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Other Forms of Dysautonomia

There are many conditions classified as dysautonomia, this is by no means a complete list. Find links here to resources, news articles, and journal articles about each disorder/disease.

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Autoimmune Autonomic Ganglionopathy (AAG):

​​The NIH defines AAG as, "...a rare disorder characterized by the presence of autonomic failure in association with specific antibodies directed against a specific receptor of the autonomic ganglia."

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AAG was formerly known as Autoimmune Autonomic Neuropathy, until a specific antibody was found in conjunction with it, the acetylcholine receptor ganglionic (G-AchR) antibody.   Not everyone with AAG has this antibody, and AAG has been found to closely resemble Pure Autonomic Failure (PAF), and is sometimes indistinguishable from PAF. The Mayo Clinic estimates that 10-15% of their POTS patients have AAG.

 

Symptoms include severe long term orthostatic hypotension (drop in blood pressure upon standing), urinary retention, constipation, dry mouth and dry eyes, and fainting. AAG has no known cause or cure, and can affect both sexes of any age.

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AAG Sources, Resources, and News:

 

Dopamine β-Hydroxylase Deficiency (DBHD):

​​The NIH defines this disorder as, "a congenital syndrome of severe orthostatic hypotension, noradrenergic failure, and ptosis of the eyelids in two young adults. The syndrome differs from familial dysautonomia and various autonomic disorders seen in adults in that the defect can be localized to the noradrenergic and adrenergic tissues. There is virtual absence of norepinephrine, epinephrine, and their metabolites. However, there is greatly increased dopamine in plasma, cerebrospinal fluid, and urine."

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This means that these individuals are missing the types of adrenalin  called norepinepherine and epinepherine, but have increased dopamine.  It seems to be present from birth, but the diagnosis is not usually made until late childhood. Symptoms include drooping eyelids, severe orthostatic hypotension (blood pressure drops upon standing), nasal stuffiness, sexual disfunction, vomiting, dehydration, hypotension, hypothermia, profound hypoglycemia requiring repeated hospitalization, and children have reduced exercise capacity.​

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This is a genetic disorder, and identification of DBHD has led to the discovery of other genetic disorders involving catecholemines (the name for the chemicals that are generally referred to as adrenalin). This is an extremely rare disorder, and life expectancy is unknown.

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DBHD Sources, Resources, and News:

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Complex Regional Pain Syndrome (CRPS)/Reflex Sympathetic Dystrophy (RSD):

​RDS is a type of CRPS, and both are nerve disorders that include burning, intense pain that is out of proportion with the injury sustained (if an injury occurred). Symptoms include: burning pain, excessive sweating, discoloration of the area, loss of use of the affected area, change in skin texture, and increased sensitivity. This usually affects the arms, hands, legs, and feet and has no apparent cause. Treatment and prognosis are very individualized, and can include surgery, medication, and physical therapy.

 

 

 

 

RSD/CRPS Support and Resources:​​

Familial Dysautonomia (FD):

​​According to the NIH, Familial Dysautonomia (also known as Riley-Day syndrome) is, "a genetic disease present at birth in male and female Ashkenazi Jews, primarily causing dysfunction of the autonomic and sensory nervous systems. Dysfunction is a result of an incomplete development of the neurons (nerve fibers) of these systems."

 

This is a very rare, serious, and life threatening disease. Riley-Day syndrome is passed down through families (inherited). A person must inherit a copy of the defective gene from each parent to develop the condition. Recent advancements in treatment have extended the life expectancy of children born with FD significantly. It is now estimated that 50% will live to 30 years of age.​

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For more information, please check out the NIH Autonomic Consortium Page and The Dysautonomia Foundation.

 

Other Resources and News:

 

 

 

 

 

Baroreflex Failure (BF):

​The NIH defines Baroreflex Failure (BR) as, "a rare disorder due to a damaged baroreflex arc, prevents the body’s buffering of high and low blood pressures. It is characterized by marked lability of BP with episodic severe hypertension and increased heart rate during stress and hypotension and normal or reduced heart rate during rest. There is often also headache, diaphoresis, emotional lability, and refractoriness of heart rate in response to exogenous vasoactive substances. It may resemble another rare disorder, pheochromocytoma, a catecholamine-secreting tumor."

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BF causes swings to extremely high blood pressures and heart rates, as well as headaches and excessive sweating. These symptoms are similar to Pheochromocytomas, which must be ruled out. ​

 

 

Baroreflex Failure Sources and Resources:

 

 

 

 

Holmes-Adie Syndrome (HAS):

​According to NIH, "Holmes-Adie syndrome (HAS) is a neurological disorder affecting the pupil of the eye and the autonomic nervous system.  It is characterized by one eye with a pupil that is larger than normal and constricts slowly in bright light  (tonic pupil), along with the absence of deep tendon reflexes, usually in the Achilles tendon.   HAS is thought to be the result of a neurotrophic (acting on neurons, or nerve cells) viral infection that causes inflammation and damage to neurons in the ciliary ganglion, an area of the brain that controls eye movements, and the dorsal root ganglion, an area of the spinal cord involved in the response of the autonomic nervous system."​

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This disorder usually affects women, and can coccur in conjunction with other disorders such as Sjogren's, Lyme dosease, and Migraines. It sometimes involves excessive sweating on one side of the body, can sometimes have cardiac implications, and is not life threatening.

 

HAS Resources, Support, & Sources:

 

 

 

 

 

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