eMedicine Specialties > Neurology > Neuromuscular Diseases
Autonomic Neuropathy: Treatment & Medication
Updated: Jun 25, 2009
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
Treatment
Medical Care
General management
The first objective of management of a patient with autonomic neuropathy is to administer specific treatment for treatable conditions. For example, if an autoimmune neuropathy is present, attempted management with immunomodulatory therapies should be considered. If diabetes mellitus is the underlying cause, strict control of blood glucose to prevent further worsening is essential. However, many of the autonomic neuropathies are not treatable with specific therapy. In these cases, symptomatic therapy becomes vitally important.
In cases of orthostatic intolerance, conservative therapy should be attempted first. Maintenance of high intakes of fluid and salt, as tolerated, can be attempted. The action of simply drinking 1-2 glasses of water can have a significant effect on systolic blood pressure. In patients with severe neurogenic orthostatic hypotension, intake of this volume led to an increase in systolic blood pressure of more than 30 mm Hg.63 Plasma norepinephrine in this patient group increased, and this vasopressor response was almost completely abolished by intravenous ganglion blockade. Therefore, simply drinking water increases blood pressure not only by increasing volume status, but also by increasing sympathetic activity.
Avoidance of alcohol, which could lead to a delayed hypovolemic state, as well as a second cause of autonomic neuropathy, should be advised. Slow cautious movements between different body postures should be emphasized.
Encourage patients to sit or lie down upon the initiation of orthostatic symptoms. The head of the bed can be elevated so the patient sleeps at a 15-20° angle to stimulate nocturnal mineralocorticoid release. Physical counter-maneuvers should also be attempted.64 The maneuvers include crossing the legs, squatting, and tensing the leg muscles, abdominal muscle, buttocks, or whole body.
Compressive stockings should be used. The thigh-high moderate compression stockings give the most benefit. Although they are difficult to put on and can be uncomfortable for patients, they should be strongly encouraged to use these as much as possible.
Gentle isometric exercises to help build up muscle tone is essential for patients with orthostatic intolerance. We often time recommend water aerobics or water jogging. If a place to perform these types of exercises is not available, then patients are encouraged to start gentle aerobic exercises often times with a recumbent bicycle, to avoid putting them in a position where they may experience loss of consciousness or fall.
Conservative treatment for other symptoms may also be tried, including eating smaller frequent meals, artificial tears for dry eyes, antiperspirants for hyperhidrosis and avoidance of hot environments for patients with anhidrosis.
Pharmacologic therapy
Pharmacologic therapy of orthostatic intolerance should be attempted in more difficult cases or when conservative therapy is unsuccessful.
Several medications are effective in controlling orthostatic intolerance. In less severe cases, such as in patients with POTS, medications such as beta-blockers for controlling heart rate may be sufficient.65 In more severe cases, volume expansion with fludrocortisone66 or venoconstriction with the α 2-adrenergic agonist midodrine67 may be necessary. Remembering that both of these medications may lead to supine hypertension is important and a balance may be difficult to strike. Pyridostigmine has been used successfully for treatment of both POTS and orthostatic hypotension.68 In addition, selective serotonin reuptake inhibitors (SSRIs)69 and phenobarbital70 have been shown to benefit specific patients.
Erythropoietin therapy can be effective in treating orthostatic hypotension in some patients, particularly patients with diabetes who have anemia and orthostatic hypotension.71 Erythropoietin may increase norepinephrine levels, thereby improving vasomotor tone. Also, erythropoietin promotes increased vascular sensitivity to angiotensin II, possibly through nitric oxide, and it may have direct pressor effects on vascular smooth muscle cells. DDAVP (vasopressin) produces an antidiuretic function at the renal tubuli, preventing nocturesis and elevating morning blood pressure.72
If an autoimmune cause of the autonomic neuropathy is found or strongly suspected, then immunomodulatory therapy may be considered. Intravenous immunoglobulin (IVIG)73 plasmapheresis74 and oral immunosuppressant medications75 have been used successfully.
Possible management for gastrointestinal autonomic neuropathy in patients with diabetes may include aminoguanidine, which can prevent diabetes-induced changes in nitric oxide synthase–related changes in animal models of ileum autonomic neuropathy.76
Treatments for Specific Conditions
Treatment of specific conditions can be tailored to the particular disease or syndrome .Urogenital dysfunction
Bladder dysfunction should be investigated with a urodynamic study initially before therapies are introduced.
Conservative therapy may be sufficient in mild dysfunction, such as a strict fluid schedule and bladder training. In cases of spastic bladder activity, medications such as tolterodine and oxybutynin may be useful. In cases of detrusor areflexia, cholinergic medications such as bethanechol may be helpful.
Refractory situations may require intermittent catheterization. Surgical options such as artificial sphincters may be necessary in some patients.
Sexual dysfunction may require treatment with agents such as sildenafil, tadalafil, or vardenafil. The efficacy and safety of these agents in patients with diabetes who have autonomic failure and orthostatic hypotension is largely unknown. Less commonly, prosthetic or assistive devices may be required.
Gastrointestinal conditions
Gastrointestinal difficulties can be present in many autonomic neuropathies, and may not be recognized by either the patient or the physician as symptomatic of autonomic dysfunction.
Changes in diet, small frequent meals, increased fiber ingestion, and increased fluid intake can be attempted first. In patients with Sjögren syndrome or thyroiditis, problems with hyposalivation can lead to difficulties in oral hygiene, and the patient should be reminded about regular dental checkups. Over-the-counter saliva replacements may be tried. Pyridostigmine can increase saliva output. Occasionally, cyproheptadine can be useful in treatment of altered sense of taste.
Pandysautonomia
The treatment of pandysautonomia is mainly supportive until spontaneous recovery can occur. In some patients, orthostatic hypotension may be relieved by fludrocortisone 0.1-0.2 mg daily or midodrine hydrochloride at 5-15 mg daily. Erythropoietin may be helpful in some patients with orthostatic hypotension.71 No definite evidence of course-modifying treatment exists, although glucocorticoid therapy, plasma exchange, and IVIG therapy have all been attempted. Most patients have a good prognosis once the acute episode is over.
Postural orthostatic tachycardia syndrome
The treatment of POTS may require a high-salt diet and high fluid intake. Beta-adrenergic agonists, pyridostigmine, midodrine, fludrocortisone, SSRIs and erythropoietin may be useful in some patients.77
Dysautonomia
Management of dysautonomia in cases of GBS is difficult.37,38 Primary therapy of the condition consists of supportive measures and IVIG or plasma exchange therapy. Vasoactive therapy is occasionally required and should be administered in an intensive care unit with intra-arterial blood pressure monitoring. Patients with severe bradyarrhythmias can require pacemaker assessment.
Lambert-Eaton myasthenic syndrome
The treatment of LEMS involves treatment of underlying malignancy in appropriate cases. The use of immunosuppressive therapies such as prednisone, azathioprine, plasma exchange, and IVIG has also been successful. Autonomic dysfunction in LEMS may also respond to 3,4 diaminopyridine, which may also lead to improvements in strength.
Amyloidoses
In amyloidoses associated with myeloma, treatment must be directed against the myeloma.
Sjögren syndrome
Sjögren syndrome is probably autoimmune. Although a trial may be indicated in particular patients, responses to immunosuppression are largely unsatisfying. Symptoms of xerophthalmia may be treated with artificial tears.
Hypohidrosis or hyperhidrosis
Patients who have lack of sweat output need to be educated about the risk of heat intolerance. They should be encouraged to avoid excessive and prolonged heat exposure as they may have poor thermoregulation and be at risk for heat stroke.
For patients who have increased sweat output, several medication choices may be of benefit. Botulinum toxin has been used for focal hyperhidrosis.78 If patient's symptoms are more generalized, medications with anticholinergic action or side effects may be tried. These include amitriptyline, glycopyrrolate, scopolamine patch, and hyoscyamine and belladonna tincture.
Porphyria
Porphyria can be treated by intravenous infusion of hematin, glucose, and vitamin B 6 .
Surgical Care
Liver transplant should be considered for patients with FAP that is associated with a transthyretin defect. Transthyretin is a serum transport protein synthesized primarily in the liver, and transplantation prevents its production in the abnormal form and thus prevents its deposition.79,80
Liver transplant may also be considered in patients with other hepatic disease related neuropathies. The neuropathies may be reversible in particular cases.
Uremic neuropathy with autonomic dysfunction has shown some reversibility with renal transplantation, whereas dialysis does not appear to improve the autonomic deficit.
Consultations
Consultations should be considered based upon the underlying pathophysiology of the autonomic neuropathy.
Infectious conditions, such as HIV infection, Chagas disease, leprosy, diphtheria, and Lyme disease may require the input of an infectious diseases expert.
Consultations with internal medicine specialists, including endocrinologists, hepatologists, and nephrologists, are often useful in the diagnosis and management of forms of amyloidosis, porphyria, diabetes mellitus, thyroiditis, hepatic failure, and renal failure.
A rheumatologist can be of great assistance in diagnosing and managing cases of Sjögren syndrome, rheumatoid arthritis, systemic lupus erythematosus, and other connective tissue disorders.
Diet
The treatment of patients with orthostatic intolerance may require a high-salt diet and high fluid intake as noted above.
Medication
The goals of pharmacotherapy are to reduce morbidity and prevent complications. Many of the medications used to treat dysautonomia are considered off label.
Sympathomimetic agents
These are used in orthostatic hypotension if simple measures yield no improvement.
Midodrine (ProAmatine)
Prodrug metabolized to desglymidodrine, a selective alpha1-adrenoreceptor agonist. Effects via arterioconstriction and venoconstriction.
Adult
2.5-5 mg PO bid/tid
Pediatric
Not established
Drugs stimulating alpha-adrenergic agonists may enhance or potentiate pressor effects; cardiac glycosides may precipitate or worsen bradycardia; psychopharmacologic agents or beta-blockers may precipitate or worsen AV block or arrhythmia
Documented hypersensitivity; acute renal disease; severe organic heart disease; pheochromocytoma; urinary retention; persistent and excessive supine hypertension
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Caution in diabetes mellitus or visual complications; discontinue and reevaluate if any signs or symptoms suggesting bradycardia occur
Mineralocorticoids
These agents can be used to treat orthostatic hypotension.
Fludrocortisone (Florinef)
Promotes increased reabsorption of sodium and loss of potassium at renal distal tubules.
Adult
0.1-0.2 mg/d PO
Pediatric
Not established
Antagonizes effects of anticholinergics; rifampin, hydantoins, and barbiturates decrease effects; decreases salicylate levels
Documented hypersensitivity; systemic fungal infections
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Taper dose gradually when therapy discontinued; caution in Addison disease, potassium loss, and sodium retention
Anticholinergic agents
These agents are useful in cases of difficult bladder emptying.
Oxybutynin (Ditropan)
Commonly used drug in bladder disorder. Known for anticholinergic-antispasmodic effects. Smooth muscle relaxing effect distal to cholinergic receptor site. Long-acting form available for qd dosing.
Adult
5 mg PO tid
Pediatric
2.5 mg PO tid
Coadministration with other anticholinergic agents may exacerbate anticholinergic adverse effects, including dry mouth, drowsiness, constipation; CNS effects increase when administered concurrently with other CNS depressants
Documented hypersensitivity; glaucoma; partial or complete GI obstruction; myasthenia gravis; ulcerative colitis; toxic megacolon
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Caution in urinary tract obstruction, reflux esophagitis, heart disease
Tolterodine tartrate (Detrol)
Competitive muscarinic receptor antagonist for overactive bladder, but differs from other anticholinergic types because of selectivity for urinary bladder over salivary glands. High specificity for muscarinic receptors. Minimal activity or affinity for other neurotransmitter receptors and other potential targets (eg, calcium channels).
Adult
2 mg PO bid; can be adjusted to 1 mg PO bid
Pediatric
Not established
Patients receiving macrolide antibiotics or antifungal agents should not receive doses > 1 mg PO bid
Documented hypersensitivity; urinary retention; gastric retention; uncontrolled narrow-angle glaucoma
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Do not administer doses > 1 mg PO bid to patients with significantly reduced hepatic function; caution in renal impairment
Beta-adrenergic blocker
This agent can be used to treat orthostatic tachycardia in POTS patients.
Metoprolol (Lopressor, Toprol XL)
For treatment of orthostatic tachycardia. Inhibits beta-adrenergic input.
Adult
12.5 mg PO qd /bid; titrate to patients symptoms with target dose of 50 mg of long-acting formulation daily
Pediatric
Not established
Aluminum salts, barbiturates, NSAIDs, penicillins, calcium salts, cholestyramine, and rifampin may decrease bioavailability and plasma levels of metoprolol, possibly resulting in decreased pharmacologic effects; toxicity of metoprolol may increase with coadministration of sparfloxacin, phenothiazines, astemizole, calcium channel blockers, quinidine, flecainide, and contraceptives; metoprolol may increase toxicity of digoxin, flecainide, clonidine, epinephrine, nifedipine, prazosin, verapamil, and lidocaine
Documented hypersensitivity; uncompensated congestive heart failure, bradycardia, asthma, cardiogenic shock, depression, and A-V conduction abnormalities
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Beta-adrenergic blockade may reduce signs and symptoms of acute hypoglycemia and may decrease clinical signs of hyperthyroidism; abrupt withdrawal may exacerbate symptoms of hyperthyroidism, including thyroid storm; monitor patient closely and withdraw the drug slowly; during IV administration, carefully monitor blood pressure, heart rate, and ECG
Vasopressin analogues
Oral or nasal spray agents acting to prevent nocturnal urinary production.
Desmopressin acetate (DDAVP)
Vasopressin analogue without effect on V1 receptors responsible for vasopressin-induced vasoconstriction. Acts on V2 receptors at renal tubuli, increasing cellular permeability of collecting ducts, responsible for antidiuretic effect. Prevents nocturnal diuresis and elevated morning BP, resulting in renal water reabsorption. Nasal spray and tab (more convenient).
Adult
2-4 mcg IM administered at 8:00 pm as single dose
Pediatric
Not established
Coadministration with demeclocycline and lithium decreases effects; fludrocortisone and chlorpropamide increase effects
Documented hypersensitivity; hemophilia; von Willebrand disease
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Avoid hyponatremia; monitor osmolality and plasma sodium levels q6wk; minimize fluid intake in the evening before administration (not to exceed 8 oz with dinner and 8 oz after dinner; nothing 2 h prior hs); caution in coagulation disorders and predisposition to thrombus formation and in fluid and electrolyte imbalance, hypertension, or severe cardiovascular disease
Acetylcholinesterase inhibitor
This agent can be used to treat orthostatic hypotension or orthostatic tachycardia.
Pyridostigmine (Mestinon)
Increases acetylcholine neurotransmission at peripheral
autonomic ganglia, which likely increases peripheral vasoconstriction sympathetic nerve fiber transmission. May also increase vagal cardiac input in POTS patients.
Adult
60 mg tid or 180 mg SR qd; lower initial doses may be used
Pediatric
Not established
Pyridostigmine increases effects of depolarizing neuromuscular blockers; increases toxicity of edrophonium
Documented hypersensitivity; gastrointestinal or urinary obstruction
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Caution in bronchial asthma and those receiving a cardiac glycoside
Overdose may cause cholinergic crisis, which may be fatal; atropine IV should be readily available for treatment of cholinergic reactions
Cholinergic agents
These agents stimulate cholinergic receptors in the smooth muscle of the urinary bladder for stimulation of bladder emptying.
Bethanechol hydrochloride (Duvoid, Urecholine)
Used for selective stimulation of the bladder to produce contraction to initiate micturition and empty bladder. Most useful in bladder hypocontractility, if sphincters functional and coordinated. Rarely used because of GI stimulation and difficulty in timing effect.
Adult
10-50 mg PO tid/qid
Pediatric
Not established
Concurrent ganglion-blocking compounds may critically decrease BP
Documented hypersensitivity; peptic ulcer disease; obstructive pulmonary disease; bradycardia; vasomotor instability; hypotension; AV conduction defects; hyperthyroidism; epilepsy; mechanical GI/GU obstruction
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Urinary retention secondary to possible urine reflux into kidneys
Phosphodiesterase inhibitors
These oral agents act peripherally to induce smooth muscle relaxation of the corpora cavernosa.
Sildenafil (Viagra)
Selective inhibitor of PDE5 that inactivates cGMP, allowing attenuation of the vasodilatory effect of NO. Effective in men with mild-to-moderate erectile dysfunction. Take on an empty stomach about 1 h before sexual activity. Sexual stimulation is necessary to activate response. The increased sensitivity for erections may last 24 h. Available as 25-, 50-, and 100-mg tabs.
Adult
25-100 mg PO 1 h before sexual activity
Pediatric
Not established
Potentiates vasodilatory effect of NO, resulting in potentially fatal drop in blood pressure; coadministration with ketoconazole, erythromycin, or cimetidine increases plasma sildenafil concentrations; coadministration with rifampin decreases plasma levels of sildenafil
Documented hypersensitivity; concurrent or intermittent use of organic nitrates in any form
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
May cause headaches (16%), flushing (10%), upset stomach (7%), nasal congestion (4%), blue haze at the periphery of vision (3%); adverse effects more common in men taking 100 mg; serious adverse effects may occur in severe heart disease and in those taking nitrates; rates of MI are 1.7 (drug) vs 1.4 (placebo) per 100 man-years
Neuromuscular blocker agent, toxin
Used in patients with hyperhydrosis localized to palmar or axillary region.
Botulinum toxin type A (Botox)
One of several toxins produced by clostridium botulinum. Blocks neuromuscular transmission through a 3-step process: (1) Blockade of neuromuscular transmission; botulinum toxin type A (BTA) binds to the motor nerve terminal. The binding domain of the type A molecule appears to be the heavy chain, which is selective for cholinergic nerve terminals. (2) BTA is internalized via receptor-mediated endocytosis, a process in which the plasma membrane of the nerve cell invaginates around the toxin-receptor complex, forming a toxin-containing vesicle inside the nerve terminal. After internalization, the light chain of the toxin molecule, which has been demonstrated to contain the transmission-blocking domain, is released into the cytoplasm of the nerve terminal. (3) BTA blocks acetylcholine release by cleaving SNAP-25, a cytoplasmic protein that is located on the cell membrane and that is required for the release of this transmitter. The affected terminals are inhibited from stimulating muscle contraction. Toxin does not affect synthesis or storage of acetylcholine or conduction of electrical signals along the nerve fiber. Prevents calcium-dependent release of acetylcholine and produces a state of denervation at the neuromuscular junction and postganglionic sympathetic cholinergic nerves in the sweat glands.
Typically, a 24-72 h delay between administration of toxin and onset of clinical effects exists, which terminate in 2-6 mo.
This purified neurotoxin complex is a vacuum-dried form of purified BTA, which contains 5 ng of neurotoxin complex protein per 100 U.
BTA has to be reconstituted with 2 mL of 0.9% sodium chloride diluent. With this solution, each 0.1 mL results in 5 U dose. Patient should receive 5-10 injections per visit.
Must be reconstituted from vacuum-dried toxin into 0.9% sterile saline without preservative using manufacturer's instructions to provide injection volume of 0.1 mL; must be used within 4 h of storage in refrigerator at 2-8°C.
Preconstituted dry powder must be stored in freezer at <5°C. Each injection produces an area of anhydrosis approximately 1.2 cm in diameter. Results in anhydrosis lasting 4-12 months.
Injections of botulinum toxin must be repeated at varying intervals to maintain long-term results.
Adult
Palmar hyperhidrosis: 50 subepidermal injections of 2 U per palm (total 100 U per palm)
Pediatric
Not established
Aminoglycosides or drugs that interfere with neuromuscular transmission may potentiate effects of botulinum toxin
Documented hypersensitivity; infection present at injection site
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Do not exceed recommended dosages and frequencies of administration; presence of antibodies to botulinum toxin type A may reduce effects of therapy; when used for cervical dystonia, may cause dysphagia, upper respiratory infection, neck pain, or headache; ptosis may occur when used for blepharism or strabismus; weakness of hand muscles and blepharoptosis may occur when used for palmar or facial hyperhidrosis, respectively
When used cosmetically for glabellar lines may cause headache, respiratory infection, flu syndrome, blepharoptosis, or nausea
Colony-stimulating Factor
May be used in patients with orthostatic hypotension or POTS.
Epoetin alfa (Epogen, Procrit)
Purified glycoprotein produced from mammalian cells modified with gene coding for human erythropoietin (EPO). Amino acid sequence is identical to that of endogenous EPO. Biological activity mimics human urinary EPO, which stimulates division and differentiation of committed erythroid progenitor cells and induces release of reticulocytes from bone marrow into the blood stream.
Has been shown to increase the functional capacity of patients with MSA, particularly those who have the characteristic mild anemia associated with this disease. Up to 38% of patients with severe autonomic failure are anemic. Lack of sympathetic stimulation may lead to a decrease of erythropoietin production and development of anemia. Sympathetic impairment and low plasma norepinephrine levels have been found to correlate with severity of anemia. Therapy with recombinant erythropoietin, even low doses (25-50 units/kg body weight SC, 3 times a week) has successfully corrected anemia and improved upright BP.
Adult
25-50 U/kg body weight SC weekly titrated for hematocrit of 50%
Pediatric
Not established
None reported
Documented hypersensitivity; uncontrolled hypertension
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Caution in porphyria, hypertension, history of seizures; decrease dose if hematocrit increase exceeds 4 U in any 2-wk period; the multidose preserved formulation contains benzyl alcohol and may increase risk of neurologic toxicity in infants (use preservative-free formulation); treatment results depend on adequate iron supplementation
Anticholinergic agent
Used for hyperhidrosis.
Glycopyrrolate (Robinul)
Acts in smooth muscle, CNS, and secretory glands to blocks action of acetylcholine at parasympathetic sites.
Adult
1-2 mg PO bid/tid initially, then titrate to effective dose
Pediatric
<12 years: Not recommended
>12 years: Administer as in adults
Levodopa decreases effects of glycopyrrolate; both amantadine and cyclopropane increase glycopyrrolate toxicity
Documented hypersensitivity; narrow-angle glaucoma, tachycardia, ulcerative colitis, paralytic ileus, or acute hemorrhage
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Glycopyrrolate may increase chances of developing megacolon, hyperthyroidism, CHF, CAD, hiatal hernia, and BPH; not recommended for children <12 years and patients with down syndrome
Immune globulin
Used for autoimmune causes of autonomic neuropathy.
Immune globulin intravenous (CarimuneGammagard S/D, Gammar-P, Gamunex, Polygam S/D)
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%).
Adult
2 g/kg IV over 2-5 d
Pediatric
Not established
Globulin preparation may interfere with immune response to live virus vaccine (MMR) and reduce efficacy (do not administer within 3 months of vaccine)
Documented hypersensitivity; IgA deficiency
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Check serum IgA before IVIG (use an IgA-depleted product if deficient, eg, Gammagard S/D); infusions may increase serum viscosity and thromboembolic events; infusions may increase risk of migraine attacks, aseptic meningitis (10%), urticaria, pruritus, or petechiae (2-30 d postinfusion)
Increases risk of renal tubular necrosis in elderly patients and in patients with diabetes, volume depletion, and preexisting kidney disease; lab result changes associated with infusions include elevated antiviral or antibacterial antibody titers for 1 mo, 6-fold increase in ESR for 2-3 wk, and apparent hyponatremia
Anti-inflammatory agent
Can be used if an inflammatory cause of the autonomic neuropathy is considered to be autoimmune in nature.
Prednisone (Deltasone, Sterapred, Orasone)
Immunosuppressant for treatment of autoimmune disorders; may decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. Stabilizes lysosomal membranes and also suppresses lymphocyte and antibody production.
Adult
5-60 mg/d PO qd or divided bid/qid; taper over 2 wk, as symptoms resolve
Pediatric
4-5 mg/m2/d PO; alternatively, 0.05-2 mg/kg PO divided bid/qid; taper over 2 wk, as symptoms resolve
Coadministration with estrogens may decrease clearance; concurrent use with digoxin may cause digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics
Documented hypersensitivity; viral infection, peptic ulcer disease, hepatic dysfunction, connective tissue infections, and fungal or tubercular skin infections; GI bleeding or ulceration
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Abrupt discontinuation of glucocorticoids may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections may occur with glucocorticoid use
More on Autonomic Neuropathy |
| Overview: Autonomic Neuropathy |
| Differential Diagnoses & Workup: Autonomic Neuropathy |
 Treatment & Medication: Autonomic Neuropathy |
| Follow-up: Autonomic Neuropathy |
| References |
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Further Reading
Keywords
syndrome of acute pandysautonomia, postural orthostatic tachycardia syndrome, POTS, Guillain-Barré syndrome, GBS, acute inflammatory demyelinating polyneuropathy, AIDP, Lambert-Eaton myasthenic syndrome, LEMS, Holmes-Adie syndrome, idiopathic distal small-fiber neuropathy, human immunodeficiency virus, HIV, Chagas disease, Chagas' disease, botulism, chronic idiopathic anhidrosis, familial amyloid polyneuropathy, FAP, diabetes mellitus, uremic neuropathy, hepatic disease, vitamin B-12 deficiency, paraneoplastic autonomic neuropathy, Sjögren syndrome, acute intermittent porphyria, variegate porphyria, hereditary sensory autonomic neuropathy, HSAN, Fabry disease, autonomic dysreflexia, AD, acquired immunodeficiency syndrome, AIDS, autonomic nervous system, ANS, autonomic reflex screen, ARS, composite autonomic scoring scale, CASS, collapsin response–mediator family, CRMP-5, cerebrospinal fluid, CSF, vasopressin, DDAVP, electromyography, EMG, inhibitor of k-light polypeptide gene enhancer inB-cells, IKBKAP, mitochondrial neurogastrointestinal encephalomyopathy, MNGIE, M-phase phosphoprotein-1, MPPI, multiple system atrophy, MSA, nerve conduction studies, NCS, progressive autonomic failure, PAF, primary biliary cirrhosis, PBC, Purkinje cell cytoplasmic antibody-2, PCA-2, Parkinson disease, PD, positron emission tomography, PET, peripheral nervous system, PNS, quantitative sudomotor axon reflex test, QSART, quantitative sensory testing, QST, single-photon emission computed tomography, SPECT, serine palmitoyltransferase, SPT, sympathetic skin responses, SSR, selective serotonin reuptake inhibitor, SSRI, thermoregulatory sweat test, TST
