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At least 19 recordsLinked to original sources

Autonomic dysfunction in women with mitral valve prolapse syndrome.

Autonomic cardiovascular regulation was evaluated in 35 women, 19 with mitral valve prolapse and 16 healthy controls. Heart rate responses to the diving reflex and to phenylephrine infusion were diminished in patients. Noninvasive measures of cardiac output, heart rate, blood pressure, forearm flow and leg volume during lower body negative pressure (LBNP) showed that patients had less lower extremity pooling of blood and had lower forearm conductance. Blood pressures during LBNP rose or remained unchanged despite decreases in cardiac output of 20--25%. These data indicate that mitral valve prolapse patients have an increased venous and arterial vasoconstrictor activity. Cardiac output at rest and echocardiographic indices of contractility were normal. Patients with a history of significant ventricular arrhythmias had higher heart rates and lower forward stroke volumes than the other patients or controls. The combined data demonstrate autonomic dysfunction in women with the mitral valve prolapse syndrome and suggest decreased parasympathetic, increased alpha- and normal beta-adrenergic tone and responsiveness.

Adult

Adult onset autonomic dysfunction coexistent with familial dysautonomia in a consanguineous family.

A consanguineous family is described in which autonomic dysfunction developed in the father during adult life while the son had familial dysautonomia at birth. The father's condition is felt to be secondary to olivopontocerebellar atrophy. The concurrence in this family of an adult and a childhood form of dysautonomia may be an expression of the same genetic defect at different stages of development.

Adult

The Riley-Day syndrome. Familial dysautonomy, central autonomic dysfunction.

The Riley-Day syndrome is characterized by a dysfunction of the autonomous nervous system, sensory disturbances, neurological disorders, psychical anomalies and important ophthalmological symptoms, such as absence of tears, corneal anaesthesia, keratinized conjunctiva and cornea; myosis after instillation of methacholine. The diagnosis is based on the absence of fungiform papillae of the tongue and the absence of reaction after intradermic injection of histamine. The inheritance is autosomal recessive. The disease results probably from an enzymatic insufficiency.

Child, Preschool

Autonomic dysfunction in the Landry-Guillain-Barre syndrome.

Tests of autonomic function have been performed on 7 patients with the Landry-Guillain-Barre syndrome and 5 control subjects. These included a sweat test, and measurement of both postural hypotension and baroreflex sensitivity. Sweating was definitely abnormal in 5 patients. Postural hypotension was present in 4 patients. The baroreflex sensitivity was significantly reduced (p less than 0.01) in the patients when compared with the control group. The results demonstrate that both the sympathetic and parasympathetic nervous systems may be affected in the Landry-Guillain-Barre syndrome.

Adult

Autonomic dysfunction in botulism B: a clinical report.

Nine cases of botulism B with preponderant effects upon cholinergic autonomic innervation are presented. Blurred vision and dry mouth were constant symptoms. Impairment of salivary and lacrimal secretion were detectable for months. In the absence of clinical signs, electromyographic studies did not reveal neuromuscular involvement. Administration of antitoxin in the late course of such cases is not recommended, but guanidine can be used. Doubt is cast upon a new clinical entity called "acute autonomic neuropathy."

Adult

Sleep apnoea syndrome: states of sleep and autonomic dysfunction.

Eleven patients with upper airway apnoea during sleep (one with SHY-Drager syndrome) were monitored polygraphically for wakefulness, sleep, and cardiovascular variables. Systemic hypertension and most of the severe arrhythmias recorded during sleep were secondary to repetitive obstructive apnonea and were mediated through the autonomic nervous system. Sleep related elevations of pulmonary arterial pressure were not influenced by atropine or impaired autonomic functions. Upper airway sleep apnoea is sleep related; the type of sleep (REM or NREM) is critical in the appearance of abnormalities. The distinction between two patient subgroups (total sleep dependent and NREM sleep dependent) has haemodynamic, and possibly long-term, implications. Sleep apnoea syndrome should be looked for in pateints with the Shy-Drager syndrome.

Adult

Pheochromocytoma and autonomic dysfunction.

Patients with hypertension due to the release of large amounts of NE from a pheochromocytoma and patients with PAD and postural hypotension due to diminished NE release from adrenergic axon terminals represent the extremes of the spectrum of adrenergic pathophysiolgic findings. It is reasonable to suspect that some more common disorders lie between these extremes and to anticipate that potential roles of the adrenergic nervous system in the pathogenesis of these disorders will be extensively studied with the availability of sensitive isotope-derivative methods for the measurement of catecholamine levels in plasma and in tissue.

Adolescent

Diabetes mellitus and autonomic dysfunction after vacor rodenticide ingestion.

A case of N-3 pyridylmethyl-N' 4 nitrophenyl urea (Vacor) rodenticide poisoning in a 52-year-old man is presented. Vacor is structurally related to alloxan and streptozotocin, agents that have been used extensively to produce diabetes mellitus in laboratory animals. Seven days after ingestion of Vacor, the patient presented in diabetic ketoacidosis complicated by postural hypotension and adynamic ileus. The patient recovered from ketoacidosis but has continued to require insulin. With infusion of arginine, glucagon rose from 185 to 650 pg./ml. and C-peptide from 0.5 to 3.4 ng./ml. Six weeks after onset of diabetes, no anti-islet-cell antibodies were detected. Muscle capillary basement membrane thickness on electron microscopy was found to be 1,918 +/- 194 A. The absence of hyperglycemia after Vacor ingestion should not lead to complacency on the part of the attending physician. The patient must be observed closely for development of ketoacidosis and treated prophylactically with nicotinamide, the suggested antidote.

Autonomic Nervous System

Evaluating dysfunction of the autonomic nervous system.

Simple bedside measurements of blood pressure and systolic pressure response to the Valsalva maneuver will confirm a clinical impression of orthostatic hypotension. Careful questioning of the patient usually elicits other symptoms of autonomic nervous system dysfunction, such as impotence, urinary and fecal incontinence, constipation or diarrhea, blurred vision, or sweating changes. Drugs are the most common cause of autonomic dysfunction, and their benefits should be weighed against the severity of the dysfunction. In addition, diabetes mellitus, uremia, amyloidosis, acute intermittent porphyria, myeloma, tabes dorsalis, and alcohol-nutritional problems may produce symptoms of autonomic dysfunction. Thus, patients who present with autonomic features but no history of dysfunction-producing drugs should undergo complete laboratory evaluation. A regimen of tyramine or L-dopa or a diet rich in cheese, processed meats, and wine (a monoamine), coupled with a monoamine oxidase inhibitor have beneficial effects in patients with orthostatic hypotension due to preganglionic autonomic dysfunction. Patients who do not respond to catecholamine precursors have stable, isolated orthostatic hypotension or a polyneuropathy such as that caused by diabetes.

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