Ambulatory Infusion of Noradrenaline for Long-Term Treatment of Shy-Drager Syndrome

Abstract

A 70-year-old female patient with advanced Shy-Drager syndrome exhibited severe orthostatic hypotension, low serum catecholamine levels, and autonomic dysfunction. She was bedridden despite oral medication with fludrocortisone, etilefrin, dihydroergotamine, L-dopa, yohimbine, and amezinium methyl sulfate. Only intravenous application of noradrenaline (30 ng/kg/min) provided complete mobilization. After implantation of a port-a-cath system, intravenous noradrenaline treatment could be continued on an outpatient basis. Over the following 5 years, the patient was throughout sufficiently mobile and did not show any significant side effects of this treatment. However, during the 5th year she suffered from nonhemorrhagic brain stem infarction due to cerebral hypoperfusion after orthostatic stress in the absence of noradrenaline infusion. We conclude that ambulatory noradrenaline infusion is a new valuable tool for long-term treatment of advanced Shy-Drager syndrome.

Temporary elimination of orthostatic hypotension by norepinephrine infusion

“…The computer-controlled approach proved inadequate. Since then, case studies have noted sometimes spectacular improvement in orthostatic tolerance in patients receiving norepinephrine by vein or subcutaneously [4-8], without continuous pressure monitoring or titration of the norepinephrine infusion rate. Whether manually titrated i.v.…”