In this section, find brief overviews of main types of dysautonomia, links to journal articles, and resources.
Dysautonomia is an umbrella term that describes conditions and diseases of the Autonomic Nervous System. They are cross specialty, easily misdiagnosed, and are more common than previously estimated. If your patient presents some of these seemingly unrelated symptoms, they may have a type of dysautonomia:
-Syncope, pre-syncope -Fatigue
-Postural tachycardia -Palpitations
-Bradycardia -Gastroparesis
-IBS -Urinary incontinence or urgency -Tingling/numbness/pain in the extremities -Dysphagia
-Unexplained chronic pain -Shortness of breath
-Fluctuations in blood pressure, -Angina
many times postural -Sleep disturbances
-Cognitive impairment -Anxiety
-Headaches/Migraines -Tremulousness
-Loss or increase in sweating -Blood pooling in limbs
-Supine hypertension -Orthostatic hypotension
Types of dysautonomia, starting with the most common form, Postural Tachycardia Syndrome (POTS):
Dysautonomia Overviews for the Medical Professional
Postural Tachycardia Syndrome (POTS):
Def: characterized by a sustained heart rate increment of ≥ 30 beats/min within 10 min of standing or head-up tilt in the absence of orthostatic hypotension. The standing heart rate for all subjects is often ≥ 120 beats/min. These criteria may not be applicable for individuals with low resting heart rates. For individuals aged 12–19 years the required increment is at least 40 beats/min. The orthostatic tachycardia may be accompanied by symptoms of cerebral hypoperfusion and autonomic over-activity that are relieved by recumbency.
Sources:
2.The Postural Tachycardia Syndrome (POTS): Pathophysiology, Diagnosis & Management, Satish R Raj, MD MSCI, Indian Pacing Electrophysiol J. 2006 Apr-Jun; 6(2): 84–99.
3. NIH Rare Disease Network Autonomic Disorders Consortium, Information For Professionals
Neurally Mediated Syncope (NMS) and Syncopal Disorders:
Def: Neurally mediated (reflex) syncope, e.g., vasovagal, situational (cough, swallowing, micturition) or carotid sinus syncope refers to a heterogeneous group of conditions in which there is a relatively sudden change in autonomic nervous system activity leading to a fall in blood pressure, heart rate and cerebral perfusion. Neurally mediated syncope is best understood as a reflex with afferent, central and efferent pathways. The term ‘neurocardiogenic syncope’ is frequently used to describe reflex syncope but it should be abandoned
because the origin of the reflex is rarely in the heart. (1)
Sources:
2.The elusive pathophysiology of neurally mediated syncope. Mosqueda-Garcia R, Furlan R, Tank J, Fernandez-Violante R., Circulation. 2000 Dec 5;102(23):2898-906.
3. NIH Rare Disease Network Autonomic Disorders Consortium, Information For Professionals
Neurogenic Orthostatic Hypotension:
Def: Orthostatic hypotension is a sustained reduction of systolic blood pressure of at least 20 mm Hg or diastolic blood pressure of 10 mm Hg within 3 min of standing or head-up tilt to at least 60° on a tilt table.
Orthostatic hypotension is a clinical sign and may be symptomatic or asymptomatic. In patients with supine hypertension, a reduction in systolic blood pressure of 30 mm Hg may be a more appropriate criterion for orthostatic hypotension because the magnitude of the orthostatic blood pressure fall is dependent on the baseline blood pressure. Orthostatic hypotension is caused by an excessive fall of cardiac output or by defective or inadequate vasoconstrictor mechanisms. The focus of this consensus statement is neurogenic orthostatic hypotension, i.e., orthostatic hypotension due to inadequate release of norepinephrine
from sympathetic vasomotor neurons leading to vasoconstrictor failure. (1)
Sources:
2.Mortality and prognosis in patients with neurogenic orthostatic hypotension. Maule S, Milazzo V, Maule MM, Di Stefano C, Milan A, Veglio F.,Funct Neurol. 2012 Apr-Jun;27(2):101-6.
3. Management of neurogenic orthostatic hypotension: an update. Low PA, Singer W., Lancet Neurol. 2008 May;7(5):451-8.
Multiple System Atrophy:
Def: Multiple system atrophy (MSA) is a progressive neurodegenerative disorder characterized by symptoms of autonomic nervous system failure such as fainting spells and bladder control problems, combined with motor control symptoms such as tremor, rigidity, and loss of muscle coordination. MSA affects both men and women primarily in their 50s. Although what causes MSA is unknown, the disorder's symptoms reflect the loss of nerve cells in several different areas in the brain and spinal cord that control the autonomic nervous system and coordinate muscle movements. The loss of nerve cells may be due to the buildup of a protein called alpha-synuclein in the cells that produce dopamine, a neurotransmitter that relays motor commands in the brain. (1)
Sources:
1. NINDS Multiple System Atrophy Information Page
2. Premotor signs and symptoms of multiple system atrophy. Jecmenica-Lukic M, Poewe W, Tolosa E, Wenning GK. Lancet Neurol. 2012 Apr;11(4):361-8. Epub 2012 Mar 19.
3. Multiple system atrophy: a clinical and neuropathological perspective. Ubhi K, Low P, Masliah E. Trends Neurosci. 2011 Nov;34(11):581-90. Epub 2011 Sep 29.
Pure Autonomic Failure:
Def: Pure autonomic failure (PAF), or Bradbury-Eggleston syndrome, is a degenerative disorder of the autonomic nervous system presenting in middle to late life, affecting men more often than women. The disorder appears to be confined to the sympathetic and parasympathetic nervous systems. (1) The adrenal medulla is relatively spared. Extreme orthostatic hypotension, reduced catecholemine levels with little change upon standing, and erectile dyfunction in men are prominent features. Differentiating between PAF and MSA is extremely important.
Sources:
1. Vanderbilt Autonomic Dysfunction Center, Information for Professionals
The Other Dysautonomias:
Find information and links to other forms of dysautonomia HERE.
Find Journal Article links to other forms of dysautonomia HERE.