Small-fiber neuropathy with cardiac denervation in postural tachycardia syndrome
Abstract
Introduction: Postural tachycardia syndrome (POTS) is a disorder of orthostatic intolerance characterized by excessive tachycardia of unknown etiology. Our objective in this study was to evaluate the correlation between C‐fiber involvement as shown by skin biopsy and adrenergic cardiac metaiodobenzylguanadine (MIBG) uptake in POTS patients. Methods: Skin biopsies of 84 patients with POTS were examined by Protein Gene Product 9.5 (PGP9.5) immunohistochemistry and were compared with MIBG myocardial scintigraphy imaging data. Results: Mean intraepidermal nerve fiber (IENF) density was in the lower normal age‐adjusted range, 7.2 ± 2.9/mm (normal ≥7/mm), and was slightly below the normal range in 45% of POTS patients. MIBG uptake was reduced in 21% of patients. Low IENF density correlated with reduced cardiac MIBG uptake (r = 0.39, P = 0.001). Conclusions: A subset of neuropathic POTS patients may harbor mild small fiber neuropathy with abnormalities of unmyelinated nerve fibers in the skin associated with reduced myocardial postganglionic sympathetic innervation. Muscle Nerve 50: 956–961, 2014
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In one study, more than 50% of POTS patients were noted to have loss of sweat function in distal lower extremities, measured as impaired sweat volume on quantitative sudomotor axon reflex testing or reduced intraepidermal C-fiber density on skin biopsy.
“In one study, >50% of POTS patients (6) were noted to have loss of sweat function in distal lower extremities (32), measured as impaired sweat volume on quantitative sudomotor axon reflex testing (32) or reduced intraepidermal C-fiber density on skin biopsy (33).”
From Postural Orthostatic Tachycardia Syndrome (2019) · cites this paper as [33]
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References 48 · Cited by 79
- Authors
- Haensch, C., Tosch, M., Katona, I., Weis, J., & Isenmann, S.
- Affiliations
- Witten/Herdecke University (DE)
- RWTH Aachen University (DE)
- Corresponding author
- Haensch, Carl‐Albrecht
- Venue
- Muscle & Nerve
- Publisher
- Wiley
- Published
- 2014-08-29
- Volume
- 50
- Issue
- 6
- Pages
- 956-961
- ISSN
- 0148-639X (ISSN-L)
- 1097-4598
- OpenAlex
- W1902912997
- Cited by
- 79
- References
- 48
- Citations by year
- 2026: 6 · 2025: 10 · 2024: 12 · 2023: 4 · 2022: 7 · 2021: 5 · 2020: 10 · 2019: 5 · 2018: 5 · 2017: 5 · 2016: 5 · 2015: 2 · 2014: 3
- FWCI
- 4.96
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Muscle & Nerve (publishedVersion)
- PubMed (publishedVersion)
- RWTH Publications (RWTH Aachen) (submittedVersion)
- Topics
- Health Sciences › Medicine › Surgery › Cardiovascular Syncope and Autonomic Disorders
- Health Sciences › Medicine › Neurology › Peripheral Neuropathies and Disorders
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Heart Rate Variability and Autonomic Control
- Keywords
- postural orthostatic tachycardia syndrome, small fiber neuropathy, cardiac denervation, intraepidermal nerve fiber density, MIBG myocardial scintigraphy, orthostatic intolerance, skin biopsy
- MeSH terms
- Adolescent, Adult, Biopsy, Erythromelalgia, Female, Heart, Humans, Male, Myocardium, Nerve Degeneration, Retrospective Studies, Skin, Cohort Studies, Comorbidity, Tomography, Emission-Computed, Single-Photon, Incidence, 3-Iodobenzylguanidine, Nerve Fibers, Unmyelinated, Postural Orthostatic Tachycardia Syndrome, Young Adult
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