Peripheral sudomotor abnormalities in postural tachycardia syndrome (POTS)
Anna Newman1, Guillaume Lamotte2, Jordan Langford1, Luke Heyliger2, Eric Tokita1, Melissa Cortez2
1University of Utah School of Medicine, 2University of Utah Neurology
Objective:

To evaluate the frequency and distribution of sudomotor abnormalities in individuals who presented to the autonomic laboratory and, subsequently, received a diagnosis of postural tachycardia syndrome (POTS).

Background:

Neuropathic POTS is one of several physiologically defined subtypes and has been investigated using quantitative sudomotor axon reflex testing (QSART), skin biopsy for intra-epidermal nerve fiber density (IENFD), quantitative sensory testing (QST), autonomic testing, and other investigative methods. Sympathetic neuronal denervation, leading to excessive venous pooling and reflex tachycardia, has been hypothesized as a key contributor to POTS. Sudomotor abnormalities have been reported in 38-75% of POTS patients, though sample sizes were small and/or relatively selected (6-152 subjects).

Design/Methods:

The University of Utah Autonomic Physiology Laboratory database was used to retrospectively investigate the proportion of patients with quantifiable sudomotor dysfunction as measured by > 1 abnormal sudomotor sites on QSART testing during standardized autonomic reflex screening, based on a cutoff of <5th-percentile for sex (Sletten et al. 2015). This study was IRB approved, and consent waiver obtained. Inclusion criteria: laboratory diagnosis of POTS, confirmed by 10-minute head-up tilt table; age > 20. Exclusion: sudomotor medication effect; missing data.

Results:

374 patients, aged 20-65 were analyzed (2/2015-3/2022); 86% female. Of these, 79 (21%) had > 1 abnormal QSART site: 27 (34%) foot, 27 (34%) distal leg, 33 (42%) proximal leg, 29 (37%) forearm. The composite autonomic severity score (CASS) was > 1 in 243 (65%).

Conclusions:

Our findings suggest that peripheral sudomotor impairment may be present in a lower proportion of POTS patients than previously reported. However, CASS was > 1 in over half of subjects. Studies using multimodal assessment to categorize neuropathic POTS tended to support a higher percentage of POTS subjects with neuropathic features, suggesting that QSART alone may be insufficient to detect neuropathic POTS.

10.1212/WNL.0000000000203954