Dermal microdialysis provides evidence for hypersensitivity to noradrenaline in patients with familial dysautonomia. Issue 3 (1st September 2002)

• Record Type: Journal Article
• Authors: Bickel, A; Axelrod, F B; Schmelz, M; Marthol, H; Hilz, M J
• Is Part Of: Journal of neurology, neurosurgery and psychiatry. Volume 73:Issue 3(2002)
• Page Start: 299
• Page End: 302
• Publication Date: 2002-09-01
• Subjects: familial dysautonomia -- microdialysis -- catecholamine hypersensitivity; FD, familial dysautonomia (Riley-Day syndrome) -- NA, noradrenaline -- pU, perfusion units -- SBF, skin blood flow -- TPC, total protein content; Neurology -- Periodicals; Nervous system -- Surgery -- Periodicals; Psychiatry -- Periodicals; 616.8
• DOI: 10.1136/jnnp.73.3.299
• Languages: English
• ISSNs: 0022-3050

Abstract

Abstract : 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.