Objective: In this report, we describe eight patients with PAF with nOH and imaging evidence of cardiac sympathetic denervation by ¹²³I-metaiodobenzylguanidine (¹²³I-MBG) single photon emission tomography (SPECT).

Background: Pure autonomic failure (PAF) is a rare disease primarily affecting the autonomic nervous system and manifested by neurogenic orthostatic hypotension (nOH), with a significant impact on quality of life. The natural history of PAF varies; PAF defined clinically may evolve into a central synucleinopathy. There is a need for pathophysiologically relevant biomarkers of preclinical central synucleinopathies.

Method: The patients were followed over a mean of 9.7 years for the development of Parkinson’s disease (PD) or neuroimaging evidence of central dopamine deficiency by ¹⁸F-DOPA positron emission tomography (PET).

Results: Five were diagnosed with PD during follow-up. Two showed non-diagnostic extrapyramidal signs, and 1 showed asymptomatic, unilaterally reduced ¹⁸F-DOPA-uptake. In conclusion, in this series, most patients with PAF and imaging evidence of cardiac sympathetic denervation progressed to PD during follow-up, and the remainder developed clinical or laboratory evidence of nigrostriatal dopamine deficiency.

Conclusion: This form of PAF seems to represent a body-first course toward PD.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/longitudinal-follow-up-of-pure-autonomic-failure-with-cardiac-sympathetic-denervation-relevance-to-the-body-first-theory-of-parkinsons-disease/