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Review
. 2023 Dec;33(6):737-747.
doi: 10.1007/s10286-023-00987-1. Epub 2023 Oct 16.

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

Abhishek Lenka  1   2 , Risa Isonaka  1 , Courtney Holmes  1 , David S Goldstein  3
Affiliations
Review

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

Abhishek Lenka et al. Clin Auton Res. 2023 Dec.

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.

Keywords: Dementia with Lewy bodies; Fluorodopamine; Multiple system atrophy; Parkinson’s disease; Pure autonomic failure.

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