L-DOPS therapy for refractory orthostatic hypotension in autoimmune autonomic neuropathy.
The authors report a 46-year-old woman with antibodies to the nicotinic acetylcholine receptor (NiAchR) of the autonomic ganglia. She presented with severe orthostatic intolerance refractory to treatment with midodrine, fludrocortisone, erythropoietin, vasopressin, salt, and fluid loading. Addition of L-threo-3,4-dihidroxyphenylserine (L-DOPS) substantially improved blood pressure and orthostatic tolerance. L-DOPS may benefit patients with severe orthostatic intolerance and be particularly effective in patients with ganglionic NiAchR antibodies.
Gibbons
Christopher H
CH
Beth Israel Deaconess Medical Center, Boston, MA 02215, USA.
Vernino
Steven A
SA
Kaufmann
Horacio
H
Freeman
Roy
R
D000322:Adrenergic Agonists / Q000494:pharmacology; D000978:Antiparkinson Agents / Q000627:therapeutic use; D001323:Autoantibodies / Q000276:immunology; D020274:Autoimmune Diseases of the Nervous System / Q000150:complications* / Q000276:immunology* / Q000503:physiopathology; D001342:Autonomic Nervous System Diseases / Q000150:complications* / Q000276:immunology* / Q000503:physiopathology; D001794:Blood Pressure / Q000187:drug effects / Q000502:physiology; D015103:Droxidopa / Q000627:therapeutic use*; D005260:Female; D005725:Ganglia, Autonomic / Q000187:drug effects / Q000276:immunology / Q000503:physiopathology; D006801:Humans; D007024:Hypotension, Orthostatic / Q000188:drug therapy* / Q000276:immunology / Q000503:physiopathology; D008875:Middle Aged; D009638:Norepinephrine / Q000378:metabolism; D011978:Receptors, Nicotinic / Q000276:immunology; D016896:Treatment Outcome
D000322:Adrenergic Agonists; D000978:Antiparkinson Agents; D001323:Autoantibodies; D011978:Receptors, Nicotinic; D015103:Droxidopa; D009638:Norepinephrine