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Results for “Autonomic dysfunction”

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PubMed1993

Effects of ponalrestat, an aldose reductase inhibitor, on neutrophil killing of Escherichia coli and autonomic function in patients with diabetes mellitus.

In diabetic subjects, polyol pathway activity might inhibit neutrophil function and cause nerve damage. The effects of ponalrestat, an aldose reductase inhibitor, were assessed on neutrophil intracellular killing of Escherichia coli and on autonomic function in diabetic subjects in a randomized double-blind, placebo-controlled, crossover trial. We studied 31 diabetic subjects with autonomic dysfunction and 21 age- and sex-matched control subjects. During two 12-wk treatment periods, the diabetic subjects took either 600 mg of ponalrestat or matching placebo once daily. Neutrophil killing of E. coli was measured by a microbiological assay technique. Kmax by neutrophils from the diabetic subjects was lower than in the control group (Kmax of diabetic subjects 54.5 +/- 26.4 vs. control subjects 67.3 +/- 16.3, P = 0.045). Ponalrestat significantly increased bacterial killing in the diabetic subjects (Kmax of ponalrestat 75.1 +/- 16.5 vs. placebo 58.2 +/- 20.8, P = 0.003) so that there was no longer any significant difference in Kmax between the control subjects and the diabetic subjects on active treatment. Ponalrestat had no significant effect on a range of standard cardiovascular autonomic nerve function tests. We conclude that neutrophil killing of E. coli is impaired in diabetic subjects with autonomic dysfunction. This is restored to normal by ponalrestat.

Aldehyde Reductase↗

PubMed2003

Autonomic Nervous System Dysfunction in Parkinson's Disease.

Autonomic nervous system (ANS) dysfunction is common in Parkinson's disease (PD), affects 70% to 80% of patients, and causes significant morbidity and discomfort. Autonomic nervous system dysfunction symptoms in PD include sexual dysfunction, swallowing and gastrointestinal disorders, bowel and bladder abnormalities, sleep disturbances, and derangements of cardiovascular regulation, particularly, orthostatic hypotension. Autonomic nervous system dysfunction in PD may be caused by an underlying degenerative process that affects the autonomic ganglia, brainstem nuclei, and hypothalamic nuclei. Anti-parkinsonian medications can cause or worsen symptoms of ANS dysfunction. The care of a PD patient with ANS dysfunction relies on its recognition and directed treatment, including coordinated care between the neurologist and appropriate subspecialist. Pharmacotherapy may be useful to treat orthostasis, gastrointestinal, urinary, and sexual dysfunction.

Journal Article↗

PubMed2003

Impaired cardiac autonomic functions in patients with environmental asbestos exposure: a study of time domain heart rate variability.

Environmental asbestos exposure is related to diffuse pleural disease (thickening and calcification) and restrictive pulmonary disease. To assess cardiac autonomic system, we investigated the time domain heart rate variability (HRV) by Holter monitoring and their correlation with pulmonary function tests in patients with pleural disease caused by environmental asbestos exposure. We studied 45 patients (26 men, 19 women, aged 62.67 +/- 10.1 years) and 35 healthy patients who had similar sex and age profile to the patients (24 men, 11 women, aged 59.31 +/- 8.4 years). The asbestosis group was divided into 3 subgroups according to the severity of forced vital capacity (FVC) severe (group 1) (n = 12): FVC less than 50% of expected, moderate (group 2) (n = 16): FVC 64%-51% of expected and mild (group 3) (n = 17): FVC 65%-80% of expected. HRV parameters were significantly different among all groups (P<.0001). Comparing the 4 groups (subgroups and control group), group 1 had the lowest mean HRV values and controls had the highest mean HRV values (P<.0001). Severity of autonomic dysfunction was correlated with the severity of FVC and arterial oxygen pressure. Right ventricular end-diastolic internal diameter (RVEDID) and right ventricular end-systolic internal diameter (RVESID) values were significantly increased in patients (P <.0001, P < 0.0001, respectively). Pulmonary acceleration time (AcT) values were shorter in all patient groups than control group (P <.0001). It was shortest in group 1. Group 2 and 3 had shorter AcT values than control group. HRV parameters were correlated positively with AcT values and negatively with RVEDID and RVESID values. In conclusion, patients with restrictive pulmonary disease due to environmental asbestos exposure had autonomic dysfunction, which was correlated with the severity of restriction. This was thought to be the result of chronic hypoxia, pulmonary hypertension, and right ventricular enlargement.

Analysis of Variance↗

PubMed1996

Electrophysiological evaluation of peripheral autonomic function in leprosy patients, leprosy contacts and controls.

Since there is immunocytochemical evidence that the initial damage in leprosy is directed at distal, small, unmyelinated nerve fibers, we investigated several electrophysiological methods for their potential value in detecting peripheral autonomic dysfunction in leprosy contacts and leprosy patients. Fingertip blood flow velocity and its control by vasomotor reflexes (VMR) with a laser Doppler flowmeter, fingertip skin temperature, and the sympathetic skin response (SSR) to exosomatic stimuli were studied in 89 leprosy patients, 36 leprosy contacts and 47 normal subjects. Whereas there were no significant differences between the groups in fingertip skin temperature and resting blood flow velocity measurements, there were significant differences in the prevalence of impaired fingertip VMR and absent SSR. The prevalence of absent SSR in leprosy patients was 60.9%, in contacts 13.8%, in controls 6.3%. The prevalence of abnormal VMR in leprosy patients was 61.2%, in contacts 34.7% and in controls 10.6%. VMR testing is a more sensitive test method for autonomic dysfunction compared with the SSR. The implication of impairment in vasomotor and sudomotor function in leprosy contacts needs yet to be determined. However, we propose this to be a response to exposure to Mycobacterium leprae, which represents either ongoing nerve damage or nonprogressive residual autonomic nerve damage. We suggest that VMR testing and SSR are valuable methods to evaluate early leprous neuropathy.

Adolescent↗

PubMed2001

Psychological and physiological factors associated with tilt table testing for neurally mediated syncopal syndromes.

This study investigated psychological and physiological factors in two groups of patients who had tilt table testing for autonomic dysfunction. The first group of 61 patients completed assessments of depression, anxiety, and symptom effects on lifestyle. The 25 patients identified as tilt positive were younger (30.5 years) and had higher mean depression scores (7.6) compared to the tilt-negative response group (n = 36); the latter averaged 40 years of age and had mean depression scores of 4.6. These differences were statistically significant. Women testing tilt positive were significantly more depressed than tilt-negative women (P = 0.02). More severe depressive symptoms were associated with lower blood pressure (BP) (P < 0.05). A second group of 52 patients was monitored during tilt for BP, heart rate (HR), skin temperature (TEMP), skin conductance level (SCL), and forehead muscle tension (EMG). Twenty-seven tested positive and 23 were negative. There were statistically significant group differences in systolic BP and diastolic BP (P < 0.05). There was a significant interaction between tilt status (positive or negative) and time (P = 0.03) in HR. TEMP increased 2 degrees over time in both groups (P < 0.05). The decrease in SCL from 13.7 to 10.4 mu omega in the tilt-positive response group compared to the slight increase in the tilt-negative group was significantly different (P < 0.05). Identification of psychological factors correlated with BP and physiological changes that accompany decreases in BP in tilt-positive response patients could guide management of patients with autonomic dysfunction.

Adult↗

PubMed2003

Sweating dysfunction in Parkinson's disease.

We sought to determine the prevalence and nature of sweating disturbances in patients with Parkinson's disease (PD), and investigated their correlation with other clinical features and with Quality of Life (QoL) measures. A questionnaire on symptoms and consequences of sweating dysfunction was completed by 77 consecutive outpatients, from three movement disorder clinics, and 40 controls. QoL was assessed using the disease-specific Parkinson's Disease Questionnaire (PDQ)-39 and generic EuroQoL (EQ)-5D rating scales. Patients also underwent a clinical examination, including assessment with the Unified Parkinson's Disease Rating Scale and the Hoehn and Yahr staging system. Sweating disturbances, either hypohidrosis or in particular, hyperhidrosis, were reported by 64% of patients and by 12.5% of controls (P < 0.005) and were often localised or asymmetric. Complaints of sweating disturbances were not correlated with disease severity, but did correlate with other symptoms of autonomic dysfunction. Sweating problems occurred predominantly in off periods and in on periods with dyskinesias. Sweating disturbances were not correlated with overall QoL scores, but we did observe a significant correlation with the pain dimension of the PDQ-39 and the visual analogue scale of the EQ-5D. Furthermore, many patients reported physical, social, and emotional impairment due to sweating. Sweating disturbances are common and distressing symptoms of PD that are related mainly to autonomic dysfunction, off periods, and dyskinesias.

Aged↗

PubMed1999

RR interval variation, sympathetic skin reflex and QT dispersion in the assessment of autonomic function in peripheral neuropathy.

In recent years, two simple non-invasive tests, sympathetic skin response (SSR) and RR interval variation (RRIV) have been used to assess autonomic function (Wang et al., 1994; Drory et al., 1995; Chassande et al., 1996; Bordet et al., 1996; Spitzer et al., 1997). Their easy performance by an electromyographic (EMG) machine and successful clinical correlation (Shahani et al., 1990) have made them routine in autonomic assessment in several EMG laboratories. More recently QT dispersion on electrocardiogram (ECG) has been shown to have increased in patients with diabetic neuropathy and several autonomic dysfunctions (Wei et al., 1995; Lengyel et al., 1997; Langen et al., 1997; Axelrod et al., 1997). In order to evaluate the diagnostic value of the RR interval variation, sympathetic skin response and QT dispersion combined, we performed the three tests on a group of 37 patients with several peripheral neuropathies with and without clinical signs of dysautonomia. All patients were studied using an electromyograph (EMG). The results of the SSR, RRIV and QT dispersion combined have shown abnormal values in all patients with neuropathy and clinical dysautonomia and in some patients without clinical signs of dysautonomia, suggesting a subclinical autonomic dysfunction. The principal finding of this study is that the evaluation of the RRIV, SSR and QT dispersion combined may contribute to the assessment of dysautonomia in patients with somatic neuropathy and that they may be currently performed by an electromyograph.

Adult↗

PubMed2006

[Idiopathic Lambert-Eaton myasthenic syndrome: report of one case].

Lambert-Eaton myasthenic syndrome (LEMS) is an autoinmune idiopathic or paraneoplastic syndrome producing antibodies agaisnt presynaptic voltage calcium channels. The clinical features of patients with LEMS are muscle weakness and autonomic dysfunction. We report a 40 years old man with a four years history of proximal weakness, absent tendon reflexes and dry mouth. The diagnosis was confirmed by characteristic electromyographic findings, showing a low-amplitude muscle response that increased dramatically after activation. Circulating antibodies to voltage-gated calcium channel were present. The search for malignant tumors was negative. The patient was treated with prednisone and azathioprine and after four months, he was able to walk and signs of autonomic dysfunction started to subside.

Adult↗

PubMed2001

Modulatory effects of respiration.

Respiration is a powerful modulator of heart rate variability, and of baro- and chemoreflex sensitivity. Abnormal respiratory modulation of heart rate is often an early sign of autonomic dysfunction in a number of diseases. In addition, increase in venous return due to respiration may help in maintaining blood pressure during standing in critical situations. This review examines the possibility that manipulation of breathing pattern may provide beneficial effects in terms not only of ventilatory efficiency, but also of cardiovascular and respiratory control in physiologic and pathologic conditions, such as chronic heart failure. This opens a new area of future research in the better management of patients with cardiovascular autonomic dysfunction.

Arrhythmia, Sinus↗

PubMed2002

Cerebral metabolic changes in early multiple system atrophy: a PET study.

Previous positron emission tomography (PET) studies have shown widespread hypometabolism in the brain of advanced MSA but the time course of these metabolic abnormalities is largely unknown. In order to clarify the principal disease processes in multiple system atrophy (MSA) in the early stage, we investigated regional cerebral glucose metabolism (rCMGglc) and nigral dopaminergic function in nine patients with early stage of MSA using [(18)F]fluorodeoxyglucose (FDG) and 6-L-[(18)F]fluorodopa ((18)F-Dopa) positron emission tomography (PET) (two men and seven women; age, 59.3+/-5.4 years; disease duration, 29.7+/-14.6 months). The rCMRglc in the early MSA patients significantly decreased in the cerebellum, brainstem, and striatum compared with that in nine normal subjects. A significant correlation was found between the severity of autonomic dysfunction and rCMRglc within the brainstem. The severity of extrapyramidal signs also correlated with the decline of F-Dopa uptake but not that of rCMRglc within the striatum. The degree of atrophy on MRI has correlated with neither the clinical symptoms nor rCMRglc at the cerebellum and the brainstem. Our PET studies demonstrated widespread metabolic abnormalities except for the cerebral cortex in the brain of MSA even in the early stage. The hypometabolism in the brainstem was tightly linked to the autonomic dysfunction. Not the striatal dysfunction but the nigral damage may be responsible for the extrapyramidal symptoms in early MSA.

Aged↗

PubMed1993

Effect of diabetes and aging on human sympathetic autonomic ganglia.

Although autonomic dysfunction frequently complicates the clinical course of patients with diabetes, relatively little is known of its underlying neuropathology. Using experimental animal models as a guide, the prevertebral superior mesenteric (SMG) and paravertebral superior cervical (SCG) sympathetic ganglia have been examined in a series of adult autopsied diabetic and non-diabetic patients of various ages using histochemical, ultrastructural, morphometric, and immunohistochemical methods. Quantitative studies demonstrated that markedly swollen argyrophilic terminal axons (neuroaxonal dystrophy) containing large numbers of disorganized neurofilaments developed in the SMG but not SCG as a function of diabetes, increasing age, and gender (males were more severely affected than females). As in experimental animals, diabetic (types I and II) patients developed histologically identical lesions prematurely and in greater numbers than age-matched nondiabetic patients. Morphometric studies showed a small but statistically significant decrease in neuronal density in the SMG but not SCG of diabetic patients. The dimensions of individual sympathetic neurons were not significantly different in aging or diabetes. The pathological lesions identified in the SMG may contribute to the autonomic dysfunction so commonly observed in diabetic patients.

Adolescent↗

PubMed1999

Noninvasive exploration of cardiac autonomic neuropathy. Four reliable methods for diabetes?

OBJECTIVE: The purpose of this work was to assess relevant information that could be provided by various mathematical analyses of spontaneous blood pressure (BP) and heart rate (HR) variabilities in diabetic cardiovascular neuropathy. RESEARCH DESIGN AND METHODS: There were 10 healthy volunteers and 11 diabetic subjects included in the study. Diabetic patients were selected for nonsymptomatic orthostatic hypotension in an assessment of their cardiovascular autonomic impairment. Cardiac autonomic function was scored according to Ewing's methodology adapted to the use of a Finapres device. The spontaneous beat-to-beat BP and HR variabilities were then analyzed on a 1-h recording in supine subjects. The global variabilities were assessed by standard deviation, fractal dimension, and spectral power. The cardiac baroreflex function was estimated by cross-spectral sequences and Z analyses. RESULTS: In diabetic patients, Ewing's scores ranged from 1 to 4.5, confirming cardiovascular autonomic dysfunction. In these diabetic patients, global indices of variabilities were consistently lower than in healthy subjects. Furthermore, some of them (standard deviation and fractal dimension of HR, spectral power of systolic blood pressure and HR) were significantly correlated with the Ewing's scores. The Z methods and the spectral analysis found that the cardiac baroreflex was less effective in diabetic subjects. However, the baroreflex sensitivity could not be reliably assessed in all the patients. The sequence method pointed out a decreased number of baroreflex sequences in diabetic subjects that was correlated to the Ewing's score. CONCLUSIONS: Indices of HR spontaneous beat-to-beat variability are consistently related to the degree of cardiac autonomic dysfunction, according to Ewing's methodology. The Z method and spectral analysis confirmed that the cardiac baroreflex was impaired in diabetic patients. These methods might be clinically relevant for use in detecting incipient neuropathy in diabetic patients.

Adult↗

PubMed1992

Neuroleptic malignant syndrome among acute psychiatric admissions in Barbados.

The main features of the Neuroleptic Malignant Syndrome (NMS), a complication of neuroleptic therapy, are fever, muscle rigidity, autonomic dysfunction, and an alteration in consciousness level. We describe five cases of NMS comprising 0.6% of acute neuroleptically-treated admissions to a psychiatric hospital over a one-year period. All patients, four females aged 26 to 63 years, and one male, aged 65 years, were of African origin and received multiple neuroleptic drugs, at least one of which was a depot preparation. Four were being treated for functional psychiatric disorders while one had dementia. All patients had fever and depressed consciousness level while four had rigidity and autonomic dysfunction. Serum creatine phosphokinase was elevated in 4 cases, and there was indirect evidence of myoglobinuria in 3 cases suggested by a positive urine dipstick test for blood despite the absence of red cells on microscopy. Rhabdomyolysis was associated with renal failure in one case. Both bromocriptine mesylate and dantrolene sodium were given in two cases. Three patients died in hospital, one with persistent rigidity and progressive decubitus ulceration, one from peritonitis following peritoneal dialysis, and another suddenly. Early recognition of NMS is important; it should be considered in any patient on neuroleptic therapy who develops fever, rigidity or alteration in consciousness level.

Adult↗

PubMed2005

Factors influencing outcome in Guillain-Barré Syndrome: comparison of plasma adsorption against other treatments.

OBJECTIVE: This study was performed to evaluate which factors influence the outcome of Guillain-Barré Syndrome (GBS), focusing on the choice of treatments. METHODS: Sixty-three GBS patients were retrospectively studied and the following factors were evaluated: sex, age, days from onset of disease to the start of treatment, severity of symptoms, prior infection, autonomic dysfunction, bulbar palsy, anti-ganglioside antibody, and disease form, as well as the choice of treatment. Plasma adsorption (PA, n=39), plasma exchange (PE, n=14), or immunoglobulin treatment (IVIg, n=10) were performed in this study. Outcomes were evaluated using the functional grading scale (FGS) of Hughes. RESULTS: The number of days needed for one functional grade improvement was significantly longer in the elderly, the severe symptom group, and patients with acute motor axonal form, and days needed for two functional grade improvement was significantly longer in the elderly, patients with autonomic dysfunction, and acute motor axonal form. The choice of treatments (PA, PE, or IVIg) did not significantly influence the outcome as determined by both univariate and multivariate analysis. CONCLUSION: Although patient age, symptoms, and disease form influenced the outcome, treatment methods did not significantly influence the outcome. Since PA does not result in a risk of unknown infection, choosing a PA treatment may be justified, especially for patients (or doctors) who may be anxious about a possibility of unknown infection.

Adolescent↗

PubMed2004

Aromatic L-amino acid decarboxylase deficiency: clinical features, treatment, and prognosis.

BACKGROUND: Deficiency of aromatic L-amino acid decarboxylase (AADC) is associated with severe developmental delay, oculogyric crises (OGC), and autonomic dysfunction. Treatment with dopamine agonists and MAO inhibitors is beneficial, yet long-term prognosis is unclear. OBJECTIVE: To delineate the clinical and molecular spectrum of AADC deficiency, its management, and long-term follow-up. RESULTS: The authors present six patients with AADC deficiency and review seven cases from the literature. All patients showed reduced catecholamine metabolites and elevation of 3-O-methyldopa in CSF. Residual plasma AADC activity ranged from undetectable to 8% of normal. Mutational spectrum was heterogeneous. All patients presented with hypotonia, hypokinesia, OGC, and signs of autonomic dysfunction since early life. Diurnal fluctuation or improvement of symptoms after sleep were noted in half of the patients. Treatment response was variable. Two groups of patients were detected: Group I (five males) responded to treatment and made developmental progress. Group II (one male, five females) responded poorly to treatment, and often developed drug-induced dyskinesias. CONCLUSIONS: The molecular and clinical spectrum of AADC deficiency is heterogeneous. Two groups, one with predominant male sex and favorable response to treatment, and the other with predominant female sex and poor response to treatment, can be discerned.

Adolescent↗

PubMedDate not supplied

Electrocardiographic measures and heart rate variability in patients with familial dysautonomia.

Cardiovascular abnormalities are prominent in the genetic disorder, familial dysautonomia (FD). To determine if autonomic dysfunction involves cardiac, as well as peripheral vascular integrity, noninvasive tests were performed in 10 FD patients and 8 healthy control subjects while supine and at 90 degrees tilt. Simultaneous blood pressure (BP) and heart rate (HR) and QTc were obtained, while performing signal-averaged electrocardiography (SAECG) and heart rate variability (HRV). FD subjects were tested on 2 separate days, before and 1 h after oral fludrocortisone or midodrine; controls were tested once without medication. With tilt, all FD subjects decreased mean BP > or = 24 mm Hg by 5 min. On SAECG, 70% of supine FD subjects had a prolonged tQRS; only 2 FD subjects shortened tQRS with tilt. The QTc interval was prolonged (> 440 ms) in 2 supine FD subjects; with tilt, the QTc prolonged in a third. Frequency domain analysis of HRV revealed that mid (MF) and high frequency band areas were significantly decreased when supine, but not when upright. On time domain analysis, the pNN50 was significantly decreased in FD subjects (8.9 +/- 1.7 vs. 17.7 +/- 3.6%, p < 0.01). Fludrocortisone lowered supine BP and HR and increased supine MF area. Midodrine raised supine and erect BP, lowered erect HR, and shortened erect QTc. Although all FD subjects have abnormal orthostatic BP and HR responses, cardiac tone, as assessed by electrocardiographic and HRV responses, varies. Prolongation of the tQRS appears to be a sensitive but not specific indicator of autonomic dysfunction. QTc prolongation may indicate more extensive sympathetic dysfunction. HRV data suggest some FD patients have abnormalities in parasympathetic, as well as sympathetic, cardiac tone.

Adolescent↗

PubMedDate not supplied

[Vascular tone in adolescents with autonomic vascular dysfunction of hypotensive type (according to the data of tetrapolar rheovasography)].

408 teenagers at the age of 11-14 y.o. have been investigated. All of them underwent tetrapolar longitudinal reovasography. 339 were found with hypotensive type vegetovascular dysfunction. It was established, that the vascular component plays an essential part in genesis of vegetovascular disfunction; vessels are losing their elasticity, that leads to decreasing of vascular tone, blood filling of some parts of the body, decelerating pulse-wave velocity, at the end transforming in steady decreasing of arterial tension.

Adolescent↗

PubMed1990

[Nosological correlation between autonomic failure with Lewy bodies and Parkinson's disease--comparative assessment of clinical features in both diseases].

Autonomic failure with Lewy bodies (AF-LB) was first described by Fichefet et al. in 1965, and more than ten cases have been reported to date. AF-LB and Parkinson's disease (PD) share the neuropathological findings characterized by widely distributed Lewy bodies in the central nervous system including the substantia nigra and locus coeruleus. However, clinical manifestations of AF-LB are far different from PD in which autonomic dysfunction, if present, is not a predominant feature. In the present study, clinical features were comparatively analysed in AF-LB and PD to investigate the nosological relation between PD and AF-LB. The subjects were 94 patients with PD and 11 reported cases of AF-LB in the literature. A test of 70 degrees passive head-up tilt was performed upon the patients with PD in our laboratory. Based on the results in tilting test, the patients with PD were divided into two groups; PD-I (69 cases) with an orthostatic fall of systolic blood pressure less than 30 mmHg, and PD-II (25 cases) with a fall of 30 mmHg or more. Autonomic dysfunctions were more extent in PD-II than in PD-I, because the incidences of anhidrosis, impotence, neurogenic bladder and constipation were higher in PD-II. All of the cases of AF-LB were contained in the previous literatures with reasonably full clinical descriptions. Mean age at onset of the disease was 62.1 +/- 8.7 (mean +/- SD) years old in PD-I, 64.5 +/- 7.5 years old in PD-II and 63.9 +/- 9.0 years old in AF-LB, and no significant differences were found among the three groups.(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗