Pure autonomic failure
Encyclopedia
Pure autonomic failure, also known as Bradbury-Eggleston syndrome or idiopathic orthostatic hypotension, is a form of dysautonomia
that first occurs in middle age or later in life; men are affected more often than women. It is one of three diseases classified as primary autonomic failure
.
, symptoms include dizziness
and fainting (caused by orthostatic hypotension
), visual disturbances and neck pain. Chest pain, fatigue
and sexual dysfunction
are less common symptoms that may also occur. Symptoms are worse when standing; sometimes one may relieve symptoms by laying down.
of pure autonomic failure is not yet completely understood. However, a loss of cells in the intermediolateral column of the spinal cord
has been documented, as has a loss of catecholamine
uptake and catecholamine fluorescence in sympathetic postganglionic neurons. In general, levels of catecholamines in these patients are very low while lying down, and do not increase much upon standing.
, midodrine
, somatostatin
, erythropoietin
, and other vasopressor agents. However, often a patient with pure autonomic failure can mitigate his or her symptoms with far less costly means. Compressing the legs and lower body, through crossing the legs, squatting, or the use of compression stockings
can help. Also, ingesting more water than usual can increase blood pressure and relieve some symptoms.
Dysautonomia
Dysautonomia is a broad term that describes any disease or malfunction of the autonomic nervous system. This includes postural orthostatic tachycardia syndrome , inappropriate sinus tachycardia , vasovagal syncope, mitral valve prolapse dysautonomia, pure autonomic failure, neurocardiogenic...
that first occurs in middle age or later in life; men are affected more often than women. It is one of three diseases classified as primary autonomic failure
Primary autonomic failure
There are three diseases classified as primary autonomic failure: pure autonomic failure, multiple system atrophy , and Parkinson disease.-References:* *...
.
Symptoms
A degenerative disease of the autonomic nervous systemAutonomic nervous system
The autonomic nervous system is the part of the peripheral nervous system that acts as a control system functioning largely below the level of consciousness, and controls visceral functions. The ANS affects heart rate, digestion, respiration rate, salivation, perspiration, diameter of the pupils,...
, symptoms include dizziness
Dizziness
Dizziness refers to an impairment in spatial perception and stability. The term is somewhat imprecise. It can be used to mean vertigo, presyncope, disequilibrium, or a non-specific feeling such as giddiness or foolishness....
and fainting (caused by orthostatic hypotension
Orthostatic hypotension
Orthostatic hypotension, also known as postural hypotension, orthostasis, and colloquially as head rush or dizzy spell, is a form of hypotension in which a person's blood pressure suddenly falls when the person stands up or stretches. The decrease is typically greater than 20/10 mm Hg, and may be...
), visual disturbances and neck pain. Chest pain, fatigue
Fatigue (physical)
Fatigue is a state of awareness describing a range of afflictions, usually associated with physical and/or mental weakness, though varying from a general state of lethargy to a specific work-induced burning sensation within one's muscles...
and sexual dysfunction
Sexual dysfunction
Sexual dysfunction or sexual malfunction refers to a difficulty experienced by an individual or a couple during any stage of a normal sexual activity, including desire, arousal or orgasm....
are less common symptoms that may also occur. Symptoms are worse when standing; sometimes one may relieve symptoms by laying down.
Pathology
The pathologyPathology
Pathology is the precise study and diagnosis of disease. The word pathology is from Ancient Greek , pathos, "feeling, suffering"; and , -logia, "the study of". Pathologization, to pathologize, refers to the process of defining a condition or behavior as pathological, e.g. pathological gambling....
of pure autonomic failure is not yet completely understood. However, a loss of cells in the intermediolateral column of the spinal cord
Spinal cord
The spinal cord is a long, thin, tubular bundle of nervous tissue and support cells that extends from the brain . The brain and spinal cord together make up the central nervous system...
has been documented, as has a loss of catecholamine
Catecholamine
Catecholamines are molecules that have a catechol nucleus consisting of benzene with two hydroxyl side groups and a side-chain amine. They include dopamine, as well as the "fight-or-flight" hormones adrenaline and noradrenaline released by the adrenal medulla of the adrenal glands in response to...
uptake and catecholamine fluorescence in sympathetic postganglionic neurons. In general, levels of catecholamines in these patients are very low while lying down, and do not increase much upon standing.
Treatment
Pharmacological methods of treatment include fludrocortisoneFludrocortisone
Fludrocortisone is a synthetic corticosteroid with moderate glucocorticoid potency and much greater mineralocorticoid potency. The brand name in the U.S. and Canada is Florinef.-Uses:...
, midodrine
Midodrine
Midodrine is a vasopressor/antihypotensive agent. Midodrine was approved in the United States by the Food and Drug Administration in 1996 for the treatment of orthostatic hypotension...
, somatostatin
Somatostatin
Somatostatin is a peptide hormone that regulates the endocrine system and affects neurotransmission and cell proliferation via interaction with G-protein-coupled somatostatin receptors and inhibition of the release of numerous secondary hormones.Somatostatin...
, erythropoietin
Erythropoietin
Erythropoietin, or its alternatives erythropoetin or erthropoyetin or EPO, is a glycoprotein hormone that controls erythropoiesis, or red blood cell production...
, and other vasopressor agents. However, often a patient with pure autonomic failure can mitigate his or her symptoms with far less costly means. Compressing the legs and lower body, through crossing the legs, squatting, or the use of compression stockings
Compression stockings
Compression stockings and socks are specialised hosiery items designed to increase blood circulation. Their main theraputic purpose is to provide graduated pressure on the lower leg and foot and, in some cases, the thigh, to alleviate circulatory problems such as edema, phlebitis and...
can help. Also, ingesting more water than usual can increase blood pressure and relieve some symptoms.