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.

• Authors: Carl-Albrecht Haensch, Marco Tosch, Istvan Katona, Joachim Weis, Stefan Isenmann
• Journal: Muscle & nerve (Muscle Nerve) Vol. 50 Issue 6 Pg. 956-61 (Dec 2014) ISSN: 1097-4598 [Electronic] United States
• PMID: 24647968 (Publication Type: Comparative Study, Journal Article)
• Copyright: © 2014 Wiley Periodicals, Inc.

Chemical References

• 3-Iodobenzylguanidine

Topics

• 3-Iodobenzylguanidine (metabolism)
• Adolescent
• Adult
• Biopsy
• Cohort Studies
• Comorbidity
• Erythromelalgia (diagnosis, epidemiology, pathology)
• Female
• Heart (diagnostic imaging, innervation)
• Humans
• Incidence
• Male
• Myocardium (metabolism, pathology)
• Nerve Degeneration (diagnosis, epidemiology, pathology)
• Nerve Fibers, Unmyelinated (pathology)
• Postural Orthostatic Tachycardia Syndrome (complications, pathology)
• Retrospective Studies
• Skin (innervation, pathology)
• Tomography, Emission-Computed, Single-Photon
• Young Adult