A Insuficiência Autonômica Pura (IAP) é uma doença neurodegenerativa que afeta a parte simpática do sistema nervoso autônomo e que se manifesta com pressão baixa ao se levantar.
Introdução
O que você precisa saber de cara
A Insuficiência Autonômica Pura (IAP) é uma doença neurodegenerativa que afeta a parte simpática do sistema nervoso autônomo e que se manifesta com pressão baixa ao se levantar.
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
+ 8 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 10 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
Nenhum gene associado encontrado
Os dados genéticos desta condição ainda estão sendo catalogados.
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 — Falência autonômica pura
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Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.
Pesquisa ativa
Ensaios clínicos abertos e novidades científicas recentes
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Outros ensaios clínicos
70 ensaios clínicos encontrados, 14 ativos.
Publicações mais relevantes
Pure autonomic failure: a natural history study of the Queen Square cohort.
The current research challenge in pure autonomic failure (PAF) lies in identifying specific biomarkers that can differentiate it from the other Lewy body disorders (Parkinson's disease, Parkinson's disease dementia, dementia with Lewy bodies) and multiple system atrophy in the early stages and predict phenoconversion trajectories to more widespread impairment. In this study, we described the natural history of our cohort of patients with PAF over five decades and validated a cluster of clinical, autonomic, and neuroimaging biomarkers that help identify clinical profiles susceptible to further neurodegeneration, working towards a biological definition of PAF. Consecutive patients with an initial diagnosis of PAF were recruited and monitored through key milestones (disease onset, first and repeat autonomic assessment, phenoconversion, and death/final contact). A subset underwent brain MRI and DaTSCAN (dopamine transporter single-photon emission CT scan). Uni- and multivariate regression analyses explored the associations among different factors, survival times, and phenoconversion, and were used to predict the probability of phenoconversion. Altogether, 281 patients with PAF were followed for a median of 10 years. Of these, 33% (91) converted to a more widespread synucleinopathy, and 41% (115) died during follow-up, of whom 53% (61) retained a PAF phenotype. Baseline cardiovascular autonomic biomarkers were key in differentiating disease trajectories and repeat testing indicated worsening of autonomic failure during the disease course. Median survival of patients with PAF was 15 years from orthostatic symptoms onset and was mostly influenced by age and the severity of orthostatic hypotension. Overall, 39% of patients had abnormal DaTSCAN results up to 7 years before phenoconversion, with 84% of these patients progressing to more widespread synucleinopathy. Male sex, older age, dream enactment behaviour and supine noradrenaline levels >200 pg/ml correlated with the risk of phenoconversion to Lewy body disorders, whereas younger age, bladder dysfunction, catheter use and dream enactment behaviour were associated with phenoconversion to multiple system atrophy. Our natural history study involves the largest single-centre longitudinal cohort of patients with an initial diagnosis of PAF and identifies robust clinical, autonomic, and neuroimaging biomarkers that, when used together, could serve as a novel and sensitive screening tool for early identification and stratification of patients at risk of phenoconversion to more widespread synucleinopathy.
Pure autonomic failure as an essential window into progression of synucleinopathies.
Differential patterns of central synucleinopathy and catecholaminergic abnormalities in Lewy body diseases and multiple system atrophy.
Parkinson's disease (PD), pure autonomic failure (PAF), and multiple system atrophy (MSA) feature intracellular deposition of alpha-synuclein and catecholamine deficiency in the putamen or heart. This retrospective, cross-sectional, observational study assessed relationships of cerebrospinal fluid alpha-synuclein seed amplification assay (CSF SAA) data with catecholamine deficiency indicated by positron emission tomography (PET). In groups with PD, PAF, parkinsonian MSA (MSA-P), or cerebellar MSA (MSA-C) SAAs were conducted by Rocky Mountain Laboratories (RML) and Amprion, Inc. using different assay conditions. 18F-DOPA PET examined putamen dopaminergic innervation, and 18F-dopamine PET assessed cardiac noradrenergic innervation. CSF SAAs by both RML and Amprion separated PD or PAF from MSA. The Amprion assay detected type 1 seeding associated with Lewy body diseases (LBDs) in 22/24 (92%) PD patients and 15/16 (94%) PAF patients and type 2 seeding associated with MSA in 9/10 (90%) MSA-P and 3/4 (75%) MSA-C patients (p < 0.0001). All of 24 PD and all of 9 MSA-P patients had low putamen/occipital cortex ratios of 18F-DOPA-derived radioactivity, while 11/13 (85%) PAF patients and 4/4 (100%) MSA-C patients had normal ratios. Contingency analyses of CSF SAA and brain 18F-DOPA PET efficiently separated the 4 groups (p < 0.0001). CSF SAAs and cardiac 18F-dopamine PET distinguish LBDs from MSA but not PAF from PD or MSA-P from MSA-C. 18F-DOPA PET separates PAF from PD and MSA-P from MSA-C. Combining biomarkers differentiates among these synucleinopathies.
Emerging Role of Liquid Chromatography-Mass Spectrometry in the Clinical Laboratory Evaluation of Chronic Autonomic Failure.
Liquid chromatography with electrochemical detection (LC-ED) after batch alumina extraction has been the mainstay for assaying levels of catecholamines and related 3,4-dihydroxy compounds (catechols) as part of the clinical laboratory workup of patients with neurogenic orthostatic hypotension, especially in the setting of the autonomic synucleinopathies Parkinson disease with orthostatic hypotension (PD + OH), pure autonomic failure (PAF), and multiple system atrophy (MSA). Liquid chromatography with tandem mass spectrometry (LC-MS/MS) is faster and measures catechols and non-catechol metabolites simultaneously but has not yet been validated sufficiently against LC-ED or used to assess catechol vs. non-catechol neurochemical abnormalities in autonomic synucleinopathies. We measured plasma catechols by LC-MS/MS and LC-ED in patients with PAF, PD + OH, or MSA and healthy controls. Cardiac sympathetic neuroimaging by 18F-dopamine positron emission tomography (PET) was used to indicate myocardial norepinephrine (NE) content in the same subjects. Across 41 participants (12 PAF, 9 PD + OH, 10 MSA, 10 controls) individual values for plasma 3,4-dihydroxyphenylglycol (DHPG), NE, and 3,4-dihydroxyphenylalanine (DOPA) by LC-MS/MS correlated positively with values by LC-ED (r = 0.97, 0.98, and 1.00, p < 0.0001 each). The PAF group had low mean NE, DHPG, normetanephrine, 3-methoxy-4-hydroxyphenylglycol, epinephrine, and metanephrine compared to the PD + OH group, while cardiac PET did not separate the 2 groups. We therefore conclude that LC-MS/MS validly assays plasma catechols. Several catechol and non-catechol biomarkers of generalized catecholamine deficiency separate PAF from PD + OH but not PD + OH from MSA, while 18F-dopamine PET separates PAF and PD + OH from MSA but not PAF from PD + OH. Combining LC-MS/MS with cardiac sympathetic neuroimaging efficiently differentiates among these conditions.
Association of Autonomic Dysfunction With Long COVID: Evaluation Using Quantitative Autonomic Testing.
Persistent symptoms (eg, heart palpitations, lightheadedness, fatigue) despite resolution of acute COVID-19 infection is termed "long COVID syndrome" or simply "long COVID." Long COVID is believed to be associated with autonomic dysfunction, but the nature and severity of any autonomic disturbances are not well understood. This study sought to compare autonomic function measures in patients with long COVID, control subjects, and individuals with pure autonomic failure. Patients referred for autonomic testing were classified into 3 groups: long COVID (acute COVID-19 infection ≥12 weeks before testing), control subjects (COVID-19 negative, normal autonomic tests), and pure autonomic failure (COVID-19 negative, abnormal autonomic testing). Heart rate and blood pressure were recorded during active standing, Valsalva maneuver, respiratory sinus arrhythmia, and tilt-table testing. Compared with control subjects, patients with long COVID exhibited both a greater heart rate increase and blood pressure drop with active standing and tilt-table testing (all P < 0.05). They also had lower Valsalva ratios and respiratory sinus arrhythmia values than did control subjects (both P < 0.05). Compared with pure autonomic failure patients, patients with long COVID had a greater heart rate increase but a lower drop in blood pressure with active standing and tilt-table testing and lesser respiratory sinus arrhythmia values and Valsalva ratios (all P < 0.001). After age and sex adjustment, autonomic dysfunction measures in patients with long COVID were comparable with those in the pure autonomic failure group. Further, autonomic testing abnormalities were observed in patients referred up to 40 months after infection. When adjusted for age and sex, patients with long COVID may demonstrate persistent autonomic dysfunction that is similar to patients with pure autonomic failure.
Publicações recentes
CSF alpha-Synuclein Seed Amplification Assay results in routine clinically collected samples.
Skin-Based α-Synuclein Deposits Detection Across the Prodromal Continuum of Synucleinopathies: Updated Evidence and Perspectives.
Differential patterns of central synucleinopathy and catecholaminergic abnormalities in Lewy body diseases and multiple system atrophy.
Emerging Role of Liquid Chromatography-Mass Spectrometry in the Clinical Laboratory Evaluation of Chronic Autonomic Failure.
Pure autonomic failure as an essential window into progression of synucleinopathies.
📚 EuropePMC144 artigos no totalmostrando 190
Differential patterns of central synucleinopathy and catecholaminergic abnormalities in Lewy body diseases and multiple system atrophy.
Parkinsonism & related disordersEmerging Role of Liquid Chromatography-Mass Spectrometry in the Clinical Laboratory Evaluation of Chronic Autonomic Failure.
Journal of neurochemistryPure autonomic failure as an essential window into progression of synucleinopathies.
Brain : a journal of neurologyShould Neurogenic Supine Hypertension Be Treated? Insights from Hypertension-Mediated Organ Damage Studies-A Narrative Review.
BiomedicinesLong COVID as Intermediate Physiology: Rethinking Autonomic Dysfunction and Medical Uncertainty.
Journal of the American College of CardiologyAssociation of Autonomic Dysfunction With Long COVID: Evaluation Using Quantitative Autonomic Testing.
Journal of the American College of CardiologyQuality of life and disability in pure autonomic failure: More than a prodromal synucleinopathy.
Parkinsonism & related disordersEstablishing minimally clinically important differences for the orthostatic hypotension questionnaire (OHQ).
Clinical autonomic research : official journal of the Clinical Autonomic Research SocietyIdiopathic polyneuropathy with neurogenic autonomic failure - an early manifestation of Lewy body disease? a case report.
BMC neurologyCardiac MIBG scintigraphy in pure autonomic failure: A systematic review.
Journal of the neurological sciencesMultitracer PET to Assess Cardiac Sympathetic Innervation and Vesicular Storage in Lewy Body Diseases.
Journal of nuclear medicine : official publication, Society of Nuclear MedicineApproach to Cardiovascular Autonomic Dysfunction in Patients with Synucleinopathies.
Movement disorders clinical practiceCardiac 123I-Meta-Iodobenzylguanidine Imaging as a Biomarker for Body-First Parkinson's Disease: Linking Peripheral α-Synuclein to Clinical Subtyping.
Journal of movement disordersHarlequin syndrome: using clinical features and autonomic testing to unmask the disorder.
Clinical autonomic research : official journal of the Clinical Autonomic Research SocietyMultimodal Autonomic Biomarkers Predict Phenoconversion in Pure Autonomic Failure.
Annals of clinical and translational neurologyAutonomic nervous system dysregulation in neurodegenerative diseases: Bridging brain and heart.
Progress in brain researchThe MSA Atrophy Index (MSA-AI): An Imaging Marker for Diagnosis and Clinical Progression in Multiple System Atrophy.
Annals of clinical and translational neurologyCrosstalk between bladder-cardiovascular autonomic nervous system in synucleinopathies.
Autonomic neuroscience : basic & clinicalPrevalence of Supine Hypertension in Alpha-Synucleinopathies: A Systematic Review and Meta-Analysis.
Movement disorders clinical practicePure autonomic failure: a natural history study of the Queen Square cohort.
Brain : a journal of neurologyThe Usefulness of 131 I-MIBG Myocardial Scintigraphy in a Case of Pure Autonomic Failure - First Report in the Literature.
Annals of Indian Academy of NeurologyPure autonomic failure, phenoconversion phenomenon and autonomic non-motor subtype of Parkinson disease: The chicken or the egg?
Parkinsonism & related disordersThe phenotype of "pure" autonomic failure.
Clinical autonomic research : official journal of the Clinical Autonomic Research SocietySustained therapeutic effect of spinal cord stimulation on improving severe neurogenic orthostatic hypotension in a patient with pure autonomic failure converting to multiple system atrophy.
Journal of neurologyCentral Involvement in Pure Autonomic Failure: Insights from Neuromelanin-Sensitive Magnetic Resonance Imaging and 18F-Fluorodopa-Positron Emission Tomography.
Movement disorders : official journal of the Movement Disorder SocietyPathophysiological Significance of α-Synuclein in Sympathetic Nerves: In Vivo Observations.
NeurologyClinical Correlates of Efficacy of Pyridostigmine in the Treatment of Orthostatic Hypotension.
Hypertension (Dallas, Tex. : 1979)Decreased urinary excretion of norepinephrine and dopamine in autonomic synucleinopathies.
Clinical autonomic research : official journal of the Clinical Autonomic Research SocietyA-synuclein prion strains differentially adapt after passage in mice.
PLoS pathogensCutaneous phosphorylated-synuclein: an early diagnostic biomarker for pure autonomic failure.
Journal of neurology, neurosurgery, and psychiatrySARS-CoV-2 Infection and Alpha-Synucleinopathies: Potential Links and Underlying Mechanisms.
International journal of molecular sciencesPelvic autonomic dysfunction is common in patients with pure autonomic failure.
European journal of neurologyIntrasubject reproducibility of supine norepinephrine plasma concentrations in patients with cardiovascular sympathetic failure.
Autonomic neuroscience : basic & clinicalBiofluid Detection of Pathological α-Synuclein in the Prodromal Phase of Synucleinopathies.
Journal of Parkinson's diseaseCerebrospinal fluid concentration gradients of catechols in synucleinopathies.
Journal of neurochemistryAn update on multiple system atrophy.
Current opinion in neurologyParkinson's Disease and Dementia with Lewy Bodies: One and the Same.
Journal of Parkinson's diseaseSkin Biopsy Detection of Phosphorylated α-Synuclein in Patients With Synucleinopathies.
JAMAClinical Characteristics and Outcomes in Young-Onset Multiple System Atrophy.
Movement disorders clinical practiceTilt-evoked, breathing-driven blood pressure oscillations: Independence from baroreflex-sympathoneural function.
Clinical autonomic research : official journal of the Clinical Autonomic Research SocietyPhenoconversion in pure autonomic failure: a multicentre prospective longitudinal cohort study.
Brain : a journal of neurologyUnveiling autonomic failure in synucleinopathies: Significance in diagnosis and treatment.
Revue neurologiqueHemodynamic recognition of pure autonomic failure: A case report.
Journal of cardiology casesAn overview on pure autonomic failure.
Revue neurologiquePrimary Sjogren's syndrome presenting as an isolated severe autonomic dysfunction treated with steroids.
BMJ case reportsAbnormal dopamine transporter imaging in pure autonomic failure: a potential biomarker of central nervous system involvement.
European journal of neurologyHemodynamic Effects of the Osmopressor Response: A Systematic Review and Meta-Analysis.
Journal of the American Heart AssociationCardiac 18F-dopamine positron emission tomography predicts the type of phenoconversion of pure autonomic failure.
Clinical autonomic research : official journal of the Clinical Autonomic Research SocietyAutonomic failure: Clinicopathologic, physiologic, and genetic aspects.
Handbook of clinical neurologyExploring the impact of caring for an individual with neurogenic orthostatic hypotension: a qualitative study.
Neurodegenerative disease managementPost-Traumatic Stress Disorder and Risk of Degenerative Synucleinopathies: Systematic Review and Meta-Analysis.
The American journal of geriatric psychiatry : official journal of the American Association for Geriatric PsychiatryAlpha-synuclein in Parkinson's disease and other synucleinopathies: from overt neurodegeneration back to early synaptic dysfunction.
Cell death & diseaseD-685 Reverses Motor Deficits and Reduces Accumulation of Human α-Synuclein Protein in Two Different Parkinson's Disease Animal Models.
ACS chemical neuroscienceSeed amplification assay for the detection of pathologic alpha-synuclein aggregates in cerebrospinal fluid.
Nature protocolsContinuous Positive Airway Pressure for the Treatment of Supine Hypertension and Orthostatic Hypotension in Autonomic Failure.
Hypertension (Dallas, Tex. : 1979)Imaging in autonomic failure: a window into real-time physiological mechanisms and other updates on recent autonomic research.
Clinical autonomic research : official journal of the Clinical Autonomic Research SocietyHealth-related quality-of-life and burden for caregivers of individuals with neurogenic orthostatic hypotension.
Neurodegenerative disease managementReal-Time Magnetic Resonance Imaging to Study Orthostatic Intolerance Mechanisms in Human Beings: Proof of Concept.
Journal of the American Heart AssociationAlterations in electrochemical skin conductance as a marker of autonomic dysfunction in multiple system atrophy.
Parkinsonism & related disordersPure Autonomic Failure-A Localized Alpha Synucleinopathy with a Potential for Conversion to More Extensive Alpha Synucleinopathies.
Annals of Indian Academy of NeurologyRenal Transplantation in Pure Autonomic Failure.
Transplantation direct[Evaluation of Disopyramide Efficacy for Refractory Syncope in Heart Failure with Preserved Ejection Fraction Using Holter Electrocardiography: A Case Report].
Yakugaku zasshi : Journal of the Pharmaceutical Society of JapanCerebrovascular regulation in patients with vasovagal syncope and autonomic failure due to familial amyloidotic polyneuropathy.
Autonomic neuroscience : basic & clinicalRecent advances in establishing fluid biomarkers for the diagnosis and differentiation of alpha-synucleinopathies - a mini review.
Clinical autonomic research : official journal of the Clinical Autonomic Research SocietyDiagnosing Premotor Multiple System Atrophy: Natural History and Autonomic Testing in an Autopsy-Confirmed Cohort.
Neurology[Dizziness upon standing: consider autonomic dysfunction].
Nederlands tijdschrift voor geneeskundeA Case of Pure Autonomic Failure Initially Presenting with Hemihypohidrosis: Twelve-Year Follow-Up.
Case reports in neurologyAlpha-Synuclein Strain Variability in Body-First and Brain-First Synucleinopathies.
Frontiers in aging neuroscienceSynuclein-One study: skin biopsy detection of phosphorylated α-synuclein for diagnosis of synucleinopathies.
Biomarkers in medicine[Autonomic Dysfunction in Lewy Body Disease].
Brain and nerve = Shinkei kenkyu no shinpo[Pathological Study of Pure Autonomic Failure].
Brain and nerve = Shinkei kenkyu no shinpoPassive Immunization in Alpha-Synuclein Preclinical Animal Models.
BiomoleculesMachine learning applied to ambulatory blood pressure monitoring: a new tool to diagnose autonomic failure?
Journal of neurologyRelationships Between Cardiovascular Autonomic Profile and Work Ability in Patients With Pure Autonomic Failure.
Frontiers in human neurosciencePure autonomic failure: the first report in Poland?
Polish archives of internal medicine[A case of pure autonomic failure that eventually developed into multiple system atrophy 5 years later].
Nihon Ronen Igakkai zasshi. Japanese journal of geriatricsThe role of cardiovascular autonomic failure in the differential diagnosis of α-synucleinopathies.
Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical NeurophysiologyInhibition of the norepinephrine transporter to treat neurogenic orthostatic hypotension: is this the end of the story?
Clinical autonomic research : official journal of the Clinical Autonomic Research SocietyLewy pathology of the submandibular gland in Lewy body disease: A report of autopsy cases.
Neuropathology : official journal of the Japanese Society of NeuropathologyDifferent phenoconversion pathways in pure autonomic failure with versus without Lewy bodies.
Clinical autonomic research : official journal of the Clinical Autonomic Research SocietyPure Autonomic Failure: A Case Report of Recurrent Orthostatic Hypotension.
JNMA; journal of the Nepal Medical AssociationPure autonomic failure and the differential diagnosis of autonomic peripheral neuropathies.
Current opinion in neurologyCardiology meets neurology: clinical presentation and management of patients with primary neurogenic disorders and orthostatic intolerance.
Herzschrittmachertherapie & ElektrophysiologieSynuclein in neurodegeneration.
Advances in clinical chemistryPredictors of the Pressor Response to the Norepinephrine Transporter Inhibitor, Atomoxetine, in Neurogenic Orthostatic Hypotension.
Hypertension (Dallas, Tex. : 1979)The new frontier in pure autonomic failure: getting a grip on cerebral blood flow.
Clinical autonomic research : official journal of the Clinical Autonomic Research SocietyDifferential abnormalities of cerebrospinal fluid dopaminergic versus noradrenergic indices in synucleinopathies.
Journal of neurochemistryDysautonomia: A Forgotten Condition - Part 1.
Arquivos brasileiros de cardiologiaAlpha-Synuclein Oligomers and Neurofilament Light Chain Predict Phenoconversion of Pure Autonomic Failure.
Annals of neurologyLocal Passive Heat for the Treatment of Hypertension in Autonomic Failure.
Journal of the American Heart AssociationDo indices of baroreflex failure and peripheral noradrenergic deficiency predict the magnitude of orthostatic hypotension in Lewy body diseases?
Clinical autonomic research : official journal of the Clinical Autonomic Research SocietyNeurogenic Orthostatic Hypotension. Lessons From Synucleinopathies.
American journal of hypertensionElevated cerebral blood flow in patients with pure autonomic failure.
Clinical autonomic research : official journal of the Clinical Autonomic Research SocietyCan novel non-invasive autonomic tests help discriminate between pure autonomic failure and multiple system atrophy?
Autonomic neuroscience : basic & clinicalClinical and neurohormonal characteristics in African Americans with neurogenic orthostatic hypotension.
Clinical autonomic research : official journal of the Clinical Autonomic Research SocietyCardioselective peripheral noradrenergic deficiency in Lewy body synucleinopathies.
Annals of clinical and translational neurologyLewy pathology of the esophagus correlates with the progression of Lewy body disease: a Japanese cohort study of autopsy cases.
Acta neuropathologicaRepeated hypoglycemia remodels neural inputs and disrupts mitochondrial function to blunt glucose-inhibited GHRH neuron responsiveness.
JCI insightComparison of 123I-MIBG scintigraphy and phosphorylated α-synuclein skin deposits in synucleinopathies.
Parkinsonism & related disordersPharmacologic treatment of orthostatic hypotension.
Autonomic neuroscience : basic & clinicalCardiac sympathetic innervation and vesicular storage in pure autonomic failure.
Annals of clinical and translational neurologyThe "Sick-but-not-Dead" Phenomenon Applied to Catecholamine Deficiency in Neurodegenerative Diseases.
Seminars in neurologyAutonomic Dysfunction in the Synucleinopathies.
Seminars in neurologyCerebrospinal fluid fistula in a patient with chronic constipation related to an autonomic dysfunction and revealed by bacterial meningitis - A case report.
Surgical neurology internationalIs Ross Syndrome a New Type of Synucleinopathy? A Brief Research Report.
Frontiers in neurosciencePredicting phenoconversion in pure autonomic failure.
NeurologyThe impact of supine hypertension on target organ damage and survival in patients with synucleinopathies and neurogenic orthostatic hypotension.
Parkinsonism & related disordersAn update on MSA: premotor and non-motor features open a window of opportunities for early diagnosis and intervention.
Journal of neurology"Sick-but-not-dead": multiple paths to catecholamine deficiency in Lewy body diseases.
Stress (Amsterdam, Netherlands)Ultrasensitive RT-QuIC assay with high sensitivity and specificity for Lewy body-associated synucleinopathies.
Acta neuropathologicaMechanisms of alpha-synuclein toxicity: An update and outlook.
Progress in brain researchDiagnosing multiple system atrophy at the prodromal stage.
Clinical autonomic research : official journal of the Clinical Autonomic Research SocietyAutonomic ganglionic injection of α-synuclein fibrils as a model of pure autonomic failure α-synucleinopathy.
Nature communicationsSynucleinopathies: Where we are and where we need to go.
Journal of neurochemistryAutonomic dysfunction in Parkinson's disease: Implications for pathophysiology, diagnosis, and treatment.
Neurobiology of diseaseThe catecholaldehyde hypothesis: where MAO fits in.
Journal of neural transmission (Vienna, Austria : 1996)Correlations of Neuropsychological and Metabolic Brain Changes in Parkinson's Disease and Other α-Synucleinopathies.
Frontiers in neurologyAutonomic dysfunction: Diagnosis and management.
Handbook of clinical neurologyOrthostatic Hypotension in Parkinson Disease.
Clinics in geriatric medicineSensitivity and specificity of cardiac 123I-MIBG scintigraphy for diagnosis of early-phase Parkinson's disease.
Journal of the neurological sciencesLong-term trends in myocardial sympathetic innervation and function in synucleinopathies.
Parkinsonism & related disordersCognitive and Behavioral Changes in Patients Treated With Droxidopa for Neurogenic Orthostatic Hypotension: A Retrospective Review.
Cognitive and behavioral neurology : official journal of the Society for Behavioral and Cognitive NeurologyPure autonomic failure presenting as Harlequin syndrome.
Autonomic neuroscience : basic & clinicalA case of autonomic failure in post-craniectomy syndrome of the trephined.
Clinical autonomic research : official journal of the Clinical Autonomic Research SocietySmall fiber neuropathy and phosphorylated alpha-synuclein in the skin of E46K-SNCA mutation carriers.
Parkinsonism & related disordersOrthostatic hypotension and REM sleep behaviour disorder: impact on clinical outcomes in α-synucleinopathies.
Journal of neurology, neurosurgery, and psychiatryRole of nurses and nurse practitioners in the recognition, diagnosis, and management of neurogenic orthostatic hypotension: a narrative review.
International journal of general medicineAutonomic Dysfunction in α-Synucleinopathies.
Frontiers in neurologyUsefulness of Blood Pressure Variability Indices Derived From 24-Hour Ambulatory Blood Pressure Monitoring in Detecting Autonomic Failure.
Journal of the American Heart AssociationDopamine transporter imaging versus myocardial MIBG scintigraphy for the diagnosis of prodromal synucleinopathies-and other updates on autonomic research.
Clinical autonomic research : official journal of the Clinical Autonomic Research SocietyDroxidopa for Hypotension of Different Etiologies: Two Case Reports.
P & T : a peer-reviewed journal for formulary managementBlood pressure circadian rhythm alterations in alpha-synucleinopathies.
Journal of neurologyMIBG Myocardial Scintigraphy Identifies Premotor PD/DLB During a Negative DAT Scan Period: Second Report.
Movement disorders clinical practiceDroxidopa for the treatment of neurogenic orthostatic hypotension in neurodegenerative diseases.
Expert opinion on pharmacotherapyMoving forward the in vivo diagnosis of the synucleinopathies.
Clinical autonomic research : official journal of the Clinical Autonomic Research SocietyPure Autonomic Failure with Asymptomatic Hypertensive Urgency: A Case Report and Literature Review.
Case reports in neurologySkin nerve α-synuclein deposits in Parkinson's disease and other synucleinopathies: a review.
Clinical autonomic research : official journal of the Clinical Autonomic Research SocietySkin α-synuclein deposits differ in clinical variants of synucleinopathy: an in vivo study.
Scientific reportsSupine plasma NE predicts the pressor response to droxidopa in neurogenic orthostatic hypotension.
NeurologyOrthostatic Hypotension: JACC State-of-the-Art Review.
Journal of the American College of CardiologyCharacterization of the symptoms of neurogenic orthostatic hypotension and their impact from a survey of patients and caregivers.
BMC neurologyRoles of cardiac sympathetic neuroimaging in autonomic medicine.
Clinical autonomic research : official journal of the Clinical Autonomic Research SocietyRapid eye movement sleep behavior disorder and the link to alpha-synucleinopathies.
Clinical neurophysiology : official journal of the International Federation of Clinical NeurophysiologyAutonomic dysfunction in genetic forms of synucleinopathies.
Movement disorders : official journal of the Movement Disorder SocietyTreatment of autonomic dysfunction in Parkinson disease and other synucleinopathies.
Movement disorders : official journal of the Movement Disorder SocietyOrthostatic heart rate changes in patients with autonomic failure caused by neurodegenerative synucleinopathies.
Annals of neurologySpectrum of abnormalities of sympathetic tyrosine hydroxylase and alpha-synuclein in chronic autonomic failure.
Clinical autonomic research : official journal of the Clinical Autonomic Research SocietySympathetic neurograms showing characteristics of both muscle and skin sympathetic nerve activity in a case with pure autonomic failure.
Clinical autonomic research : official journal of the Clinical Autonomic Research SocietyAutonomic dysfunction predicts poor outcome in stroke: Updates on recent autonomic research.
Clinical autonomic research : official journal of the Clinical Autonomic Research SocietyNeuropathology of autonomic dysfunction in synucleinopathies.
Movement disorders : official journal of the Movement Disorder SocietyThe effects of recurrent hypoglycaemia and opioid antagonists on the adrenal catecholamine synthetic capacity in a rat model of HAAF.
Autonomic neuroscience : basic & clinicalDoes sympathetic dysfunction occur before denervation in pure autonomic failure?
Clinical science (London, England : 1979)Diagnosis of multiple system atrophy.
Autonomic neuroscience : basic & clinicalSymptomatic therapy of multiple system atrophy.
Autonomic neuroscience : basic & clinicalTreatment of supine hypertension in autonomic failure: a case series.
Clinical autonomic research : official journal of the Clinical Autonomic Research SocietyThe heart of PD: Lewy body diseases as neurocardiologic disorders.
Brain researchHypothalamic Glucose Transport in Humans During Experimentally Induced Hypoglycemia-Associated Autonomic Failure.
The Journal of clinical endocrinology and metabolismLet's not forget about the vagus and other updates on recent autonomic research.
Clinical autonomic research : official journal of the Clinical Autonomic Research SocietyEpidemiology, Diagnosis, and Management of Neurogenic Orthostatic Hypotension.
Movement disorders clinical practiceManaging neurogenic orthostatic hypotension in a patient presenting with pure autonomic failure who later developed Parkinson disease.
Clinical autonomic research : official journal of the Clinical Autonomic Research SocietyPossible Involvement of Hypotension in Postprandial Headache: A Case Series.
HeadacheInitiating droxidopa for neurogenic orthostatic hypotension in a patient with Parkinson disease.
Clinical autonomic research : official journal of the Clinical Autonomic Research SocietyDroxidopa for Symptomatic Neurogenic Hypotension.
Cardiology in reviewIodine-123-meta-iodobenzylguanidine Myocardial Scintigraphy in Isolated Autonomic Failure: Potential Red Flag for Future Multiple System Atrophy.
Frontiers in neurologyREM Sleep Behavior Disorder in Parkinson's Disease and Other Synucleinopathies.
Movement disorders : official journal of the Movement Disorder SocietyLetter on "Natural history of pure autonomic failure: A United States prospective cohort".
Annals of neurologyVery late-onset pure autonomic failure.
Movement disorders : official journal of the Movement Disorder SocietyIs pure autonomic failure a distinct nosologic entity?
Clinical autonomic research : official journal of the Clinical Autonomic Research SocietyIs pure autonomic failure an early marker for Parkinson disease, dementia with Lewy bodies, and multiple system atrophy? And other updates on recent autonomic research.
Clinical autonomic research : official journal of the Clinical Autonomic Research SocietyPure autonomic failure without alpha-synuclein pathology: an evolving understanding of a heterogeneous disease.
Clinical autonomic research : official journal of the Clinical Autonomic Research SocietyPure autonomic failure: Predictors of conversion to clinical CNS involvement.
NeurologyPure autonomic failure without synucleinopathy.
Clinical autonomic research : official journal of the Clinical Autonomic Research SocietyPure autonomic failure versus prodromal dysautonomia in Parkinson’s disease: Insights from the bedside.
Movement disorders clinical practiceCardiovascular Safety of Droxidopa in Patients With Symptomatic Neurogenic Orthostatic Hypotension.
The American journal of cardiologyNatural history of pure autonomic failure: A United States prospective cohort.
Annals of neurologyThe recommendations of a consensus panel for the screening, diagnosis, and treatment of neurogenic orthostatic hypotension and associated supine hypertension.
Journal of neurologyThe Pharmacology of Autonomic Failure: From Hypotension to Hypertension.
Pharmacological reviewsAutoimmunity-associated autonomic failure with sympathetic denervation.
Clinical autonomic research : official journal of the Clinical Autonomic Research SocietySafety and Durability of Effect with Long-Term, Open-Label Droxidopa Treatment in Patients with Symptomatic Neurogenic Orthostatic Hypotension (NOH303).
Journal of Parkinson's diseaseA case series of REM sleep behavior disorder in pure autonomic failure.
Clinical autonomic research : official journal of the Clinical Autonomic Research SocietyLong-term safety of droxidopa in patients with symptomatic neurogenic orthostatic hypotension.
Journal of the American Society of Hypertension : JASHAnalysis of number needed to treat for droxidopa in patients with symptomatic neurogenic orthostatic hypotension.
BMC neurologySuperficial siderosis associated with peripheral autonomic failure and tetraventricular hydrocephalus: a case report.
Clinical autonomic research : official journal of the Clinical Autonomic Research SocietyElevated cerebrospinal fluid ratios of cysteinyl-dopamine/3,4-dihydroxyphenylacetic acid in parkinsonian synucleinopathies.
Parkinsonism & related disordersMeta-analysis of the safety and efficacy of droxidopa for neurogenic orthostatic hypotension.
Clinical autonomic research : official journal of the Clinical Autonomic Research SocietyCongenital Insensitivity to Pain (HSNA type IV).
Pediatric neurology briefs(123)I-meta-iodobenzylguanidine (MIBG) cardiac scintigraphy in α-synucleinopathies.
Ageing research reviewsMineralocorticoid Receptor Activation Contributes to the Supine Hypertension of Autonomic Failure.
Hypertension (Dallas, Tex. : 1979)Skin nerve misfolded α-synuclein in pure autonomic failure and Parkinson disease.
Annals of neurology[Acute Sensory Neuropathies and Acute Autonomic Neuropathies].
Brain and nerve = Shinkei kenkyu no shinpoAssociações
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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.
- Pure autonomic failure: a natural history study of the Queen Square cohort.
- Pure autonomic failure as an essential window into progression of synucleinopathies.
- Differential patterns of central synucleinopathy and catecholaminergic abnormalities in Lewy body diseases and multiple system atrophy.
- Emerging Role of Liquid Chromatography-Mass Spectrometry in the Clinical Laboratory Evaluation of Chronic Autonomic Failure.
- Association of Autonomic Dysfunction With Long COVID: Evaluation Using Quantitative Autonomic Testing.
- CSF alpha-Synuclein Seed Amplification Assay results in routine clinically collected samples.
- Skin-Based α-Synuclein Deposits Detection Across the Prodromal Continuum of Synucleinopathies: Updated Evidence and Perspectives.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:441(Orphanet)
- MONDO:0018608(MONDO)
- GARD:10428(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Q896883(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
