Observational studyBrain : a journal of neurology2024
Phenoconversion in pure autonomic failure: a multicentre prospective longitudinal cohort study.
Observational study in Brain : a journal of neurology, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT01799915 (Natural History Study of Synucleinopathies), which is not on this map. Cited by 20 papers.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Natural History Study of Synucleinopathies
Who cites it
20 citing papers in PubMed, 27 citations in OpenAlex.
- Utility and best practices for clinical diagnostic testing of the autonomic nervous system: A Consensus Statement Endorsed by the American Autonomic Society and the American Association of Neuromuscular & Electrodiagnostic Medicine.Clinical autonomic research : official journal of the Clinical Autonomic Research Society · 2026Review
- Multiple system atrophy: cure and care.Journal of neural transmission (Vienna, Austria : 1996) · 2026Review
- Prospective Validation of the Movement Disorder Society Prodromal Multiple System Atrophy Criteria in Pure Autonomic Failure.Movement disorders : official journal of the Movement Disorder Society · 2026Article
- Lower Urinary Tract Symptoms as an Indicator of Occult Neurologic Disease: A System-first Framework for Urologic Practice.Current urology reports · 2026Review
- Skin-Based α-Synuclein Deposits Detection Across the Prodromal Continuum of Synucleinopathies: Updated Evidence and Perspectives.Biomolecules · 2026Review
- How to treat cardiovascular autonomic failure in Parkinson's disease.Journal of neural transmission (Vienna, Austria : 1996) · 2026Review
- Characteristics and mechanisms of cognitive impairment in Parkinson disease.Nature reviews. Neurology · 2026Review
- Preclinical Catecholaminergic Biomarkers of Central Lewy Body Diseases.Journal of clinical neurology (Seoul, Korea) · 2026Review
- A case report of syndrome of inappropriate antidiuretic hormone secretion unveiling hypothalamic involvement in multiple system atrophy.Frontiers in endocrinology · 2026Article
- Approach to Cardiovascular Autonomic Dysfunction in Patients with Synucleinopathies.Movement disorders clinical practice · 2025Review
- Autonomic dysfunction in multiple system atrophy: from pathophysiology to clinical manifestations.Annals of medicine · 2025Review
- 3,4-Dihydroxyphenylglycol levels separate multiple system atrophy from Parkinson disease with orthostatic hypotension.Clinical autonomic research : official journal of the Clinical Autonomic Research Society · 2025Observational
- Multimodal Autonomic Biomarkers Predict Phenoconversion in Pure Autonomic Failure.Annals of clinical and translational neurology · 2025Observational
- Blood Pressure Variability as a Diagnostic Marker for α-Synucleinopathy in Patients With Orthostasis.Journal of the American Heart Association · 2025Article
- The Gut-Brain Axis in Parkinson disease: Emerging Concepts and Therapeutic Implications.Movement disorders clinical practice · 2025Review
- The phenotype of "pure" autonomic failure.Clinical autonomic research : official journal of the Clinical Autonomic Research Society · 2025Article
- Sympathetic and parasympathetic subtypes of body-first Lewy body disease observed in postmortem tissue from prediagnostic individuals.Nature neuroscience · 2025Article
- Decreased urinary excretion of norepinephrine and dopamine in autonomic synucleinopathies.Clinical autonomic research : official journal of the Clinical Autonomic Research Society · 2025Article
- Central Involvement in Pure Autonomic Failure: Insights from Neuromelanin-Sensitive Magnetic Resonance Imaging andMovement disorders : official journal of the Movement Disorder Society · 2025Article
- Blunted tachycardia and cardiac sympathetic denervation in isolated rapid eye movement sleep behavior disorder.BMC neurology · 2024Article
Corrections and comments
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Authors and funding
18 authors at 8 institutions in 2 countries.
Funding
Abstract
We aimed to describe the clinical features of patients with pure autonomic failure (PAF) preceding phenoconversion that could be useful as predictive markers for advancing α-synuclein-associated neurodegeneration of the brain. Patients diagnosed with PAF were evaluated at eight centres (seven US-based and one European) and enrolled in a longitudinal observational cohort study (NCT01799915). Subjects underwent detailed assessments of motor, sleep, olfactory, cognitive and autonomic function and were followed prospectively to determine whether they developed parkinsonism or dementia for up to 10 years. We identified incident cases of Parkinson's disease (PD), dementia with Lewy bodies (DLB) or multiple system atrophy (MSA) and computed hazard ratios for phenoconversion as functions of clinical features. A total of 209 participants with PAF with a median disease duration of 6 years (IQR: 3-10) were enrolled. Of those, 149 provided follow-up information at an office or telemedicine visit. After a mean follow-up duration of 3 years, 48 (33%) participants phenoconverted (42% to PD, 35% to DLB and 23% to MSA). Faster phenoconversion from study enrolment to any diagnosis was associated with urinary and sexual dysfunction [hazard ratio (HR) 5.9, 95% confidence interval (CI): 1.6-22 and HR: 3.6, 95% CI: 1.1-12] followed by subtle motor signs (HR: 2.7, 95% CI: 1.2-6), trouble swallowing (HR 2.5, 95% CI: 1.4-4.5) and changes in speech (HR:2.4, 95% CI:1.1-4.8) at enrolment. Subjects reporting deterioration of handwriting were more likely to phenoconvert to PD (HR: 2.6, 95% CI: 1.1-5.9) and those reporting difficulty handling utensils were more likely to phenoconvert to DLB (HR: 6.8, 95% CI: 1.2-38). Patients with a younger age of PAF onset (HR: 11, 95% CI: 2.6-46), preserved olfaction (HR: 8.7, 95% CI: 1.7-45), anhidrosis (HR: 1.8, 95% CI: 1-3.1, P = 0.042) and severe urinary problems (HR 1.6, 95% CI: 1-2.5, P = 0.033) were more likely to phenoconvert to MSA. The best autonomic predictor of PD was a blunted heart rate increase during the tilt-table test (HR: 6.1, 95% CI: 1.4-26). Patients with PAF have an estimated 12% (95% CI: 9-15%) per year annual risk following study entry of phenoconverting to a manifest CNS synucleinopathy.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.