A Paralisia Supranuclear Progressiva Atípica (PSP Atípica) é um grupo de problemas de saúde que aparecem por causa de uma alteração cerebral na proteína tau (o principal problema da PSP), mas que não se encaixam na forma clássica de apresentação da PSP (chamada Síndrome de Richardson), uma doença neurodegenerativa rara que surge em idade avançada. Este grupo inclui o Parkinsonismo-PSP (PSP-P), a PSP-Acinesia pura com congelamento da marcha (PSP-PAGF), a PSP-Síndrome corticobasal (PSP-CBS) e a PSP-Afasia progressiva não-fluente (PSP-PNFA).
Introdução
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A Paralisia Supranuclear Progressiva Atípica (PSP Atípica) é um grupo de problemas de saúde que aparecem por causa de uma alteração cerebral na proteína tau (o principal problema da PSP), mas que não se encaixam na forma clássica de apresentação da PSP (chamada Síndrome de Richardson), uma doença neurodegenerativa rara que surge em idade avançada. Este grupo inclui o Parkinsonismo-PSP (PSP-P), a PSP-Acinesia pura com congelamento da marcha (PSP-PAGF), a PSP-Síndrome corticobasal (PSP-CBS) e a PSP-Afasia progressiva não-fluente (PSP-PNFA).
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
+ 47 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 77 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.
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.
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 — Síndrome de paralisia supranuclear progressiva atípica
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Publicações mais relevantes
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.
Longitudinal Evaluation of Polyneuropathy in Atypical Parkinsonian Syndromes.
Background: In Parkinson's disease (PD), a higher prevalence of polyneuropathy (PNP) is increasingly recognized, although the causal association is still under debate. In contrast, PNP in atypical parkinsonian syndromes (APS) has been insufficiently addressed, despite preliminary evidence suggesting elevated prevalence. Methods: Nerve conduction studies were performed on 13 patients with multiple system atrophy (MSA) and 9 patients with progressive supranuclear palsy (PSP) at baseline. PNP was diagnosed according to standard electrophysiological criteria after exclusion of common secondary causes. Comprehensive clinical evaluation included motor and non-motor assessments over two years of follow-up. Results: At baseline, PNP was present in 53.8% of MSA patients and 66.7% of PSP patients. MSA patients with PNP showed greater motor symptom severity (UPDRS III score; p = 0.046) and worse cognitive performance (MoCA; p = 0.044) compared to those without PNP. Over two years, a significant reduction in the tibial nerve amplitude was observed exclusively in MSA patients (p = 0.039), paralleling disease progression. Conclusions: This study provides the first longitudinal evaluation of clinical and electrophysiological PNP progression in MSA and PSP. A high comorbidity of PNP in patients with APS could contribute to motor and sensory impairments in these patients. Our findings indicate that PNP progression may reflect disease progression in MSA. Given the limited sample size, larger-scale longitudinal studies are needed to further investigate biomarker potential of PNP in APS and to clarify differences in peripheral nerve involvement between synucleinopathies and tauopathies.
Multi-model Diffusion MRI Signatures in Atypical Parkinsonian Disorders.
Distinguishing atypical parkinsonian disorders (APS) from Parkinson's disease (PD) remains challenging due to overlapping clinical features, yet accurate differentiation is critical for prognosis and treatment. Here, we employed multi-model diffusion MRI (dMRI) analysis to characterize microstructural alterations across corticobasal syndrome (CBS), progressive supranuclear palsy-Richardson syndrome (PSP-RS) and PD, with the aim of identifying which dMRI model provides optimum differentiation. We analyzed 25 CBS, 42 PSP-RS, and 21 PD participants compared to 35 age and sex-matched controls. Using a clinically feasible 3-shell high angular resolution diffusion imaging (HARDI) protocol, we applied 11 metrics from five complementary dMRI models-diffusion tensor imaging (DTI), free-water-eliminated model of DTI (FWE), neurite orientation dispersion and density imaging (NODDI), tissue-weighted NODDI, and Fixel Density (FD) in fixel-based analysis (FBA) -to comprehensively assess regional white and gray matter integrity. Group differentiation was assessed using Cohen's d effect sizes and spearman correlations were assessed between dMRI metrics and clinical scales. Distinct microstructural signatures were observed across disorders and the sensitivity of the dMRI models differed. In group contrasts, DTI and NODDI-derived metrics consistently captured the strongest effects in midbrain and peduncular pathways for PSP-RS, whereas precentral and corticospinal alterations in CBS were most prominent using NODDI and FBA measures. Free-water-corrected metrics showed attenuated group differences. Across clinical-diffusion analyses, NODDI metrics exhibited the most robust associations with disease severity, while DTI and FWE measures detected more limited, regionally constrained effects. Together, these findings highlight complementary yet distinct sensitivities of tensor, free-water, multi-compartment, and fixel-based models to APS-related neurodegeneration.
Neuroimaging and Pathology Biomarkers in Parkinson's Disease and Parkinsonism.
The "Neuroimaging and Pathology Biomarkers in Parkinson's Disease" course held on 12-13 September 2025 in Milan, Italy, convened an international faculty to review state-of-the-art biomarkers spanning neurotransmitter dysfunction, protein pathology and clinical translation. Here, we synthesize the four themed sessions and highlights convergent messages for diagnosis, stratification and trial design. The first session focused on neuroimaging markers of neurotransmitter dysfunction, highlighting how positron emission tomography (PET), single photon emission computed tomography (SPECT), and magnetic resonance imaging (MRI) provided complementary insights into dopaminergic, noradrenergic, cholinergic and serotonergic dysfunction. The second session addressed in vivo imaging of protein pathology, presenting recent advances in PET ligands targeting α-synuclein, progress in four-repeat tau imaging for progressive supranuclear palsy and corticobasal syndromes, and the prognostic relevance of amyloid imaging in the context of mixed pathologies. Imaging of neuroinflammation captures inflammatory processes in vivo and helps study pathophysiological effects. The third session bridged pathology and disease mechanisms, covering the biology of α-synuclein and emerging therapeutic strategies, the clinical potential of seed amplification assays and skin biopsy, the impact of co-pathologies on disease expression, and the "brain-first" versus "body-first" model of pathological spread. Finally, the fourth session addressed disease progression and clinical translation, focusing on imaging predictors of phenoconversion from prodromal to clinically overt stages of synucleinopathies, concepts of neural reserve and compensation, imaging correlates of cognitive impairment, and MRI approaches for atypical parkinsonism. Biomarker-informed pharmacological, infusion-based, and surgical strategies, including network-guided and adaptive deep brain stimulation, were discussed as examples of how multimodal biomarkers may inform personalized management. Across all sessions, the need for harmonization, longitudinal validation, and pathology-confirmed outcome measures was consistently emphasized as essential for advancing biomarker qualification in multicentre research and clinical practice.
Publicações recentes
An autopsy case of progressive supranuclear palsy with severe corticospinal tract degeneration.
Training high level balance and stepping responses in atypical progressive supranuclear palsy: a case report.
Association Between Globular Glial Tauopathies and Frontotemporal Dementia-Expanding the Spectrum of Gliocentric Disorders: A Review.
Typical or atypical progressive supranuclear palsy: a comparative clinicopathologic study of three Chinese cases.
A case of atypical progressive supranuclear palsy.
📚 EuropePMCmostrando 200
[Basal ganglia and movement disorders : Part 1: the parkinsonian spectrum-Neuroradiological patterns and diagnostic considerations].
Radiologie (Heidelberg, Germany)Chronological Diagnostic Algorithm Predicting Neuropathology in Parkinsonism.
Annals of neurologyEarly clinical characteristics of pathologically confirmed progressive supranuclear palsy and corticobasal degeneration.
Journal of neurologyImproving access to care through transportation and internet use for patients with atypical parkinsonism: A pilot study.
Clinical parkinsonism & related disorders[MRI in neurodegenerative Parkinsonian syndromes].
Radiologie (Heidelberg, Germany)Longitudinal Evaluation of Polyneuropathy in Atypical Parkinsonian Syndromes.
Neurology internationalA data-driven SSM/PCA analysis approach for differential diagnosis of parkinsonism using 11C-PE2I PET.
NeuroImage. ClinicalJuggling Under Controlled Hypoxia as a Multimodal Coordinative and Cognitive Training in Parkinson's Disease-A Narrative Review.
Journal of functional morphology and kinesiologyCholinergic 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 sciencesCurrent Neuroimaging Modalities to Distinguish Parkinson's Disease from its Mimics: Imaging Features and Implications for Clinical Practice.
The neuroradiology journalPercutaneous endoscopic gastrostomy in atypical parkinsonian syndromes: survival and aspiration outcomes from a retrospective international cohort.
The New Zealand medical journalRetinal Thickness Profiles in Parkinsonian Syndromes: Discerning Parkinson's Disease, Multiple System Atrophy, and Progressive Supranuclear Palsy via Optical Coherence Tomography.
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Brain sciencesVoice and Speech in Atypical Parkinsonian Disorders.
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Journal of neural transmission (Vienna, Austria : 1996)Richardson Syndrome Variant of Progressive Supranuclear Palsy: A Case Report.
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NeuroSciNo 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)Cross-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)Patterns and causes of supranuclear vertical gaze palsy: A retrospective, single-institutional study in 113 patients.
Clinical parkinsonism & related disordersImaging of Proteinopathies in the Brains of Parkinsonian Disorders.
CellsThe Progressive Supranuclear Palsy Clinical Deficits Scale accurately reflects functional as well as patient- and caregiver-reported outcomes.
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International journal of molecular sciencesMidsagittal Midbrain Area and Midbrain-to-Pons-Ratio Cannot Distinguish Overlap Syndromes Between Amyotrophic Lateral Sclerosis and Progressive Supranuclear Palsy.
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Movement disorders : official journal of the Movement Disorder SocietySerious Illness Conversation in the Care of Atypical Parkinsonian Disorders: A Practical Guide for Neurology Clinicians.
Movement disorders clinical practiceDistinct microRNA signatures define sporadic PSP-RS and PD in patient-derived midbrain organoids.
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PloS oneSpectral domain and angiography optical coherence tomography in atypical parkinsonisms and Parkinson disease: an explorative study.
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Journal of clinical medicinePlasma miRNA Biomarker Signatures in Parkinsonian Syndromes.
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AJNR. American journal of neuroradiologyAn audit on the assessment and management of osteoporosis in a Parkinson's and related diseases clinic in Australia.
Journal of neurology[Chronic epidural spinal cord stimulation for gait impairments in Parkinson's disease and atypical parkinsonism].
Zhurnal nevrologii i psikhiatrii imeni S.S. KorsakovaPossible Impact of Peripheral Inflammatory Factors and Interleukin-1β (IL-1β) on Cognitive Functioning in Progressive Supranuclear Palsy-Richardson Syndrome (PSP-RS) and Progressive Supranuclear Palsy-Predominant Parkinsonism (PSP-P).
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Clinical parkinsonism & related disordersQuality of life in patients with progressive supranuclear palsy: a review of literature and implications for practice.
Frontiers in neurologyCognitive impairment in Parkinson's disease and other parkinsonian syndromes.
Journal of neural transmission (Vienna, Austria : 1996)Differentiating atypical parkinsonian syndromes with hyperbolic few-shot contrastive learning.
NeuroImageJapanese longitudinal biomarker study in progressive supranuclear palsy and corticobasal degeneration: Clinical features of the first registered patients and short-term follow-up analysis.
Clinical parkinsonism & related disordersThe Gunslinger's sign in atypical parkinsonism.
Journal of the neurological sciencesSpeech profile in different clinical PSP phenotypes: an acoustic-perceptual study.
Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical NeurophysiologyAutoimmune Encephalitis and Paraneoplastic Neurological Syndromes with Progressive Supranuclear Palsy-like Manifestations.
Brain sciencesAcute Levodopa Challenge in Atypical Parkinsonism: Comprehensive Analysis of Individual Motor Responses.
Brain sciencesThe possible connection between neutrophil-to-high-density lipoprotein ratio and cerebral perfusion in clinically established corticobasal syndrome: a pilot study.
Frontiers in neurologyAsymmetry in Atypical Parkinsonian Syndromes-A Review.
Journal of clinical medicinePrecision Imaging in Neurodegeneration: The Superiority of Diffusion Tensor Imaging Over Conventional MRI in Differentiating Parkinson's Disease From Atypical Parkinsonian Syndromes.
CureusSEND-PD in Parkinsonian Syndromes: Results of a Monocentric Cross-Sectional Study.
Neuropsychiatric disease and treatmentDiagnostic value of six plasma biomarkers in progressive supranuclear palsy, multiple system atrophy, and Parkinson's disease.
Clinica chimica acta; international journal of clinical chemistryAtypical Parkinsonian Syndromes: Structural, Functional, and Molecular Imaging Features.
AJNR. American journal of neuroradiologyCSF α-Synuclein Seed Amplification Assay in Patients With Atypical Parkinsonian Disorders.
NeurologyImpact of the Anticholinergic Burden on Disease-Specific Symptoms in Parkinsonian Syndromes.
Brain sciencesClinical Practices and Opinions toward Gastrostomy Use in Patients with Atypical Parkinsonian Syndromes: A National Survey in the UK.
Movement disorders clinical practiceA General Neurologist's Practical Diagnostic Algorithm for Atypical Parkinsonian Disorders: A Consensus Statement.
Neurology. Clinical practiceThe associations between common neuroimaging parameters of Progressive Supranuclear Palsy in magnetic resonance imaging and non-specific inflammatory factors - pilot study.
Frontiers in immunologyThe association of vagal atrophy with parameters of autonomic function in multiple system atrophy and progressive supranuclear palsy.
Therapeutic advances in neurological disordersStructural parameters are superior to eigenvector centrality in detecting progressive supranuclear palsy with machine learning & multimodal MRI.
HeliyonDeterminants of care partner burden in atypical Parkinsonian syndromes: A retrospective, multi-center analysis.
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Brain sciencesUpper motor neuron-predominant motor neuron disease presenting as atypical parkinsonism: A clinicopathological study.
Brain pathology (Zurich, Switzerland)The potential significance of hepcidin evaluation in progressive supranuclear palsy.
Brain and behaviorUsefulness of Olfactory Bulb Measurement in 3D-FIESTA in Differentiating Parkinson Disease from Atypical Parkinsonism.
AJNR. American journal of neuroradiologyIron deposition in subcortical nuclei of Parkinson's disease: A meta-analysis of quantitative iron-sensitive magnetic resonance imaging studies.
Chinese medical journalAnti-IgLON5 disease as a differential diagnosis of multiple system atrophy.
Parkinsonism & related disordersEffects of physiotherapy and home-based training in parkinsonian syndromes: protocol for a randomised controlled trial (MobilityAPP).
BMJ openParkinson's disease and Parkinsonism syndromes: Evaluating iron deposition in the putamen using magnetic susceptibility MRI techniques - A systematic review and literature analysis.
HeliyonHistologic tau lesions and magnetic resonance imaging biomarkers differ across two progressive supranuclear palsy variants.
Brain communicationsHow can we manage progressive supranuclear palsy syndrome with pharmacotherapy?
Expert opinion on pharmacotherapyDetecting Abnormal Eye Movements in Patients with Neurodegenerative Diseases - Current Insights.
Eye and brainClinical and Pathological Features of FTDP-17 with MAPT p.K298_H299insQ Mutation.
Movement disorders clinical practiceNew knowledge on anti-IgLON5 disease.
Current opinion in neurologyFrequency and outcomes of gastrostomy insertion in a longitudinal cohort study of atypical parkinsonism.
European journal of neurologyImpulse Control Disorders in Parkinson's Disease and Atypical Parkinsonian Syndromes-Is There a Difference?
Brain sciencesThe significance of glial cell line-derived neurotrophic factor analysis in Progressive Supranuclear Palsy.
Scientific reportsDissecting the Clinical Heterogeneity and Genotype-Phenotype Correlations of MAPT Mutations: A Systematic Review.
Frontiers in bioscience (Landmark edition)Monash-Alfred protocol for assessment of atypical parkinsonian syndromes (MAP-APS).
BMJ neurology openGlucose Metabolism and Cognitive Decline in Progressive Supranuclear Palsy and Corticobasal Syndrome: A Preliminary Study.
Journal of clinical medicineComparison of autonomic dysfunction in patients with Parkinson's Disease, progressive supranuclear palsy, and multiple system atrophy.
Neurologia i neurochirurgia polskaCoenzyme Q10: A Biomarker in the Differential Diagnosis of Parkinsonian Syndromes.
Antioxidants (Basel, Switzerland)The MAPT p.E342K and p.R406W mutations are associated with progressive supranuclear palsy with atypical features.
Parkinsonism & related disordersProgressive supranuclear palsy: A case report and brief review of the literature.
Radiology case reportsClinical Phenotypes of Progressive Supranuclear Palsy-The Differences in Interleukin Patterns.
International journal of molecular sciencesSerum GDF-15 Levels in Patients with Parkinson's Disease, Progressive Supranuclear Palsy, and Multiple System Atrophy.
Neurology internationalAtypical findings: Atypical parkinsonian syndromes or Atypical parkinsonian syndromes look-alikes.
Clinical neurology and neurosurgeryClinical features of progressive supranuclear palsy.
Frontiers in aging neurosciencemicroRNA and circRNA in Parkinson's Disease and atypical parkinsonian syndromes.
Advances in clinical chemistryLarge-scale proximity extension assay reveals CSF midkine and DOPA decarboxylase as supportive diagnostic biomarkers for Parkinson's disease.
Translational neurodegenerationImpaired skeletal muscle health in Parkinsonian syndromes: clinical implications, mechanisms and potential treatments.
Journal of cachexia, sarcopenia and musclePSP-Richardson syndrome mimics: An overview and pragmatic approach.
Revue neurologique[Autoimmune encephalitis and paraneoplastic neurological syndromes presenting atypical parkinsonism: a scoping review].
Rinsho shinkeigaku = Clinical neurology[18F]-FDopa positron emission tomography imaging in corticobasal syndrome.
Brain imaging and behaviorLarge-scale activation likelihood estimation meta-analysis of parkinsonian disorders.
Brain communicationsNeurological update: non-motor symptoms in atypical parkinsonian syndromes.
Journal of neurologyWhole-Brain Magnetic Resonance Spectroscopy Reveals Distinct Alterations in Neurometabolic Profile in Progressive Supranuclear Palsy.
Movement disorders : official journal of the Movement Disorder SocietyCase report of a patient with unclassified tauopathy with molecular and neuropathological features of both progressive supranuclear palsy and corticobasal degeneration.
Acta neuropathologica communicationsSlowing of Frontal β Oscillations in Atypical Parkinsonism.
Movement disorders : official journal of the Movement Disorder SocietyOptimal Protocol and Clinical Usefulness of 123I-MIBG Cardiac Scintigraphy for Differentiation of Parkinson's Disease and Dementia with Lewy Body from Non-Parkinson's Diseases.
Nuclear medicine and molecular imagingDiagnosis across a cohort of "atypical" atypical and complex parkinsonism.
Parkinsonism & related disordersSignificance of clinical symptoms and red flags in early differential diagnosis of Parkinson's disease and atypical Parkinsonian syndromes.
Journal of neural transmission (Vienna, Austria : 1996)Genetics of Multiple System Atrophy and Progressive Supranuclear Palsy: A Systemized Review of the Literature.
International journal of molecular sciencesProgression of atypical parkinsonian syndromes: PROSPECT-M-UK study implications for clinical trials.
Brain : a journal of neurologySleep disturbances in progressive supranuclear palsy syndrome (PSPS) and corticobasal syndrome (CBS).
Neurologia i neurochirurgia polskaComparative analysis of freezing of gait in distinct Parkinsonism types by diffusion tensor imaging method and cognitive profiles.
Journal of neural transmission (Vienna, Austria : 1996)CSF Aβ42 and Aβ42/Aβ40 Ratio in Alzheimer's Disease and Frontotemporal Dementias.
Diagnostics (Basel, Switzerland)Retinal Structure Abnormalities in Parkinson's Disease and Atypical Parkinsonism.
BiomoleculesThe challenging quest of neuroimaging: From clinical to molecular-based subtyping of Parkinson disease and atypical parkinsonisms.
Handbook of clinical neurologyComplement system changes in blood in Parkinson's disease and progressive Supranuclear Palsy/Corticobasal Syndrome.
Parkinsonism & related disordersComparison of mean diffusivity, R2* relaxation rate and morphometric biomarkers for the clinical differentiation of parkinsonism.
Parkinsonism & related disordersHigher prevalence of idiopathic normal pressure hydrocephalus-like MRI features in progressive supranuclear palsy: An imaging reminder of atypical parkinsonism.
Brain and behaviorMulticlass prediction of different dementia syndromes based on multi-centric volumetric MRI imaging.
NeuroImage. ClinicalDifferences in Striatal Metabolism in [18F]FDG PET in Parkinson's Disease and Atypical Parkinsonism.
Diagnostics (Basel, Switzerland)Neuromelanin-Sensitive Magnetic Resonance Imaging Changes in the Locus Coeruleus/Subcoeruleus Complex in Patients with Typical and Atypical Parkinsonism.
Movement disorders : official journal of the Movement Disorder SocietyPDQ-8: A Simplified and Effective Tool Measuring Life Quality in Progressive Supranuclear Palsy.
Journal of Parkinson's diseaseThe Use of Cerebellar Hypoperfusion Assessment in the Differential Diagnosis of Multiple System Atrophy with Parkinsonism and Progressive Supranuclear Palsy-Parkinsonism Predominant.
Diagnostics (Basel, Switzerland)Oligomeric α-synuclein and tau aggregates in NDEVs differentiate Parkinson's disease from atypical parkinsonisms.
Neurobiology of diseaseA meta-analysis of the diagnostic utility of biomarkers in cerebrospinal fluid in Parkinson's disease.
NPJ Parkinson's diseaseClinical outcomes and cognitive impairments between progressive supranuclear palsy and multiple system atrophy.
Brain and behaviorRhythmic auditory cueing in atypical parkinsonism: A pilot study.
Frontiers in neurologySpeech acoustic indices for differential diagnosis between Parkinson's disease, multiple system atrophy and progressive supranuclear palsy.
NPJ Parkinson's diseaseQualitative evaluation of the Rey-Osterrieth Complex Figure Test in patients with progressive supranuclear palsy.
The Clinical neuropsychologistProgressive supranuclear palsy and corticobasal degeneration: novel clinical concepts and advances in biomarkers.
Arquivos de neuro-psiquiatriaThe Cognitive Profile of Atypical Parkinsonism: A Meta-Analysis.
Neuropsychology reviewThe Significance of Asymmetry in the Assessment of Brain Perfusion in Atypical Tauopathic Parkinsonian Syndromes.
Diagnostics (Basel, Switzerland)Parkinsonian Syndromes in Motor Neuron Disease: A Clinical Study.
Frontiers in aging neuroscienceCharacterization and diagnostic potential of R2* in early-stage progressive supranuclear palsy variants.
Parkinsonism & related disordersProbable progressive supranuclear palsy in a patient with chronic schizophrenia: A case report.
Experimental and therapeutic medicine[Atypical Parkinson's syndrome in old age].
Zeitschrift fur Gerontologie und GeriatrieCase Report: Presence of Anti-MAG in the CSF Can Be Associated With a Neurodegenerative Process With Frontal Involvement.
Frontiers in neurologyIs MRPI 2.0 More Useful than MRPI and M/P Ratio in Differential Diagnosis of PSP-P with Other Atypical Parkinsonisms?
Journal of clinical medicineThe promise of amplification assays for accurate early detection of α-synucleinopathies: A review.
Experimental gerontologyDecoding the dopamine transporter imaging for the differential diagnosis of parkinsonism using deep learning.
European journal of nuclear medicine and molecular imagingAdvanced diffusion imaging to track progression in Parkinson's disease, multiple system atrophy, and progressive supranuclear palsy.
NeuroImage. ClinicalMicroRNA as Candidate Biomarkers in Atypical Parkinsonian Syndromes: Systematic Literature Review.
Medicina (Kaunas, Lithuania)Differential diagnosis between Parkinson's disease and atypical parkinsonism based on gait and postural instability: Artificial intelligence using an enhanced weight voting ensemble model.
Parkinsonism & related disordersUtility of 18F FDG-PET in Parkinsonism in an African population.
eNeurologicalSciRegional Selectivity of Neuromelanin Changes in the Substantia Nigra in Atypical Parkinsonism.
Movement disorders : official journal of the Movement Disorder SocietyAutomated Differentiation of Atypical Parkinsonian Syndromes Using Brain Iron Patterns in Susceptibility Weighted Imaging.
Diagnostics (Basel, Switzerland)Patterns of Mixed Pathologies in Down Syndrome.
Journal of Alzheimer's disease : JADDopaminergic Correlates of Regional Cerebral Blood Flow in Parkinsonian Disorders.
Movement disorders : official journal of the Movement Disorder Society[Autonomic Dysfunction in Tauopathies].
Brain and nerve = Shinkei kenkyu no shinpoA Panel of Plasma Biomarkers for Differential Diagnosis of Parkinsonian Syndromes.
Frontiers in neuroscienceDifferential Diagnosis of Parkinsonism Based on Deep Metabolic Imaging Indices.
Journal of nuclear medicine : official publication, Society of Nuclear MedicineDifferential Diagnosis of Rare Subtypes of Progressive Supranuclear Palsy and PSP-Like Syndromes-Infrequent Manifestations of the Most Common Form of Atypical Parkinsonism.
Frontiers in aging neuroscienceThe Strengths and Obstacles in the Differential Diagnosis of Progressive Supranuclear Palsy-Parkinsonism Predominant (PSP-P) and Multiple System Atrophy (MSA) Using Magnetic Resonance Imaging (MRI) and Perfusion Single Photon Emission Computed Tomography (SPECT).
Diagnostics (Basel, Switzerland)Is the Phenotype Designation by PSP-MDS Criteria Stable Throughout the Disease Course and Consistent With Tau Distribution?
Frontiers in neurologyDiffusion microstructure imaging in progressive supranuclear palsy: reduced axonal volumes in the superior cerebellar peduncles, dentato-rubro-thalamic tracts, ventromedial thalami, and frontomesial white matter.
Cerebral cortex (New York, N.Y. : 1991)Complementary Utility of Dopamine Transporter and Tau PET Imaging in the Diagnosis of Progressive Supranuclear Palsy: A Case Report.
Clinical nuclear medicineEye-of-the-Tiger Sign with an Unexpected Pathological Diagnosis.
Movement disorders clinical practiceRole of Apolipoprotein E in the Clinical Profile of Atypical Parkinsonian Syndromes.
Alzheimer disease and associated disordersPain in neurodegenerative diseases with atypical parkinsonism: a systematic review on prevalence, clinical presentation, and findings from experimental studies.
Journal of integrative neuroscienceTherapeutic Application of rTMS in Atypical Parkinsonian Disorders.
Behavioural neurologyPresence of Skin α-Synuclein Deposits Discriminates Parkinson's Disease from Progressive Supranuclear Palsy and Corticobasal Syndrome.
Journal of Parkinson's diseaseCerebrospinal Fluid Levels of 5-Hydroxyindoleacetic Acid in Parkinson's Disease and Atypical Parkinsonian Syndromes.
Neuro-degenerative diseasesNeuroimaging in Parkinson's Disease: necessity or exaggeration?
Neurologia i neurochirurgia polskaNeurofilament light chain and α-synuclein RT-QuIC as differential diagnostic biomarkers in parkinsonisms and related syndromes.
NPJ Parkinson's disease"Parkinson's disease" on the way to progressive supranuclear palsy: a review on PSP-parkinsonism.
Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical NeurophysiologyNovel Therapies for Parkinsonian Syndromes-Recent Progress and Future Perspectives.
Frontiers in molecular neuroscienceCorpus callosum hypersignals and focal atrophy: Neuroimaging findings in globular glial tauopathy type I.
European journal of neurologyThe Genetic Landscape of Parkinsonism-Related Dystonias and Atypical Parkinsonism-Related Syndromes.
International journal of molecular sciencesLower urinary tract dysfunction in Parkinsonian syndromes.
Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology123I-Metaiodobenzylguanidine Myocardial Scintigraphy in Discriminating Degenerative Parkinsonisms.
Movement disorders clinical practicePrevalence and Characteristics of Polyneuropathy in Atypical Parkinsonian Syndromes: An Explorative Study.
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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.
- Chronological Diagnostic Algorithm Predicting Neuropathology in Parkinsonism.
- Early clinical characteristics of pathologically confirmed progressive supranuclear palsy and corticobasal degeneration.
- Longitudinal Evaluation of Polyneuropathy in Atypical Parkinsonian Syndromes.
- Multi-model Diffusion MRI Signatures in Atypical Parkinsonian Disorders.
- Neuroimaging and Pathology Biomarkers in Parkinson's Disease and Parkinsonism.
- An autopsy case of progressive supranuclear palsy with severe corticospinal tract degeneration.
- Training high level balance and stepping responses in atypical progressive supranuclear palsy: a case report.
- Association Between Globular Glial Tauopathies and Frontotemporal Dementia-Expanding the Spectrum of Gliocentric Disorders: A Review.
- Typical or atypical progressive supranuclear palsy: a comparative clinicopathologic study of three Chinese cases.
- A case of atypical progressive supranuclear palsy.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:99750(Orphanet)
- MONDO:0020488(MONDO)
- Esclerose Lateral Amiotrofica(PCDT · Ministério da Saúde)
- GARD:4507(GARD (NIH))
- Variantes catalogadas(ClinVar)
- Busca completa no PubMed(PubMed)
- Q55346109(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
