|Other names||Autonomic dysfunction|
|The autonomic nervous system|
|Causes||Inadequacy of sympathetic, or parasympathetic, components of autonomic nervous system|
|Risk factors||Alcoholism and Diabetes|
|Diagnostic method||Ambulatory Blood pressure, as well as EKG monitoring|
|Treatment||Symptomatic and supportive|
Dysautonomia or autonomic dysfunction is a condition in which the autonomic nervous system (ANS) does not work properly. This may affect the functioning of the heart, bladder, intestines, sweat glands, pupils, and blood vessels. Dysautonomia has many causes, not all of which may be classified as neuropathic. A number of conditions can feature dysautonomia, such as Parkinson's disease, HIV/AIDS, multiple system atrophy, autonomic failure, postural orthostatic tachycardia syndrome, Ehlers-Danlos syndrome, autoimmune autonomic ganglionopathy, and autonomic neuropathy.
The diagnosis is achieved through functional testing of the ANS, focusing on the affected organ system. Investigations may be performed to identify underlying disease processes that may have led to the development of symptoms or autonomic neuropathy. Symptomatic treatment is available for many symptoms associated with dysautonomia, and some disease processes can be directly treated.
Signs and symptoms
The symptoms of dysautonomia, which are numerous and vary widely for each individual, are due to inefficient or unbalanced efferent signals sent via both systems. The primary symptoms in individuals with dysautonomia include
- Blurry or double vision
- Bowel incontinence
- Brain fog
- Difficulty swallowing
- Exercise intolerance
- Chronic fatigue
- Low blood pressure
- Orthostatic hypotension
- Tunnel vision
- Urinary incontinence or urinary retention
Dysautonomia may be due to inherited or degenerative neurologic diseases (primary dysautonomia) or it may occur due to injury of the autonomic nervous system from an acquired disorder (secondary dysautonomia). The most common causes of dysautonomia include
- Autoimmune disease, such as Sjögren's syndrome or systemic lupus erythematosus (lupus), and autoimmune autonomic ganglionopathy
- Craniocervical instability
- Eaton-Lambert syndrome
- Ehlers-Danlos syndrome
- Guillain-Barré syndrome
- HIV and AIDS
- Lyme disease
- Multiple sclerosis
- Multiple system atrophy
- Paraneoplastic syndrome
- Parkinson's disease
- Spinal cord injury
- Surgery or injury involving the nerves
- Toxicity (vincristine)
In the sympathetic nervous system (SNS), predominant dysautonomia is common along with fibromyalgia, chronic fatigue syndrome, irritable bowel syndrome, and interstitial cystitis, raising the possibility that such dysautonomia could be their common clustering underlying pathogenesis.
In addition to sometimes being a symptom of dysautonomia, anxiety can sometimes physically manifest symptoms resembling autonomic dysfunction. A thorough investigation ruling out physiological causes is crucial, but in cases where relevant tests are performed and no causes are found or symptoms do not match any known disorders, a primary anxiety disorder is possible, but should not be presumed. For such patients, the anxiety sensitivity index may have better predictivity for anxiety disorders, while the Beck anxiety inventory may misleadingly suggest anxiety for patients with dysautonomia.
The autonomic nervous system is a component of the peripheral nervous system and comprises two branches: the sympathetic nervous system (SNS) and the parasympathetic nervous system (PSNS). The SNS controls the more active responses such as increasing heart rate and blood pressure. The PSNS slows down the heart rate and aids in digestion, for example. Symptoms typically arise from abnormal responses of either the sympathetic or parasympathetic systems based on situation or environment.
The diagnosis of dysautonomia depends on the overall function of three autonomic functions – cardiovagal, adrenergic, and sudomotor. A diagnosis should, at a bare minimum, include measurements of blood pressure and heart rate while lying flat, and after at least 3 minutes of standing. The best way to achieve a diagnosis includes a range of testing, notably an autonomic reflex screen, tilt table test, and testing of the sudomotor response (QSART or thermoregulatory sweat test).
Additional tests and examinations to determine a diagnosis of dysautonomia include
- Ambulatory blood pressure and EKG monitoring
- Cold pressor test
- Deep breathing
- Hyperventilation test
- Nerve biopsy for small fiber neuropathy
- Quantitative sudomotor axon reflex test (QSART)
- Testing for orthostatic intolerance
- Thermoregulatory sweat test
- Tilt table test
- Valsalva maneuver
Tests to elucidate the cause of dysautonomia can include:
Particularly in the Russian literature, a subtype of dysautonomia which particularly affects the vascular system has been called vegetative-vascular dystonia. The term "vegetative" reflects an older name for the autonomic nervous system: the vegetative nervous system.
The treatment of dysautonomia can be difficult; since it is made up of many different symptoms, a combination of drug therapies is often required to manage individual symptomatic complaints. Therefore, if an autoimmune neuropathy is the case, then treatment with immunomodulatory therapies is done, or if diabetes mellitus is the cause, control of blood glucose is important. Treatment can include proton-pump inhibitors and H2 receptor antagonists used for digestive symptoms such as acid reflux.
For the treatment of genitourinary autonomic neuropathy medications may include sildenafil (a guanine monophosphate type-5 phosphodiesterase inhibitor). For the treatment of hyperhidrosis, anticholinergic agents such as trihexyphenidyl or scopolamine can be used, also intracutaneous injection of botulinum toxin type A can be used for management in some cases.
The prognosis of dysautonomia depends on several factors; individuals with chronic, progressive, generalized dysautonomia in the setting of central nervous system degeneration such as Parkinson's disease or multiple system atrophy have a generally poorer long-term prognosis. Consequently, dysautonomia could be fatal due to pneumonia, acute respiratory failure, or sudden cardiopulmonary arrest.
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