Autonomic Neuropathy Treatment & Management

  • Author: Steven D Arbogast, DO; Chief Editor: Nicholas Lorenzo, MD   more...
 
Updated: Feb 6, 2012
 

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.[67] 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.[68] 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.[69] In more severe cases, volume expansion with fludrocortisone[70] or venoconstriction with the α 2-adrenergic agonist midodrine[71] 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.[72] In addition, selective serotonin reuptake inhibitors (SSRIs)[73] and phenobarbital[74] 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.[75] 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.[76]

If an autoimmune cause of the autonomic neuropathy is found or strongly suspected, then immunomodulatory therapy may be considered. Intravenous immunoglobulin (IVIG)[77] plasmapheresis[78] and oral immunosuppressant medications[79] 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.[80]

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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 Sj ö gren 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.[75] 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.[81]

Dysautonomia

Management of dysautonomia in cases of GBS is difficult.[39, 40] 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.

Sj ö gren syndrome

Sj ö gren 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.[82] 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 .

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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.[83, 84]

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.

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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 Sj ö gren syndrome, rheumatoid arthritis, systemic lupus erythematosus, and other connective tissue disorders.

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Diet

The treatment of patients with orthostatic intolerance may require a high-salt diet and high fluid intake as noted above.

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Contributor Information and Disclosures
Author

Steven D Arbogast, DO  Fellow, Neuromuscular Medicine, University Hospitals Case Medical Center, Cleveland

Steven D Arbogast, DO is a member of the following medical societies: American Academy of Neurology and American Osteopathic Association

Disclosure: Nothing to disclose.

Coauthor(s)

J Douglas Miles, MD, PhD  Assistant Professor of Neuroscience, Marshall University School of Medicine, and Clinical Instructor of Neurology, Case Western Reserve University School of Medicine

J Douglas Miles, MD, PhD is a member of the following medical societies: American Academy of Neurology, American Medical Association, and Society for Neuroscience

Disclosure: Nothing to disclose.

Bashar Katirji, MD, FACP  Director, Neuromuscular Center and EMG Laboratory, The Neurological Institute, University Hospitals Case Medical Center; Professor of Neurology, Case Western Reserve University School of Medicine

Bashar Katirji, MD, FACP is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, American College of Physicians, and American Neurological Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Paul E Barkhaus, MD  Professor, Department of Neurology, Medical College of Wisconsin; Director of Neuromuscular Diseases, Milwaukee Veterans Affairs Medical Center

Paul E Barkhaus, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, and American Neurological Association

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Glenn Lopate, MD  Associate Professor, Department of Neurology, Division of Neuromuscular Diseases, Washington University School of Medicine; Director of Neurology Clinic, St Louis ConnectCare; Consulting Staff, Department of Neurology, Barnes-Jewish Hospital

Glenn Lopate, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, and Phi Beta Kappa

Disclosure: Baxter Grant/research funds Other; Amgen Grant/research funds None

Selim R Benbadis, MD  Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, Tampa General Hospital, University of South Florida College of Medicine

Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association

Disclosure: UCB Pharma Honoraria Speaking, consulting; Lundbeck Honoraria Speaking, consulting; Cyberonics Honoraria Speaking, consulting; Glaxo Smith Kline Honoraria Speaking, consulting; Pfizer Honoraria Speaking, consulting; Sleepmed/DigiTrace Honoraria Speaking, consulting

Chief Editor

Nicholas Lorenzo, MD  Consulting Staff, Neurology Specialists and Consultants

Nicholas Lorenzo, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Neurology, and American College of Physician Executives

Disclosure: Nothing to disclose.

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Table. Types of HSAN
HSANMode of InheritanceOnsetSymptomsSigns
Type IAutosomal dominant, point mutations in SPT, 9q22.1-9q22.3Second decade of lifeDistal lower-limb involvement, ulceration of the feet, particularly the solesLow sensory action potential amplitude
Type II, Morvan diseaseAutosomal recessiveCongenital onsetPansensory loss of upper and lower limbs, also trunk and forehead; early ulcersLoss of myelinated and unmyelinated fibers
Type III, Riley-Day syndrome or familial dysautonomia)Autosomal recessive, 9q31Childhood onset, predominantly Ashkenazi JewsPallor in infancy, irregularities in temperature and blood pressure; Difficulties in eating and swallowingAbsence of unmyelinated fibers
Type IVAutosomal recessive, 1q21-1q22Congenital onsetWidespread anhidrosis, lost sense of pain, mental retardationLoss of myelinated and small unmyelinated fibers
Type VAutosomal recessiveCongenital onsetPain insensitivity in extremitiesNot applicable
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