Doença neurodegenerativa rara de início tardio caracterizada por paralisia do olhar supranuclear, instabilidade postural, rigidez progressiva e demência leve.
Introdução
O que você precisa saber de cara
Doença neurodegenerativa rara de início tardio caracterizada por paralisia do olhar supranuclear, instabilidade postural, rigidez progressiva e demência leve.
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 64 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 112 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Genes associados
1 gene identificado com associação a esta condição. Padrão de herança: Not applicable.
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
Variantes genéticas (ClinVar)
152 variantes patogênicas registradas no ClinVar.
Classificação de variantes (ClinVar)
Distribuição de 17 variantes classificadas pelo ClinVar.
Vias biológicas (Reactome)
3 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 — Paresia supranuclear progressiva
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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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Ensaios clínicos abertos e novidades científicas recentes
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Outros ensaios clínicos
165 ensaios clínicos encontrados, 34 ativos.
Publicações mais relevantes
Mostrando amostra de 200 publicações de um total de 3.063
Pan-neurodegeneration proteomics reveals disease subtypes and molecular signatures.
Neurodegenerative diseases (NDs) pose clinical challenges due to their complexity and molecular heterogeneity. Here, we present a pan-neurodegeneration atlas (PanNDA) from multilayer, deep proteomic analysis of 2,279 human brain samples spanning 6 major NDs: Alzheimer's disease (AD), Lewy body dementia (LBD), frontotemporal lobar degeneration with TDP-43 pathology, progressive supranuclear palsy with tau pathology, vascular dementia, and Parkinson's disease. PanNDA integrates data from whole proteome, detergent-insoluble proteome, and posttranslational modifications (phosphorylation and ubiquitination), enabling intra- and inter-disease comparisons. Intra-disease analyses uncover distinct molecular subtypes (e.g., three in AD and four in LBD), reveal dysregulated pathways, and prioritize top-ranked proteins. Inter-disease comparisons identify shared alterations in NDs, such as GPNMB in microglial and lysosomal activation and NPTX2 in synaptic regulation, alongside disease-specific changes and hub regulators within protein networks. Overall, PanNDA provides a systems-level framework for understanding ND mechanisms and serves as a foundational resource that is accessible via an interactive website: https://penglab.shinyapps.io/pannda.
Fronto-medial theta instability error signal correlates with postural sway in Parkinson's disease and progressive supranuclear palsy.
Postural instability is a common feature in progressive supranuclear palsy (PSP) and idiopathic Parkinson's disease (PD). Understanding the neural mechanisms behind the loss of postural stability may aid identification of patients at an increased risk of falls. To examine differences in postural sway between PSP and PD cohorts during dual-task assessment with electroencephalography (EEG) recording to identify neural mechanisms associated with postural control in Parkinsonism patients. We assessed static postural stability in healthy controls (HC; n = 15), people with PD (n = 18), and people with PSP (n = 9), during the following conditions: Eyes open, eyes closed and during cognitive tasks. EEG data were simultaneously recorded using a 24-channel electrode array. The groups were matched for age, sex and disease duration. The PSP cohort demonstrated greater executive dysfunction during cognitive task (p ≤ 0.01). PD and PSP groups exhibited greater instability during different postural task conditions compared to the control group; however, no significant differences were found between PD and PSP groups. The PSP group exhibited a significant reduction in fronto-medial theta power compared to the PD group (p < 0.03). A negative correlation was observed between fronto-medial theta and measures of postural sway for Parkinsonism cohorts (p < 0.05 for all). Increased fronto-medial theta activity, which has been shown to reflect anterior cingulate cortex generated 'error signals' when stability is challenged, is associated with postural stability in PD and PSP. The differences in this 'error signal' in PD and PSP possibly reflect differing abilities to compensate for challenges in stability.
[A case of progressive supranuclear palsy with progressive non-fluent agrammatic variant primary progressive aphasia who could sing fluently].
The patient was a 65-year-old man. He visited the hospital complaining of gait disturbances and difficulty speaking. He was diagnosed with progressive supranuclear palsy because of slow vertical eye movements, frequent falls, and freezing gait; these symptoms were levodopa-resistant. The patient also had aphasia similar to non-fluent agrammatic variant of primary progressive aphasia, stuttering, and palilalia. However, he was able to sing a familiar song fluently. In contrast, for an unfamiliar song, he could not begin singing without the lyrics, and was more accurate at reading aloud than singing. Nonfluent aphasia with preserved singing ability has been reported in patients with stroke and trauma. However, this has rarely been reported for neurodegenerative diseases. This phenomenon may be due to the functional preservation of the right hemisphere and the left temporal lobe. The familiar song may have been stored in long-term memory as a set of lyrics and melody, and he may have produced it as automatic language.
Chronological Diagnostic Algorithm Predicting Neuropathology in Parkinsonism.
Pre-mortem diagnosis of parkinsonism is often challenging due to atypical presentations, overlapping syndromes, and co-pathologies. This study aimed to develop a machine learning-based algorithm predicting neuropathology in parkinsonism using chronological clinical presentations, which has previously been underexplored. Clinical information was automatically abstracted from medical records of the Mayo Clinic Brain Bank using fine-tuned Generative Pre-trained Transformer 4 models. Patients who developed parkinsonism within 3 years of disease onset were included. Six machine learning models were trained with age, sex, family history, and 197 clinical presentations paired with onset information to predict neuropathologic diagnoses, including co-pathologies. Among 7,825 donors, 949 met inclusion criteria, representing 9 neuropathologic categories: Lewy body disease (LBD; n = 128), LBD with Alzheimer's disease (AD; n = 136), progressive supranuclear palsy (PSP; n = 303), PSP with AD (n = 56), PSP with LBD (n = 27), multiple system atrophy (MSA; n = 120), corticobasal degeneration (CBD; n = 99), AD (n = 43), and frontotemporal lobar degeneration (FTLD; n = 37). The CatBoost algorithm achieved an area under the receiver operating characteristic curve of 0.83 across the 9 diagnostic categories at 3 years after onset. Important predictors included age at onset, restricted eye movement, and tremor. The model remained robust to incomplete data, requiring only 23 of 200 parameters for reliable predictions with an area under the curve of 0.80. The algorithm was implemented into a user-friendly program providing diagnostic probabilities with visualizations of parameter contributions. This neuropathology-confirmed diagnostic algorithm provides a cost-effective and interpretable screening tool for parkinsonism, bridging biomarker testing and molecular-targeted therapies. ANN NEUROL 2026.
Early clinical characteristics of pathologically confirmed progressive supranuclear palsy and corticobasal degeneration.
Although the clinical features of progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) are well documented, their early clinical presentations from the perspective of initial clinical consultation remain less well understood. This study aimed to characterize the early clinical features of PSP and CBD to inform clinicians on diagnostic assessment and management strategies. Data were obtained from the National Alzheimer's Coordinating Center (NACC) database (2005-March 2025 data freeze). Neuropathologically confirmed cases of PSP (n = 278) and CBD (n = 149) were included. Analyses were restricted to first clinical visits, focusing on demographic, neuropsychiatric, neurological, and neuropsychological variables. Memory symptoms were reported in more than half of both groups, whereas language impairment was more common in CBD (> 70%) and associated with higher odds of CBD diagnosis. Depression was frequent (~ 50%), with PSP showing higher odds for depressive symptoms but lower odds for disinhibition. Although 20-30% of both PSP and CBD patients exhibited no parkinsonian signs at presentation, gait disturbance, falls, and slowness were common and strongly associated with PSP. Approximately half of both groups presented with cognitive-predominant onset, and nearly one-fifth were initially misdiagnosed as Alzheimer's disease. A substantial proportion of PSP and CBD patients lack parkinsonian signs at first presentation, and cognitive-onset presentations are frequent, leading to early diagnostic uncertainty. Clinicians should recognize these overlapping features when evaluating early atypical parkinsonian or cognitive syndromes.
Publicações recentes
Biomarkers of Leucine-Rich Repeat Kinase 2 (LRRK2) and Lysosomal Dysfunction in Progressive Supranuclear Palsy.
Clinicopathological features of primary tauopathies: a brain bank series.
Exploring the phenotypic spectrum of frontotemporal lobar degeneration.
Elucidating the Neurobiological Underpinnings of Mild Behavioral Impairment in Tauopathies: Clinical and Molecular Insights.
Metabolomic Cerebrospinal Fluid Biomarkers for the Diagnosis of Atypical Parkinsonian Syndromes.
📚 EuropePMC2.732 artigos no totalmostrando 196
Pan-neurodegeneration proteomics reveals disease subtypes and molecular signatures.
CellLower body parkinsonism: rethinking MRI planimetry.
Parkinsonism & related disordersFronto-medial theta instability error signal correlates with postural sway in Parkinson's disease and progressive supranuclear palsy.
Journal of the neurological sciencesNatural Killer Subset Changes and Vascular Endothelial Growth Factor-A Plasma Profile in Progressive Supranuclear Palsy: The NKscape Study.
Movement disorders : official journal of the Movement Disorder Society[Basal ganglia and movement disorders : Part 1: the parkinsonian spectrum-Neuroradiological patterns and diagnostic considerations].
Radiologie (Heidelberg, Germany)Unveiling discrepancies in depression detection among persons with dementia: A comparative analysis of caregiver and self-report.
Journal of Alzheimer's disease : JADBilateral reverse shoulder arthroplasty in a patient with progressive supranuclear palsy: a case report.
International journal of surgery case reports[A case of progressive supranuclear palsy with progressive non-fluent agrammatic variant primary progressive aphasia who could sing fluently].
Rinsho shinkeigaku = Clinical neurologyChronological Diagnostic Algorithm Predicting Neuropathology in Parkinsonism.
Annals of neurologyEarly clinical characteristics of pathologically confirmed progressive supranuclear palsy and corticobasal degeneration.
Journal of neurologyHypothalamic atrophy in progressive supranuclear palsy, assessed by convolutional neural network-based automatic segmentation.
Journal of neurologyGV1001 reduces pathological 4R tau and functional deficits in models relevant to progressive supranuclear palsy.
Scientific reportsPlasma tau-species positive neuron-derived extracellular vesicles in progressive supranuclear palsy.
Brain : a journal of neurologyLongitudinal Videofluorographic Dysphagia Measures in Progressive Supranuclear Palsy.
Movement disorders clinical practiceTranscriptomic signature of frontotemporal lobar degeneration with TDP-43 type C pathology.
Brain : a journal of neurologySafety, tolerability and biomarker results of bepranemab in participants with progressive supranuclear palsy: a randomised, multicentre, double-blind, placebo-controlled, phase 1b trial.
BMJ neurology openREM sleep behavior disorder as a shared motor phenotype: A multidimensional clinical study.
Sleep medicineFrontotemporal lobar degeneration-TDP mimicking progressive supranuclear palsy: A perspective on clinicopathology, genetics, and biomarkers.
Neural regeneration researchImproving access to care through transportation and internet use for patients with atypical parkinsonism: A pilot study.
Clinical parkinsonism & related disordersShort tandem repeat expansions in patients with neurodegenerative dementia.
EBioMedicine[MRI in neurodegenerative Parkinsonian syndromes].
Radiologie (Heidelberg, Germany)Revisiting Minamata disease through computational phenotypic similarity analysis.
PloS oneLongitudinal Evaluation of Polyneuropathy in Atypical Parkinsonian Syndromes.
Neurology internationalFunctional MRI in Multiple System Atrophy: A Promising Biomarker for Clinical Applications.
Neuropsychiatric disease and treatmentLongitudinal monitoring of tau aggregation in progressive supranuclear palsy with [18F]PI-2620 PET.
Alzheimer's & dementia : the journal of the Alzheimer's AssociationAssociation between the prevalence of neurological diseases and the density of board-certified neurologists: ecological cross-sectional analysis.
BMC neurologyIncidence and Survival Rates of Frontotemporal Lobar Degeneration: Population-Based Registry Study.
NeurologyNovel Variants in DCTN1 Associated with Perry Disease: A Case Series from a Chinese Parkinsonism Cohort.
Movement disorders : official journal of the Movement Disorder SocietyAccuracy of clinical diagnosis in neurodegenerative diseases - a study of 455 autopsy cases.
Journal of neurologyA data-driven SSM/PCA analysis approach for differential diagnosis of parkinsonism using 11C-PE2I PET.
NeuroImage. ClinicalNanoscopic tau aggregates are not shared intermediates but disease-specific entities across tauopathies.
Cell reportsJuggling Under Controlled Hypoxia as a Multimodal Coordinative and Cognitive Training in Parkinson's Disease-A Narrative Review.
Journal of functional morphology and kinesiologyArtificial microRNAs targeting tau enable post-symptomatic functional recovery in aged tauopathy mice.
Molecular therapy. Nucleic acidsProgressive Supranuclear Palsy PERK haplotype B selectively translates DLX1 promoting tau toxicity.
The Journal of neuroscience : the official journal of the Society for NeuroscienceClinical implications of progressive ventilatory impairment in multiple system atrophy and progressive supranuclear palsy.
Journal of the neurological sciencesExecutive anosognosia in progressive supranuclear palsy versus Parkinson's disease.
Frontiers in neurologyVulnerability of anterior cingulate Von Economo neurons to FTLD-tauopathies in behavioral variant frontotemporal dementia.
Cerebral cortex (New York, N.Y. : 1991)Dementia with Lewy bodies and additional progressive supranuclear palsy presenting with early postural instability and frequent falls: an autopsy case.
BMC neurologySpontaneous tauopathy with parkinsonism in an aged cynomolgus macaque.
Frontiers in aging neuroscienceSuicide attempt by a patient with an early phase of progressive supranuclear palsy: a case report.
BMC neurologyPhosphorylated alpha-synuclein skin biopsy alters diagnosis in two out of three patients: Initial findings in a tertiary center.
Parkinsonism & related disordersClassification of tauopathies from human brain homogenates through salt-modulated tau amplification.
Alzheimer's & dementia : the journal of the Alzheimer's AssociationReduced synaptic density in patients with progressive supranuclear palsy: An 18F-SynVest-1 PET study.
Parkinsonism & related disordersCholinergic substrates of gait and postural impairments in Progressive Supranuclear Palsy.
Research squareMulti-model Diffusion MRI Signatures in Atypical Parkinsonian Disorders.
medRxiv : the preprint server for health sciencesPatient-reported Vision Quality-of-life in Parkinsonian Syndromes and Ataxias and Association with Clinical Oculomotor Findings.
medRxiv : the preprint server for health sciencesReal-world evaluation of Armstrong's criteria in corticobasal degeneration: Phenotypic overlap and diagnostic challenges.
Parkinsonism & related disordersBalancing Accuracy and Precision: Optimal b-values for Diffusion Tensor Imaging Along the Perivascular Space.
AJNR. American journal of neuroradiologyCurrent Neuroimaging Modalities to Distinguish Parkinson's Disease from its Mimics: Imaging Features and Implications for Clinical Practice.
The neuroradiology journalDyrk1a inhibition with the Novel Compound DYR533: A Cross-Disease Therapeutic Strategy Targeting Amyloidosis, Tau Pathogenesis, and Neuroinflammation.
bioRxiv : the preprint server for biologyAutomated differentiation of parkinsonian disorders: an ROI-based analysis of subcortical shape and cortical surface features.
Cognitive neurodynamicsMolecular features of human pathological tau distinguish tauopathy-associated dementias.
CellPercutaneous endoscopic gastrostomy in atypical parkinsonian syndromes: survival and aspiration outcomes from a retrospective international cohort.
The New Zealand medical journalFluid Biomarkers of Disease Burden and Cognitive Dysfunction in Progressive Supranuclear Palsy.
Annals of clinical and translational neurologyOculogyric crisis induced by high-dose apomorphine in a patient with advanced Parkinson's disease.
Parkinsonism & related disordersRetinal Thickness Profiles in Parkinsonian Syndromes: Discerning Parkinson's Disease, Multiple System Atrophy, and Progressive Supranuclear Palsy via Optical Coherence Tomography.
BiomedicinesNeuroimaging and Pathology Biomarkers in Parkinson's Disease and Parkinsonism.
Brain sciencesPotential role of MRI to optimize clinical trial design for progressive supranuclear palsy and corticobasal degeneration.
The journal of prevention of Alzheimer's diseaseSubtracting First Principal Component May Improve 4R Tau Detectability on [18F]Flortaucipir Tau PET.
Journal of nuclear medicine : official publication, Society of Nuclear MedicineDysphagia and Dysarthria in Neurodegenerative Diseases: A Multisystem Network Approach to Assessment and Management.
Audiology researchLocalized Iron Deposition and Shape Changes of Cerebellar Dentate in Progressive Supranuclear Palsy Clinical Variants.
Movement disorders : official journal of the Movement Disorder SocietyDementia in Progressive Supranuclear Palsy: A Narrative Review.
Neurology and therapyBrainstem and Cerebellar Volume Loss and Associated Clinical Features in Progressive Supranuclear Palsy.
Annals of clinical and translational neurologyHydrocephalus should not be a mandatory exclusion criterion in PSP: Emerging pathologic and clinical evidence urges reconsideration.
Parkinsonism & related disordersRapidly progressive dysphagia and vertical supranuclear gaze palsy: a key diagnostic clue for progressive supranuclear palsy in Levodopa-Resistant parkinsonism.
Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical NeurophysiologyMoving beyond the hospital: in-depth characterization of daily-life mobility in patients with atypical Parkinsonian disorders.
NPJ Parkinson's diseaseRevealing the conformational landscape of tau P → L mutants in R23 tau macromolecule: A pathway to targeted therapeutic design.
International journal of biological macromoleculesVoice and Speech in Atypical Parkinsonian Disorders.
Movement disorders clinical practiceFirst Korean Case of C9orf72-Related Amyotrophic Lateral Sclerosis With Progressive Supranuclear Palsy-Like Features.
Journal of clinical neurology (Seoul, Korea)CORTICOBASAL SYNDROME PRESENTING AS A PROGRESSIVE HEMIPARETIC SYNDROME: A CASE REPORT.
Georgian medical newsAssociation between Physical Function at Discharge and Fall Frequency One Month after Discharge in Patients with Progressive Supranuclear Palsy.
Physical therapy researchCatestatin ameliorates tauopathy and amyloidogenesis via adrenergic inhibition.
bioRxiv : the preprint server for biologyTreatment of Disturbed Sleep in Progressive Supranuclear Palsy: a randomized, remote, double-blinded, 6-week cross-over design study protocol comparing zolpidem, suvorexant, and placebo.
TrialsWernicke's encephalopathy unmasking progressive supranuclear palsy and Alzheimer's pathology: a diagnostic challenge in an older adult.
BMJ case reportsThe spectrum of movement disorders in neurosyphilis: A systematic review.
Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical NeurophysiologyRefining a mouse model of progressive supranuclear palsy through inoculation of human post-mortem brain-derived tau.
BMC research notesPET evaluation of cholinergic system differences in progressive supranuclear palsy and age-matched controls using [18F]VAT.
Nuclear medicine and biology4R-tau seeding activity reveals molecular subtypes in progressive supranuclear palsy.
Nature communications18 F-FDG, 18 F-FP-CIT, and 18 F-Florzolotau PET Imaging in Progressive Supranuclear Palsy : Region-Specific Correlations Between Glucose Metabolism, Dopaminergic Function, and Tau Pathology.
Clinical nuclear medicineThe Utility of Olfactory Testing to Discriminate Parkinson's Disease From Diagnostic Mimics: A Systematic Review and Meta-Analysis.
European journal of neurologyPraja1 E3 ubiquitin ligase and the role it plays in neurodegeneration.
The FEBS journalInflammatory and Neurotrophic Factors and Their Connection to Quality of Life in Progressive Supranuclear Palsy-Single-Center Study.
International journal of molecular sciencesUnlocking the Sugar Code: Implications and Consequences of Glycosylation in Alzheimer's Disease and Other Tauopathies.
BiomedicinesDistinct plasma inflammatory signatures reflect disease severity and progression in progressive supranuclear palsy: a prospective cohort study.
Journal of neural transmission (Vienna, Austria : 1996)Between Habit and Mannerism: Idiosyncratic Motor Behavior.
Movement disorders clinical practiceParaneoplastic progressive Supranuclear palsy: a case report and literature review.
Oxford medical case reportsWhite matter hyperintensities in the deep cerebral venous territory differ between subcortical and cortical 4-repeat tauopathies.
Parkinsonism & related disordersPlasma NFL, GFAP, sTREM2, pTau231, and clinical severity in progressive supranuclear palsy.
Journal of neurologyThe Paper-Toss Test: enhancing bedside recognition of corticobasal syndrome.
Frontiers in neurologyProgressive Supranuclear Palsy With Post-traumatic Frontal Lobe Damage Mimicking Anti-IgLON5 Antibody Disease.
CureusDeep Learning-Based Brainstem Segmentation and Multi-Class Classification for Parkinsonian Syndrome.
Journal of magnetic resonance imaging : JMRIIncreased 18F-AV1451 Retention in Soft Tissue of Patients With Progressive Supranuclear Palsy.
Clinical nuclear medicineBrain atrophy and cholinergic denervation in progressive supranuclear palsy: an MRI and [18F]-FEOBV PET study.
Frontiers in neuroscienceInflammation PET and plasma neurofilament light predict survival in people with progressive supranuclear palsy.
Brain communicationsClinical Framework for Motor Rehabilitation in Parkinsonism: Integrating Individualized and Syndrome-Specific Approaches.
Brain & NeuroRehabilitationDiagnostic utility of biomarkers in progressive supranuclear palsy: toward a biotyping framework.
Journal of neurologyPatient with Progressive Supranuclear Palsy Initially Diagnosed as Anxiety and Depression.
Revista Colombiana de psiquiatriaProgressive supranuclear palsy and corticobasal syndrome: cross-sectional study of palliative care needs.
BMJ supportive & palliative carePlasma pTau217 and Neurofilament Light Chain as Differentiative Markers for Progressive Supranuclear Palsy From Parkinson Disease.
NeurologyQuantification of Neuromelanin as a Neuroimaging Biomarker for Parkinson's Disease.
The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiquesComparative [18F]Florzolotau and [18F]FDG PET Imaging Patterns in Anti-IgLON5 Disease and Progressive Supranuclear Palsy.
Clinical nuclear medicineNDPACX: a newly defined X-linked Parkinsonian syndrome associated with SLC9A6 hemizygote mutation.
Brain communicationsProgressive Supranuclear Palsy or Something More? Unveiling Late-Onset Wilson's Disease.
Annals of Indian Academy of NeurologyUpdates of nuclear medicine imaging in neurodegenerative disorders.
Journal of the Formosan Medical Association = Taiwan yi zhiNeurofilament light chain as a diagnostic and prognostic biomarker in atypical parkinsonisms: current evidence, new data, challenges, and future directions.
Journal of neural transmission (Vienna, Austria : 1996)Richardson Syndrome Variant of Progressive Supranuclear Palsy: A Case Report.
CureusMovement Disorders Associated with 22q11.2 Microdeletion: A Scoping Review.
Movement disorders clinical practiceSingle-cell Spatial Transcriptional Profiling Uncovers Heterogeneous Cellular Responses to Pathogenic Tau in a Mouse Model of Neurodegeneration.
bioRxiv : the preprint server for biologyCerebrospinal fluid biomarkers for diagnosis of Parkinson's disease: a systematic review and network meta-analysis.
Journal of neurologyBeyond Parkinson's Disease: A Narrative Review of Neuromelanin MRI in Neurodegenerative Diseases.
Journal of neuroimaging : official journal of the American Society of NeuroimagingMethodological considerations for MRI biomarkers in progressive supranuclear palsy.
Journal of the neurological sciencesERBB4 colocalizes with phosphorylated tau aggregates in multiple tauopathies.
Neurochemistry internationalPatterns of Longitudinal Regional Brain Atrophy in Patients With Different Progressive Supranuclear Palsy Clinical Variants.
NeurologyThe Role of MRI in Capturing Phenotypic Variability in Progressive Supranuclear Palsy: Lessons for Future Clinical Trials.
NeurologyAcoustic Vowel Metrics as Correlates of Dysphagia and Dysarthria in Brainstem Neurodegenerative Diseases.
Audiology researchConsensus meta-analysis of genome-wide association studies for Alzheimer's disease and related dementia.
medRxiv : the preprint server for health sciencesRemote Cortical Network for Frontal Cognitive Symptoms Derived from Tau Accumulation in Progressive Supranuclear Palsy.
bioRxiv : the preprint server for biologyInterpeduncular Angle: A Possible Marker to Differentiate Atypical Parkinsonism from Idiopathic Parkinson's Disease.
Annals of Indian Academy of NeurologyDuloxetine for PSP-Related Speech Disorders.
Movement disorders clinical practiceFTLD-MAPT mutations and short 5'UTR Tau mRNAs increase Tau translation.
NAR molecular medicineDynamic multi-scale deep learning with mixture of experts for differentiating iNPH and PSP using MRI.
International journal of computer assisted radiology and surgeryUtility of Progression Rate Since Symptom Onset in Predicting Subsequent Survival in Progressive Supranuclear Palsy.
Movement disorders : official journal of the Movement Disorder SocietyBackward walking as a mobility assessment and exercise intervention for persons with neurologic disorders: A scoping review.
Gait & postureThe genetic landscape of frontotemporal lobar degeneration: investigation of a diagnostic cohort of 2747 probands.
Brain : a journal of neurologySafety, Tolerability, Pharmacokinetics, and Brain Target Occupancy of the OGA Inhibitor ASN90 in Healthy Participants.
Movement disorders : official journal of the Movement Disorder SocietyAssociation between visual hallucinations and α-synuclein oligomers in patients with dementia with Lewy bodies.
Alzheimer's & dementia : the journal of the Alzheimer's AssociationMultiple System Atrophy Without Dysautonomia: An Autopsy-Confirmed Study.
NeurologyTDP-43-proteinopathy at the crossroads of tauopathy: on copathology and current and prospective biomarkers.
Frontiers in cellular neurosciencePotential Role of Transferrin and Vascular Cell Adhesion Molecule 1 in Differential Diagnosis Among Patients with Tauopathic Atypical Parkinsonian Syndromes.
Diagnostics (Basel, Switzerland)Automated MRI-Based Classification of Parkinsonism: A Deep Learning Approach to Distinguish PD From PSP.
CNS neuroscience & therapeuticsDeep transcranial magnetic stimulation in progressive supranuclear palsy: a randomized double-blind, cross-over study.
Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical NeurophysiologyLevodopa exposure and nigral neuroinflammation in parkinsonian disorders: A postmortem study of 63 cases.
Scientific reportsRefining the diagnostic accuracy of Parkinsonian disorders using metaphenomic annotation of the clinicopathological literature.
NPJ Parkinson's diseaseProgressive supranuclear palsy-like parkinsonism ensuing from anti-N-methyl-Daspartate receptor encephalitis.
Hong Kong medical journal = Xianggang yi xue za zhiProximity Labeling of the Tau Repeat Domain Enriches RNA-Binding Proteins That Are Altered in Alzheimer's Disease and Related Tauopathies.
Molecular & cellular proteomics : MCPData-Driven differentiation of idiopathic Normal-Pressure hydrocephalus and progressive supranuclear palsy via automated volumetric analysis.
NeuroradiologyThe discriminative linguistic profiles of progressive supranuclear palsy and Parkinson's disease.
NeurologiaPeripheral immunity patterns, imaging features, and clinical outcomes in patients with gait impairment and ventriculomegaly on brain MRI.
Frontiers in aging neuroscienceIntracellular trafficking SNARE protein, syntaxin-6, modifies prion cellular phenotypes and risk of disease development in vivo.
Acta neuropathologicaUtility of Locus Coeruleus Signal Intensity on High-resolution T1-weighted MR Imaging with Magnetization Transfer for Differentiating Parkinson's Disease from Atypical Parkinsonism.
Magnetic resonance in medical sciences : MRMS : an official journal of Japan Society of Magnetic Resonance in MedicineLeveraging Proteomics to Explore the Molecular Mechanisms of Primary Tauopathies.
Journal of neurochemistryHybrid Tau-PET/MRI study: Quantitative susceptibility mapping in progressive supranuclear palsy and its correlation with Tau-PET.
European journal of nuclear medicine and molecular imagingThe underlying causes of eye movement disorders and ataxia.
Neurodegenerative disease managementSpatial Metabolic Covariance Networks in Progressive Supranuclear Palsy: Implications for Symptomatology and Their Neural Basis.
Movement disorders : official journal of the Movement Disorder SocietyDeceleration Capacity as a Marker of Autonomic Cardiac Modulation in Prodromal and Manifest Parkinson's Disease, Multiple System Atrophy, and Progressive Supranuclear Palsy.
European journal of neurologyBarcelona Progressive Supranuclear Palsy (PSP) Registry: Clinical, Oculomotor, and Cerebrospinal Fluid Markers; from Suggestive to Definite Cases.
Movement disorders : official journal of the Movement Disorder SocietyNeuronal-specific antibody in patients with multiple system atrophy and progressive supranuclear palsy.
Journal of the Formosan Medical Association = Taiwan yi zhiA Mathematical Model of Cellular Aggregation Predicts Patterns of Tau Accumulation in Neurodegenerative Disease.
Advanced science (Weinheim, Baden-Wurttemberg, Germany)Reviewer Comment on Kazemi et al. "Assessment of Clinical and Demographic Factors Influencing the Severity of Levodopa-Induced Dyskinesia".
The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiquesToward Biology-Driven Diagnosis of Atypical Parkinsonian Disorders.
NeuroSciAbsence of STN Neuronal Activity during MER in DBS Surgery: Diagnostic and Clinical Implications.
Stereotactic and functional neurosurgeryLRRK2 kinase-mediated accumulation of lysosome-associated phospho-Rabs in tauopathies and synucleinopathies.
Acta neuropathologicaComparing and combining TSPO-PET tracers in tauopathies.
European journal of nuclear medicine and molecular imagingProgressive supranuclear palsy against everyone else: a look into the specificity of MRI markers.
Journal of the neurological sciencesInvestigating the impact of white matter hyperintensities on longitudinal progression in progressive supranuclear palsy.
Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical NeurophysiologyEarly subtypes and progressions of progressive supranuclear palsy: a data-driven brain bank study.
Journal of neurologyA Short Version of Carers' Quality of Life Questionnaire for Parkinsonism: Data from Progressive Supranuclear Palsy Network.
Movement disorders clinical practiceProtein fingerprints of brain-derived extracellular vesicles predict types of tau pathology.
Alzheimer's research & therapyBrain linear measurement index in the differential diagnosis between idiopathic normal pressure hydrocephalus and progressive supranuclear palsy.
Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical NeurophysiologyImmune system modifications in atypical parkinsonism related to autoimmunity - a case control study.
Neurodegenerative disease managementNo anti-IgLON5 antibody in carefully diagnosed PSP patients with "atypical" or variant clinical features.
Parkinsonism & related disordersPolyneuropathy in Parkinson's disease and atypical Parkinsonian syndromes: clinical impact and risk factors.
Journal of neural transmission (Vienna, Austria : 1996)A Case of Progressive Supranuclear Palsy with Predominant Speech/Language Disorder.
Internal medicine (Tokyo, Japan)Anti-tau VHH therapy against PHF6: a safe approach to slowing the phenotype of tau pathology.
Alzheimer's research & therapyA novel lncRNA FAM151B-DT regulates degradation of aggregation prone proteins.
Molecular psychiatryCross-sectional and longitudinal validation of short and long versions of the progressive supranuclear palsy quality of life scale.
Journal of neural transmission (Vienna, Austria : 1996)A Biomarker-Based Classification of Corticobasal Syndrome.
Movement disorders : official journal of the Movement Disorder Society[Classification and Clinical Characteristics of Dementia].
No shinkei geka. Neurological surgeryVisual interpretation of [18F]Florzolotau Tau-PET imaging for differentiating alzheimer's disease and progressive supranuclear palsy.
European journal of nuclear medicine and molecular imagingA Bayesian analysis of diagnostic timelines across Alzheimer's disease, frontotemporal dementia, and other neurodegenerative conditions.
Alzheimer's & dementia (Amsterdam, Netherlands)Understanding Tau pathology: Insights from animal models.
Zoological researchAZP2006 in Progressive Supranuclear Palsy: Outcomes from a Phase 2a Multicenter, Randomized Trial, and Open-Label Extension on Safety, Biomarkers, and Disease Progression.
Movement disorders : official journal of the Movement Disorder SocietyImaging of Proteinopathies in the Brains of Parkinsonian Disorders.
CellsDisease-specific tau polymorphs define unique protein interaction networks across proteinopathies.
bioRxiv : the preprint server for biologyCNS-Tau Specific Antibodies Illuminate Disease Signatures Across Tauopathies.
bioRxiv : the preprint server for biologyDynamic Stability Analysis of Progressive Supranuclear Palsy Affected Gait Using Lyapunov Floquet Theory.
IEEE transactions on neural systems and rehabilitation engineering : a publication of the IEEE Engineering in Medicine and Biology SocietyFacilitated α-synuclein oligomer sharing among glial cells by a centrally acting connexin inhibitor attenuates a rapidly progressive multiple system atrophy-cerebellar type model by reducing the neuronal α-synuclein burden.
Acta neuropathologica communicationsThe Progressive Supranuclear Palsy Clinical Deficits Scale accurately reflects functional as well as patient- and caregiver-reported outcomes.
Parkinsonism & related disorders[18F]THK5351 uptake in multiple system atrophy compared with other parkinsonian disorders.
Journal of neurologyNeuroanatomical normative modelling in frontotemporal lobar degeneration: higher heterogeneity in the behavioural variant.
Journal of neurologyElevated levels of cerebrospinal fluid soluble triggering receptor expressed on myeloid cells 2 in multiple system atrophy: a marker of disease-associated microglial activation.
Journal of neural transmission (Vienna, Austria : 1996)α-Synuclein seed amplification assay positivity beyond synucleinopathies.
EBioMedicineLow synaptic and neurosecretory proteins in cerebrospinal fluid in early parkinsonian disease.
Journal of the neurological sciencesQuantitative Susceptibility Mapping and Iron Deposition in Autopsy-Confirmed Progressive Supranuclear Palsy.
Movement disorders clinical practiceThe temporal order of genetic, environmental, and pathological risk factors in Parkinson's disease: paving the way to prevention.
The Lancet. NeurologyCircadian Rhythms of Sleep-Wake, Thermoregulatory and Cardiovascular Function in Progressive Supranuclear Palsy.
Movement disorders clinical practiceThe "gunslinger" sign in neurological disorders.
Journal of the neurological sciencesA Review of FDG-PET in Progressive Supranuclear Palsy and Corticobasal Syndrome.
International journal of molecular sciencesMidsagittal Midbrain Area and Midbrain-to-Pons-Ratio Cannot Distinguish Overlap Syndromes Between Amyotrophic Lateral Sclerosis and Progressive Supranuclear Palsy.
Clinical neuroradiologyDigital speech assessments and machine learning for differentiation of neurodegenerative diseases.
Clinical parkinsonism & related disordersEnhancing interpretability of AI with radiomics-based deep neural network: proof of concept in the classification of Parkinsonian syndromes with 18F-FDG PET imaging.
European journal of nuclear medicine and molecular imagingIncidence and Prevalence of Frontotemporal Dementia: A Systematic Review and Meta-Analysis.
JAMA neurologyAcetylation Mimetic and Null Mutations Within the Filament Core of P301L Tau Have Varied Effects on Susceptibility to Seeding and Aggregation.
Journal of neurochemistryPrevalence and correlated factors of REM sleep behavior disorder in patients with progressive supranuclear palsy.
Sleep medicineRevisiting Parkinson's disease definition and classification: insights from two emerging biological frameworks.
Journal of neural transmission (Vienna, Austria : 1996)Progressive Supranuclear Palsy-A Global Review.
Movement disorders clinical practiceAssociações
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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.
- Pan-neurodegeneration proteomics reveals disease subtypes and molecular signatures.
- Fronto-medial theta instability error signal correlates with postural sway in Parkinson's disease and progressive supranuclear palsy.
- [A case of progressive supranuclear palsy with progressive non-fluent agrammatic variant primary progressive aphasia who could sing fluently].
- Chronological Diagnostic Algorithm Predicting Neuropathology in Parkinsonism.
- Early clinical characteristics of pathologically confirmed progressive supranuclear palsy and corticobasal degeneration.
- Biomarkers of Leucine-Rich Repeat Kinase 2 (LRRK2) and Lysosomal Dysfunction in Progressive Supranuclear Palsy.
- Clinicopathological features of primary tauopathies: a brain bank series.
- Exploring the phenotypic spectrum of frontotemporal lobar degeneration.
- Elucidating the Neurobiological Underpinnings of Mild Behavioral Impairment in Tauopathies: Clinical and Molecular Insights.
- Metabolomic Cerebrospinal Fluid Biomarkers for the Diagnosis of Atypical Parkinsonian Syndromes.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:683(Orphanet)
- MONDO:0019037(MONDO)
- Esclerose Lateral Amiotrofica(PCDT · Ministério da Saúde)
- GARD:7471(GARD (NIH))
- Variantes catalogadas(ClinVar)
- Busca completa no PubMed(PubMed)
- Artigo Wikipedia(Wikipedia)
- Q945930(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
