Medication Summary
Several medications are available to help manage symptoms of autonomic dysfunction. The most commonly used are listed below.
Mineralocorticoids
Class Summary
These agents play a role in hemodynamics and can be used to control orthostatic hypotension.
Fludrocortisone (Florinef)
Used to increase standing blood pressure. Acts to increase sodium retention and expand plasma volume.
Alpha-adrenergic agonists
Class Summary
These agents improve the hemodynamic status by increasing blood pressure. Midodrine forms the active metabolite desglymidodrine, which is an alpha1-agonist. Desglymidodrine exerts its actions via activation of the alpha-adrenergic receptors of the arteriolar and venous vasculature, producing an increase in vascular tone and elevation of blood pressure. Droxidopa increases blood pressure by conversion to norepinephrine, but has no clinically significant effect on standing or supine heart rates in patients with autonomic failure.
Midodrine (ProAmatine)
Alpha-adrenergic agonist used in orthostatic hypotension to increase standing blood pressure. Acts at level of resistance vessels and is useful for peripherally mediated hypotension.
Droxidopa (Northera)
Droxidopa is a norepinephrine precursor that is metabolized to norepinephrine by dopa-decarboxylase. Norepinephrine increases blood pressure by inducing peripheral arterial and venous vasoconstriction. It is indicated for symptomatic neurogenic orthostatic hypotension (NOH) in patients with primary autonomic failure (Parkinson's disease, multiple system atrophy, and pure autonomic failure), dopamine beta-hydroxylase deficiency, and nondiabetic autonomic neuropathy.
Beta-adrenergic blocking agents
Class Summary
These agents limit heart rate and reduce blood pressure.
Propranolol (Inderal)
Nonselective beta-blocker that is lipophilic (penetrates CNS).
Vasopressors
Class Summary
These agents augment both coronary and cerebral blood flow that occurs during the low flow state associated idiopathic hypotension.
Desmopressin (DDAVP, Stimate)
Increases cellular permeability of collecting ducts, resulting in reabsorption of water by kidneys. Helpful for symptoms of nocturia.
Erythropoietins
Class Summary
Anemia may occur due to low blood levels of endogenous erythropoietin, which can result from a lack of sympathetic innervation. Erythropoietins may also increase blood pressure through other mechanisms.
Epoetin alfa (Epogen, Procrit)
Stimulates RBC production in bone marrow. Increases sensitivity to pressor effects of angiotensin II, intravascular volume, cytosolic free calcium in vascular smooth muscle, and plasma endothelin level. Enhances renal tubular reabsorption.
Gastroprokinetic agents
Class Summary
These agents promote motility of the GI tract.
Metoclopramide (Reglan)
Dopamine agonist helpful in relieving GI paresis.
Anticholinesterase inhibitors
Class Summary
These agents inhibit acetylcholinesterase (AChE), raising the concentration of ACh at cholinergic synapses and increasing the chance of activating the AChR.
Pyridostigmine bromide (Mestinon)
Stimulates muscarinic AChR, increasing salivation and gastric motility.
Bulk agents
Class Summary
Used for the chronic treatment of constipation.
Psyllium (Metamucil, Fiberall)
Must be taken with water or may cause obstruction. Increase dose gradually. Inform patient that effect not immediate.
Antispasmodic agents
Class Summary
These agents may be helpful for urinary symptoms.
Oxybutynin (Ditropan)
Useful for urinary urgency. Inhibits action of ACh on smooth muscle and direct antispasmodic effect on smooth muscle, which increases bladder capacity and decreases uninhibited contractions.
Cholinergic agents
Class Summary
These agents stimulate cholinergic receptors in the smooth muscle of the urinary bladder for stimulation of bladder emptying.
Bethanechol hydrochloride (Duvoid, Urecholine)
For selective stimulation of the bladder to produce contraction to initiate micturition and empty bladder. Most useful in bladder hypotonia. Rarely used because of GI stimulation and difficulty in timing effect.
Phosphodiesterase inhibitors
Class Summary
These oral agents act peripherally to induce smooth muscle relaxation of the corpora cavernosa.
Sildenafil (Viagra)
Selective PDE5 inhibitor that inactivates cGMP, attenuating vasodilatory effect of NO. Effective in mild-to-moderate erectile dysfunction. Patient should take on an empty stomach about 1 h before sexual activity. Sexual stimulation necessary to activate response. Increased sensitivity for erections may last 24 h.
Corticosteroids
Class Summary
These agents regulate key factors in the immune system.
Prednisone (Deltasone, Orasone, Meticorten)
Shorten duration of symptoms and improves overall prognosis in acute pandysautonomia.
Immune globulins
Class Summary
These agents are used to improve clinical and immunologic aspects of the disease. May decrease autoantibody production, and increase solubilization and removal of immune complexes.
Immune globulins intravenous (IVIG, Gammagard, Gamimune)
Shortens duration of symptoms and improves overall prognosis in acute pandysautonomia. Clinical improvements have been reported within few days of administration, with normalization of autonomic parameters.
Neutralize circulating myelin antibodies through antiidiotypic antibodies; down-regulates proinflammatory cytokines, including INF-gamma; blocks Fc receptors on macrophages; suppresses inducer T and B cells and augments suppressor T cells; blocks complement cascade; promotes remyelination; may increase CSF IgG (10%).
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- Overview
- Presentation
- DDx
- Workup
- Treatment
- Medication
- Medication Summary
- Mineralocorticoids
- Alpha-adrenergic agonists
- Beta-adrenergic blocking agents
- Vasopressors
- Erythropoietins
- Gastroprokinetic agents
- Anticholinesterase inhibitors
- Bulk agents
- Antispasmodic agents
- Cholinergic agents
- Phosphodiesterase inhibitors
- Corticosteroids
- Immune globulins
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- Follow-up
- Questions & Answers
- References
