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Acta Neuropathol 1997 Aug;94(2):192-6

Department of Pathology (Division of Neuropathology), Mount Sinai Medical Center, New York, NY 10029, USA.

Pure autonomic failure (PAF; also known as idiopathic orthostatic hypotension or Bradbury-Eggleston syndrome) is an uncommon sporadic disorder, characterized by autonomic failure without other neurological deficits and histopathologically by cell loss in intermediolateral columns and sympathetic ganglia. Few postmortem studies of patients with PAF have been reported in the literature, and none have demonstrated Lewy bodies in distal axons, although this has been described as a feature in Parkinson's disease with autonomic failure. We report a patient with PAF who had orthostatic hypotension and urinary symptoms for 15 years prior to death at the age of 63 years. Read More

Int Angiol 1993 Jun;12(2):93-102

Autonomic Dysfunction Center, Vanderbilt University, Nashiville, Tennessee.

Recent advances in our understanding of the pathophysiology of cardiovascular regulation and the metabolism of catecholamines have enabled us to develop an improved system of classification of autonomic disorders. Patients with autonomic impairment, clinically unassociated with other neurological abnormalities, are considered to have the Bradbury-Eggleston syndrome (idiopathic orthostatic hypotension, pure autonomic failure). Individuals whose autonomic failure is accompanied by degeneration in other neurological systems are classified as having the Shy-Drager syndrome (multiple system atrophy with autonomic failure). Read More

Dtsch Med Wochenschr 1992 Jul;117(30):1146-8

Innere Abteilung, Evangelisches Krankenhaus Gelsenkirchen.

For 15 years a now 70-year-old woman had been having occasional episodes of circulatory collapse which 7 years ago were diagnosed as being caused by severe idiopathic orthostatic hypotension. These episodes had recently become much more frequent and she was hardly able to be upright for more than one minute. In the Schellong test the blood pressure fell from 80/50 mm Hg when lying to 70/30 mm Hg on standing, the pulse rate remaining unchanged at 60/min. Read More

Rinsho Shinkeigaku 1989 Nov;29(11):1364-9

Idiopathic orthostatic hypotension (IOH) and primary sympathicotonic orthostatic hypotension (PSOH) are conspicuous orthostatic hypotension syndromes without overt somatic neurologic signs. IOH, also referred as pure autonomic failure, is a syndrome of chronic pandysautonomia, and its clinical features include supine hypertension, anhidrosis, impotence, neurogenic urinary and bowel disturbances. PSOH is different from IOH in which it is not accompanied with autonomic features outside of cardiovascular symptoms, and has been most commonly described in German-Scandinavian literatures. Read More

Minerva Med 1981 Apr;72(13):831-6

A patient with idiopathic orthostatic hypotension not accompanied by other neurologic disorders was examined. Pulmonary function tests demonstrated the failure of the autonomic innervation of airways. A syndrome of chronic primary alveolar hypoventilation was present, with the vocal cord paralysis. Read More

Can Anaesth Soc J 1979 Jul;26(4):322-7

Orthostatic hypotension due to autonomic failure may occur secondary to systemic disease states (notably diabetes) or as a disease entity in its own right with a variable degree of neurological involvement that has resulted in a confused classification. The diagnosis, classification and treatment of these latter forms of orthostatic hypotension is reviewed. The pathology is in the central and efferent autonomic pathway, resulting in a disordered baro-receptor reflex, postural hypotension, abnormal responses to tilting and the Valsalva manoeuvre, an inappropriately fixed heart rate and other autonomic features. Read More

Fortschr Neurol Psychiatr Grenzgeb 1978 May;46(5):231-47

The idiopathic orthostatic hypotension can be characterized as a presenile degenerative process with a liability to attack especially multiple systems. In the course of the disease clinically the autonomic functional failure stands in the beginning. It also determines the serious illness in the later stages, which is associated with an additional variable neurologic syndrome. Read More

Lancet 1976 Nov;2(7996):1164-7

Six patients with neurogenic orthostatic hypotension were treated with a chemical preparation of tyramine and tranylcypromine ("Parnate"), a monoamine oxidase inhibitor (M.A.O. Read More