1/286. Postural hypotension in idiopathic Parkinson's disease. Etiopathology. Postural changes in blood pressure were recorded in all 391 patients suffering from Parkinson's syndrome over a period of six years. Intraarterial blood pressure studies were carried out in those with significant postural hypotension. Histological examination of the entire central nervous system and the sympathetic ganglia was performed in six patients suffering from idiopathic Parksinson's disease. Five of the six patients had lewy bodies in the sympathetic ganglia. Loss of nerve cells was noted in the sympathetic ganglia in those patients that demonstrated postural hypotension. The severity of the lesions in the ganglia correlated with the severity of postural hypotension in idiopathic Parkinson's disease, One case of shy-drager syndrome was similarly studied to demonstrate the differences in spinal cord and sympathetic ganglia lesions in the two conditions. ( info) |
2/286. Orthostatic hypotension improved after bilateral carotid endarterectomy--case report. A 60-year-old male with recurrent syncopal attacks presented with orthostatic hypotension on the head-up tilt test. angiography also showed severe stenosis of the bilateral extracranial carotid arteries. He underwent two-staged bilateral carotid endarterectomy. After the operations, the orthostatic hypotension resolved and the syncopal attacks have disappeared completely. Orthostatic hypotension in this patient was due to vasodepressor-type carotid sinus syndrome caused by compression of the carotid baroreceptors by atherosclerotic plaques. ( info) |
3/286. Acute pulmonary edema associated with placement of waist-high, custom-fit compression stockings. Compression stockings are a safe, noninvasive treatment for patients with symptomatic orthostatic hypotension due to autonomic nervous system dysfunction. In this report, we describe a 75-year-old man who had development of pulmonary edema approximately 45 minutes after placement of compression stockings on the first postoperative day following a carotid endarterectomy. No sudden changes were noted on an electrocardiogram or echocardiogram or in the cardiac isoenzymes associated with the pulmonary edema. The patient had a history of coronary artery disease, diabetes mellitus-induced autonomic nervous system dysfunction, and recent surgery near the carotid baroreceptor. All these factors may have limited his ability to compensate for a rapid increase in central blood volume. The temporal relationship of the patient's respiratory distress to the placement of the compression stockings, in the absence of laboratory findings of primary cardiac dysfunction, make stocking-related fluid shift the likely precipitating event in the formation of acute pulmonary edema. This case suggests that compression stockings should be used with caution in patients with limited cardiac reserve. ( info) |
4/286. Supine hypotensive syndrome caused by intra-abdominal mass: a case report. An obese woman who presented with 3 separate intra-abdominal masses developed a supine hypotensive syndrome following induction of general anesthesia. The hypotension was corrected by positioning the patient in a left lateral tilt and by releasing intra-abdominal pressure. Following decompression of the vena cava, arterial and central venous pressure rose and remained at a high level. urine output was poor until IV furosemide was administered. ( info) |
5/286. The use of oral vasopressors in the management of autonomic dysfunction and orthostatic hypotension. OBJECTIVE: To describe a patient with hypotension secondary to autonomic dysfunction who was successfully treated with oral vasopressors. CASE SUMMARY: A 76-year-old African-American man with a history of cerebrovascular accident with right hemiparesis 30 years prior to admission was admitted from another hospital four days after a new posterior inferior cerebellar artery occlusion and poor distal flow as manifested by weakness and hypotension. This was treated with intravenous fluids and dopamine. The dopamine was weaned and changed to phenylephrine to maintain systolic blood pressure >80 mm Hg. fludrocortisone 0.3 mg orally once daily was initiated; pressure support garments were used for the management of orthostatic hypotension. ephedrine 25 mg po tid was added and titrated up to 50 mg p.o. tid. yohimbine 5.4 mg po every eight hours was added due to continued dependence on phenylephrine to maintain adequate blood pressure. yohimbine was titrated up to 10.8 mg p.o. tid in an unsuccessful effort to wean the patient from phenylephrine. fludrocortisone was decreased to 0.1 mg po tid and the phenylephrine was tapered off. The patient developed a pan-sensitive escherichia coli urinary tract infection that was treated with oral trimethoprim/sulfamethoxazole. Over the subsequent days, an 80% left subclavian stenosis was detected; yohimbine and pressure support garments were discontinued. Subsequently, oral ephedrine was tapered off over two days, and fludrocortisone was tapered to 0.1 mg p.o. bid. The patient was transferred in a stable normotensive condition to an inpatient rehabilitation unit. The fludrocortisone was later discontinued with no further hypotension or orthostatic symptoms. DISCUSSION: In this case, orthostatic hypotension associated with autonomic dysfunction was successfully managed with a combination of intravenous vasopressors and hydration, pressure support garments, oral mineralocorticoids, and oral vasopressors. Oral vasopressors and mineralocorticoids are effective treatment options in the management of the vasopressor-dependent patient. In our patient the adverse effects were tolerable. After continued therapy, the oral vasopressors were withdrawn without a return of orthostatic symptoms. CONCLUSIONS: Orthostatic hypotension due to autonomic dysfunction may be successfully managed with combination oral therapy after initial treatment with intravenous vasopressors as evidenced by the absence of orthostasis. ( info) |
6/286. fatigue that doesn't go away. The classic profile of the chronic fatigue syndrome (CFS) patient is a white, middle-age female. Characterized by profound fatigue, CFS often starts with an acute viral infection. While today's medicine provides symptomatic relief, research is offering innovative insights. With this research, is a cure for these patients just around the corner? ( info) |
7/286. Autonomic failure and proximal skeletal myopathy in a patient with primary Sjogren syndrome. Autonomic failure and proximal skeletal myopathy are rare features of the Sjogren syndrome (SS). We describe a 51-year-old woman with primary SS who had development of esophageal dysmotility, urinary retention, severe orthostatism, and skeletal myopathy during a 3-month period after the diagnosis of SS. Her symptoms and signs responded well to corticosteroid therapy. Although dysfunction of the peripheral nervous system has a prevalence rate of 20% in patients with SS, most commonly the nerve dysfunction is a sensory deficit, and autonomic neuropathy is less frequent. Autonomic neuropathy due to SS may be underreported. The cause of our patient's myopathy remains undetermined. We speculate that the myopathy was due to either a form of polymyositis or an immune-mediated neuropathy with muscle involvement. ( info) |
8/286. Clinical and physiological characteristics of autonomic failure with Parkinson's disease. We analyzed the clinical and physiological features of autonomic failure with Parkinson's disease (AF-PD) in seven patients and compared them with those of autonomic failure with multiple system atrophy (AF-MSA). In AF-PD, parkinsonism was more gradually progressive than in AF-MSA, and symptoms were responsive to L-dopa. All seven patients with AF-PD had orthostatic hypotension, postprandial hypotension, and constipation, but no urinary retention. Of these, three had hypohidrosis and five had frequent urination; five patients had subnormal plasma norepinephrine (NE) concentrations. Supersensitivity to NE infusion was observed in all patients. head-up tilting (HUT) test resulted in no increase of plasma NE concentrations in both groups, but a significant increase of the plasma arginine vasopressin (AVP) concentrations in the patients with AF-PD. Urodynamic studies revealed that urinary bladder function was relatively well preserved in AF-PD in contrast to AF-MSA. In conclusion, there exists some clinical and physiological differences in autonomic features between AF-PD and AF-MSA, and postganglionic involvement predominates in AF-PD. ( info) |
9/286. Interaction of genetic predisposition and environmental factors in the pathogenesis of idiopathic orthostatic intolerance. BACKGROUND: The hemodynamic and autonomic abnormalities in idiopathic orthostatic intolerance (IOI) have been studied extensively. However, the mechanisms underlying these abnormalities are not understood. If genetic predisposition were important in the pathogenesis of IOI, monozygotic twins of patients with IOI should have similar hemodynamic and autonomic abnormalities. methods: We studied two patients with IOI and their identical twins. Both siblings in the first twin pair had orthostatic symptoms, significant orthostatic tachycardia, increased plasma norepinephrine levels with standing, and a greater than normal decrease in systolic blood pressure with trimethaphan infusion. RESULTS: Both siblings had a normal response of plasma renin activity to upright posture. In the second twin pair, only one sibling had symptoms of orthostatic intolerance, an orthostatic tachycardia, and raised plasma catecholamines with standing. The affected sibling had inappropriately low plasma renin activity with standing and was 8-fold more sensitive to the pressor effect of phenylephrine than the unaffected sibling. CONCLUSIONS: We conclude that in some patients, IOI seems to be strongly influenced by genetic factors. In others, however, IOI may be mainly caused by nongenetic factors. These findings suggest that IOI is heterogenous, and that both genetic and environmental factors contribute individually or collectively to create the IOI phenotype. ( info) |
10/286. pupil abnormality in amyloidosis with autonomic neuropathy. darkness pupil diameters, light reflexes, and redilatation times have been recorded with infrared TV pupillometry in 12 consecutive patients with systemic amyloidosis associated with sensory motor and autonomic neuropathy. Nine of the patients had AL amyloidosis, two had familial amyloidosis associated with a transthyretin abnormality, and one was untyped. The pupils were abnormal in all 12 patients. On the basis of redilatation lag without pupillotonia, six patients had bilateral Horner's syndrome and in one of them amyloid deposits were found in a sympathetic ganglion and in the attached sympathetic chain obtained at necropsy. Four patients had bilateral tonic pupils with light-near dissociation and two had abnormally small pupils with reduced light reactions which could not be characterised. It seems that in patients with systemic amyloidosis generalised autonomic neuropathy is strongly associated with pupil abnormality as shown by tonic reactions with light-near dissociation, by redilatation lag, or by reduced size in darkness. ( info) |