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[Concept and classification of autonomic failure].

The autonomic nervous system innervates every visceral and peripheral organs in the body. It has as complex a neural organization in the brain, spinal cord, and periphery as the somatic nervous system, but remains largely involuntary or automatic. Accurate diagnosis is essential for proper management of autonomic failure but in attempting to classify autonomic disease there is a philosophical point to be borne in mind. Since it is very difficult to comprehend and evaluate systematically the aspect of autonomic dysfunction in each organ in various diseases, little works have been performed to classify autonomic diseases. We introduce several comprehensible works of classification of autonomic failure. We also presented some typical autonomic diseases according to those classifications in this paper.

Autonomic Nervous System↗

[Sulpiride in the prophylactic treatment of migraine].

The aim of the work was investigation of both efficacy of sulpiride, atypical neuroleptic drug with pronounced antidepressive activity, and its influence on central nervous system. The study was performed in the course of prophylactic treatment of migraine using contingent negative variation (CNV) index. It is demonstrated that sulpiride is effective in prophylactic treatment of migraine complicated with psychologic disorders and autonomic dysfunctions. Such effect may be caused by the drug's influence on depression, anxiety, autonomic symptoms, tension-type headaches. This conclusion was confirmed by CNV data, change of which after sulpiride administration have indicated normalization of mental performance and increase of active adaptive abilities.

Contingent Negative Variation↗

Erectile dysfunction predicts generalised cardiovascular disease: evidence from a case-control study.

AIMS: To determine whether idiopathic erectile dysfunction, in the absence of overt cardiovascular disease or cardiovascular risk factors, is associated with vascular or autonomic dysfunction. METHODS: We studied 49 men with ED (without known cardiovascular risk factors or disease) and 50 age-matched controls, aged 40-70 years. Macrovascular endothelial function was examined by brachial artery ultrasonography and microvascular function by venous occlusion plethysmography. Blood pressure measurement and electrocardiography were performed lying and standing, and the 30:15 RR ratio calculated. RESULTS: Body mass index, testosterone, fasting lipids and glucose did not differ significantly between groups. Standing pulse pressure was higher (50+/-1mm Hg versus 43+/-2mm Hg, p<0.004) and 30:15 RR ratio lower (0.97+/-0.01 versus 1.01+/-0.01, p<0.02) in the ED group. Flow-mediated dilatation of the brachial artery was not significantly different between groups. Flow debt repayment during forearm reactive hyperaemia was lower in the ED group (7.2+/-0.7 ml versus 9.5+/-0.8 ml per 100ml, p<0.02) than in controls. CONCLUSIONS: Men with idiopathic ED have evidence of endothelial dysfunction in forearm resistance vessels, increased pulse pressure and impaired heart rate variability. This supports the concept that erectile dysfunction is a predictor of cardiovascular dysfunction and a precursor of clinical cardiovascular disease.

Adult↗

Ocular autonomic function in progressive autonomic failure.

Ocular autonomic function was assessed in 4 patients with progressive autonomic failure (PAF) and age-matched control subjects, by measurement of the pupil cycle time, and determination of autonomic denervation hypersensitivity of the iris. Pupil cycle time was abnormal in all patients with PAF; sustained pupil cycling was absent in 5 of the 8 eyes tested of the PAF patients, compared with only 16 eyes from 70 control subjects. Pupil constriction in response to 2.5% methacholine, indicative of parasympathetic denervation hypersensitivity, was significantly increased in patients with PAF (p less than 0.001), whilst pupil dilation in response to 0.5% phenylephrine, indicative of sympathetic denervation hypersensitivity, was also significantly higher in the PAF patients (p less than 0.001). The results suggest that ocular autonomic function may provide a sensitive early indicator of generalised autonomic dysfunction.

Aged↗

Autonomic cardiovascular neuropathy in primary Sjögren's syndrome.

The case of a 65-year-old female with primary Sjögren's syndrome and severe autonomic cardiovascular neuropathy, manifested by incapacitating postural hypotension, is presented. It is postulated that the patient's Sjögren's syndrome was responsible for the autonomic neuropathy, probably via a vasculitic mechanism. Treatment with hydrocortisone resulted in improvement in the patient's symptoms but it did not significantly alter the objective findings of autonomic dysfunction. We suggest that it is probably worth studying Sjögren's patients for evidence of autonomic neuropathy.

Aged↗

Asymptomatic coronary artery disease in diabetes: associated with autonomic neuropathy?

To elucidate the potential association of diabetic autonomic neuropathy with increased prevalence of silent coronary artery disease (CAD), 138 asymptomatic diabetic subjects were screened using exercise ECG. 24-h ambulatory ECG and dynamic thallium scintigraphy. Fourteen patients with exercise-induced myocardial ischaemia and angiographically confirmed CAD (greater than or equal to 50% coronary artery narrowing) were found using this protocol. Their autonomic nervous function was assessed using standard cardiovascular tests and compared with that of 23 consecutive diabetic patients catheterised because of symptomatic CAD (mean New York Heart Association class 3.0). The diabetic patients with symptomatic CAD had more severe coronary atherosclerosis than the diabetic patients with asymptomatic CAD assessed by jeopardy score (P less than 0.01). The groups did not, however, differ with respect to autonomic function tests. Five patients (22%) with symptomatic CAD and 3 patients (21%) with asymptomatic CAD had definite autonomic dysfunction, i.e. two or more abnormal tests. Thus, our results suggest that the frequency of autonomic neuropathy is not increased in diabetic patients with asymptomatic CAD. The contribution of diabetic autonomic neuropathy to the absence of cardiac pain needs further clinical and pathological studies.

Autonomic Nervous System↗

Stress management techniques: are they all equivalent, or do they have specific effects?

This article evaluates the hypothesis that various stress management techniques have specific effects. Studies comparing various techniques are reviewed, as well as previous literature reviews evaluating the effects of individual techniques. There is evidence that cognitively oriented methods have specific cognitive effects, that specific autonomic effects result from autonomically oriented methods, and that specific muscular effects are produced by muscularly oriented methods. Muscle relaxation and/or EMG biofeedback have greater muscular effects and smaller autonomic effects than finger temperature biofeedback and/or autogenic training. EMG biofeedback produces greater effects on particular muscular groups than progressive relaxation, and thermal biofeedback has greater finger temperature effects than autogenic training. Disorders with a predominant muscular component (e.g., tension headaches) are treated more effectively by muscularly oriented methods, while disorders in which autonomic dysfunction predominates (e.g., hypertension, migraine headaches) are more effectively treated by techniques with a strong autonomic component. Anxiety and phobias tend to be most effectively treated by methods with both strong cognitive and behavioral components.

Autogenic Training↗

[Autonomic innervation of the female pelvis. Anatomic basis].

The pelvic plexus with its sympathetic (hypogastric nerves, sacral sympathetic trunk) and parasympathetic (pelvic splanchnic nerves) sources provides and distributes the autonomic nervous supply for the pelvic organs. This homogeneous autonomic nerve plate is located within and deeply to the peritoneal fold (recto-uterine in the female pelvis) lying medially to the internal iliac vessels. Autonomic nerve fibres emerge from the pelvic plexus and travel to the anterolateral aspect of the rectum and the inferolateral and posterolateral aspects of the bladder and female genital tract. In addition, pelvic organs are innervated by nerves accompanying the vessels and the ureter which penetrate the pelvic plexus. Knowledge of the topography of the pelvic plexus indicates the most common sites for iatrogenic lesions and should help us in avoiding postoperative autonomic dysfunction.

Autonomic Pathways↗

Advances in the diagnosis and therapy of syncope and palpitations in children.

Although advances in diagnosis and therapy of congenital heart disease have received most of the attention, in the past few years advances in the more common troublesome problems of adolescents with syncope and palpitations have been as dramatic. Syncope may be due to a cardiac arrhythmia, but is more commonly due to an autonomic dysfunction of blood pressure control. In cases where the diagnosis is equivocal by history alone, autonomic testing, including head-up tilt table testing, may be useful. Treatment with a salt-enriched diet and a mineralocorticoid is usually effective in reducing or eliminating the incidence of syncope. In resistant cases beta-blockade, scopolamine, disopyramide, theophylline, or fluoxetine may be helpful. Palpitations are usually benign. When they are due to a supraventricular tachycardia, especially when associated with the Wolff-Parkinson-White syndrome, they may be annoying and, in those with a short refractory period, dangerous. Radiofrequency ablation of the abnormal pathway is being increasingly used to eliminate the arrhythmia and may be superior to a lifetime of pharmacologic suppression in symptomatic individuals.

Arrhythmias, Cardiac↗

Assessment of autonomic nervous function in human hypertension.

In spite of the popular belief that "nervous tension," "stress," and hypertension are causally related, it has been difficult to prove a role of autonomic dysfunction in the genesis of essential hypertension. There are three major reasons for this: 1) autonomic function cannot be assessed with one simple test, 2) the short-term "phasic" and long-term "tonic" autonomic control of blood pressure are not necessarily interrelated, and 3) hypertension is a dynamic process in the course of which there are changes in autonomic control. Some of the controversies in the field stem from the lack of appreciation of these limitations.

Autonomic Nervous System↗

An investigation of antibodies to nerve growth factor in diabetic autonomic neuropathy.

Nerve growth factor (NGF) is required for development and maintenance of autonomic nervous function and is highly expressed in the iris. An association between sensory and autonomic diabetic neuropathy and NGF function has been postulated. In light of the evidence for an autoimmune component to the pathogenesis of autonomic neuropathy in Type 1 diabetes mellitus, an enzyme linked immunosorbent assay (ELISA) was established to investigate the presence of autoantibodies to mNGF in: 20 patients with long-standing Type 1 diabetes with abnormal autonomic function tests, of whom 14 had symptomatic autonomic neuropathy, 3 had an episode of iritis, and 6 had no autonomic symptoms; 9 age-matched patients with Type 1 diabetes and no complications; 10 healthy control subjects. Insulin antibodies by ELISA and autoantibodies to other endocrine targets were also measured. The specificity of anti-mNGF autoantibody ELISA was further confirmed by immunodepletion on mNGF-Sepharose 4B. No differences in antibody binding to mNGF were detected, between any of the patient groups and control subjects. There was no relationship between age, sex, and diabetes duration and mNGF binding. High levels of anti-mNGF autoantibodies were found in only one diabetic patient who had no evidence of neuropathy, raising the possibility that an autoimmune component to NGF might precede the development of autonomic dysfunction. It remains to be established whether autoantibodies to NGF play a role in diabetic autonomic neuropathy and prospective studies will be required to investigate whether the autoantibodies are a feature of evolving but not established neuropathy.

Adult↗

Functional restoration of the traumatically injured spinal cord in cats by clonidine.

The long-term, chronic, paralysis resulting from spinal cord injury in the cat has been reversed by the use of an alpha 2-adrenergic receptor agonist, clonidine. Administration of this drug resulted in "normalization" of sensory-motor and autonomic dysfunctions. Preliminary studies of the clonidine in humans with traumatically injured spinal cord indicate that autonomic dysreflexia can be controlled and spasticity minimized. The data suggest that biochemical and pharmacologic manipulation of receptors may ameliorate paralysis following traumatic injury to the spinal cord as well as to the brain and brainstem.

Adrenergic beta-Agonists↗

Spectral analysis of heart rate and blood pressure variability in primary Sjögren's syndrome.

BACKGROUND: Autonomic dysfunction has been described in primary Sjögren's syndrome (SS). OBJECTIVE: To investigate the circulatory autonomic regulation in patients with primary SS by power spectral analysis of heart rate and blood pressure variability. METHODS: Forty three (42 female) patients with primary SS, mean age 52 years (range 23-80), with a mean disease duration of eight years (range 1-30) and 30 (15 female) healthy controls, mean age 43 years (range 21-68) were studied. In each patient blood pressure, heart rate, and respiration were measured continuously during supine rest and orthostatic challenge (60 degrees head-up tilt). Power spectral analysis was performed to determine possible differences in short term sympathetic and parasympathetic autonomic regulation between patients and controls. Furthermore, spectral parameters were studied in relation to illness severity and disease duration of the patients with primary SS. RESULTS: After controlling for differences in age, heart rate variability of the mid-frequency band and the variation coefficient of systolic blood pressure were significantly lower in patients with primary SS than in controls during supine rest. During 60 degrees tilt patients with primary SS showed a significantly higher mean heart rate, mean systolic blood pressure, and variation coefficient of diastolic blood pressure, and a significantly lower baroreflex index than controls. After controlling for age, no differences were found either in heart rate variability, blood pressure results, and baroreflex sensitivity during supine rest and tilt between the subgroups divided according to disease duration, Schirmer test results, or between the subgroups with different fatigue scores. No differences were found in spectral data between the groups with and without positive antinuclear antibody serology. CONCLUSION: For the group no differences in sympathetic and parasympathetic cardiac control were seen between patients with primary SS and controls, as assessed by spectral techniques, although some cardiovascular differences were found, particularly during orthostatic challenge.

Adult↗

[Deep breathing test type I diabetic patients and parameters of spectral analysis].

UNLABELLED: Nervous system injury, affecting also its autonomic part, occurs in the natural course of chronic complications in patients with type 1 and 2 diabetes. The aim of the study was to compare heart rate frequency analysis parameters at rest and after deep breathing test in healthy persons and in the group of type 1 diabetes patients without complications. The study was performed in 34 patients with type 1 diabetes without chronic complications (N0), hospitalized in the Chair and Department of Metabolic Diseases in 1993-1996. The control group (K) consisted of 23 healthy persons. All diabetic patients underwent examinations to identify chronic complications of the disease. Aiming at answering the question how simple physiological tests influence frequency parameters of heart rate variability, assessment of VLF, LF and HF parameters was performed at rest and after the deep breathing test. In both groups K and N0 no changes were observed in the spectrum power within VLF values after deep breathing compared to the initial values. In the group K the deep breathing test significantly (p < 0.001) increased spectrum power within LF values, whereas in the group N0 the test did not produce statistically significant changes of this parameter compared to the initial values. In the control group K as well as in diabetic patients N0 the test caused significant (p < 0.01 and p < 0.001, respectively) decrease in the HF spectrum power in comparison to the values at rest. VLF/LF ratio after the deep breathing test as well as LF/HF ratio at rest were significantly lower in the group K compared to the group N0. CONCLUSION: Analysis of the autonomic nervous system imbalance may be an indispensable tool in the early diagnostics of patients with type 1 diabetes. Results of spectrum analysis may be useful in cases when classical Ewing battery tests do not identify autonomic dysfunction.

Adult↗

[A case of acute idiopathic pandysautonomia complicated with acute pancreatitis and liver injury].

A case of acute idiopathic pandysautonomia (AIPD) complicated with acute pancreatitis and liver injury was reported. A 43-year-old woman acutely developed abdominal pain, low grade fever, lymphoadenopathy and generalized erythema in May 1992. She was found to have autonomic dysfunctions, such as orthostatic hypotension, hypohidrosis, paralytic ileus and urinary retention. A systematic investigation of autonomic function revealed an impairment of both sympathetic and parasympathetic post-ganglionic nerves. Serum concentrations of amylase, lipase, elastase 1, and transaminase and amylase creatinine clearance ratio were elevated in the early stage of the disease. No significant serological findings for viral infections were detected to suggest any special etiology of the disease; the symptoms gradually improved, although incompletely, after several months from the onset. AIPD complicated with acute pancreatitis or liver injury is uncommon. A possible link between undetectable viral infection and AIPD was discussed.

Acute Disease↗

Autonomic peripheral neuropathy.

The autonomic neuropathies are a group of disorders in which the small, lightly myelinated and unmyelinated autonomic nerve fibres are selectively targeted. Autonomic features, which involve the cardiovascular, gastrointestinal, urogenital, sudomotor, and pupillomotor systems, occur in varying combination in these disorders. Diabetes is the most common cause of autonomic neuropathy in more developed countries. Autonomic neuropathies can also occur as a result of amyloid deposition, after acute infection, as part of a paraneoplastic syndrome, and after exposure to neurotoxins including therapeutic drugs. Certain antibodies (eg, anti-Hu and those directed against neuronal nicotinic acetylcholine receptor) are associated with autonomic signs and symptoms. There are several familial autonomic neuropathies with autosomal dominant, autosomal recessive, or X-linked patterns of inheritance. Autonomic dysfunction can occur in association with specific infections. The availability of sensitive and reproducible measures of autonomic function has improved physicians' ability to diagnose these disorders.

Amyloidosis↗

An Italian family with Ala-47 transthyretin mutation associated with cardiomyopathy and polyneuropathy.

We describe two Italian first cousins with familial amyloidotic polyneuropathy associated with transthyretin variant consisting of the substitution of alanine for glycine at codon 47 (TTR Ala-47), from a family with a history of cardiac failure. The 40-year-old patient presented with autonomic dysfunction and the 44-year-old cousin with congestive heart failure. Both developed sensorimotor and autonomic polyneuropathy. Since a similar clinical picture has been described in another Italian family, the cardiac involvement must be regarded as a salient and early feature of the TTR Ala-47 mutation.

Adult↗

The effects of diabetes and autonomic neuropathy on parotid salivary flow in man.

Stimulated parotid salivary flow studies were performed on 20 non-diabetic subjects and on 30 patients with insulin-dependent diabetes mellitus who had been screened for autonomic neuropathy using tests of cardiovascular reflexes. Electrical gustometry was performed to detect the taste threshold. The stimulated parotid salivary flow rates of diabetics with no autonomic neuropathy or early parasympathetic autonomic dysfunction alone were similar to those of non-diabetic subjects. Diabetic patients with established parasympathetic autonomic neuropathy had significantly higher salivary flow rates as did those with combined sympathetic and parasympathetic neuropathies. Taste thresholds did not correlate either with the duration of the diabetes or with the presence of autonomic neuropathy. The cause of the increased salivary flow associated with diabetic autonomic neuropathy is unclear. It may represent the removal of a neural inhibitory mechanism which modulates salivary flow, or the effects of long-standing diabetes on salivary secretion.

Adult↗