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Cardiac 18F-dopamine positron emission tomography predicts the type of phenoconversion of pure autonomic failure

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Abstract

Purpose

Pure autonomic failure (PAF) is a rare disease characterized by neurogenic orthostatic hypotension (nOH), no known secondary cause, and lack of a neurodegenerative movement or cognitive disorder. Clinically diagnosed PAF can evolve (“phenoconvert”) to a central Lewy body disease [LBD, e.g., Parkinson’s disease (PD) or dementia with Lewy bodies (DLB)] or to the non-LBD synucleinopathy multiple system atrophy (MSA). Since cardiac 18F-dopamine-derived radioactivity usually is low in LBDs and usually is normal in MSA, we hypothesized that patients with PAF with low cardiac 18F-dopamine-derived radioactivity would be more likely to phenoconvert to a central LBD than to MSA.

Methods

We reviewed data from all the patients seen at the National Institutes of Health Clinical Center from 1994 to 2023 with a clinical diagnosis of PAF and data about 18F-dopamine positron emission tomography (PET).

Results

Nineteen patients (15 with low 18F-dopamine-derived radioactivity, 4 with normal radioactivity) met the above criteria and had follow-up data. Nine (47%) phenoconverted to a central synucleinopathy over a mean of 6.6 years (range 1.5–18.8 years). All 6 patients with low cardiac 18F-dopamine-derived radioactivity who phenoconverted during follow-up developed a central LBD, whereas none of 4 patients with consistently normal 18F-dopamine PET phenoconverted to a central LBD (p = 0.0048), 3 evolving to probable MSA and 1 upon autopsy having neither a LBD nor MSA.

Conclusion

Cardiac 18F-dopamine PET can predict the type of phenoconversion of PAF. This capability could refine eligibility criteria for entry into disease-modification trials aimed at preventing evolution of PAF to symptomatic central LBDs.

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Funding

The research reported here was supported (in part) by the Division of Intramural Research, NINDS, NIH, Bethesda, MD, USA. None of the authors has a financial disclosure to make.

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Authors and Affiliations

  1. Autonomic Medicine Section, National Institute of Neurological, Disorders and Stroke (NINDS), National Institutes of Health (NIH), CNP/DIR/NINDS/NIH, 9000 Rockville Pike MSC-1620, Building 10 Room 8N260, Bethesda, MD, 20892, USA

    Abhishek Lenka, Risa Isonaka, Courtney Holmes & David S. Goldstein

  2. Parkinson’s Disease Center and Movement Disorders Clinic, Department of Neurology, Baylor College of Medicine, Houston, TX, USA

    Abhishek Lenka

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  1. Abhishek Lenka
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  2. Risa Isonaka
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Correspondence to David S. Goldstein.

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Lenka, A., Isonaka, R., Holmes, C. et al. Cardiac 18F-dopamine positron emission tomography predicts the type of phenoconversion of pure autonomic failure. Clin Auton Res 33, 737–747 (2023). https://doi.org/10.1007/s10286-023-00987-1

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