of Frontotemporal Dementia, Alzheimer's Disease, and Mild Cognitive Impairment.
Journal of Alzheimer's disease : JADImpulsivity, decreased social exploration, and executive dysfunction in a mouse model of frontotemporal dementia.
Neurobiology of learning and memoryTau-directed approaches for the treatment of Alzheimer's disease: focus on leuco-methylthioninium.
Expert review of neurotherapeuticsAffective mentalizing and brain activity at rest in the behavioral variant of frontotemporal dementia.
NeuroImage. ClinicalAssociações
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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.
- Multimodal semantic knowledge of emotion concepts in frontotemporal dementia.
- CSF1R mutations in an Italian population of early-onset dementia: a case series.
- Exploring neuropsychiatric and behavioral symptoms, and social cognitive deficits in the differential diagnosis of behavioral variant of frontotemporal dementia and primary psychiatric disorders: A systematic literature review.
- Repetitive behaviors in syndromes associated with frontotemporal lobar degeneration.
- A Psychosocial Intervention for Managing Disinhibition in People With the Behavioral Variant of Frontotemporal Dementia: A Matter of Focusing.
- Dysphagia in behavioural variant of frontotemporal dementia- a systematic review.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:275864(Orphanet)
- MONDO:0017160(MONDO)
- GARD:7392(GARD (NIH))
- Variantes catalogadas(ClinVar)
- Busca completa no PubMed(PubMed)
- Q55345980(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
