What is Pure Autonomic Failure?

Pure Autonomic Failure (PAF), also known as Bradbury-Egglestone Syndrome, is dysautonomic disorder affecting people during midlife but can occur at all ages. It is the presence of orthostatic hypotension (OH), not caused by outside sources, and no evidence of other neurological problems. Many medications cause OH particularly antidepresents. The autonomic nervous system controls the respiratory and cardiovascular systems of the body. This enables different body systems to work together in harmony to carry out daily activities such as exercise and digestion.

The main symptom is orthostatic hypotension; there may be other autonomic symptoms like decreased sweating, heat intolerance, urinary retention, erectile dysfunction, constipation, pupillary abnormalities.

Diagnosis is difficult due to the vagueness and fluctuations of symptoms but is accomplished by exclusion. Often, factors that affect diagnosis is medications and enviormental factors. Hypotension diagnosis must be done with a detailed history of the patients blood pressure as well as other symptoms. Blood pressure is taken in the sitting, standing and laying down positions. Other testing that is often accomplished include blood tests and brain scans to rule out other diseases.

Pure autonomic failure has a nearly normal life expectancy, but due to the condition can be life altering. An increase of symptoms is followed by long periods of stability. It is a chronic ilness and therefore can be disabling.

Treatments include avoiding the situations that bring the symptoms on, changes to diet, raising and controlling blood pressure and medication. Medications include Fludrocortisone (Florinef), Midodrine (ProAmatine), Propranolol (Inderal), Desmopressin (DDAVP, Stimate), Epoetin alfa (Epogen, Procrit), Metoclopramide (Reglan), Pyridostigmine bromide (Mestinon) , Psyllium (Metamucil, Fiberall), Oxybutynin (Ditropan), Bethanechol hydrochloride (Duvoid, Urecholine), Prednisone (Deltasone, Orasone, Meticorten) nad Immune globulins intravenous (IVIG, Gammagard, Gamimune). With any medicines, side effects should be monitored closely and reported to doctors as necessary for dosing adjustment. 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.