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Orthostatic hypotension in familial amyloid polyneuropathy

Treatment with DL-threo-3,4-di-hydroxyphenylserine

Third Department of Internal Medicine (Drs. Suzuki, Higa, Sakodar, Hayashi, and Yamamura), Osaka University Hospital, Osaka, the Arao City Hospital (Dr. Takaba), and the Nakajima Medical Clinic (Dr. Nakajima), Arao. Kumamoto, Japan.

we measured plasma norepinephrine levels in patients with familial amyloid polyneuropathy. Patients with orthostatic hypotension had low basal plasma norepinephrine levels, which did not increase after postural change. On the basis of biochemical findings that suggest depletion of peripheral norepinephrine, DL-threo-3,4-di-hydroxyphenylserine, an immediate precursor of norepinephrine, was given orally. Six hundred mg of this drug induced substantial and sustained elevation of blood pressure for several hours, and plasma norepinephrine content, increased. Daily administration for 4 weeks improved postural dizziness and syncope, and daily activity increased.

Address correspondence and reprint requests to Dr. Suzuki, The Third Department of Internal Medicine, Osaka University Hospital, Fuku-shima-ku, Osaka 553. Japan.

Accepted for publication March 9, 1981.

This work was supported by grants for Specific Diseases and for Monitoring Study of Congenital Disorders from the Ministry of Health and Welfare, grants for Inborn Errors of Metabolism from the Ministry of Education (No. 212003), and grants from Yamanouchi Foundation of Metabolism and Diseases.