Abstract
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.
MeSH terms
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Adrenergic Agonists / pharmacology
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Antiparkinson Agents / therapeutic use
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Autoantibodies / immunology
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Autoimmune Diseases of the Nervous System / complications*
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Autoimmune Diseases of the Nervous System / immunology*
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Autoimmune Diseases of the Nervous System / physiopathology
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Autonomic Nervous System Diseases / complications*
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Autonomic Nervous System Diseases / immunology*
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Autonomic Nervous System Diseases / physiopathology
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Blood Pressure / drug effects
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Blood Pressure / physiology
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Droxidopa / therapeutic use*
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Female
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Ganglia, Autonomic / drug effects
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Ganglia, Autonomic / immunology
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Ganglia, Autonomic / physiopathology
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Humans
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Hypotension, Orthostatic / drug therapy*
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Hypotension, Orthostatic / immunology
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Hypotension, Orthostatic / physiopathology
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Middle Aged
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Norepinephrine / metabolism
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Receptors, Nicotinic / immunology
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Treatment Outcome
Substances
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Adrenergic Agonists
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Antiparkinson Agents
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Autoantibodies
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Receptors, Nicotinic
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Droxidopa
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Norepinephrine