Sections

Follow-up

Deterrence/Prevention

Maneuvers to avoid complications of orthostatic hypotension, particularly falls in elderly patients, as described above, are important to avoid further associated morbidity.

Bladder and gastrointestinal difficulties must be monitored to prevent anuria and bowel obstruction.

Patients with hypohidrosis/anhidrosis must be cautious in warm climates to avoid excessive heat, preventing heat stroke.

Foot care is essential in patients with small-fiber neuropathy and diabetic neuropathy. Patients should be instructed to test temperatures with a sensitive limb and to avoid trauma that could have ulcerative complications.

Patients with dry mouth should be instructed to seek regular dental checkups and be instructed about proper methods of oral hygiene.

Patients with dry eyes should be given advice regarding proper hydration of the eyes to avoid conjunctival difficulties.

Complications

Many complications of autonomic neuropathy exist, as described above. The most severe are cardiac arrest, cardiac dysrhythmia, blood pressure fluctuations, and risk of cerebral and cardiac ischemia

Prognosis

The prognosis depends on the particular syndrome causing autonomic neuropathy. In many cases, the course is gradually progressive in nature. In specific cases, the prognosis may be improved by controlling diabetes mellitus, limiting alcohol intake, and treating correctable syndromes or diseases as applicable to prevent progression. In the case of acute autonomic neuropathies, such as acute pandysautonomia and GBS, the prognosis is often good after resolution of the acute illness.

Patient Education

Patient education begins in the primary care office or with the neurologist as a consultant. Discussion of the following simple questions should be encouraged:

What is autonomic neuropathy?

Autonomic neuropathy is damage to nerves controlling many everyday body activities. It can be caused by a number of different diseases, each of which affects the nerves forming the autonomic nervous system. Some of the functions regulated by the autonomic nervous system are control of heart rate, blood pressure, digestion, bladder function, bowel function, sweating, and even breathing. These are unconscious vital functions important to the body.

How does it occur?

Different diseases may damage the nervous system, which includes the autonomic nervous system. The most common of these diseases is probably diabetes mellitus, but other diseases of nerves can do this as well.

What are the symptoms?

See the list below:

Lightheadedness and low blood pressure upon rising, which can lead to unconsciousness in severe cases
Heart-rate variability
Heart-rhythm variability with irregular beats
Lack of tears within the eyes
Lack of saliva
Feeling full earlier than expected with a meal
Nausea and vomiting
Problems with swallowing
Constipation, which if severe can result in bowel obstruction
Diarrhea
Incontinence of bladder and bowel functions
Urgency of bladder (rushing to the bathroom)
Problems with penile erection
Lack of sweating

How is it treated?

In many cases, no specific treatment is available for autonomic neuropathy. In some cases, such as with diabetes mellitus, the best approach is to control the diabetes to prevent progression. In mild cases, changes in diet, sleeping position, and nonmedicinal changes can help some patients. In more severe cases, drugs can be used to maintain blood pressure to prevent fainting. Medications can help with bladder function and erectile function.

How can a patient take care of him or herself?

See the list below:

Avoid rising too quickly.
Drink water regularly if standing blood pressure falls too much.
Follow physician guidelines about how to treat specific symptoms.
Avoid excessive alcohol intake, which may reduce blood pressure and lead to a neuropathy, which can worsen the autonomic problems.
Men who have trouble having erections should talk to their health care providers. Medications such as sildenafil, tadalafil, or vardenafil can help a man achieve and maintain an erection. In some cases, prosthetic devices or other devices to assist in erection may be useful.

How long will the effects last?

In most cases, autonomic neuropathy is permanent. It may become worse as the disease progresses. The goal of treatment is to relieve symptoms of the disease, or to prevent disease progression when possible.

Where can further information be obtained?

The Dysautonomia Foundation
The National Dysautonomia Research Foundation
eMedicineHealth's Brain and Nervous System Center.
eMedicineHealth's patient education articles Guillain-Barré Syndrome, Bladder Control Problems, and Chronic Fatigue Syndrome.

Questions

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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, 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, Society for Neuroscience

Disclosure: Nothing to disclose.

Bashar Katirji, MD, FACP Director, Neuromuscular Center and EMG Laboratory, The Neurological Institute, University Hospitals Cleveland 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, American Neurological Association

Disclosure: Nothing to disclose.

Specialty Editor Board

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

Disclosure: Received salary from Medscape for employment. for: Medscape.

Glenn Lopate, MD Associate Professor, Department of Neurology, Division of Neuromuscular Diseases, Washington University in St Louis School of Medicine; 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, Phi Beta Kappa

Disclosure: Nothing to disclose.

Chief Editor

Nicholas Lorenzo, MD, CPE, MHCM, FAAPL Co-Founder and Former Chief Publishing Officer, eMedicine and eMedicine Health, Founding Editor-in-Chief, eMedicine Neurology; Founder and Former Chairman and CEO, Pearlsreview; Founder and CEO/CMO, PHLT Consultants; Former Chief Medical Officer, MeMD Inc

Nicholas Lorenzo, MD, CPE, MHCM, FAAPL is a member of the following medical societies: Alpha Omega Alpha, American Academy of Neurology, American Association for Physician Leadership

Disclosure: Nothing to disclose.

Additional Contributors

Paul E Barkhaus, MD, FAAN, FAANEM Professor of Neurology and Physical Medicine and Rehabilitation, Chief, Neuromuscular and Autonomic Disorders Program, Director, ALS Program, Department of Neurology, Medical College of Wisconsin

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

Disclosure: Nothing to disclose.

HSAN	Mode of Inheritance	Onset	Symptoms	Signs
Type I	Autosomal dominant, point mutations in SPT, 9q22.1-9q22.3	Second decade of life	Distal lower-limb involvement, ulceration of the feet, particularly the soles	Low sensory action potential amplitude
Type II, Morvan disease	Autosomal recessive	Congenital onset	Pansensory loss of upper and lower limbs, also trunk and forehead; early ulcers	Loss of myelinated and unmyelinated fibers
Type III, Riley-Day syndrome or familial dysautonomia)	Autosomal recessive, 9q31	Childhood onset, predominantly Ashkenazi Jews	Pallor in infancy, irregularities in temperature and blood pressure; Difficulties in eating and swallowing	Absence of unmyelinated fibers
Type IV	Autosomal recessive, 1q21-1q22	Congenital onset	Widespread anhidrosis, lost sense of pain, mental retardation	Loss of myelinated and small unmyelinated fibers
Type V	Autosomal recessive	Congenital onset	Pain insensitivity in extremities	Not applicable

Autonomic Neuropathy Follow-up