Orthostatic intolerance

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Orthostatic intolerance (OI) is the development of symptoms when standing upright that are relieved when reclining. [1] There are many types of orthostatic intolerance. OI can be a subcategory of dysautonomia, a disorder of the autonomic nervous system [2] occurring when an individual stands up. [3] Some animal species with orthostatic hypotension have evolved to cope with orthostatic disturbances. [4] [5]

Contents

A substantial overlap is seen between syndromes of orthostatic intolerance on the one hand, and either chronic fatigue syndrome or fibromyalgia on the other. [6] It affects more women than men (female-to-male ratio is at least 4:1), usually under the age of 35. [7] OI can also be a symptom of mitochondrial cytopathy. [8]

Orthostatic intolerance occurs in humans because standing upright is a fundamental stressor, so requires rapid and effective circulatory and neurologic compensations to maintain blood pressure, cerebral blood flow, and consciousness. When a human stands, about 750 ml of thoracic blood are abruptly translocated downward. People who have OI lack the basic mechanisms to compensate for this deficit. [1] Changes in heart rate, blood pressure, and cerebral blood flow that produce OI may be caused by abnormalities in the interactions between blood volume control, the cardiovascular system, the nervous system, and circulation control system. [9]

Signs and symptoms

Orthostatic intolerance can be classified as acute OI and chronic OI.[ citation needed ]

Acute orthostatic intolerance

Patients who have acute OI usually manifest the disorder by a temporary loss of consciousness and posture, with rapid recovery (simple faints, or syncope), as well as remaining conscious during their loss of posture. This is different from a syncope caused by cardiac problems because the triggers for the fainting spell (standing, heat, emotion) and identifiable prodromal symptoms (nausea, blurred vision, headache) are known. As Dr. Julian M. Stewart, an expert in OI from New York Medical College states, "Many syncopal patients have no intercurrent illness; between faints, they are well." [1]

Symptoms: [9]

A classic manifestation of acute OI is a soldier who faints after standing rigidly at attention for an extended period of time.

Chronic orthostatic intolerance

Patients with chronic orthostatic intolerance have symptoms on most or all days. Their symptoms may include most of the symptoms of acute OI, plus:

Causes

Symptoms of OI are triggered by:

Diagnosis

Many patients go undiagnosed or misdiagnosed and either untreated or treated for other disorders. Current tests for OI (tilt table test, NASA Lean Test, [13] adapted Autonomic Profile (aAP), [14] autonomic assessment, and vascular integrity) can also specify and simplify treatment. [9] Patients with dysautonomia symptoms can be referred to a cardiologist, neurologist, or even a gastroenterologist for treatment and management. [15] [16]

Management

Most patients experience an improvement of their symptoms, but for some, OI can be gravely disabling and can be progressive in nature, particularly if it is caused by an underlying condition that is deteriorating. The ways in which symptoms present themselves vary greatly from patient to patient; as a result, individualized treatment plans are necessary. [17]

OI is treated pharmacologically and non-pharmacologically. Treatment does not cure OI; rather, it controls symptoms.[ citation needed ]

Physicians who specialize in treating OI agree that the single most important treatment is drinking more than 2 liters (8 cups) of fluids each day. A steady, large supply of water or other fluids reduces most, and for some patients all, of the major symptoms of this condition. Typically, patients fare best when they drink a glass of water no less frequently than every two hours during the day, instead of drinking a large quantity of water at a single point in the day. [6]

For most severe cases and some milder cases, a combination of medications is used. Individual responses to different medications vary widely, and a drug that dramatically improves one patient's symptoms may make another patient's symptoms much worse. Medications focus on three main issues: [6]

Medications that increase blood volume:

Medications that inhibit acetylcholinesterase:

Medications that improve vasoconstriction:

Behavioral changes that patients with OI can make are:

Notable case

A notable person with OI is Greg Page, founding member and original lead singer of the Australian children's music group The Wiggles. Due to being diagnosed with this illness, Page left the group in late 2006, and was replaced by his understudy, Sam Moran. [18] [19] Two years later in late 2008, he went on to create his own fund for OI to help fund research into this then-little known disorder. [20] Page recovered enough to temporarily return to The Wiggles in early 2012 to help with the transition to the next generation of Wiggles, after which he again left the group at the end of 2012 and was replaced by Emma Watkins and again by Tsehay Hawkins. [21]

See also

Related Research Articles

Orthostatic hypotension, also known as postural hypotension, is a medical condition wherein a person's blood pressure drops when they are standing up (orthostasis) or sitting down. Primary orthostatic hypotension is also often referred to as neurogenic orthostatic hypotension. The drop in blood pressure may be sudden, within 3 minutes or gradual. It is defined as a fall in systolic blood pressure of at least 20 mmHg or diastolic blood pressure of at least 10 mmHg after 3 minutes of standing. It occurs predominantly by delayed constriction of the lower body blood vessels, which is normally required to maintain adequate blood pressure when changing the position to standing. As a result, blood pools in the blood vessels of the legs for a longer period, and less is returned to the heart, thereby leading to a reduced cardiac output and inadequate blood flow to the brain.

Autonomic neuropathy is a form of polyneuropathy that affects the non-voluntary, non-sensory nervous system, affecting mostly the internal organs such as the bladder muscles, the cardiovascular system, the digestive tract, and the genital organs. These nerves are not under a person's conscious control and function automatically. Autonomic nerve fibers form large collections in the thorax, abdomen, and pelvis outside the spinal cord. They have connections with the spinal cord and ultimately the brain, however. Most commonly autonomic neuropathy is seen in persons with long-standing diabetes mellitus type 1 and 2. In most—but not all—cases, autonomic neuropathy occurs alongside other forms of neuropathy, such as sensory neuropathy.

<span class="mw-page-title-main">Dysautonomia</span> Any disease or malfunction of the autonomic nervous system

Dysautonomia, autonomic failure, 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, multiple system atrophy, dementia with Lewy bodies, Ehlers–Danlos syndromes, autoimmune autonomic ganglionopathy and autonomic neuropathy, HIV/AIDS, mitochondrial cytopathy, pure autonomic failure, autism, and postural orthostatic tachycardia syndrome.

<span class="mw-page-title-main">Hypotension</span> Abnormally low blood pressure

Hypotension, also known as low blood pressure, is a cardiovascular condition characterized by abnormally reduced blood pressure. Blood pressure is the force of blood pushing against the walls of the arteries as the heart pumps out blood and is indicated by two numbers, the systolic blood pressure and the diastolic blood pressure, which are the maximum and minimum blood pressures within the cardiac cycle, respectively. A systolic blood pressure of less than 90 millimeters of mercury (mmHg) or diastolic of less than 60 mmHg is generally considered to be hypotension. Different numbers apply to children. However, in practice, blood pressure is considered too low only if noticeable symptoms are present.

<span class="mw-page-title-main">Dizziness</span> Neurological condition causing impairment in spatial perception and stability

Dizziness is an imprecise term that can refer to a sense of disorientation in space, vertigo, or lightheadedness. It can also refer to disequilibrium or a non-specific feeling, such as giddiness or foolishness.

<span class="mw-page-title-main">Reflex syncope</span> Brief loss of consciousness due to a neurologically induced drop in blood pressure

Reflex syncope is a brief loss of consciousness due to a neurologically induced drop in blood pressure and/or a decrease in heart rate. Before an affected person passes out, there may be sweating, a decreased ability to see, or ringing in the ears. Occasionally, the person may twitch while unconscious. Complications of reflex syncope include injury due to a fall.

<span class="mw-page-title-main">Familial dysautonomia</span> Medical condition

Familial dysautonomia (FD), also known as Riley–Day syndrome, is a rare, progressive, recessive genetic disorder of the autonomic nervous system that affects the development and survival of sensory, sympathetic, and some parasympathetic neurons in the autonomic and sensory nervous system.

<span class="mw-page-title-main">Postural orthostatic tachycardia syndrome</span> Abnormally high heart rate after a postural change

Postural orthostatic tachycardia syndrome (POTS) is a condition characterized by an abnormally large increase in heart rate upon sitting up or standing. POTS is a disorder of the autonomic nervous system that can lead to a variety of symptoms, including lightheadedness, brain fog, blurred vision, weakness, fatigue, headaches, heart palpitations, exercise intolerance, nausea, diminished concentration, tremulousness (shaking), syncope (fainting), coldness or pain in the extremities, numbness or tingling in the extremities, chest pain, and shortness of breath. Other conditions associated with POTS include myalgic encephalomyelitis/chronic fatigue syndrome, migraine headaches, Ehlers–Danlos syndrome, asthma, autoimmune disease, vasovagal syncope, and mast cell activation syndrome. POTS symptoms may be treated with lifestyle changes such as increasing fluid, electrolyte, and salt intake, wearing compression stockings, gentle and slow postural changes, avoiding prolonged bedrest, medication, and physical therapy.

<span class="mw-page-title-main">Midodrine</span> Vasopressor/antihypotensive agent

Midodrine, sold under the brand names ProAmatine and Orvaten among others, is a vasopressor or antihypotensive medication used to treat orthostatic hypotension and urinary incontinence. It is taken by mouth.

Lightheadedness is a common and typically unpleasant sensation of dizziness or a feeling that one may faint. The sensation of lightheadedness can be short-lived, prolonged, or, rarely, recurring. In addition to dizziness, the individual may feel as though their head is weightless. The individual may also feel as though the room is "spinning" or moving (vertigo). Most causes of lightheadedness are not serious and either cure themselves quickly or are easily treated.

<span class="mw-page-title-main">Tilt table test</span> Medical procedure often used to diagnose dysautonomia or syncope

A tilt table test (TTT), occasionally called upright tilt testing (UTT), is a medical procedure often used to diagnose dysautonomia or syncope. Patients with symptoms of dizziness or lightheadedness, with or without a loss of consciousness (fainting), suspected to be associated with a drop in blood pressure or positional tachycardia are good candidates for this test.

<span class="mw-page-title-main">Pure autonomic failure</span> Medical condition

Pure autonomic failure (PAF) is an uncommon, sporadic neurodegenerative condition marked by a steadily declining autonomic regulation. Bradbury and Eggleston originally described pure autonomic failure in 1925.

<span class="mw-page-title-main">Inappropriate sinus tachycardia</span> Syndrome where the sinus heart rate is inexplicably faster than expected

Inappropriate sinus tachycardia (IST) is defined as sinus tachycardia that is not caused by identifiable medical ailments, a physiological reaction, or pharmaceuticals (a diagnosis of exclusion) and is accompanied by symptoms, frequently invalidating and affecting quality of life. IST symptoms include palpitations, chest discomfort, exhaustion, shortness of breath, presyncope, and syncope.

<span class="mw-page-title-main">Syncope (medicine)</span> Transient loss of consciousness and postural tone

Syncope, commonly known as fainting or passing out, is a loss of consciousness and muscle strength characterized by a fast onset, short duration, and spontaneous recovery. It is caused by a decrease in blood flow to the brain, typically from low blood pressure. There are sometimes symptoms before the loss of consciousness such as lightheadedness, sweating, pale skin, blurred vision, nausea, vomiting, or feeling warm. Syncope may also be associated with a short episode of muscle twitching. Psychiatric causes can also be determined when a patient experiences fear, anxiety, or panic; particularly before a stressful event, usually medical in nature. When consciousness and muscle strength are not completely lost, it is called presyncope. It is recommended that presyncope be treated the same as syncope.

<span class="mw-page-title-main">Orthostatic headache</span> Medical condition

Orthostatic headache is a medical condition in which a person develops a headache while vertical and the headache is relieved when horizontal. Previously it was often misdiagnosed as different primary headache disorders such as migraine or tension headaches. Increasing awareness of the symptom and its causes has prevented delayed or missed diagnosis.

<span class="mw-page-title-main">Dopamine beta hydroxylase deficiency</span> Medical condition

Dopamine beta (β)-hydroxylase deficiency is a human medical condition involving inadequate dopamine beta-hydroxylase. It is characterized by increased amounts of serum dopamine and the absence of norepinephrine (NE) and epinephrine.

Orthostatic syncope refers to syncope resulting from a postural decrease in blood pressure, termed orthostatic hypotension.

Heat intolerance is a symptom characterized by feeling overheated in warm environments or when the surrounding environment's temperature rises. Typically, the person feels uncomfortably hot and sweats excessively.

<span class="mw-page-title-main">Orthostatic hypertension</span> Medical condition

Orthostatic hypertension is a medical condition consisting of a sudden and abrupt increase in blood pressure (BP) when a person stands up. Orthostatic hypertension is diagnosed by a rise in systolic BP of 20 mmHg or more when standing. Orthostatic diastolic hypertension is a condition in which the diastolic BP raises to 98 mmHg or over in response to standing, but this definition currently lacks clear medical consensus, so is subject to change. Orthostatic hypertension involving the systolic BP is known as systolic orthostatic hypertension.

<span class="mw-page-title-main">Autoimmune autonomic ganglionopathy</span> Medical condition

Autoimmune autonomic ganglionopathy is a type of immune-mediated autonomic failure that is associated with antibodies against the ganglionic nicotinic acetylcholine receptor present in sympathetic, parasympathetic, and enteric ganglia. Typical symptoms include gastrointestinal dysmotility, orthostatic hypotension, and tonic pupils. Many cases have a sudden onset, but others worsen over time, resembling degenerative forms of autonomic dysfunction. For milder cases, supportive treatment is used to manage symptoms. Plasma exchange, intravenous immunoglobulin, corticosteroids, or immunosuppression have been used successfully to treat more severe cases.

References

  1. 1 2 3 4 Julian M. Stewart. "Orthostatic Intolerance: An overview". WebMD . Retrieved 2007-08-20.
  2. ↑ "What is dysautonomia?". National Dysautonomia Research Foundation (NDRF). Retrieved 2007-08-20.
  3. ↑ Definition at Dorland's Illustrated Medical Dictionary Retrieved through web archive on 2008-10-09.
  4. ↑ Lillywhite, Harvey B. (November 1993). "Orthostatic Intolerance of Viperid Snakes". Physiological Zoology. 66 (6): 1000–1014. doi:10.1086/physzool.66.6.30163751. JSTOR   30163751. S2CID   88375293.
  5. ↑ Nasoori, Alireza; Taghipour, Ali; Shahbazzadeh, Delavar; Aminirissehei, Abdolhossein; Moghaddam, Sharif (September 2014). "Heart place and tail length evaluation in Naja oxiana, Macrovipera lebetina, and Montivipera latifii". Asian Pacific Journal of Tropical Medicine. 7: S137–S142. doi: 10.1016/S1995-7645(14)60220-0 . PMID   25312108.
  6. 1 2 3 4 5 Peter C. Rowe. "General information brochure on Orthostatic Intolerance and its treatment". The Pediatric Network. Archived from the original on 2007-07-28. Retrieved 2007-08-21.
  7. ↑ "Vanderbilt autonomic dysfunction center". Vanderbilt Medical Center. Archived from the original on 2007-08-08. Retrieved 2007-08-20.
  8. ↑ Kanjwal, Khalil; Karabin, Beverly; Kanjwal, Yousuf; Saeed, Bilal; Grubb, Blair P. (October 2010). "Autonomic dysfunction presenting as orthostatic intolerance in patients suffering from mitochondrial cytopathy". Clinical Cardiology. 33 (10): 626–629. doi:10.1002/clc.20805. ISSN   1932-8737. PMC   6653231 . PMID   20960537.
  9. 1 2 3 Julian M. Stewart. "Orthostatic Intolerance". New York Medical College. Archived from the original on 2007-08-06. Retrieved 2007-08-20.
  10. ↑ "Orthostatic Intolerance: Background, Pathophysiology, Etiology". 2023-06-30.{{cite journal}}: Cite journal requires |journal= (help)
  11. 1 2 Joyner, Michael J.; Masuki, Shizue (December 2008). "POTS versus deconditioning: the same or different?". Clinical Autonomic Research. 18 (6): 300–307. doi:10.1007/s10286-008-0487-7. PMC   3770293 . PMID   18704621.
  12. ↑ Pertab, Jon L.; Merkley, Tricia L.; Cramond, Alex J.; Cramond, Kelly; Paxton, Holly; Wu, Trevor (2018). "Concussion and the autonomic nervous system: An introduction to the field and the results of a systematic review". NeuroRehabilitation. 42 (4): 397–427. doi:10.3233/NRE-172298. ISSN   1878-6448. PMC   6027940 . PMID   29660949.
  13. ↑ Lee, Jihyun; Vernon, Suzanne D.; Jeys, Patricia; Ali, Weam; Campos, Andrea; Unutmaz, Derya; Yellman, Brayden; Bateman, Lucinda (2020-08-15). "Hemodynamics during the 10-minute NASA Lean Test: evidence of circulatory decompensation in a subset of ME/CFS patients". Journal of Translational Medicine. 18 (1): 314. doi: 10.1186/s12967-020-02481-y . ISSN   1479-5876. PMC   7429890 . PMID   32799889.
  14. ↑ M, Sivan; J, Corrado; C, Mathias (2022-08-08). "The adapted Autonomic Profile (aAP) home-based test for the evaluation of neuro-cardiovascular autonomic dysfunction". ACNR, Advances in Clinical Neuroscience and Rehabilitation. doi: 10.47795/qkbu6715 .
  15. ↑ "Finding the Right Doctor for You". 9 August 2011.
  16. ↑ "Dysautonomia: Symptoms, types, and treatment". 16 December 2021.
  17. ↑ "National Dysautonomia Research Foundation". National Dysautonomia Research Foundation (NDRF). Retrieved 2007-08-21.
  18. ↑ "Greg Page leaves the Wiggles". The Wiggles Home Page. Archived from the original on 2007-09-28. Retrieved 2007-08-21.
  19. ↑ Maddox, Greg. "Life without a skivvie". The Sydney Morning Herald Online. Retrieved 2008-11-29.
  20. ↑ "Orthostatic Intolerance".
  21. ↑ "Original Yellow Wiggle Greg Page gets his skivvy back". The Daily Telegraph. 2012-01-18.
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