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Dermal microdialysis provides evidence for hypersensitivity to noradrenaline in patients with familial dysautonomia

¹ Neurologische Klinik, Universität Erlangen-Nürnberg, Schwabachanlage 6, 91054 Erlangen, Germany
² New York University Medical Center, 530 First Avenue, Suite 9Q, New York, NY 10016, USA
³ Institut für Physiologie I, Universität Erlangen-Nürnberg, Universitätsstrasse 17, 91054 Erlangen, Germany
⁴ New York University Medical Center, 550 First Avenue, Suite 7 W 11, New York, NY 10016, USA

Correspondence to:
Correspondence to:
Dr M J Hilz;
max.hilz{at}neuro.imed.uni-erlangen.de

Objectives: To use the technique of dermal microdialysis to examine sensitivity of skin vessels to noradrenaline (NA) in patients with familial dysautonomia (FD) and in healthy controls.

Methods: In 14 patients with FD and 12 healthy controls, plasma extravasation, local laser Doppler blood flow, and skin blanching were observed before, during, and after application of 10^-6 M NA through a microdialysis membrane, located intradermally in the skin of the lower leg.

Results: Maximum local vasoconstriction measured by laser Doppler blood flow did not differ between patients with FD and controls. In contrast, patients with FD had an earlier onset of vasoconstriction (p = 0.02). Moreover, reaction to NA was more prominent and prolonged in FD, shown by a larger zone of skin blanching around the microdialysis membrane (p < 0.001) and delayed reduction of the protein content in the dialysate after termination of NA application (p = 0.03).

Conclusion: These data support the hypothesis that peripheral blood vessels of patients with FD show a denervation hypersensitivity to catecholamines. This may be one mechanism contributing to the major hypertension that frequently occurs during "dysautonomic crises" in FD.

Keywords: familial dysautonomia; microdialysis; catecholamine hypersensitivity

Abbreviations: FD, familial dysautonomia (Riley-Day syndrome); NA, noradrenaline; pU, perfusion units; SBF, skin blood flow; TPC, total protein content

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				F. B. Axelrod, G. G. Chelimsky, and D. E. Weese-Mayer Pediatric Autonomic Disorders Pediatrics, July 1, 2006; 118(1): 309 - 321. [Abstract] [Full Text] [PDF]

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