Shy-Drager syndrome, a rare cause of orthostatic hypotension

History and admission findings: A 71-year-old man was admitted because of t reatment-resistant orthostatic hypotension of unknown aetiology. When aged 64 years he developed some impotence and later urinary incontinence and uri nary Frequency. At 68 years he noted vertigo on physical activity, and a ye ar later he had signs of reversible cerebral ischaemia. At this point the S chellong test demonstrated vasovagal circulatory dysfunction. after his 70t h birthday the unsteadiness on walking and standing got worse and he had re current syncopes. He was in a wheel-chair when hospitalized and even the un steady walk he could maintain for only a few seconds. Investigations: Plasm a and urinary concentrations of catecholamines were at the lower limit of n ormal but failed to increase during orthostasis. Hormonal, cardiological an d infectious causes of the orthostatic hypotension were excluded. Orthostat ic tests after Schellong and with the tilting table showed orthostatic hypo tension without increased sympathetic activity but hypertensive blood press ure levels during the recumbent period. Intravenous infusion of norepinephr ine produced an excess rise in blood pressure (raised norepinephrine sensit ivity). The recurrent urinary infection was shown to be due to a hypotonic bladder detrusor muscle. Neurological examination revealed cerebellar dysfu nction, signs of pyramidal tract abnormality and sensory polyneuropathy. A Shy-Drager syndrome was diagnosed on the basis of the history, absent blood pressure rise and lack of catecholamine release during orthostasis with in creased epinephrine sensitivity and characteristic neurological signs. Trea tment and course: Physiotherapy and elastic stockings with administration o f mineralocorticoids as well as of one direct (norfenefrine) and one indire ct (amezenium) sympathomimetic drug failed to improve adequately the abnorm al orthostatic response. But on additional administration of an alpha(2-)re ceptor antagonist (yohimbine) the patient was able to stand and walk for a few minutes, but the urinary incontinence and the other neurological signs remained treatment resistant. Conclusion: If orthostatic hypotension occurs together with neurological symptoms, a Shy-Drager syndrome should be taken into account.