Category: Parkinson's Disease: Non-Motor Symptoms
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.
To cite this abstract in AMA style:
T. Fay Karmon, S. Hassin-Baer, Z. Shabtai, E. Druker, A. Leibowitz, Y. Sharabi. Longitudinal follow-up of pure autonomic failure with cardiac sympathetic denervation: Relevance to the body-first theory of Parkinson’s disease [abstract]. Mov Disord. 2023; 38 (suppl 1). 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/. Accessed September 7, 2024.« Back to 2023 International Congress
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/