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PubMed2005

Harlequin syndrome with crossed sympathetic deficit of the face and arm.

Harlequin syndrome is characterized by unilateral hyperhidrosis and flushing, which are predominantly induced by heat or exercise. Usually, the sympathetic deficits confine to the face. Rarely, the autonomic deficits involve the arm or the parasympathetic neurons in the ciliary ganglia. We report a 43-yr-old woman who presented with facial flushing and sweating in the right side, which were mainly induced by exercise. The facial flushing accompanied relative coldness in the right arm. Valsalva maneuver, cold pressure and 0.125% pilocarpine test, and computed tomography of the chest were normal. The crossed sympathetic deficit in the left face and right arm suggested that the lesions were multifocal. The sympathetic impairment in our patient may lie on a spectrum of pre- and postganglionic autonomic dysfunction, which was observed in Holmes-Adie, Ross, and Guillain-Barre syndromé.

Adult↗

PubMed1992

Rhinorrhea after neurotologic surgery.

Rhinorrhea after neurotologic surgery may be indicative of a cerebrospinal fluid (CSF) leak or may be secondary to autonomic dysfunction as a result of injury to preganglionic parasympathetic fibers. Differentiation between these two conditions is essential because undetected CSF rhinorrhea may lead to meningitis and death.

Autonomic Nervous System Diseases↗

PubMed1989

[Cold pressor test in the evaluation of autonomic neuropathy].

Neurogenic orthostatic hypotension is due to interruption of the baroreceptor reflex. To optimize the pharmacological therapy in this clinical setting the lesion should be carefully pinpointed and the degree of denervation established. To this purpose 7 patients with autonomic failure underwent ambulatory intraarterial monitoring, which showed the reversal of the normal 24-hour blood pressure (BP) pattern. During daytime BP gradually rose from its lowest point early in the morning to a peak during late evening. No BP fall was observed during sleep. Tilting test, Valsalva maneuver, hyperventilation, cold pressor test, handgrip and pharmacological tests showed interruption of the efferent pathway of the reflex. The BP fall 60 seconds after assuming the orthostatic position significantly correlated with the BP response to the cold pressor test (r = 0.89; p less than 0.01), while it did not with the BP changes during hand grip and hyperventilation and with the orthostatic increase of heart rate. Therefore, the BP response to the cold pressor test seems the most reliable indicator of the degree of autonomic dysfunction in the lesions of the efferent pathway of the reflex.

Aged↗

PubMed1996

Postganglionic cholinergic dysautonomia with incomplete recovery: a clinical, neurophysiological and immunological case study.

A 26-year-old man presented with signs and symptoms of marked postganglionic cholinergic autonomic dysfunction manifested by non-reacting dilated pupils, paresis of accommodation, decreased salivation, dry skin, atony of the bladder, erectile impotence and complete gastrointestinal paresis. Standard neurophysiological tests for myelinated sensory and motor fibre function and quantitative methods to examine unmyelinated parasympathetic, sympathetic and afferent fibres were performed: parasympathetic function was measured by heart rate variation tests. Sympathetic cutaneous vasoconstrictor responses induced by deep inspiration were examined with laser Doppler flowmetry. Cutaneous nociceptive C-fibre function was assessed by measurement of axon reflex vasodilatation and flare size induced by histamine iontophoresis. The findings confirmed that the abnormalities were restricted exclusively to the cholinergic postganglionic autonomic systems. All other functions were completely preserved. Modern neurophysiological methods of testing sympathetic and afferent small fibre function might help in the diagnosis of cholinergic postganglionic dysautonomia in the early stages. The specificity of the dysfunction argues in favour of an immunological pathogenesis. However, antibody screening including acetylcholine receptor antibodies and voltage-gated calcium channel antibodies gave negative results. Whatever autoimmunological mechanism might be involved, the postulated antibodies act highly specifically on unknown structures of the cholinergic postganglionic autonomic neurons.

Adult↗

PubMed2006

Heart rate variability and sympathetic skin response in male patients suffering from acute alcohol withdrawal syndrome.

BACKGROUND: Many symptoms of alcohol withdrawal (AW) such as tachycardia or elevated blood pressure might be explained by increased peripheral and central adrenergic activity. In contrast to many neurochemical studies of sympathetic activation during AW, only very few studies investigated autonomic balance using neurophysiological methods. METHODS: We investigated heart rate variability (HRV) and sympathetic skin response (SSR) in male patients suffering from mild AW syndrome (n = 20, no treatment required) and in patients with moderate to severe AW syndrome (n = 20, clomethiazole treatment) in the acute stage. Sympathovagal influence was quantified using measures of time and frequency domain of HRV as well as modern nonlinear parameters (compression entropy). Furthermore, we obtained latencies and amplitudes of SSR to quantify isolated sympathetic influence. Measures were obtained during the climax of withdrawal symptomatology before treatment, 1 day after climax, and shortly before discharge from hospital. Alcohol withdrawal scores were obtained and correlated to autonomic measures. RESULTS: Ambulatory blood pressure and AW scores revealed characteristic withdrawal symptoms in both patient groups. Apart from the nonlinear parameter compression entropy, Hc, measures of HRV revealed no sign of autonomic dysfunction in contrast to the significantly increased heart rates at the time of admission. Latencies and amplitudes of SSR did not indicate any increase of sympathetic activity. A negative correlation was found between Hc and mental withdrawal symptoms. CONCLUSIONS: We show here that classical measures for autonomic nervous system activity such as HRV and SSR are not suitable for describing the autonomic changes seen in acute AW, although a major role for the sympathetic nervous system has been proposed. This might be due to multiple dysregulation of metabolites in AWS or to subtle alcohol-induced damage to neuronal structures, issues that should be addressed in future studies.

Adult↗

PubMed1997

Nerve conduction and autonomic nerve function in diabetic children. A 10-year follow-up study.

In order to study the long-term development of diabetic neuropathy in children with newly diagnosed diabetes mellitus, 144 children were entered in a prospective study of nerve conduction and autonomic nervous function. Neurophysiological recordings of nerve conduction and parasympathetic function (R-R variations) were made at onset of diabetes and after 2, 5 and 10 years. Low sensory nerve conduction and autonomic dysfunction were found in approximately 25% of the children at onset of diabetes when the patients were not yet in complete remission. During years 0-2, an initial improvement of sensory conduction velocities was found. After 2 years, deteriorations in sensory and motor nerve conduction and autonomic nerve function were common and further deterioration was seen over time. A correlation was found between nerve conduction and glycaemic control.

Adolescent↗

PubMed2004

Cardiovascular alterations after spinal cord injury: an overview.

The recent developments in the management of spinal cord injury (SCI) have led to a reduction in mortality and in the consequences, resulting from incomplete spinal cord damage in those who survive. In this respect, it is noteworthy that SCI not only results in paraplegia or tetraplegia, but also in systemic, cardiovascular and metabolic alterations secondary to autonomic dysfunction. After SCI there is a decrease in sympathetic discharge and an increase in parasympathetic drive, resulting in profound changes in arterial blood pressure and heart rate. When SCI is induced in experimental animals, an immediate hypotension occurs (acute phase) which has been attributed to an autonomic imbalance involving a predominance of parasympathetic activity. Subsequently, an episodic hypertension may develop (chronic phase) as a part of a condition denominated autonomic dysreflexia. This hypertension is caused by afferent stimulation below the level of injury and can be so severe that sometimes may lead to cerebral haemorrhage, seizures, and death. In the light of the above lines of evidence, experimental SCI may provide an ideal model to study the nature of cardiovascular mechanisms following traumatic injury. Thus, the present review will deal with an update of the possible cardiovascular complications associated to SCI (including spinal shock, autonomic dysreflexia, deep venous thrombosis, and risk for coronary heart disease). This will be discussed within the context of the development of drugs with potential therapeutic usefulness in the acute and chronic stages of SCI.

Animals↗

PubMed2002

Effects of vital exhaustion on cardiac autononomic nervous functions assessed by heart rate variability at rest in middle-aged male workers.

We investigated the effects of vital exhaustion (VE) on cardiac autonomic functions in relation to working conditions such as overtime and frequent business trips, and to lifestyles such as smoking on 52 healthy middle-aged male workers. VE was evaluated by an abbreviated Maastricht Vital Exhaustion Questionnaire. Cardiac autonomic function at supine rest was assessed by spectral analysis of heart rate variability in an annual health checkup. The mean amplitude of the high frequency (HF: 0.15-0.4 Hz) component was lower in the high-VE group, whereas no significant difference in the ratio of the low frequency (LF: 0.04-0.15 Hz) component power to HF power (the LF/HF ratio) was observed among VE groups. There were significant interactive effects of VE and smoking on HF amplitude, and of VE and frequent business trips on the LF/HF ratio. VE symptoms were related to the suppression of the cardiac para-sympathetic nervous function at rest in middle-aged male workers, but not to the alteration in sympathovagal balance. Smoking and overwork such as frequent business trips may amplify the autonomic dysfunction in relation to VE among workers with a pronounced feeling of VE.

Adult↗

PubMed2004

Eighteen years of fair glycemic control preserves cardiac autonomic function in type 1 diabetes.

OBJECTIVE: To study the association between 18 years of mean HbA(1c) and cardiac autonomic function in type 1 diabetic patients having used intensive insulin treatment. RESEARCH DESIGN AND METHODS: A total of 39 patients with type 1 diabetes were followed during 18 years, and HbA(1c) was measured yearly. At 18 years follow-up heart rate variability (HRV) measurements were used to assess cardiac autonomic function. Standard cardiac autonomic tests during normal breathing, deep breathing, the Valsalva maneuver, and the tilt test were performed. Maximal heart rate increase during exercise electrocardiogram and minimal heart rate during sleep were also used to describe cardiac autonomic function. RESULTS: We present the results for patients with mean HbA(1c) <8.4% (two lowest HbA(1c) tertiles) compared with those with HbA(1c) > or = 8.4% (highest HbA(1c) tertile). All of the cardiac autonomic tests were significantly different in the high- and the low-HbA(1c) groups, and the most favorable scores for all tests were seen in the low-HbA(1c) group. In the low-HbA(1c) group, the HRV was 40% during deep breathing, and in the high-HbA(1c) group, the HRV was 19.9% (P = 0.005). Minimal heart rate at night was significantly lower in the low-HbA(1c) groups than in the high-HbA(1c) group (P = 0.039). With maximal exercise, the increase in heart rate was significantly higher in the low-HbA(1c) group compared with the high-HbA(1c) group (P = 0.001). CONCLUSIONS: Mean HbA(1c) during 18 years was associated with cardiac autonomic function. Cardiac autonomic function was preserved with HbA(1c) <8.4%, whereas cardiac autonomic dysfunction was impaired in the group with HbA(1c) > or = 8.4%.

Adult↗

PubMed2002

[Value of ambulatory blood pressure monitoring in blood pressure profile determination in type 2 diabetes].

Risk assessment in the type 2 diabetic patients must take into account the major risk factors, particularly arterial hypertension which is commonly associated, increasing the risk for macrovascular and microvascular complications. The aim of this study is to determine whether ambulatory blood pressure monitoring provides additional information to clinical (office) blood pressure measurement for the detection and control of hypertension in type 2 diabetic patients. Ambulatory blood pressure monitoring is more helpful than office blood pressure measurement for the evaluation of blood pressure levels. It is very valuable in type 2 diabetic patients for detecting autonomic dysfunction and elevated blood pressure load, which are associated with cardiovascular complications.

Adult↗

PubMed1988

[Malignant neuroleptic syndrome. Literature review and case report].

The neuroleptic malignant syndrome, first described by Delay and Deniker in 1968, is a rare but severe complication of neuroleptic treatment. According to the literature the syndrome particularly affects young men undergoing rapidly intensified neuroleptic treatment ("rapid neuroleptization"). The diagnosis is based on the triad of hyperthermia, extrapyramidal signs, and autonomic dysfunction, once infectious disease has been ruled out. The clinical features, course and therapy of the syndrome are described on the basis of a case report.

Adult↗

PubMed1981

Autonomic disturbances in relation to sensorimotor peripheral neuropathy in diabetes mellitus.

Thirty patients with diabetes mellitus were investigated. Twenty-seven had clinical signs of a symmetrical sensory or sensorimotor polyneuropathy. Features of autonomic dysfunction were found in 17 patients. Nerve conduction was studied in the femoral, deep peroneal, saphenous, sural and sensory and motor fibres of the posterior tibial nerves. The postural heart rate response was tested in all of the 30 patients. There was only a weak correlation between autonomic disturbances and sensorimotor polyneuropathy, based either on clinical or on electrophysiological results. Thus a multifactorial causation is suspected.

Adult↗

PubMed2001

The effects of reboxetine on autonomic and cognitive functions in healthy volunteers.

RATIONALE: Though reboxetine, a selective noradrenaline reuptake inhibitor, causes autonomic and cognitive adverse events there is a paucity of appropriately designed studies on the cognitive and autonomic effects of the drug in the literature. OBJECTIVE: To compare the effects of reboxetine on cognitive and autonomic functions with those of placebo in healthy humans. METHOD: A randomised, double-blind, crossover study of 12 healthy male volunteers aged 25 (21-27; median, range) years. Subjects orally received 4 mg reboxetine and placebo twice daily for periods of 14 days each with at least 14 days in between. Vasoconstrictory response of cutaneous vessels (VR) and skin conductance response (SCR) following sudden deep breath were employed as parameters for autonomic function. Quantitative EEG (qEEG) and psychometric tests served as parameters for cognitive function. RESULTS: Reboxetine decreased SCR and prolonged the dilation phase of VR (P<0.05). It did not affect cognitive functions such as flicker fusion frequency, choice reaction, memory and psychomotor coordination but increased slow beta (beta1) power density in the qEEG. Tiredness (n=12), dry mouth (n=9), delayed urination (n=3) and constipation (n=1) were noted with reboxetine. CONCLUSION: Sustained peripheral and/or central sympathetic activation accounts for the prolongation of VR. The decrease of SCR and typical side effects suggest a relevant antimuscarinic drug action. Chronic administration of reboxetine at therapeutic doses causes autonomic dysfunction and subjective sedation but does not impair cognitive and psychomotor abilities in healthy humans.

Adrenergic Uptake Inhibitors↗

PubMed2004

Small fiber neuropathy: a common and important clinical disorder.

Small fiber neuropathy (SFN) is a neuropathy selectively involving small diameter myelinated and unmyelinated nerve fibers. Interest in this disorder has considerably increased during the past few years. It is often idiopathic and typically presents with peripheral pain and/or symptoms of autonomic dysfunction. Diagnosis is made on the basis of the clinical features, normal nerve conduction studies (NCS) and abnormal specialized tests of small nerve fibers. Among others, these tests include assessment of epidermal nerve fiber density, temperature sensation tests for sensory fibers and sudomotor and cardiovagal testing (QSART) for autonomic fibers. Unless an underlying disease is identified, treatment is usually symptomatic and directed towards alleviation of neuropathic pain.

Autonomic Nervous System Diseases↗

PubMed1998

Autonomic testing in patients with chronic fatigue syndrome.

The purpose of this study was to determine whether chronic fatigue syndrome (CFS) patients show autonomic dysfunction at the cardiac level and if so, to discover whether these abnormalities explain the fatiguability and/or other symptoms in CFS. The study population consisted of 21 CFS patients (Centers for Disease Control and Prevention [CDC] criteria, 1988) and 13 age- and sex-matched healthy controls. The autonomic testing consisted of: (1) postural challenge: registration of heart rate and blood pressure (BP) and heart rate variability in supine and in upright position (tilted to 70 degrees); (2) Valsalva maneuver; (3) handgrip test; (4) cold pressor test; and (5) heart rate response to deep breathing. Statistical analysis was performed using the Mann Whitney rank sum test; results of the test were considered significant at the 0.05 level. After tilting heart rate was significantly higher in CFS patients compared with healthy controls (mean CFS = 88.9 beats/min vs control = 77.9 beats/min; P <0.01). Low frequency power after tilting was significantly higher in CFS patients compared with controls (mean CFS = 0.603 vs control = 0.428; P = 0.02). There was a trend toward an increased heart rate during the cold pressor test. Other parameters did not differ between the CFS and control populations. The observed changes point toward a sympathetic overactivity in CFS patients when they are exposed to stress. Parasympathetic abnormalities could not be observed. Therefore, our findings provide no real explanation for the fatigue and intolerance to physical exertion in these patients.

Adult↗

PubMed1997

Iodine-123 metaiodobenzylguanidine scintigraphic assessment of myocardial sympathetic innervation in patients with familial amyloid polyneuropathy.

OBJECTIVES: This study attempted to assess myocardial sympathetic innervation using iodine-123 (I-123) metaidobenzylguanidine (MIBG) imaging in patients with familial amyloid polyneuropathy. BACKGROUND: Signs and symptoms of cardiac autonomic dysfunction are commonly seen in patients with cardiac amyloidosis. However, the incidence and magnitude of abnormalities in myocardial sympathetic nerve function by means of I-123 MIBG imaging and their relation to clinical findings, cardiac function and the results of thallium-201 (Tl-201) and technetium-99m pyrophosphate (Tc-99m PYP) myocardial scanning have not yet been clarified. METHODS: We performed M-mode, two-dimensional and Doppler echocardiography and I-123 MIBG, Tl-201 and Tc-99m PYP imaging of the heart in 12 patients with familial amyloid polyneuropathy and biopsy-proved cardiac amyloidosis. RESULTS: Ten of 12 patients had no clinical evidence of overt heart disease, but left ventricular (LV) wall thickening was observed in 4 of these 10. Left ventricular percent fractional shortening and Doppler transmitral flow velocity patterns were found to be normal in all 12 patients. Eight of 12 patients showed no myocardial MIBG accumulation, with limited uptake in the remaining 4 demonstrated only in the LV anterior wall. Diffuse but mild myocardial uptake of Tc-99m PYP occurred in only 4 of 12 patients, and all 12 had normal results on Tl-201 myocardial scanning. Complete defects on myocardial MIBG scans were found in five of eight patients with negative findings on Tc-99m PYP myocardial scanning. The incidence and magnitude of myocardial uptake of MIBG were independent of clinical findings, extent of endomyocardial amyloid deposition, electrocardiographic QRS voltage and ventricular wall thickness. CONCLUSIONS: Patients with familial amyloid polyneuropathy show a high incidence of myocardial adrenergic denervation with viable myocardium that can be identified very early in cardiac amyloidosis, before the development of clinically apparent heart disease, ventricular wall thickening, significant LV systolic and diastolic dysfunction and positive findings on Tc-99m PYP myocardial scanning.

3-Iodobenzylguanidine↗

PubMedDate not supplied

QT dispersion in microalbuminuric Type 1 diabetic patients without myocardial ischemia.

Microalbuminuria is associated with an increased risk of cardiac death. We assessed whether urinary albumin excretion is related to abnormalities of the QT interval independently of myocardial ischemia. Thirty-four patients with type 1 diabetes who were free from ischemic heart disease on the basis of normal stress electrocardiography and echocardiography were studied. Maximal QT interval and dispersion were significantly greater in the group with microalbuminuria (n=17) compared to controls (n=17) with normal urinary albumin excretion (394 [26.1] vs. 373.8 [27.8] ms; P=.044 and 62.4 [21.8] vs. 42.7[11.6] arbitrary units; P=.009). Autonomic function was similar between the groups. Urinary albumin excretion correlated positively with QT dispersion (P=.023). These data suggest that in type 1 diabetic patients, QT abnormalities can occur independently of autonomic dysfunction or myocardial ischemia and may be related to the processes which increase urinary albumin leakage.

Adult↗

PubMed2002

Autonomic function in elderly patients with chronic heart failure.

AIMS: Autonomic function (AF) is attenuated by heart failure (HF). Reports have been based on studies of young patients with systolic heart failure (SHF). However, HF is a disease of older patients who are more likely to have diastolic heart failure (DHF). We investigated whether age alters AF in elderly HF patients and whether the haemodynamic type of HF influences AF. METHOD AND RESULTS: Thirty-six elderly HF (Framingham criteria) patients (11 with SHF, 25 with DHF) and 21 matched healthy subjects underwent simple bedside AF tests. Compared with the reference values for healthy adults, the mean E:I ratios and the median 30:15 ratios standing were all essentially normal. The median 30:15 ratios tilt and the mean Valsalva ratios were all significantly below the reference value (P for all cases <<0.050). Comparing three groups, there were no significant differences for mean E:I ratio (P=0.111), 30:15 tilt (P=0.619) and 30:15 standing (P=0.167), whereas there were significant differences for the mean Valsalva ratios (P=0.001). The mean Valsalva ratio of the SHF patients was significantly lower than that for the DHF patients (P<0.001) which in turn was significantly lower than the result of the healthy subjects (P<0.001). CONCLUSION: There is an age-related impairment in AF with further impairment occurring in patients with HF. However, the severity of autonomic dysfunction is less in patients with DHF compared with patients with SHF.

Aged↗