Treatment of Orthostatic Hypotension in Familial Amyloidotic Polyneuropathy with L-Threo-3,4-Dihydroxyphenylserine
Type 1 familial amyloidotic polyneuropathy (FAP) is an inherited systemic amyloidosis characterized by sensory dominant polyneuropathy accompanied by severe autonomic dysfunctions. Patients with orthostatic hypotension are severely disabled by dizziness and syncope when standing. We studied the effects of L-threo-3,4-dihydroxyphenylserine (L-threo-DOPS), an unphysiologyical precursor amino acid of norepinephrine in treating orthostatic hypotension in patients with FAP.
Daily administration of 100 to 600 mg of L-threo-DOPS for 2 to 11 weeks improved dizziness in 5 of 6 (83 %), syncope in 2 of 3 (67 %) and other autonomic or mental disturbances in 3 cases.
The drug has no toxicologie effect and over all utility rate was evaluated in 75 %. Our trial suggests that L-threo-DOPS may be useful in the symptomatic treatment of FAP.
KeywordsOrthostatic Hypotension Familial Amyloid Polyneuropathy Familial Amyloidotie Polyneuropathy Syncope Attack Locus Caeruleus
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