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Treatment of Orthostatic Hypotension in Familial Amyloidotic Polyneuropathy with L-Threo-3,4-Dihydroxyphenylserine

  • Shukuro Araki
  • Shinichi Ikegawa
  • Shigehiro Yi
  • Yukio Ando
  • Akira Miyazaki
Chapter

Abstract

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.

Keywords

Orthostatic Hypotension Familial Amyloid Polyneuropathy Familial Amyloidotie Polyneuropathy Syncope Attack Locus Caeruleus 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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Copyright information

© Springer Science+Business Media New York 1988

Authors and Affiliations

  • Shukuro Araki
    • 1
  • Shinichi Ikegawa
    • 1
  • Shigehiro Yi
    • 1
  • Yukio Ando
    • 1
  • Akira Miyazaki
    • 1
  1. 1.The First Department of Internal MedicineKumamoto University Medical SchoolKumamoto 860Japan

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