☆ 4.4 Article

SMALL-FIBER NEUROPATHY WITH CARDIAC DENERVATION IN POSTURAL TACHYCARDIA SYNDROME

MUSCLE & NERVE (2014)

期刊

MUSCLE & NERVE

卷 50, 期 6, 页码 956-961

出版社

WILEY-BLACKWELL

DOI: 10.1002/mus.24245

关键词

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

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Clinical Neurology Neurosciences

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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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