Pure autonomic failure (PAF) is a form of dysautonomia that first occurs in middle age or later in life; diagnosed more often in men than in women.

Signs and symptoms

A degenerative disease of the

autonomic nervous system The autonomic nervous system (ANS), formerly referred to as the vegetative nervous system, is a division of the peripheral nervous system that supplies viscera, internal organs, smooth muscle and glands. The autonomic nervous system is a control ...

, symptoms include dizziness and fainting (caused by orthostatic hypotension), visual disturbances and neck pain. Chest pain,

fatigue Fatigue describes a state of tiredness that does not resolve with rest or sleep. In general usage, fatigue is synonymous with extreme tiredness or exhaustion that normally follows prolonged physical or mental activity. When it does not resolve ...

and sexual dysfunction are less common symptoms that may also occur.

Symptoms Signs and symptoms are the observed or detectable signs, and experienced symptoms of an disease, illness, injury, or condition. A sign for example may be a higher or lower temperature than normal, raised or lowered blood pressure or an abnormali ...

are worse when standing; sometimes one may relieve symptoms by lying down. More pervasive autonomic dysfunction involving any of the following: night sweats or abnormal lack of sweating, urogenital problems (frequent UTIs, incontinence, frequency, urgency), gastrointestinal problems (chronic constipation, chronic constipation alternating with diarrhea, poor gastric motility), or esophageal/respiratory problems ( sleep apnea, abnormal breath sounds during sleep or while awake) indicate possible autoimmune autonomic ganglionopathy or

multiple system atrophy Multiple system atrophy (MSA) is a rare neurodegenerative disorder characterized by autonomic dysfunction, tremors, slow movement, muscle rigidity, and postural instability (collectively known as parkinsonism) and ataxia. This is caused by progr ...

Pathology

The pathology 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 A neuron, neurone, or nerve cell is an electrically excitable cell that communicates with other cells via specialized connections called synapses. The neuron is the main component of nervous tissue in all animals except sponges and placozoa. N ...

. 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 fludrocortisone, midodrine,

somatostatin Somatostatin, also known as growth hormone-inhibiting hormone (GHIH) or by several other names, is a peptide hormone that regulates the endocrine system and affects neurotransmission and cell proliferation via interaction with G protein-couple ...

erythropoietin Erythropoietin (; EPO), also known as erythropoetin, haematopoietin, or haemopoietin, is a glycoprotein cytokine secreted mainly by the kidneys in response to cellular hypoxia; it stimulates red blood cell production (erythropoiesis) in the bo ...

, and other

vasopressor An antihypotensive agent, also known as a vasopressor agent or simply vasopressor, or pressor, is any substance, whether endogenous or a medication, that tends to raise low blood pressure. Some antihypotensive drugs act as vasoconstrictors to inc ...

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. Use of an abdominal binder is even more effective. Also, ingesting more water than usual can increase blood pressure and relieve some symptoms.

History

In 1925, Bradbury and Eggleston first characterized three patients seemingly with a common syndrome, with what they described as "the occurrence of syncopal attacks after or during exertion or even after standing erect for some minutes. Other features in the three patients are a slow, unchanging pulse rate, incapacity to perspire, a lowered basal metabolism and signs of slight and indefinite changes in the nervous system. Each of these patients felt much worse during the heat of summer." Further research identified multiple causes for these syndromic findings, now grouped as primary autonomic disorders (also called primary dysautonomia), including Pure Autonomic Failure, Multiple System Atrophy, and Parkinson's. The primary differentiating characteristic of Pure autonomic failure is decreased circulation and synthesis of norepinephrine, and dysfunction localized peripherally. It is relevant to note that progression to central nervous system neurodegeneration can also occur.

Eponym

It is also known as Bradbury-Eggleston syndrome, named after

Samuel Bradbury Samuel ''Šəmūʾēl'', Tiberian: ''Šămūʾēl''; ar, شموئيل or صموئيل '; el, Σαμουήλ ''Samouḗl''; la, Samūēl is a figure who, in the narratives of the Hebrew Bible, plays a key role in the transition from the bibl ...

and Cary Eggleston who first described it in 1925.S. Bradbury, C. Eggleston. Postural hypotension: A report of three cases. American Heart Journal, St. Louis, 1925, 1: 73-86.

References

External links

{{Autonomic diseases Peripheral nervous system disorders