SEARCH · PubMed Health

Results for “Autonomic dysfunction”

Explore indexed PubMed citations for clinical trials, systematic reviews and public health research. Read source abstracts and follow each citation to its original PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 991 records · Page 55Linked to original sources

PubMed1999

Diffuse Lewy body disease presenting as multiple system atrophy.

OBJECTIVES: The majority of patients with diffuse Lewy body disease have cognitive or psychiatric manifestations as part of their initial presentation. A sizable minority present with parkinsonian features alone. Autonomic features may also occur, typically after the development of cognitive changes. We aim to demonstrate that diffuse Lewy body disease may rarely also present with parkinsonism accompanied by marked autonomic dysfunction in the absence of significant cognitive or psychiatric abnormalities. METHODS: Case report based on a retrospective chart review and neuropathological examination. RESULTS: We report on a patient in whom a clinical diagnosis of multiple system atrophy was made based on a presentation of parkinsonism with prominent and early autonomic involvement. The former included postural tremor, rigidity and bradykinesia, while the latter consisted of repeated falls due to orthostasis and the subsequent development of urinary incontinence midway through the course of her illness. She was poorly tolerant of dopaminergic therapy due to accentuated orthostasis. Benefit from levodopa was limited and only evident when attempted withdrawal resulted in increased rigidity. There was no history of spontaneous or drug-induced hallucinations, delusions or fluctuating cognition, and in contrast to the prominence and progression of her parkinsonian and autonomic features over the first several years, cognitive impairment did not occur until the final stages of her illness, seven years after the onset of initial symptoms. Neuropathological examination revealed numerous Lewy bodies in both neocortical as well as subcortical structures consistent with a diagnosis of diffuse Lewy body disease. There was marked neuronal loss in the substantia nigra as well as the autonomic nuclei of the brainstem and spinal cord. CONCLUSIONS: In addition to cognitive, psychiatric, and parkinsonian presentations, diffuse Lewy body disease may present with parkinsonism and prominent autonomic dysfunction, fulfilling proposed criteria for the striatonigral form of MSA.

Aged↗

PubMed1999

Oesophageal atresia, related malformations, and medical problems: a family study.

Oesophageal atresia (OA) and tracheo-oesophageal fistula (TOF) are life-threatening malformations of generally undefined cause. Previous reports of familial cases suggest a genetic contribution. The pattern of inheritance appears non-Mendelian, i.e., multifactorial. Individuals with OA/TOF often have other malformations and medical problems. The aim of this study was to determine the association in OA/TOF cases and healthy control subjects of associated malformations, midline defects, and medical conditions. We also investigate the relationships of these conditions in the relatives of the cases and controls. The results show that infants with OA/TOF frequently have VACTERL anomalies (vertebral, 17%; anal, 12%; cardiac, 20%; renal, 16%; limb, 10%) and other midline defects (cleft lip and palate, 2%; sacral dysgenesis, 2%; urogenital anomalies, 5%). The following medical problems were also reported: oesophageal dysmotility, 21%; gastro-oesophageal reflux, 22%; chest infections, 6%; and autonomic dysfunction, 0.5%. The first-degree relatives of children with OA are much more likely to have one of the aforementioned malformations or medical conditions when compared with the control group: one or more VACTERL anomalies (P < 0.01), gastro-oesophageal reflux (P < 0.05), recurrent respiratory infections (P < 0.05), and autonomic dysfunction (P < 0.001). The more distant relatives also show an increased incidence of such problems although in this case the data must be viewed with caution. The results confirm that the associated malformations and related medical problems occur significantly more frequently in the relatives of individuals with OA/TOF. These families may prove valuable for linkage analysis in an attempt to determine the genetics of OA/TOF.

Abnormalities, Multiple↗

PubMed1992

[Shy-Drager syndrome].

Shy-Drager syndrome is characterized by severe orthostatic hypotension and other autonomic dysfunctions, cerebellar ataxia, parkinsonism, and upper and lower motor neuron symptoms. The disease starts in the 4th to 6th decades with severe dizziness, blackout or syncopi on standing up, and the duration of the illness is 6-7 years in the majority of the cases. The pathological lesions responsible for the autonomic dysfunctions are in the hypothalamus, locus saeruleus, Edinger-Westphal and other nuclei in the brain stem, intermediolateral column and Onuf's nuclei in the spinal cord. Oppenheimer has postulated a concept of multiple system atrophy to such widely distributed degenerative neurological disorders. However, olivopontocerebellar atrophy of Dejerine and Thomas, striatonigral degeneration and this syndrome can be distinguished clinically as well as pathologically.

Adult↗

PubMed1998

Autonomic function in manganese alloy workers.

The observation of orthostatic hypotension in an index case of manganese toxicity lead to this prospective attempt to evaluate cardiovascular autonomic function and cognitive and emotional neurotoxicity in eight manganese alloy welders and machinists. The subjects consisted of a convenience sample consisting of an index case of manganese dementia, his four co-workers in a "frog shop" for gouging, welding, and grinding repair of high manganese railway track and a convenience sample of three mild steel welders with lesser manganese exposure also referred because of cognitive or autonomic symptoms. Frog shop air manganese samples 9.6-10 years before and 1.2-3.4 years after the diagnosis of the index case exceeded 1.0 mg/m3 in 29% and 0.2 mg/m3 in 62%. Twenty-four-hour electrocardiographic (Holter) monitoring was used to determine the temporal variability of the heartrate (RR' interval) and the rates of change at low frequency (0.04-0.15 Hz) and high frequency (0.15-0.40 Hz). MMPI and MCMI personality assessment and short-term memory, figure copy, controlled oral word association, and symbol digit tests were used. The five frog shop workers had abnormal sympathovagal balance with decreased high frequency variability (increased ln LF/ln HF). Seven of the eight workers had symptoms of autonomic dysfunction and significantly decreased heart rate variability (rMSSD) but these did not distinguish the relative exposure. Mood or affect was disturbed in all with associated changes in short-term memory and attention in four of the subjects. There were no significant correlations with serum or urine manganese. Power spectrum analysis of 24-h ambulatory ECG indicating a decrease in parasympathetic high frequency activation of heart rate variability may provide a sensitive index of central autonomic dysfunction reflecting increased exposure to manganese, although the contribution of exposures to solvents and other metals cannot be excluded. Neurotoxicity due to the gouging, welding, and grinding of mild steel and high manganese alloys (11-25%) merits air manganese and neuropsychologic surveillance including autonomic function by Holter monitoring of cardiovagal activation.

Adult↗

PubMed1996

Prolongation of QTc interval and autonomic nervous dysfunction in diabetic patients.

We have attempted to clarify the relationships of the QTc interval to alpha and beta sympathetic, as well as parasympathetic function tests including spectral analyses of the R-R interval and systolic blood pressure. The QTc intervals were estimated in 76 diabetic patients and 76 age-matched healthy controls whose R-R intervals were comparable to those of the diabetic patients. We also investigated the relationships of the QTc interval to various clinical features of diabetes mellitus and to autonomic function tests. The QTc interval in diabetic patients was significantly greater than that in healthy controls. The QTc intervals were unrelated to diabetic control, retinopathy and nephropathy, but were prolonged in patients with a long duration of diabetes as compared to those with a short disease duration. There were significant correlations between the QTc interval and orthostatic hypotension, R-R variation and the Valsalva maneuver. Significant correlations were observed between the QTc interval and both the low and the high frequency component of spectral analysis of the R-R interval, whereas no correlations were found with spectral analysis of systolic blood pressure. An abnormal QTc interval is an indicator of cardiac sympathetic and parasympathetic nervous dysfunction, but not vasomotor dysfunction.

Blood Glucose↗

PubMed1991

Autonomic nervous dysfunction in essential hypertension.

Borderline hypertension, a condition in which the blood pressure oscillates between normal and high values, is a predictor of future more severe hypertension. Pathophysiologically, borderline hypertension is different from established hypertension. A large proportion of such patients have elevated cardiac output and a normal vascular resistance. In established hypertension, the output is normal and resistance is elevated. The elevation of cardiac output in borderline hypertension is neurogenic; it can be abolished by an autonomic blockade of the heart. In addition to an increased cardiac sympathetic drive, increased sympathetic tone to the kidney, arterioles, and veins has also been found. In parallel with the hypersympathetic state, patients with borderline hypertension also show decreased parasympathetic tone. The enhanced sympathetic tone leads to a decreased cardiac responsiveness, and eventually, the cardiac output returns to the normal range. High blood pressure causes vascular hypertrophy, and hypertrophic vessels are hyperresponsive to vasoconstriction. These secondary changes in the responsiveness of the heart and blood vessels are the basis of transition from a high cardiac output to high-resistance hypertension. These hemodynamic changes are associated with a downregulation of the sympathetic tone. A picture of an apparently nonneurogenic high-resistance hypertension emerges. Nevertheless, when assessed in regard to the enhanced pressor responsiveness, the sympathetic drive in such patients is still excessive. Despite the apparently normal tone, the sympathetic nervous system continues to play an important pathophysiological role in established hypertension. Borderline hypertension is associated with numerous metabolic abnormalities including obesity and insulin resistance. It is tempting to view all these abnormalities as a common expression of the increased sympathetic drive in hypertension. Explanation of the basis of the association of hypertension and metabolic abnormalities promises to bring new insights into the pathophysiology of two common diseases of civilization: hypertension and diabetes mellitus.

Autonomic Nervous System↗

PubMed1976

The 14C-glycocholate test in diabetic diarrhoea.

Twenty-four insulin-dependent diabetics, including seven with diabetic diarrhoea, were studied by means of the 14C-glycocholate (14C-GCA) test and various tests for autonomic dysfunction. The breath component of het test was abnormal in four of the seven patients with diarrhoea and one of the other diabetics. Three patients with diarrhoea and a positive breath test result responded to antibiotics, whereas two with diarrhoea and a negative test result did not. High faecal 14C, suggesting bile acid malabsorption, was found in only one patient with diarrhoea and he had previously failed to respond to cholestyramine. These results suggest that bacterial overgrowth in the small intestine does occur in some but not all patients with diabetic diarrhoea and that the 14C-GCA test can predict the response to antibiotics. All the patients with diabetic diarrhoea had good evidence of autonomic dysfunction.

Anti-Bacterial Agents↗

PubMed1994

Management of tetanus: a review of 18 cases.

Eighteen patients with generalized tetanus seen during an 8-year period in three regional hospitals in Hong Kong are reviewed. Eighty-nine per cent of the patients were intravenous heroin addicts who developed a severe type of generalized tetanus with autonomic dysfunction. Pain or stiffness of the neck or back was the commonest presenting symptom, followed by trismus and dysphagia. Most of the patients developed muscle spasms within 24 h of the onset of presenting symptoms. Early ventilatory support and tracheostomy, together with intensive nursing care, were the mainstay of the treatment. Pulmonary and gastrointestinal complications were common during the course of illness and treatment of autonomic dysfunction was often unsatisfactory. The case fatality rate was 25% among the heroin addicts, but full recovery was the usual outcome for those who survived. The simultaneous administration of intrathecal tetanus immunoglobulin tends to reduce mortality.

Adult↗

PubMed1992

Severe early-onset polyneuropathy in insulin-dependent diabetes mellitus. A clinical and pathological study.

BACKGROUND AND METHODS: The pathophysiologic features of diabetic neuropathy, a common and disabling long-term complication of diabetes mellitus, are poorly understood. We studied five patients, 22 to 34 years old, in whom an uncommonly severe symmetric polyneuropathy developed soon after the onset of insulin-dependent diabetes. Their autonomic function and nerve conduction were studied, and sural-nerve biopsy specimens were examined by light and electron microscopy. Other causes of neuropathy were carefully excluded. RESULTS: Four patients had autonomic dysfunction with postural hypotension, fainting, diarrhea, and Argyll Robertson pupils and peripheral neuropathy with loss of sensation of pain and changes in temperature that followed a pattern suggestive of a length-dependent degeneration of nerve fibers. In contrast, the fifth patient had muscle weakness and atrophy of limb extremities, with "glove and stocking" sensory loss, but little autonomic dysfunction. In the biopsy specimens of sural nerves, the mean (+/- SD) density of myelinated fibers was reduced to 20 +/- 14 percent of that measured in five control patients, and the density of unmyelinated fibers was reduced to 6 +/- 4 percent of that in the controls. Regenerating fibers accounted for 38 +/- 11 percent of the myelinated axons. Abnormalities of the myelin sheath affected 33 +/- 21 percent of the isolated fibers, and axonal degeneration 11 +/- 8 percent. Dying-back fibers, a characteristic of the centripetal degeneration of peripheral axons, were also identified. The dying-back process progressed at the rate of a few hundred micrometers per day. CONCLUSIONS: Early-onset symptomatic polyneuropathy in patients with diabetes mellitus is characterized by the loss of both myelinated and unmyelinated nerve fibers. Spontaneous axonal regeneration is remarkably frequent, even when neuropathy is severe.

Action Potentials↗

PubMed2000

Does severe nutcracker phenomenon cause pediatric chronic fatigue?

BACKGROUND: In the past five years we experienced 9 fatigued disabled children who were intermittently or persistently absent from school. PATIENTS: They had been suspected to be burdened with psychosomatic disorders, having orthostatic hypotension, postural tachycardia, or other autonomic dysfunction symptoms. RESULTS: Investigating the cause of moderate orthostatic proteinuria in some of them, we found by chance severe typical nutcracker phenomenon (NC), which was present in all 9 children complaining of chronic fatigue. CONCLUSION: Their symptoms filled the criteria of chronic fatigue syndrome or idiopathic chronic fatigue (CFS/CF). An association between severe NC and autonomic dysfunction symptoms in children with CFS/CF has been presented.

Adolescent↗

PubMed1995

Identifying delirious states and autonomic cardiovascular dysfunction associated with amitriptyline treatment by standardized analysis of heart rate.

Ninety-one patients treated with amitriptyline and 60 normal control subjects underwent a standardized heart rate analysis in supine posture. Tests included the determination of time- and frequency-derived measurements of heart rate variability. The patients differed significantly from the control subjects in all parameters investigated. Two-thirds of the patients treated with a tricyclic antidepressant (TCA) reached values that met the criteria for cardiovascular autonomic neuropathy. Our results provide evidence that heart rate analysis might be a more sensitive tool in diagnosing amitriptyline-associated anticholinergic delirium than determination of TCA plasma levels. Further research is needed to elucidate what implications the TCA-associated alteration of autonomic cardiovascular function might have for patients.

Adult↗

PubMed1997

The value of sympathetic skin response recordings in the assessment of the vesicourethral autonomic nervous dysfunction in spinal cord injured patients.

PURPOSE: We assessed the degree of sparing of the descending sympathetic spinal tract and correlated these findings with bladder neck function in spinal cord injured patients. MATERIALS AND METHODS: Sympathetic skin responses of the right hand and foot were recorded and compared to the urodynamic findings in 27 spinal cord injured patients. RESULTS: All tetraplegic and paraplegic patients with a lesion above the T6 level who presented with bladder neck dyssynergia associated with autonomic hyperreflexia had abnormal sympathetic skin responses in the right hand and foot. All patients with a lesion below the T6 and above the T12 levels with an abnormal sympathetic skin response in the right foot also had bladder neck dyssynergia. CONCLUSIONS: Evidence is presented that the integrity of the descending sympathetic spinal tract is necessary for a synergic function of the vesicourethral complex and that sympathetic skin responses are of value in the diagnosis of bladder neck dyssynergia. For lesions below the T12 level other investigative methods to exclude bladder neck dyssynergia are necessary.

Autonomic Nervous System↗

PubMed1997

The postural orthostatic tachycardia syndrome: a neurocardiogenic variant identified during head-up tilt table testing.

Head upright tilt table testing has emerged as an accepted modality for identifying an individual's predisposition to episodes of autonomically mediated hypotension and bradycardia that are sufficiently profound so that transient loss of consciousness ensues (neurocardiogenic syncope). However it has also become apparent that less dramatic falls in blood pressure, while not sufficient to cause full syncope, may produce symptoms such as near syncope, vertigo, dizziness, and TIA-like episodes. We have identified a subgroup of individuals with a mild form of autonomic dysfunction with symptoms of postural tachycardia and lightheadedness, disabling fatigue, exercise intolerance, dizziness, and near syncope. During baseline tilt table testing these patients demonstrated a heart rate increase of > or = 30 beats/min (or a maximum heart rate of 120 beats/min) within the first 10 minutes upright (unassociated with profound hypotension), which reproduced their symptom complex. In addition these patients exhibit an exaggerated response to isoproterenol infusions. Similar observations have been made by others who have dubbed this entity the Postural Orthostatic Tachycardia Syndrome (POTS). We conclude that POTS represents a mild (and potentially treatable) from of autonomic dysfunction that can be readily diagnosed during head upright tilt table testing.

Adult↗

PubMed2006

MR characteristics and neuropathology in adult-onset autosomal dominant leukodystrophy with autonomic symptoms.

BACKGROUND AND PURPOSE: Three families with adult-onset autosomal dominant leukodystrophy (ADLD) presenting autonomic dysfunction as the first symptom are reported. We describe detailed MR appearances of the brain in 2 new families and neuropathology in 2 patients and compare the findings with those in other adult-onset leukodystrophies. METHODS: Twenty subjects (12 women and 8 men; age range, 29-70 years) from 2 unrelated families with ADLD were examined with MR. Six subjects were asymptomatic. Fourteen had autonomic dysfunction. Eleven of them also had pyramidal signs and ataxia. The brains of 2 autopsied patients were examined histopathologically. RESULTS: Two subjects manifested no neurologic symptoms, signs, or MR pathology. Eighteen subjects displayed radiologic abnormalities ranging from subtle T2 high-signal-intensity changes in the upper corticospinal tract to extensive confluent white matter changes, predominantly in a frontoparietal distribution, along the corticospinal tracts down to the medulla oblongata and in the upper and middle cerebellar peduncles. Periventricular white matter was spared or less affected than the adjacent white matter. Histopathology revealed marked loss of cerebral and cerebellar myelin without signs of inflammation. Oligodendrocytes were relatively spared, the number of axons not markedly decreased, and reactive gliosis was modest. The number of Purkinje cells in the cerebellum was reduced. CONCLUSIONS: Two families with adult-onset ADLD with the disease entity originally reported by Eldridge et al. (N Engl J Med 1984;311:948-53) were described. We propose naming the disease "adult-onset ADLD with autonomic symptoms." The characteristic radiologic findings, combined with the clinical symptoms and mode of inheritance, enable the diagnosis.

Adult↗

PubMed2001

Assessment of coronary morphology and flow in a patient with Guillain-Barré syndrome and ST-segment elevation.

Patients with Guillain-Barré syndrome often have cardiac disturbances as a manifestation of autonomic dysfunction. Such abnormalities consist of arrhythmias and disturbances of heart rate and blood pressure. We report a case of a patient with Guillain-Barré syndrome who developed ST-segment elevation in the inferolateral leads, suggestive of an acute coronary syndrome. Cardiac catheterization revealed angiographically normal coronary arteries. Intracoronary ultrasound was also normal. Intracoronary Doppler flow measurements revealed an elevated baseline coronary flow velocity of up to 41 cm/s and decreased coronary flow reserve, particularly in the left circumflex artery. Myopericarditis as cause of the electrocardiographic changes could be ruled out by echocardiography and endomyocardial biopsy. We postulate that the intracoronary Doppler findings are caused by autonomic dysfunction with decrease of coronary resistance and redistribution of the transmural myocardial blood flow.

Autonomic Nervous System Diseases↗

PubMed1998

Diastolic ventricular interaction in chronic heart failure: relation to heart rate variability and neurohumoral status.

It is likely that abnormal baroreflex control mechanisms are at least partially responsible for autonomic dysfunction in chronic heart failure. We recently demonstrated that diastolic ventricular interaction is associated with impaired baroreflex control of vascular resistance in heart failure. We reasoned that by constraining left ventricular filling, such interaction would decrease baroreflex activity and, thereby, increase sympathetic and decrease parasympathetic outflow. We hypothesized, therefore, that diastolic ventricular interaction in chronic heart failure patients would be associated with autonomic dysfunction. We used radionuclide ventriculography to measure changes in left and right ventricular end-diastolic volumes during acute volume unloading achieved by -30 mm Hg lower-body negative pressure in 30 patients with chronic heart failure. An increase in left ventricular volume in association with a reduction in right ventricular volume indicates diastolic ventricular interaction (a larger increase indicating a greater degree of interaction). We also measured heart rate variability (n = 23) and resting venous plasma norepinephrine (n = 24), epinephrine (n = 24), and atrial natriuretic peptide (ANP) (n = 14). During lower-body negative pressure, while right ventricular volume decreased in all patients (P < 0.001), left ventricular end-diastolic volume increased (from 152 +/- 25 to 157 +/- 36 ml/m2, P = 0.01). The change in left ventricular volume was positively correlated with resting plasma norepinephrine (P < 0.01) and ANP (P < 0.005), and negatively correlated with the standard deviation of normal to normal R-R intervals (P < 0.005), the root-mean-square of differences between successive normal to normal R-R intervals (P < 0.05), total power (P < 0.01), low-frequency power (P < 0.01), and high-frequency power (P < 0.05). Diastolic ventricular interaction in patients with chronic heart failure is associated with sympathetic nervous system activation evidenced by increased plasma norepinephrine and reduced heart rate variability.

Atrial Natriuretic Factor↗

PubMed2002

Magnesium as first line therapy in the management of tetanus: a prospective study of 40 patients.

A prospective observational study was conducted to examine the efficacy and safety of magnesium sulphate for control of spasms and autonomic dysfunction in 40 patients with tetanus. Magnesium was infused intravenously, aiming to control spasms despite suppression of patellar reflex or respiratory insufficiency. Spasms were controlled in 38 of the 40 patients within a serum Mg(2+) range of 2-4 mmol.l(-1) with only two patients needing additional neuromuscular blocking drugs. Seventeen of 24 patients (< 60 years) and six of 16 patients (> or = 60 years) did not require ventilatory support. Thirty-six patients were conscious and co-operative throughout their management. Sympathetic over-activity was controlled without supplementary sedation. Overall mortality was 12%; all five deaths were in patients > or = 60 years and no deaths were due to autonomic dysfunction. We recommend magnesium as possible first line therapy in the routine management of tetanus.

Adult↗

PubMed1989

Physiological and clinical correlates of cardiorespiratory reflexes in diabetes mellitus.

Cardiorespiratory reflexes (CRRs) were studied by measuring heart-rate (HR) variation during 6 breaths/min respiration (delta R6) and Valsalva maneuver (VR) in 145 healthy and 417 type I (insulin-dependent) diabetic subjects. HR variation with breathing at 12 breaths/min and ventilatory response to hypercapnia/hypoxia were measured in fewer subjects. CRR results were compared with symptoms of autonomic dysfunction, the neurological examination, nerve conduction studies, and quantitative sweat testing. The objective was to compare the sensitivity of various methods of characterizing diabetic patients and to use this information when staging patients for clinical therapeutic trials. CRR responses were age dependent in both populations. Either delta R6 or VR was abnormal in 74% of diabetic patients, delta R6 being more sensitive. CRRs correlated well with the presence of symptoms of autonomic dysfunction, abnormalities on the neurological examination, results of nerve conduction studies, and sweating activity in the feet of the same patients. However, both CRRs and sweating were abnormal in a high proportion of patients without any clinical manifestations of neuropathy. The ventilatory reflex response to moderate hypercapnia/hypoxia was also measured. It was normal in most of the diabetic patients tested, including many with severe reduction of CRRs. We conclude from the combined results of CRR, ventilatory response, and other studies that the causative factors for abnormal CRR may not be confined to the vagus nerves, and that in most instances, the depressed CRR may be due to a decrease in the efficacy of sensorimotor nerve conduction around the reflex arc.

Adolescent↗