Treatment of Autonomic Dysfunction with L-Threo-3,4-Dihydroxyphenylserine in Patients with Familial Amyloidotic Polyneuropathy: A Multicenter Study
Type 1 familial amyloidotic polyneuropathy(FAP) is a late onset inherited disorder characterized by polyneuropathy and autonomic failure. Autonomic visceral neuropathies affect every organ system and present as extensive and severe autonomic manifestations. Conventional therapies barely relieve afflicted patients. L-threo-3,4-dihydroxyphenylserine(L-threo-DOPS), an immediate precursor of (−)-norepinephrine, has recently been adopted as a promising drug, based on findings that suggest norepinephrine depletion from sympathetic nerve endings and denervation supersensitivity in the disorder1. The beneficial effects of racemic DL-threo-DOPS on orthostatic hypotension in FAP were also reported at preceding Symposia2,3.
KeywordsOrthostatic Hypotension Familial Amyloid Polyneuropathy Familial Amylaidatic Polyneuropathy Sympathetic Nerve Ending Elevated Systolic Blood Pressure
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