Koay, S;
Vichayanrat, E;
Bremner, F;
Valerio, F;
Mackenzie, R;
Chiaro, G;
Ingle, G;
... Iodice, V; + view all
(2025)
Multimodal Autonomic Biomarkers Predict Phenoconversion in Pure Autonomic Failure.
Annals of Clinical and Translational Neurology
10.1002/acn3.70140.
(In press).
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Ann Clin Transl Neurol - 2025 - Koay - Multimodal Autonomic Biomarkers Predict Phenoconversion in Pure Autonomic Failure.pdf - Published Version Download (1MB) | Preview |
Abstract
Background: Pure autonomic failure (PAF) presents with autonomic failure without other neurological features. A third develop central neurological features, fulfilling criteria for multiple system atrophy (MSA) and Lewy body diseases (LBD), including Parkinson's disease and Dementia with Lewy bodies. We hypothesized multimodal autonomic biomarkers would identify differences between PAF, MSA, and LBD, and predict phenoconversion in patients presenting with PAF. Methods: This observational cohort study included 391 alpha-synucleinopathy patients evaluated with cardiovascular autonomic testing, plasma noradrenaline, pupillometry, autonomic symptom, and quality-of-life questionnaires. PAF patients were monitored for the emergence of central neurological features. Logistic regression modeling was used to identify autonomic biomarkers at initial assessment that predicted future phenoconversion. Results: Patients with PAF had more severe orthostatic hypotension, lower supine plasma noradrenaline, and frequent sympathetic pupillary deficits at initial assessment than MSA and LBD. 50/194 (26%) with PAF phenoconverted to MSA or LBD after a median of 13 years, with normal pupils, heart rate response to deep breathing ≥ 10 bpm, and supine plasma noradrenaline ≥ 200 pg/mL predicting future phenoconversion to MSA or LBD, with younger age at presentation and higher supine plasma noradrenaline levels associated with conversion to MSA. Conclusion: In patients presenting with PAF, normal pupillary function and supine plasma noradrenaline levels with intact cardiovagal responses were red flags for future phenoconversion. Younger patients with higher supine plasma noradrenaline levels were more likely to convert to MSA rather than LBD. A non-invasive multimodal autonomic assessment can help differentiate between alpha-synucleinopathies and predict phenoconversion from PAF to MSA or LBD.
| Type: | Article |
|---|---|
| Title: | Multimodal Autonomic Biomarkers Predict Phenoconversion in Pure Autonomic Failure |
| Location: | United States |
| Open access status: | An open access version is available from UCL Discovery |
| DOI: | 10.1002/acn3.70140 |
| Publisher version: | https://doi.org/10.1002/acn3.70140 |
| Language: | English |
| Additional information: | © 2025 The Author(s). Annals of Clinical and Translational Neurology published by Wiley Periodicals LLC on behalf of American Neurological Association. This is an open access article under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0/). |
| Keywords: | autonomic testing, multiple system atrophy, orthostatic hypotension, Parkinson's disease, pure autonomic failure |
| UCL classification: | UCL UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology > Department of Neuromuscular Diseases |
| URI: | https://discovery.ucl.ac.uk/id/eprint/10212894 |
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