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Muscle Nerve. 2014 Dec;50(6):956-61. doi: 10.1002/mus.24245. Epub 2014 Aug 29.

Small-fiber neuropathy with cardiac denervation in postural tachycardia syndrome.

Author information

1
Department of Neurology, Kliniken Maria Hilf Mönchengladbach, Faculty of Health, University of Witten/Herdecke, Viersenerstrasse 450, D-41063, Mönchengladbach, Germany.

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.

KEYWORDS:

autonomic disorder; cardiac denervation; orthostatic intolerance; postural tachycardia syndrome; small fiber neuropathy

PMID:
24647968
DOI:
10.1002/mus.24245
[Indexed for MEDLINE]

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