Amyloidosis complicated by intractable postural hypotension.

Introduction The presence of postural hypotension complicating amyloid disease has been well documented. Kyle, Kottke & Schirger (1966) reviewed the case records of 138 patients in the Mayo Clinic in whom the diagnosis had been confirmed by biopsy or necropsy. In eleven (9%) of these cases postural hypotension was found. The following signs and symptoms were attributed to an amyloid autonomic neuropathy: syncope in eight cases, peripheral neuropathy in two, impotence in two, and dyshidrosis in them all. Munsat collected sixteen cases of polyneuropathy due to amyloid from the literature and to these added three cases of his own. These patients presented with chronic sensory and motor neuropathy, gastrointestinal symptoms, hoarseness, impotence, orthostatic hypotension, dyshidrosis and trophic ulcers. It was suggested in these papers that postural hypotension is due to the presence of amyloid interfering with autonomic nervous function. Bannister, Ardill & Fentem (1967) undertook detailed physiological studies in patients with idiopathic postural hypotension and concluded that the cause of the hypotension was an impaired reflex vasoconstriction in the resistance and capacity vessels. Indirect evidence possibly indicating diminished sympathetic nervous activity in these cases comes from biochemical studies. McC. Goodall, Harlan &Alton (1 968) showed in three patients with idiopathic postural hypotension that there was a diminution in production of noradrenaline and its principle metabolites, and they suggested that this and the postural hypotension were a consequence of a defect in the neurogenic