Dysautonomia

Dysautonomia (autonomic dysfunction) is a broad term that describes any disease or malfunction of the autonomic nervous system. This includes postural orthostatic tachycardia syndrome (POTS), vasovagal syncope, mitral valve prolapse dysautonomia, pure autonomic failure, Neurocardiogenic syncope (NCS), Neurally Mediated Hypotension (NMH) autonomic instability and a number of lesser-known disorders such as cerebral salt-wasting syndrome. Dysautonomia is associated with multiple system atrophy (Shy-Drager syndrome),^[1] Ehlers-Danlos syndrome, and Marfan syndrome for reasons that are not fully understood.

Presentation

Symptoms of dysautonomia are numerous and vary widely from person to person. Since dysautonomia is a full-body condition, a large number of symptoms may be present that can greatly alter a person's quality of life. Each patient with dysautonomia is different—some are affected only mildly while others are left completely bedridden and disabled.

The primary symptoms that present in patients with dysautonomia are:

• Excessive fatigue
• Excessive thirst (polydipsia)
• Lightheadedness, dizziness or vertigo
• Feelings of anxiety or panic (not mentally induced)
• Rapid heart rate or slow heart rate
• Orthostatic hypotension, sometimes resulting in Syncope^[2]

Other symptoms frequently associated with dysautonomia include: headaches, pallor, malaise, facial flushing, constipation, diarrhea, nausea, acid reflux, visual disturbances, orthostatic hypotension, numbness, nerve pain, trouble breathing, chest pains, in some cases loss of consciousness and seizures. ^[2] A full list of symptoms may be found here.

Causes

Causes of dysautonomias are not fully understood, but they are thought to include:

• Viral illness
• Brain injury^[3]
• Genetic factors
• Bad body posture (causes compression of important arteries and/or nerves)
• Exposure to chemicals
• Pregnancy
• Hereditary connective tissue diseases, especially Ehlers-Danlos Syndrome
• Autoimmune disorders, especially diabetes
• degenerative neurological diseases such as Parkinson's disease
• Physical trauma or injury which damages the autonomic nervous system, as with Cerebral salt-wasting syndrome.^[3]

Management

There is no cure or treatment for dysautonomia. Medications are used to stabilize the condition on a long-term basis. Benzodiazepines can be used for some of the physical problems such as anxiety. In many cases treatment of primary dysautonomia is symptomatic and supportive. Measures to combat orthostatic intolerance include elevation of the head of the bed, frequent small meals, a high-salt diet, fluid intake, and compression stockings. Drugs such as fludrocortisone, midodrine, ephedrine and SSRIs can also be used to treat symptoms. Treating dysautonomia can be difficult. Treatment that helps one individual may actually worsen the symptoms of another. Often drugs and measures that are helpful are found through trial and error.

Prognosis

The outlook for patients with dysautonomia depends on the particular diagnostic category. Most forms of dysautonomia resolve within 2-3 years and are not life threatening, even if life changing in the form of minor to major limitations in activities of daily living. However patients with Ehlers-Danlos Syndrome, Marfan Syndrome or Parkinson's disease have a chronic, progressive, generalized form of dysautonomia in the setting of central nervous system degeneration, leading to a generally poor long-term prognosis. Patients can die from pneumonia, acute respiratory failure, or sudden cardiopulmonary arrest. ^[4]

There is some evidence that dysautonomia may be a factor in SIDS (sudden infant death syndrome).

History

In the nineteenth and earlier twentieth centuries, a diagnosis that was almost solely given to women was called "neurasthenia," or a "weak nervous system." (During World War One, doctors began to apply it to men exhibiting symptoms of what we now call post-traumatic stress disorder.) These women would present symptoms of fatigue, weakness, dizziness and fainting, and the doctor's orders would simply be bed rest. Some of these women died, while many others recovered. No one understood where the problems came from. With the advances in modern medicine, diagnostic criteria and treatment for various forms of dysautonomia have sharpened. Doctors and researchers are including males in their subject population for this disorder.

The prototype of dysautonomia is the ancient scourge of beriberi,a nutritional deficiency disease due to excess of simple carbohydrate and concomitant vitamin B1 deficiency. In the early stages this results in loss of functional efficiency in the central control mechanisms of the autonomic nervous system. If the nutritional deficiency continues, there is gradual degeneration of the system.Other vitamin deficiencies have been implicated in causing dysautonomia and unlike the genetically determined forms of the disease, are treatable.^[5]

See also

• Familial dysautonomia
• Dopamine beta hydroxylase deficiency
• Postural Orthostatic Tachycardia Syndrome

Footnotes

References

• A family of misunderstood disorders, by Richard N Fogoros

External links

• Dysautonomia at the Open Directory Project
• dysautonomia at NINDS
• Dysautonomia Youth Network of America, Inc.
• ndrf.org
• 12 More Pages-Live & Cope with Dysautonomia
• Dysautonomia Information Network
• Handbook for Patients with Dysautonomia, by Dr. David S. Goldstein, MD, PhD and Linda J. Smith - Free chapter by chapter PDF download