1/9. Endogenous circulating sympatholytic factor in orthostatic intolerance.Sympathotonic orthostatic hypotension (SOH) is an idiopathic syndrome characterized by tachycardia, hypotension, elevated plasma norepinephrine, and symptoms of orthostatic intolerance provoked by assumption of an upright posture. We studied a woman with severe progressive SOH with blood pressure unresponsive to the pressor effects of alpha(1)-adrenergic receptor (AR) agonists. We tested the hypothesis that a circulating factor in this patient interferes with vascular adrenergic neurotransmission. Preincubation of porcine pulmonary artery vessel rings with patient plasma produced a dose-dependent inhibition of vasoconstriction to phenylephrine in vitro, abolished vasoconstriction to direct electrical stimulation, and had no effect on nonadrenergic vasoconstrictive stimuli (endothelin-1), PGF-2alpha (or KCl). Preincubation of vessels with control plasma was devoid of these effects. SOH plasma inhibited the binding of an alpha(1)-selective antagonist radioligand ([(125)I]HEAT) to membrane fractions derived from porcine pulmonary artery vessel rings, rat liver, and cell lines selectively overexpressing human ARs of the alpha(1B) subtype but not other AR subtypes (alpha(1A) and alpha(1D)). We conclude that a factor in SOH plasma can selectively and irreversibly inhibit adrenergic ligand binding to alpha(1B) ARs. We propose that this factor contributes to a novel pathogenesis for SOH in this patient. This patient's syndrome represents a new disease entity, and her plasma may provide a unique tool for probing the selective functions of alpha(1)-ARs.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/9. Splaying of the carotid bifurcation caused by a cervical sympathetic chain schwannoma.Splaying of the carotid bifurcation revealed by an imaging study is usually indicative of a carotid body tumor, but there are other possibilities. To promote awareness of a cervical sympathetic chain schwannoma as another cause of splaying of the carotid bifurcation, we present a case of cervical sympathetic chain schwannoma, with an additional 7 cases in the English-language literature, and discuss the relationship between the great vessels of the neck and a carotid body tumor or a schwannoma of the cervical sympathetic chain or vagus nerve from an anatomic viewpoint. We conclude that splaying of the carotid bifurcation with hypervascularity suggests a carotid body tumor, whereas in cases without hypervascularity, a cervical sympathetic chain schwannoma is another possibility. vagus nerve schwannomas can separate the internal jugular vein and internal carotid artery, but seldom widen the carotid bifurcation.- - - - - - - - - - ranking = 0.33333333333333keywords = vessel (Clic here for more details about this article) |
3/9. Progressive bulbar dysfunction caused by a predominantly venous vascular malformation of the medulla oblongata.We describe a 58-year-old patient with a rare predominantly venous vascular malformation of the medulla oblongata, which caused a progressive bulbar dysfunction consisting of hiccoughs, dysphagia, hoarseness, dysarthria, gait ataxia and dysuria over a period of 11 months. On autopsy, a large dilated vein with focal marked intimal fibroelastic thickening was present on the ventral surface of the medulla. Microscopically, moderate proliferation of capillaries and veins was observed which was confined primarily to the medulla. The veins displayed abnormal dilatation and tortuosity; prominent thickening of vessel walls was also present in the veins and capillaries. The venous abnormalities were prominent in the parenchyma of the medulla, but much less apparent in its subarachnoid space. Multifocal neuronal loss and gliosis were observed, most prominently in the inferior olives, hypoglossal, dorsal vagal and ambiguus nuclei. The histopathologic findings suggested that abnormal venous drainage within the parenchyma of the medulla was the most critical factor for the pathogenesis of this patient's neurologic symptomatology.- - - - - - - - - - ranking = 0.33333333333333keywords = vessel (Clic here for more details about this article) |
4/9. Normocytic normochromic anemia due to automatic neuropathy in type 2 diabetic patients without severe nephropathy: a possible role of microangiopathy.We describe here four male patients with long-term and poorly controlled type 2 diabetes mellitus. They shared many common characteristic complications, such as severe autonomic neuropathy, proliferative retinopathy and normocytic normochromic anemia without progressive renal failure and macroangiopathy. They also showed normal levels of erythropoietin and reticulocyte, which was considered relatively low. The coefficient of variation of R-R, a useful method to estimate autonomic failure, showed markedly advanced autonomic neuropathy in all four patients. coronary angiography did not reveal stenosis, anomaly or collateral vessels, but left ventriclography showed diffuse or partial hypokinesis. Massive proteinuria, high urinary levels of N-acetyl-beta-D-glucosamidase (NAG) and beta2-microglobulin (beta2M) were detected, though creatinine clearance (Ccr) was not so deteriorated. Treatment with recombinant erythropoietin increased their hemoglobin and hematocrit levels. These common points have a possibility to be brought about by tubulointerstitial damage and microangiopathy may be involved in it.- - - - - - - - - - ranking = 0.33333333333333keywords = vessel (Clic here for more details about this article) |
5/9. Hemicrania with massive autonomic manifestations and circumscribed eyelid erythema.OBJECTIVES: To describe a unilateral headache that in addition to the typical shortlasting unilateral neuralgiform headache attacks with conjunctival injection, tearing, sweating and rhinorrhea (SUNCT) syndrome - traits with excessive and ipsilateral autonomic phenomena - had circumscribed eyelid erythema and adjacent ocular redness. OBSERVATIONS: A 60-year-old female had excessive, right-sided lacrimation and local pain at 15 years of age. Due to steadily increasing discomfort, with lacrimation and swelling over the outer part of the upper eyelid, the right lacrimal gland was removed at 20 years of age, with a suspicion of lacrimal gland adenoma. Preoperatively, symptomatic side mild-degree eyelid erythema/rhinorrhea were integral parts of the attack. After years with minor complaints, she, in the mid-twenties, experienced more long-lasting pain attacks, and pain soon became the main problem. A marked, distinct erythema on the lateral part of the right-sided eyelids and marked, localized 'eye redness' in the adjacent area were main ingredients of the attacks together with eyelid edema and viscous rhinorrhea. There were visible vessels below the eye, and telangiectasia of the upper eyelid. CONCLUSIONS: This headache has many similarities with SUNCT - but has several, grossly deviating traits: the temporal aspects, excruciatingly intense pain attacks, and above all marked, lateral eye-lid erythema, and adjacent, massive ocular reddening. This constellation probably alienates it from SUNCT.- - - - - - - - - - ranking = 0.33333333333333keywords = vessel (Clic here for more details about this article) |
6/9. Polyneuropathy in sjogren's syndrome. A case of prevalently autonomic neuropathy with tonic pupil and hypohidrosis.A 38-year-old woman suffering from primary sjogren's syndrome displayed a neurologic picture consisting of left tonic pupil, generalized tendon areflexia and left-sided hypohidrosis. Electrophysiological and pathological studies suggested a mild degree of peripheral nerve involvement. Moderate loss of large myelinated fibres and obliteration of small endoneurial vessels were seen in the sural nerve biopsy. Sympathetic skin response was absent and alterations of unmyelinated nerve fibres were found. A prevalently autonomic neuropathy with tonic pupils may represent a characteristic picture in the spectrum of peripheral nerve involvement in sjogren's syndrome. Other similar cases reported in the literature are reviewed.- - - - - - - - - - ranking = 0.33333333333333keywords = vessel (Clic here for more details about this article) |
7/9. prenalterol in the treatment of orthostatic hypotension in shy-drager syndrome.The effects of prenalterol, a selective beta 1-adrenoreceptor agonist, were studied in a patient with the shy-drager syndrome, presenting with incapacitating orthostatic hypotension. The main haemodynamic defect was an impressive postural fall in stroke volume and cardiac output pointing to denervation of the capacitance vessels. prenalterol 4 X 30 mg orally produced a marked increase in supine and standing blood pressure, along with substantial symptomatic improvement. Notable positive chronotropic and inotropic effects were observed. association of fludrocortisone 0.5 mg/day resulted in further haemodynamic and symptomatic improvement, presumably due to plasma volume expansion. Haemodynamically, prenalterol and fludrocortisone resulted in a substantial increase in standing cardiac output, primarily due to the chronotropic effects of prenalterol. In addition to the haemodynamic effects, prenalterol stimulated the renin-aldosterone system and restored the normal diurnal pattern of water and sodium excretion, the latter may have contributed to the improvement of orthostatic tolerance. prenalterol could be a valuable adjunct to the existing treatment schedules of neurogenic orthostatic hypotension.- - - - - - - - - - ranking = 0.33333333333333keywords = vessel (Clic here for more details about this article) |
8/9. Paroxysmal hypotension associated with sympathetic withdrawal. A new disorder of autonomic vasomotor regulation.We evaluated a patient who had transient episodes of hypotension with clinical and laboratory features apparently distinct from previously recognized disorders of vasomotor regulation. In between his abrupt attacks of hypotension, the patient is asymptomatic and demonstrates normal autonomic modulation of heart rate and blood pressure in response to changes in body position, valsalva maneuver, cold, and exercise. During periods of hypotension, his plasma norepinephrine falls markedly and he has blunted or absent responses to stimuli that normally have a pressor effect due to sympathetic efferent discharge. Mechanical or known hormonal disorders that produce episodic hypotension have been excluded by extensive testing. We suggest two possible causes for our patient's paroxysmal sympathetic withdrawal: first, a centrally mediated inhibition of sympathetic discharge to peripheral resistance and capacitance vessels, but with no afferent stimulus reflexly producing sympathetic withdrawal readily evident; or second, an episodic release of an unknown endogenous compound with inhibitory effects upon central or preganglionic sympathetic neurons or upon postganglionic sympathetic neurons by a presynaptic inhibition of norepinephrine release.- - - - - - - - - - ranking = 0.33333333333333keywords = vessel (Clic here for more details about this article) |
9/9. Visceral orthostatic hypotension in patients with severe autonomic dysfunction.Although changes in the blood flow of the cerebral vessels and the peripheral vessels in the extremities after changing body postures have been well examined in patients with orthostatic hypotension (OH), such changes in visceral vessels have not been well investigated. To elucidate the effect of autonomic dysfunctions on changes in the abdominal blood flow, the blood flow velocity of the portal vein was measured by Doppler ultrasonography in 11 patients with familial amyloidotic polyneuropathy (FAP) (Met30), 3 with pandysautonomia, 1 with shy-drager syndrome, and 10 healthy controls, in the supine and at the upright position. Among the 15 patients with the above-mentioned autonomic disorders, 5 of the patients showed a marked decrease in blood flow after standing, and one of these 5 patients exhibited transient hepatic and intestinal ischemia during intensive rehabilitation because of a severe decrease in visceral blood flow. Another 7 patients exhibited moderate decreases in the blood flow after standing. In contrast, no such change was observed in the 10 healthy controls. The FAP patients with L-threo-3,4-dihydroxyphenylserine (L-threo-DOPS) administration showed no significant correlation between the degree of OH and the decrease in the blood flow of the portal vein, and the patients without the drug exhibited a weak correlation. On the contrary, the pandysautonomia and shy-drager syndrome patients exhibited a linear positive correlation. These results suggest that FAP is a disease for which this kind of ultrasonographic examination should be applied, and that Doppler ultrasonography may be a helpful tool to evaluate visceral OH.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |