What are the other Names for this Condition? (Also known as/Synonyms)

• Autonomic Dysfunction
• Autonomic Nervous System Disorders

What is Dysautonomia? (Definition/Background Information)

• Dysautonomia describes a group of disorders that develops from abnormally regulated autonomic nervous system (ANS). The ANS is the part of nervous system that controls and regulates the involuntary actions of the body such as maintaining the body temperature, controlling blood flow to the organs, respiration, and digestive secretions
• The autonomic nervous system is subdivided into the sympathetic nervous system and parasympathetic nervous system. The sympathetic nervous system is responsible for the body’s “fight or flight response” and connects the brain and internal organs through the spinal nerves. The parasympathetic nervous system constitutes the cranial nerves (such as the vagus nerve, trigeminal nerve, etc.) and their functions
• The are two main types of Dysautonomia, namely primary and secondary:
  • Primary Dysautonomia: The term ‘primary’ is used to indicate that the condition develops in the absence of an underlying systemic condition
  • Secondary Dysautonomia: A secondary form of Dysautonomia is seen in the presence of certain conditions that affect the whole body. Such conditions include Marfan syndrome, celiac disease, and poorly-controlled diabetes, among others
• The signs and symptoms and the complications that develop depend on the severity of Dysautonomia, and the underlying cause it is associated with. These may include dizziness, anxiety, migraine headaches, abnormal heart rates, lack of sleep, and balance issues, among others
• Even though it is not possible to cure Dysautonomia, the condition may be adequately managed by treating the underlying cause and presenting signs and symptoms, which may include the administration of medications and leading a healthy lifestyle. The prognosis of Dysautonomia depends on its severity and the nature of the underlying condition, if any it is associated with

The following are some of the different types of Dysautonomia:

Onset at birth or during infancy (pediatric onset):

• Familial Dysautonomia (FD): Familial Dysautonomia, also known as Riley-Day Syndrome, is a rare genetic disorder that primarily affects the autonomic nervous system. It is characterized by impaired development and function of nerve cells, leading to a wide range of symptoms, including problems with temperature regulation, blood pressure, pain sensation, and digestion. Individuals with FD often experience difficulty swallowing, frequent pneumonia, and episodes of autonomic crisis. This condition is inherited in an autosomal recessive manner, and there is no cure, but supportive care can help manage symptoms
• Hereditary Sensory and Autonomic Neuropathy (HSAN): Hereditary Sensory and Autonomic Neuropathy refers to a group of inherited disorders that affect both the sensory and autonomic nervous systems. These conditions lead to problems with sensation, including the loss of pain perception, as well as abnormalities in autonomic functions like blood pressure regulation and sweating. There are several subtypes of HSAN, each with distinct genetic causes and varying degrees of severity. Symptoms can range from mild to severe, and treatment typically focuses on symptom management and supportive care
• Congenital Central Hypoventilation Syndrome (CCHS): Congenital Central Hypoventilation Syndrome, also known as Ondine's Curse, is a rare genetic disorder that affects breathing control. Individuals with CCHS often have a reduced urge to breathe during sleep, which can result in shallow or inadequate breathing. This condition is present from birth and can lead to oxygen deficiency in the blood and various complications. Treatment may involve assisted ventilation during sleep and sometimes during wakefulness to ensure adequate oxygen levels
• Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT): Catecholaminergic Polymorphic Ventricular Tachycardia is an inherited cardiac disorder characterized by abnormal heart rhythms, specifically ventricular tachycardia, which can be triggered by physical or emotional stress. CPVT can lead to fainting, seizures, or even sudden cardiac arrest. This condition is often caused by genetic mutations affecting calcium regulation in heart cells. Treatment involves medications to control heart rhythms and, in some cases, implantable devices like defibrillators to prevent life-threatening arrhythmias

Onset during teenage or adulthood (teen/adult onset)

• Postural Orthostatic Tachycardia Syndrome (POTS): Postural Orthostatic Tachycardia Syndrome is a type of dysautonomia characterized by an abnormal increase in heart rate when transitioning from lying down to an upright position. Individuals with POTS may experience symptoms such as rapid heartbeat, dizziness, lightheadedness, fatigue, and sometimes fainting. This condition is often associated with dysfunction of the autonomic nervous system and can have a significant impact on a person's daily life. Treatment for POTS typically involves lifestyle modifications, such as increased fluid and salt intake, along with medications to manage symptoms
• Neurocardiogenic Syncope/Autonomic Mediated Syncope: Neurocardiogenic Syncope, also known as Autonomic Mediated Syncope, refers to a type of fainting or loss of consciousness that occurs due to an abnormal autonomic nervous system response. It can result from triggers like sudden emotional stress or standing up quickly, leading to a drop in blood pressure and heart rate. Symptoms may include dizziness, nausea, sweating, and temporary loss of consciousness. Treatment options for neurocardiogenic syncope focus on identifying and avoiding triggers, as well as lifestyle modifications and, in some cases, medications
• Secondary Dysautonomia: Secondary Dysautonomia is a condition in which dysfunction of the autonomic nervous system is caused by an underlying medical condition or external factors. Unlike primary dysautonomia disorders, which have no clear underlying cause, secondary dysautonomia is a consequence of another medical condition, such as diabetes, Parkinson's disease, or certain infections. The treatment and management of secondary dysautonomia depend on addressing the underlying condition that is affecting the autonomic nervous system. It often involves a combination of therapies aimed at both the primary condition and the associated dysautonomia symptoms

Onset during middle age to older age (geriatric onset)

• Geriatric Dysautonomia: Geriatric Dysautonomia, often referred to as Senile Dysautonomia, is a condition primarily affecting the autonomic nervous system in elderly individuals. This disorder is characterized by dysfunction or impaired regulation of involuntary bodily functions such as blood pressure, heart rate, digestion, and temperature control. Symptoms may include orthostatic hypotension (a drop in blood pressure upon standing), dizziness, unexplained fainting, impaired temperature regulation, and gastrointestinal issues. Geriatric Dysautonomia can significantly impact the quality of life in older adults, making them more susceptible to falls and other health problems. Management typically involves addressing the specific symptoms and improving overall health and mobility in the elderly population
• Multiple System Atrophy (MSA): Multiple System Atrophy is a rare neurodegenerative disorder characterized by a progressive loss of nerve cells in various parts of the brain and spinal cord. It primarily affects the autonomic nervous system and motor functions. Symptoms may include autonomic dysfunction, parkinsonism, cerebellar ataxia, and other neurological issues. There are different subtypes of MSA, including MSA with predominant parkinsonism (MSA-P) and MSA with predominant cerebellar features (MSA-C). Treatment is generally aimed at managing symptoms and may include medications to improve quality of life
• Pure Autonomic Failure (PAF): Pure Autonomic Failure is a type of dysautonomia characterized by a significant dysfunction of the autonomic nervous system. Unlike other dysautonomia conditions, PAF primarily affects autonomic functions without substantial involvement of other organ systems. Common symptoms include orthostatic hypotension (a drop in blood pressure when standing), syncope (fainting), and other autonomic-related problems. Treatment often involves lifestyle modifications, increased salt intake, and medications to manage blood pressure and symptoms
• Neurogenic Orthostatic Hypotension (NOH): Neurogenic Orthostatic Hypotension is a condition characterized by a significant drop in blood pressure upon standing due to dysfunction in the autonomic nervous system. Symptoms typically include dizziness, lightheadedness, and sometimes fainting when changing positions. NOH is often associated with underlying conditions like Parkinson's disease, multiple system atrophy, or pure autonomic failure. Treatment involves measures to raise blood pressure and prevent orthostatic symptoms
• Idiopathic Dysautonomia: Idiopathic Dysautonomia refers to autonomic nervous system dysfunction without an identified underlying cause. It is a diagnosis of exclusion, meaning other potential causes have been ruled out. Symptoms can vary widely but may include problems with blood pressure regulation, heart rate variability, and other autonomic functions. Treatment aims to manage symptoms and improve quality of life, often through lifestyle adjustments and medications
• Autoimmune Autonomic Ganglionopathy (AAG): Autoimmune Autonomic Ganglionopathy is a rare autoimmune disorder that affects the autonomic nervous system by targeting autonomic ganglia. It can lead to a wide range of autonomic dysfunction symptoms, such as orthostatic hypotension, gastrointestinal issues, and abnormal heart rate responses. Treatment typically involves immunomodulatory therapies to suppress the autoimmune response and improve autonomic function

Onset at variable age:

• Primary Dysautonomia: Primary Dysautonomia refers to a group of disorders where dysfunction of the autonomic nervous system is the primary problem, and there is no known underlying cause or associated disease. These conditions typically result in a wide range of autonomic symptoms, including problems with blood pressure regulation, heart rate variability, and various other autonomic functions. Primary dysautonomia conditions include disorders like Pure Autonomic Failure (PAF) and Multiple System Atrophy (MSA), among others. Treatment is often aimed at managing symptoms and improving quality of life
• Inappropriate Sinus Tachycardia (IST): Inappropriate Sinus Tachycardia is a heart rhythm disorder characterized by an abnormally high resting heart rate or an excessive increase in heart rate during minimal physical activity or stress. The sinus node, the heart's natural pacemaker, generates electrical signals at an accelerated rate. This condition can result in symptoms such as palpitations, rapid heartbeat, dizziness, and exercise intolerance. Management may include medications to control heart rate and lifestyle modifications
• Baroreflex Failure (BF): Baroreflex Failure is a rare disorder in which the baroreceptor reflex, responsible for regulating blood pressure and heart rate, is impaired. As a result, individuals with BF may have difficulty maintaining stable blood pressure, particularly when changing positions. Symptoms often include severe orthostatic hypotension (a significant drop in blood pressure upon standing), dizziness, and fainting. Management involves lifestyle adjustments, increased salt intake, and medications to stabilize blood pressure

Who gets Dysautonomia? (Age and Sex Distribution)

• Dysautonomia is a common condition that may be observed in individuals of a wide age group from young children to elderly adults
• Both males and females are affected, and no gender preference is noted
• Worldwide, individuals of all racial and ethnic groups may be affected

What are the Risk Factors for Dysautonomia? (Predisposing Factors)

Currently, no risk factors have been established for primary Dysautonomia. However, there are a wide variety of risk factors for secondary Dysautonomia, and these include:

• Amyloidosis
• Autoimmune autonomic ganglionopathy
• Autonomic neuropathy
• Celiac disease
• Charcot-Marie-Tooth disease
• Chiari malformation
• Chronic alcoholism
• Crohn’s disease
• Dementia with Lewy bodies
• Ehlers-Danlos syndrome (EDS)
• Guillain-Barre syndrome (GBS)
• HIV infection and AIDS
• Lambert-Eaton syndrome
• Lyme disease
• Marfan syndrome
• Mast cell activation disorder
• Mitochondrial disorders
• Multiple sclerosis (MS)
• Parkinson's disease
• Postural orthostatic tachycardia syndrome
• Rheumatoid arthritis (RA)
• Sarcoidosis
• Sjögren's syndrome
• Systemic lupus erythematosus (SLE)
• Ulcerative colitis
• Uncontrolled diabetes
• Vitamin B and vitamin E deficiency disorder

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Dysautonomia? (Etiology)

The cause of Dysautonomia depends on whether it is of the primary or secondary subtype.

• No underlying or coexisting systemic disorders are identified for primary Dysautonomia; the cause is generally unknown
• Secondary Dysautonomia is the dysregulation of the autonomic nervous system due to certain underlying conditions or disorders. These systemic conditions may include autoimmune disorders, infections, injuries, neurodegenerative conditions, poorly-controlled diabetes, and alcoholism

In rare cases, Dysautonomia may be inherited from one’s parents. This form is referred to as familial Dysautonomia and is mostly observed in Ashkenazi Jews (of Eastern Europe).

The onset of signs and symptoms may be sometimes due to certain triggering factors such as:

• Alcohol
• Dehydration
• Exposure to sunlight
• Hot or cold temperatures
• Stress
• Tight clothing

What are the Signs and Symptoms of Dysautonomia?

The type and severity of signs and symptoms of Dysautonomia may markedly vary from one individual to another. In some, it may be mild, resulting in a delayed diagnosis of the condition. In other individuals, it may be severe. The signs and symptoms depend on the underlying condition (if any present) and nerves that are affected; several body systems may be affected.

The signs and symptoms of Dysautonomia may include (not exhaustive):

• Anxiety
• Blurred vision
• Bowel and urinary continence
• Constipation
• Dehydration
• Diminished or excessive sweating
• Dizziness
• Fainting
• Fatigue or weakness
• Headaches
• Increased sensitivity to light and/or noise
• Irregular heartbeat
• Low blood pressure (hypotension)
• Mood swings
• Nausea and vomiting
• Reduced blood sugar
• Reduced sense of awareness; lack of focus
• Sexual dysfunction
• Shortness of breath
• Sleeplessness
• Swallowing difficulties
• Tiredness after mild exercising
• Traumatic injuries
• Tunnel vision
• Vertigo and balance problems

Additionally, the signs and symptoms of the underlying condition may also be noted.

How is Dysautonomia Diagnosed?

The diagnosis of Dysautonomia may involve the following tests and examinations:

• Complete physical examination and a thorough medical history, including identifying any underlying conditions or disorders
• Assessment of the presenting signs and symptoms
• Ambulatory blood pressure and electrocardiogram (ECG) monitoring
• Autonomic reflex screen
• Tilt table test
• Quantitative sudomotor axon reflex test (QSART)
• Blood and urine tests, as required
• Neurological, motor skills, and cognitive assessment
• Electroencephalography (EEG)
• Electromyography with nerve conductivity tests
• Cold pressor and hyperventilation test
• Thermoregulatory sweat test
• Valsalva maneuver
• Imaging studies that may be performed include:
  • X-ray of head and neck and/or vertebral column
  • CT scan of the head and neck region and/or vertebral column
  • MRI scan of the central nervous system (brain and spine)
  • Cerebral angiographic studies
• Cerebrospinal fluid analysis, where a spinal tap test may be performed
• Urine and bladder function tests
• Tissue biopsy, as necessary

Note:

• Additional testing to diagnose the underlying infection/condition/disorder may be needed
• A differential diagnosis may be necessary to eliminate conditions that present similar signs and symptoms

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Dysautonomia?

The complications of Dysautonomia depend on the severity of the signs and symptoms and the nature of the associated/underlying condition, if any present. The complications may include: 

• Emotional and psychological stress
• Abnormally low or high heart rates
• Fluctuating blood pressures
• Hypersensitivity to external stimuli such as to light, sound, or temperature changes
• Severe respiratory issues
• Increased risk for fall injuries from balance issues
• Chronic and severe fatigue
• Paralysis
• Reduced performance at school, college, or work
• Severely affected quality of life due to mental health issues

Primary Dysautonomia is also known to cause the following conditions, some of which are severe and life-threatening:

• Multiple system atrophy (MSA)
• Neurocardiogenic syncope (NCS)
• Postural orthostatic tachycardia syndrome (POTS)
• Pure autonomic failure

Some complications are known to take place with treatment, and in some cases, due to treatment also.

How is Dysautonomia Treated?

In general, there is no cure for Dysautonomia; however, the condition can be adequately managed in many individuals through appropriate treatment.

The treatment of Dysautonomia depends upon a variety of factors including:

• Severity of the presenting signs and symptoms
• Regions of the body affected and the level of functioning of the affected organs
• Nature of the associated/underlying condition if any
• Overall health status of the individual and his/her age

Often, a multidisciplinary team of specialists that include general practitioners, rheumatologists, neurologists, pulmonologists, cardiovascular experts, nephrologists, ophthalmologists, among others, may be involved in the treatment.

The treatment may involve:

• Treating the underlying cause
• Symptomatic treatment, which may include:
  • Administration of suitable medications
  • Leading a healthy lifestyle that includes taking nutritious food, plenty of fluids, and regularly exercising
  • Regularly taking the prescribed medications
  • Reducing one’s stress levels; getting adequate sleep
  • Rehabilitation, where required
  • Having regular medical check-ups

How can Dysautonomia be Prevented?

Currently, it is not possible to prevent the primary forms of Dysautonomia since the cause of the condition is unknown. However, secondary Dysautonomia may be preventable in some cases by considering the following measures:

• Promptly taking appropriate treatment of the underlying/associated condition
• Adequately controlling blood sugar levels in diabetes
• Avoiding or minimising the consumption of alcohol
• Smoking cessation
• Leading a healthy lifestyle
• Taking plenty of water
• Avoid the triggering factors, where possible
• Patient education

What is the Prognosis of Dysautonomia? (Outcomes/Resolutions)

The prognosis of Dysautonomia may vary significantly from one individual to another, and it depends on a set of factors that includes the cause of the condition, severity of the presenting symptoms and/or associated complications, and the health status of the individual.

• With early diagnosis and adequate treatment, the prognosis may be improved
• However, in the setting of a severe underlying condition, the prognosis may be unfavorable

Additional and Relevant Useful Information for Dysautonomia:

The following article link will help you understand other Brain & Nerve health center:

https://www.dovemed.com/health-topics/neurological-institute/