Cases reported "Shy-Drager Syndrome"

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1/19. Clinical effects of elastic bandage on neurogenic orthostatic hypotension.

    Neurogenic orthostatic hypotension (OH) often causes troublesome symptoms such as dizziness, syncope and falling, interfering active daily life or various therapies in rehabilitation. Nonpharmacologic measures for treating patients with OH include wearing elastic leotard, head-up tilting at night, etc. Elastic garment or antigravity suits is certainly effective, but it may be uncomfortable and not practical. Although elastic bandage (EB) bound on the lower limbs has been thought to be useful, there is few clinical report about its beneficial evidence. We investigated short-term clinical effects of commercially available EB on OH, and estimated the mechanism of its effectiveness by measuring some blood pressure-related humoral variables in neurodegenerative patients with OH.
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2/19. Effect of xamoterol in shy-drager syndrome.

    BACKGROUND. xamoterol, a cardioselective beta 1-adrenoceptor partial agonist, has been reported to be effective on postural hypotension. We investigated the effect of xamoterol in five patients with shy-drager syndrome (SDS) in relation to their prevailing sympathetic nerve activity and sensitivity of beta-adrenoceptors and the change in circadian variation of blood pressure. methods AND RESULTS. Ambulatory blood pressure over 24 hours was monitored by noninvasive sphygmomanometer (model 5200, Spacelab). plasma norepinephrine levels of SDS patients were significantly lower than that of normal subjects (n = 5) both at rest (54 /- 15 versus 178 /- 83 pg/ml) and after 10-minute standing (74 /- 24 versus 318 /- 143 pg/ml). Infusion of isoproterenol (0.02 micrograms/kg/min) produced a mild rise of systolic blood pressure and tachycardia in normal subjects but resulted in marked hypotension and tachycardia in SDS subjects. After xamoterol administration (200 mg b.i.d.), systolic blood pressure and heart rate were significantly increased in the averages during the day; however, increases were more pronounced at night. In two of the five patients, the improvement in dizziness was large enough to enable them to increase their daily activities. CONCLUSIONS. Our observations suggest that 1) beta 1-selective, high intrinsic sympathomimetic activity of xamoterol increases blood pressure and heart rate in patients with SDS as a consequence of their prevailing beta 1-adrenoceptor hypersensitive state, and 2) blood pressure monitoring over 24 hours appears to have important advantages in evaluating the therapeutic effects on postural hypotension.
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3/19. Primary sjogren's syndrome presenting with generalized autonomic failure.

    A 64 year-old woman developed Raynaud's phenomenon and dry eyes/mouth. Laboratory examination revealed positive Schirmer's test, rheumatoid factor and anti-nuclear antibody, and lymphocytic sialoadenitis on salivary gland biopsy. These features strongly suggested the diagnosis of primary sjogren's syndrome. Three years later, she gradually developed generalized autonomic failure without apparent sensory neuropathy on nerve conduction study. She had systolic pressure fall of 51 mmHg on head-up tilt test, cardiovascular supersensitivity to diluted norepinephrine infusion, cardiac denervation in [123I]-MIBG scintigraphy, impaired R-R variability, decreased sweating and prolonged colonic transit time. Autoimmune autonomic ganglionopathy was mostly responsible for her autonomic failure.
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4/19. Postural hypotension: pressor effect of octreotide not mediated by norepinephrine.

    Orthostatic hypotension of the shy-drager syndrome is a chronic incapacitating condition characterized by lack of an appropriate increase in the plasma norepinephrine level in response to standing. Recently, the somatostatin analogue octreotide has been reported to induce a pressor response in patients having this syndrome. We have reported a case of shy-drager syndrome in which octreotide was effective, but the rise in blood pressure was not accompanied by an increase in the plasma norepinephrine level. Hence, the pressor effect of octreotide is not mediated by stimulation of the sympathetic nervous system, but probably through splanchnic vasoconstriction.
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5/19. Abnormal cardiovascular responses to postural changes and pharmacologic agents in a case of shy-drager syndrome.

    A 64-year-old carpenter had an unsteady gait, severe dizziness, nocturia, and a loss of erection for more than 4 years. The neurological manifestations consisted of a wide-based ataxic gait, bilateral dysmetria with intentional tremor, staccato speech, rigidity, bradykinesia, and an iris-thinning. There was reproducible orthostatic hypotension. A sweat test revealed severe anhidrosis. nicotine and methylbenzene sensitivity was absent, whereas norepinephrine infusion test showed a significant elevation of blood pressure. The resting plasma norepinephrine level on recumbency was low and a subnormal surge was noted on standing or exercise. We conclude that the clinical features caused by a degenerative process involving both the central and peripheral autonomic systems, together with atrophy of other systems in this patient, constitute the shy-drager syndrome.
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6/19. Transcranial Doppler sonographic studies of cerebral autoregulation in shy-drager syndrome.

    A study is reported of mean arterial blood pressure and heart rate in four patients suffering from shy-drager syndrome. blood flow velocity in the middle cerebral artery (MCA) was recorded by transcranial Doppler sonography. Concomitant changes in cerebral blood flow and the effect of cerebral autoregulation were thus examined. During tilt (60 degrees, head up) mean arterial blood pressure decreased by 40 mm Hg or 35%, while MCA blood flow velocity dropped by 14 cm/s or 28% (mean values). The lower percentage reduction in flow velocity may indicate a preserved cerebral autoregulation in central autonomic insufficiency.
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7/19. Non-invasive cardiovascular monitoring in shy-drager syndrome.

    The pattern of arterial blood pressure and heart rate were investigated in a patient with progressive autonomic dysfunction and nineteen healthy age-matched controls. A marked postprandial reduction of blood pressure was observed in the patient; this phenomenon was not noted in the control group; besides, the rhythmicity of blood pressure and heart rate were altered with peak values shifted approximately eight hours later with respect to the controls.
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8/19. Effect of L-threo-3,4-dihydroxyphenylserine on muscle sympathetic nerve activities in shy-drager syndrome.

    We observed changes in postganglionic efferent discharges of muscle sympathetic nerve (muscle sympathetic activity, MSA) microneurographically before and after the oral administration of L-threo-3,4-dihydroxyphenylserine (L-threo-DOPS), a precursor of norepinephrine, in a patient with shy-drager syndrome and irregular fluctuations of blood pressure. Before drug administration, MSA was only rarely observed with the patient in the supine position. There was a slight increase in MSA during head-up tilting to 40 degrees, and orthostatic hypotension (OH) occurred just after the body was tilted head upward to 40 degrees. MSA became prominent 30 minutes after the oral administration of 200 mg of L-threo-DOPS while the patient was in a 40 degree head-up position, and the OH was improved. The MSA discharge rate decreased and OH reappeared 3 hours after oral administration, when the plasma concentration of norepinephrine was at its highest level. We suggest that the OH improved mainly because of the increase in MSA due to L-threo-DOPS, and that the drug may activate sympathetic outflow at a site proximal to the sympathetic ganglion.
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9/19. sleep-related respiratory and haemodynamic changes in shy-drager syndrome: a case report.

    The sleep-related respiratory and blood pressure changes in a patient with shy-drager syndrome associated with the sleep apnoea syndrome are reported. Polygraphic recordings showed repeated apnoeic episodes during both sleep and wakefulness. Systemic arterial pressure values during sleep tended to be lower than in two other patients with shy-drager syndrome, and, unlike observations in the sleep apnoea syndrome, nocturnal swings of arterial pressure related to obstructive apnoea were markedly reduced. As a result, the total sleep time was reduced; a sleep with several features similar to REM stage was identified; during this stage the arterial pressure reached the lowest levels recorded. A review of the literature revealed that nocturnal respiratory disturbances were detectable in a high percentage of patients with shy-drager syndrome. We suggest that such an association is not a chance one.
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10/19. Treatment of orthostatic hypotension in shy-drager syndrome with DL-threo-3,4-dihydroxyphenylserine: a case report.

    Orthostatic hypotension in a patient with shy-drager syndrome was treated for 6 months with oral DL-threo-3,4-dihydroxyphenylserine (DL-threo-DOPS). Adrenergic function was evaluated before and during treatment by measurements of changes in blood pressure and plasma norepinephrine on head-up tilting and by the response of blood pressure to the valsalva maneuver and infused norepinephrine. After the patient received DL-threo-DOPS, the fall in his mean arterial blood pressure on head-up tilting was reduced and he had no syncope when standing. When peripheral decarboxylase inhibitor was combined with DL-threo-DOPS, the same beneficial effect on orthostatic hypotension was observed.
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