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PubMed1993

DC photoplethysmography in the evaluation of sympathetic vasomotor responses.

The d.c. component of the photoplethsmographic signal was used to determine the response of the finger vasculature to three standard tests of vasomotor function: (1) an inspiratory gasp (IG), (2) immersion of the contralateral hand in ice water (IW), and (3) the Valsalva manoeuvre. The vasoconstrictor response to the first two of these stimuli could be measured in all of 25 normal subjects. The response to the Valsalva manoeuvre could not be detected consistently. Seven patients with known sympathetic autonomic dysfunction showed no response to either IG or IW. In 30 patients with diabetes mellitus of over 10 years duration, 46.7% had no response to IG, and 20% had no response to IW. Absent responses correlated with abnormal autonomic cardiovascular reflexes, with absent sympathetic skin responses and with the severity of peripheral somatic neuropathy. The d.c. photoplethysmographic determination of the vasoconstrictor response in the finger after a deep inspiratory gasp and after ice water immersion offers an additional measure of the function of small (2 mu-6 mu) peripheral nerve fibres. Because of variability in the amplitude of the responses in normals, only an absent response should be accepted as abnormal.

Adult↗

PubMed1987

A case of postganglionic cholinergic dysautonomia.

A 24 year old female presented with signs and symptoms of postganglionic cholinergic autonomic dysfunction manifested by impaired lachrymation and salivation, mydriasis of the pupil, decreased gastrointestinal motility, atony of the bladder, and sweating and taste disturbance. Clinical and pharmacological studies confirmed that the abnormalities were restricted mainly to the postganglionic cholinergic autonomic systems. The titre of serum complement was low, antinuclear antibodies revealed a diffuse pattern, and anti SS-A antibody was positive, suggesting that this disorder was caused by an immune mechanism.

Adult↗

PubMed1986

Autonomic disorders in idiopathic parkinsonism.

Clinical evidences and experimental data on various signs and symptoms of autonomic dysfunction in idiopathic parkinsonism are reported and their characteristics related to the effects of the drugs usually administered for the therapy of the extrapyramidal symptoms. The use of instrumental diagnostic criteria may sometimes help to detect alterations of autonomic functions (micturition disturbances, disorders of pupil reactivity, orthostatic and postprandial hypotension, temperature dysregulation), otherwise not easily identifiable. The existence of a large involvement of autonomic structures both at central and peripheral levels underlines the concept of Parkinson's disease being part of a wide spectrum of degenerative disorders where multiple nervous structures are at risk.

Antiparkinson Agents↗

PubMed1984

Testing for autonomic neuropathy.

Autonomic neuropathy is a common complication in long-term diabetes, about 30% of the patients showing measurable signs of autonomic dysfunction after 10 years duration of disease. The diagnosis is often difficult to establish because clinical symptoms generally occur late in the course of the disease, and may be nonspecific. A number of recently developed quantifiable and reproducible autonomic nerve function tests are reviewed, with emphasis on the physiological basis of the tests and on practical applicability. Finally, diagnostic criteria, based on autonomic nerve function tests, are suggested.

Autonomic Nervous System Diseases↗

PubMed1985

Acute encephalopathy associated with continuous vincristine sulfate combination therapy: case report.

Neurotoxicity is a well-recognized and commonly observed side effect associated with the use of vincristine sulfate in cancer chemotherapy. The clinical manifestations of vincristine neuropathy cover a wide spectrum of peripheral neurologic dysfunctions that have been described to be reversible and cumulative in most instances (1, 2). Paresthesias, loss of tendon reflexes, and progressive weakness are the most common clinical features (3, 4). Sensory impairment, cranial nerve palsies, gastrointestinal disturbances, and autonomic dysfunctions including atonic bladder, impotence, and orthostatic hypotension may occur (5). Acute CNS complications, usually presenting as generalized seizures, are extremely rare and only a few cases have been reported which were without underlying biochemical or structural abnormalities (1, 5-9). We describe the case of a woman with multiple myeloma, who developed fulminant encephalopathy following 4 days of continuous vincristine, adriamycin, and day 1-4 pulse dexamethasone (VAD) combination therapy.

Antineoplastic Combined Chemotherapy Protocols↗

PubMed1994

Familial amyloidotic polyneuropathy with late-onset and well-preserved autonomic function: a Japanese kindred with novel mutant transthyretin (Ala97 to Gly).

We report the characteristics of one patient and two asymptomatic carriers from a Japanese family with familial amyloidotic polyneuropathy (FAP). The clinical features were somatic sensory and motor neuropathy with well-preserved autonomic function and late onset with slow insidious progression. These symptoms and signs are different from those of type 1 FAP. There were massive amyloid deposits with transthyretin (TTR) in the myocardium and the sural nerve. DNA sequencing of the TTR gene and amino acid sequence analysis of serum TTR revealed a new mutation in which Gly97 was substituted for Ala. We suggest that patients with somatic sensory and motor neuropathy of unknown origin without apparent autonomic dysfunction should be further studied for TTR mutation.

Age of Onset↗

PubMed2000

Cardiac autonomic function in patients suffering from primary focal hyperhidrosis.

Cardiac autonomic function in patients (n = 63) with primary focal hyperhidrosis and healthy controls (n = 28) was investigated by short-term frequency domain power spectral analysis of heart rate variability. The power of the very-low-frequency band (0.01-0.05 Hz) was significantly lower in patients with axillary hyperhidrosis than in controls. No differences between groups could be observed at investigation of the low-frequency band (0.05-0.15 Hz), which was a surprising finding because this band represents also sympathetic cardiac innervation. At the high-frequency band (0.15-0.5 Hz), which represents parasympathetic cardiac innervation, an interaction of type and position influencing spectral power was detected. Our highly interesting findings indicate that primary focal hyperhidrosis is based on a much more complex autonomic dysfunction than generalised sympathetic overactivity and seems to involve the parasympathetic nervous system as well.

Adult↗

PubMed2005

Relations of inflammatory markers to lipid levels and autonomic tone in patients with moderate and severe chronic kidney disease and in patients under maintenance hemodialysis.

BACKGROUND AND AIMS: Chronic kidney disease is associated with enhanced inflammatory response and autonomic dysfunction. Evidence exists of a potential interaction of inflammation and nervous system. We sought to investigate determinants of heart rate variability (HRV) and relations between the inflammatory markers C-reactive protein (CRP) and interleukin-6 (IL-6) and autonomic tone in patients with moderate and severe chronic kidney disease and in maintenance hemodialysis patients, and relations of CRP and IL-6 with clinical characteristics and lipid levels. METHODS: This was a cross-sectional study of 51 hemodialysis and 53 moderate and severe chronic kidney disease patients. Autonomic tone was assessed using 24-hour HRV analysis in time and frequency domain. All patients underwent measurements of high sensitivity CRP, IL-6 and lipid levels. RESULTS: CRP and IL-6 were elevated in the non-dialysis group at levels similar to hemodialysis patients. Hemodialysis patients had lower total cholesterol, LDL cholesterol and apolipoprotein B levels (p < 0.05), and in this group of patients lipids were related to CRP and IL-6 (p < 0.05). The inflammatory marker IL-6 was associated to HRV in the moderate and severe chronic kidney disease group (R = -0.4, p < 0.01 for standard deviation of RR intervals and very low frequency power, R = -0.5, p < 0.01 for standard deviation of all five-minute RR intervals, R = 0.35, p < 0.05, for total power and low frequency power). Adequacy of dialysis, but not the inflammatory markers, was associated to HRV in the hemodialysis group (R = 0.6, p < 0.01 for high frequency power). CONCLUSION: Enhanced inflammatory response occurs already in stages 3 and 4 chronic kidney disease. IL-6 is related to HRV in these patients, but not in the hemodialysis group, suggesting that IL-6 may interact with autonomic tone in that stage of disease.

Analysis of Variance↗

PubMed2002

Cardiovascular abnormalities in patients with a carcinoid syndrome.

BACKGROUND: Heart failure is an important reason for morbidity and mortality in patients with carcinoid. Carcinoid heart disease is caused by increased levels of circulating serotonin. Because carcinoids also produce catecholamines, we evaluated cardiovascular manifestations of autonomic dysfunction in patients with a carcinoid syndrome. METHODS: Twenty patients with a midgut carcinoid, who had a carcinoid syndrome with a median duration of 72 months, and markedly elevated urinary 5-hydroxyindoleacetic acid (5-HIAA) excretion were studied. RESULTS: Ten patients had no symptoms of heart failure, i.e. New York Heart Association (NYHA) functional class I, 6 had class II, and 4 class III heart failure. Transthoracic echocardiography (TTE) showed right-sided valvular abnormalities in 13 of 19 evaluable patients (mild n=8, severe n=5). Fourteen of the 20 patients (70%) had an elevated concentration of plasma N-terminal atrial natriuretic peptide (N-ANP), which correlated with NYHA class, TTE abnormalities, and increased urinary metanephrine excretion. Heart rate variability (HRV) parameters, in particular those associated with increased sympathetic activity (low frequency power, p=0.002 versus healthy individuals), were impaired but were independent of NYHA class and TTE findings and correlated with urinary metanephrine excretion (r=-0.49, p<0.05). CONCLUSION: In these 20 carcinoid patients with substantial secretory activity of the tumour, overt cardiac morphological changes were present in a minority of patients. However, N-ANP values and HRV profile were markedly abnormal, and related to enhanced urinary excretion of catecholamine and metabolites, suggesting autonomic derangement. These abnormalities possibly herald the development of more severe cardiac dysfunction and may be indicative of the need for preventive drug treatment.

Adult↗

PubMed2002

Hemicrania continua in African Americans.

OBJECTIVE: The first six cases of hemicrania continua and episodica in African Americans are reported, differences from previous accounts noted, and important diagnostic features described. BACKGROUND: Hemicrania continua is an indomethacin responsive chronic daily headache. Mild to moderate daily headache is strictly unilateral, constant but fluctuating. Superimposed severe headache attacks occur, last seconds to days, and are associated with ipsilateral orbital-nasal autonomic dysfunction. RESULTS: Severe headache attacks are usually pulsatile, occur one to four times daily, and last 40 minutes to three days. Daily unilateral background headache was typically of a pressure, sharp, dull or pulling quality. Ipsilateral orbital-nasal autonomic symptoms were noted in all. Serious concomitant medical illnesses, e.g. coronary artery disease, diabetes, and hypertension, were frequent in this population. CONCLUSIONS: This is the first report of hemicrania continua and episodica in African Americans and the second in persons of African descent in the world's literature. Late age of onset, frequent serious medical illnesses, and family history of migraine differentiate this series from previous reports. The lack of reports in African Americans most likely reflects misdiagnosis rather than true prevalence. Thus, whenever any patient presents with chronic daily unilateral headache, ipsilateral autonomic symptoms should be assessed during severe headache attacks, and an indomethacin trial considered.

Black or African American↗

PubMed2002

Circadian heart rate variability in patients with primary arterial hypertension.

INTRODUCTION: Abnormalities of autonomic nervous system function (ANS) exist in patients with hypertension and have been considered as one of the important factors in developing of essential hypertension. However the information about the effect of antihypertensive treatment (angiotensin-converting enzyme inhibitors--ACEI) on cardiac ANS activity is scarce. The main aim of our study was to evaluate circadian heart rate variability changes in primary hypertension treated with ACE inhibitors. METHODS: Fourteen patients with essential hypertension with night/day differences of mean blood pressure of more than 10% (dippers) aged 26 to 61 years (mean 52.9 +/- 9.2) and 14 healthy volunteers, 14 healthy volunteers were recruited and matched for age and gender. Ambulatory 24 hour arterial blood pressure measurement using HolCARD (Aspel, Poland) recorder with simultaneous ECG monitoring using the Mediarc-Premier IV Holter Monitoring System (DRG-International) undertaken in both groups of patients. The hypertensive patients were tested before and after one year of the enalapril treatment. The HRV analysis was performed according to a standard Fast Fourier Transformation (FFT). The time and spectral analysis parameters were compared within the examined groups of patients during day and night. RESULTS: The results obtained in the control group showed the great circadian fluctuations in sympathetic and parasympathetic activity. A simultaneous circadian HRV evaluation showed also significant increases in night RMSSD, TP, VLF, LF and HF mean values (p < 0.05) with significantly decreased night LF/HF ratio (p < 0.05) in comparison to the day time recordings. After one year of ACE inhibitors treatment, we noted the following circadian changes: Significant increase of RMSSD, pNN50 (p < 0.05), TP, VLF, LF and HF (p < 0.05) parameters with non-significant increases in LF/HF ratio during night recording. A comparison between daily HRV parameters, before and after 1 year of ACEI treatment, presented significant increases in pNN50 and RMSSD mean values (p = 0.01) and decreases in LF/HF ratio, with simultaneous withdrawal of spectral parameters. However, no significant changes in comparison of night HRV parameters before and after ACEI intake were observed. CONCLUSIONS: Our observations support an attenuation of night vagal activity as an important factor in the pathogenesis of essential hypertension. The lack of effect of ACEI treatment on circadian heart rate variability parameters in patients with essential hypertension, despite long-term normalisation of blood pressure, suggests persistent circadian cardiac autonomic dysfunction in the "dippers" subgroup of patients.

Adult↗

PubMed1994

Pathophysiology of white fingers in workers using hand-held vibrating tools.

The pathogenic events and the localization of the primary lesion in white fingers among persons using hand-held vibrating tools are still unclarified. A "vibration disease" has been proposed to be due to damage to the limbic system and other brain structures which causes autonomic dysfunction. Current common opinion regards the pathogenesis of white fingers to be a result of longterm exposure to various physical and psychological environmental stressors, but the relative importance of one stressor or other is unknown. Observations indicating a chronic autonomic disturbance include changes in cardiac functions, excessive hearing loss in persons with VWF, and reduced toe skin temperature also in the absence of acute cold or vibration exposure. Sympathetic hyperactivity alone has long been postulated to account for vibration-induced white fingers, but damage to vaso-regulatory structures and functions in the finger skin now also seems to be involved. An abnormal level of sympathetic efferents is likely to be important for producing the symptoms in white fingers. Recent findings, however, indicate that the pathogenesis also involves changes in alpha-adrenergic receptor mechanisms as well as endothelial damage with deficient function of endothelial-derived relaxing factor. The role of vessel lumen reduction due to organic changes and an increase in whole blood viscosity remains unclarified. The understanding of the influence of confounders such as cold exposure, smoking habits and variations in individual susceptibility is also lacking. In particular, the physiological complexity of the response to cold is so great and the interaction between various vaso-regulatory mechanisms so intricate that only a multifactorial etiology and pathogenesis is likely for Raynaud's phenomenon in persons using hand-held vibrating tools. A model is suggested for the manifestation of abnormally strong vasoconstriction and white fingers as a result of a narrowing of the gap between the individual symptom threshold and the level of sympathetic activity.

Fingers↗

PubMed1994

[Familial amyloid polyneuropathy type IV (Finnish type)--a clinicopathological study].

Familial amyloid polyneuropathy type IV (Finnish type, FAP IV) is one form of hereditary generalized amyloidosis with autosomal dominant trait and is characterized clinically by a triad of corneal lattice dystrophy, caudal cranial neuropathy and various skin changes. The vast majority of the families with this disorder originated from Finland. We carried out a clinicopathological study of a large FAP IV kindred recently found in Japan. This family consisted of 73 members in 5 generations with 17 affected individuals and 7 of them (ages 45 to 73) were examined in detail. All patients showed typical clinical manifestations, lacking significant peripheral neuropathy in the limbs. However, autonomic dysfunctions including orthostatic fainting and dysuria were seen in 2 patients. Congophilic amyloid deposits were commonly observed in an aspiration biopsy of abdominal fat tissues, and noradrenergic nerve fibers of the rectal mucosa were reduced in one patient with autonomic symptoms. DNA analysis using PCR revealed a single base change (G to A) at nucleotide position 654 of the gelsolin gene in 7 patients and one asymptomatic individual. The clinical pictures and gene abnormality in this Japanese family are very similar to those reported in Finland. Moreover, this study added that autonomic nerves might be involved in FAP IV patients at the advanced stage.

Adipose Tissue↗

PubMedDate not supplied

Involvement of the autonomic nervous system in patients with syringomyelia--a study with the sympathetic skin response.

In order to determine the function of the autonomic nervous system in syringomyelia, the sympathetic skin response (SSR) was performed in 13 patients with syringomyelia and 20 healthy controls. SSR was recorded from both palms and soles. In patients with syringomyelia, we found absent responses, prolonged latencies and reduced amplitudes. SSRs could be recorded in 15 out of the examined 26 upper extremities. The latencies were prolonged in 12 of these cases. In the lower limbs, 11 SSRs could be obtained. In 4 of these cases the latencies were prolonged. The SSR latencies recorded from the palms and soles were both significantly prolonged (p < 0.05) and the amplitudes were reduced (p < 0.05) as compared to normal persons. Our data strongly suggest involvement of the autonomic nervous system in syringomyelia as assessed by the SSR response (in upper and lower extremities). In our patients, the extent of autonomic dysfunction was not related to the stage or the duration of disease.

Adult↗

PubMed1997

Autonomic failure in Guamanian neurodegenerative disease.

Autonomic impairment is minor in idiopathic amyotrophic lateral sclerosis (ALS) and Alzheimer's-type dementia (D) and is usually not marked in Parkinson's disease. The autonomic status of Guamanian parkinsonism (P), ALS, and parkinsonism-dementia complex (PDC) is essentially unknown. We therefore evaluated the autonomic nervous system in Guamanian D, ALS, P, and PDC. Cardiovagal, adrenergic, and postganglionic sudomotor functions were quantitated in 16 patients and 14 paired household controls. Patients comprised PDC (N = 11), D (N = 2), P (N = 2), and ALS (N = 1). Autonomic deficit was expressed on a composite autonomic scoring scale (CASS) and its subsets that corrects for the effects of age and gender. CASS severity was rated from 0 to 10 and the maximal subset scores were 3, 3, and 4 for postganglionic sudomotor, cardiovagal, and adrenergic deficits, respectively. CASS scores for mild, moderate, and severe autonomic failure are 1 to 3, 4 to 6, and 7 to 10, respectively. Symptoms were scored by an Autonomic Symptom Profile (ASP). The affected patients were older than and had a sex distribution different from paired controls (64.2 +/- 8.0 versus 53.1 +/- 13.5; p < 0.01; male/female = 9/7 versus 2/12; p = 0.045). CASS scores were markedly increased over paired controls (6.2 +/- 2.3 versus 1.9 +/- 1.3; p < 0.001), and involvement was generalized by system. There were deficits in sudomotor, cardiovagal, and adrenergic function. Orthostatic hypotension occurred in 6 of 16 patients and 2 of 14 paired controls. Guamanian patients had more autonomic dysfunction than non-Guamanian Parkinson's disease. ASP scores were higher in patients than controls and regressed with CASS. These differences persisted when corrected for the confounding effects of age, gender, and diabetes. We conclude that Guamanian patients have autonomic failure to a greater extent than non-Guamanian Parkinson's disease or ALS. This autonomic failure suggests multisystem autonomic involvement similar to but less severe than in multiple system atrophy.

Adult↗

PubMed1997

Relationship between autonomic function and plasma fibrinogen, viscosity, and elements of fibrinolytic activity in diabetic nephropathy.

Twenty-three insulin-dependent diabetics with proteinuria (3.3 g/day: range 0.3 to 8.9) and azotemia (creatinine clearance: 58 mL/min, range 30 to 112) were tested for 24-h mean arterial blood pressure; instantaneous heart rate variations to a computerized protocol involving timed ventilation, assumption of upright posture, and Valsalva maneuver; plasma fibrinogen, viscosity, fibrinolytic activity, and plasminogen activator inhibitor. These were to test the hypothesis that autonomic dysfunction is associated with altered concentrations of plasma fibrinogen, fibrinolytic activity, viscosity, and plasminogen activator inhibitor. We have previously shown the absence of a correlation between level of blood pressure, clinical and standard laboratory testing, and the results of the autonomic function testing protocol used in this study. In this group of patients, plasma fibrinogen concentration was correlated (positively) with mean arterial pressure and (negatively) with heart rate variation in response to the Valsalva maneuver. The greater the mean arterial pressure or the worse the Valsalva results, the higher the plasma fibrinogen concentration. In addition, patients with one or no abnormal autonomic function tests had a mean fibrinogen of less than 400 mg/dL compared to the group of patients with two or more abnormal tests who had a mean fibrinogen of 500 mg/dL. In patients with demonstrated parasympathetic abnormalities, postural heart rate variation testing also discerned a differential in plasma fibrinogen. Lower concentration of plasminogen activator inhibitor throughout the day, and greater fibrinolytic activity in the morning were also noted to be present in patients with abnormal heart rate response to the Valsalva maneuver. We conclude that there are relationships between high blood pressure, autonomic function, and hemostatic factors favoring thrombogenesis that may be related by common mechanisms and treatments in the diabetic with kidney disease.

Adult↗

PubMed1979

Respiratory influence on heart rate in diabetes mellitus.

To find a simple and accurate test of autonomic nervous dysfunction in diabetes mellitus, 41 insulin-dependent diabetics and 25 controls were investigated. The diabetics, none of whom had symptoms of autonomic dysfunction, were tested for retinopathy and sensory neuropathy. Each subject also performed maximal deep-breathing procedures while undergoing electrocardiographic recording: in normal subjects the intervals are shortened during inspiration and prolonged during expiration, and a difference in the heart rate between inspiration and expiration of 10% or less seems to indicate autonomic dysfunction. This difference was calculated as an E:I ratio of the mean of the longest R-R interval during maximal expiration to the mean of the shortest during maximal inspiration. Ten of the 18 patients found to have sensory neuropathy had abnormal E:I ratios, and among those with absent ankle reflexes the proportion was even higher (9 out of 11). The E:I ratio also seemed to be as accurate as traditional tests for autonomic dysfunction and easier to perform. Diabetics with autonomic dysfunction have an increased risk of acute cardiorespiratory death during and after surgery, and maximal deep breathing and calculation of the E:I ratio may be a useful test to perform on diabetics at risk.

Adult↗

PubMed1997

Immunological and endocrinological abnormalities in paraneoplastic disorders with involvement of the autonomic nervous system.

We report a series of four patients in whom the onset of systemic cancer was heralded by dysautonomic symptoms and a neurological non-metastatic complication mediated by immunological and endocrine factors. The series includes: a patient with acute leukaemia and autonomic sensory-motor polyradiculoneuropathy, a patient affected by colon carcinoma and autonomic neuropathy and limbic encephalitis, a patient with lung cancer and autonomic neuropathy and hypercalcaemic encephalopathy, a patient with small cell lung cancer associated with autonomic neuropathy in Lambert-Eaton Myasthenic Syndrome (LEMS) and syndrome of inappropriate ADH secretion (SIADH). We underline the prognostic importance and discuss the possible etiopathogenetic role of autonomic dysfunction, which is frequently associated with paraneoplastic neurologic syndromes of autoimmune and/or dysendocrine origin.

Aged↗