Orthostatic hypotension and nicotine sensitivity in a case of multiple system atrophy.

Abstract

Shy and Drager (1960), on the basis of some 40 published case reports, described a clinical syndrome to which their names have subsequently been attached.The complete syndrome includes ortho- static hypotension, anhidrosis, urinary and faecal incontinence, sexual impotence, Parkinsonian signs, external ocular palsies, amyotrophy, and iris atrophy.A detailed post-mortem examination of one of their cases showed neuronal degeneration at many sites, including the intermediolateral columns of the spinal cord.They did not comment on the relation of this finding to the autonomic features of their patient's disorder.In 1965, Fichefet, Sternon, Franken, Demanet, and Vanderhaeghen described a case presenting with orthostatic hypotension, with subsequent development of Parkinsonism.The pathology was that of idiopathic paralysis agitans, with Lewy-type cyto- plasmic inclusions and loss of pigmented nerve cells.In addition, the authors observed some cell loss in the anterior and lateral horns of the spinal cord.In 1966, Johnson, Lee, Oppenheimer, and Spalding described the clinical, physiological, and pathological findings in two cases of orthostatic hypotension.In the first of these, the only associated disturbances were sexual impotence and loss of sweating.Lewy-type inclusions were found in the pigmented nuclei and elsewhere, but there was no detectable loss of pigmented cells.Cell loss in the intermediolateral columns was estimated at nearly 90%.In the second case, the autonomic disorder was followed, after a few years, by a progressive disorder of motor and cerebellar function.The lesions were those of olivo-ponto-cerebellar atrophy; in addition, there was cell loss, estimated at about 75 %, in the intermediolateral columns.Since then, there have been four reports on cases that have gone to necropsy-that of Nick, Contamin, Escourolle, Guillard, and Marcantoni (1967) and the two cases of Schwarz (1967)-all of which showed neuronal degeneration at various sites, including the inter-