The distribution of Lewy bodies in pure autonomic failure: autopsy findings and review of the literature.

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.

Classification of autonomic disorders.

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).

[The Shy-Drager syndrome].

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.

[Primary orthostatic hypotension syndromes without somatic neurologic signs--idiopathic orthostatic hypotension and primary sympathicotonic orthostatic hypotension].

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.

[A case of primary orthostatic hypotension of the Bradbury-Eggleston type].

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.

Anaesthetic considerations in idiopathic orthostatic hypotension and the Shy-Drager syndrome.

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.

[The idiopathic orthostatic hypotension -- Shy-Drager syndrome (author's transl)].

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.

Treatment of neurogenic orthostatic hypotension with a monoamine oxidase inhibitor and tyramine.

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