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At least 19 recordsLinked to original sources
PubMed1993

The treatment of autonomic dysfunction.

Autonomic dysfunction is responsible for much of the morbidity associated with frequently encountered neurological disorders, such as Parkinson's disease, multiple sclerosis, cerebrovascular disease, and peripheral neuropathies, as well as with the rarer primary autonomic nervous system degenerations. We review the treatment of those aspects of autonomic dysfunction that often present to the neurologist, including orthostatic hypotension, urinary incontinence and retention, and bowel dysmotility syndromes. Pathophysiology is discussed in each instance as it relates to a rational approach to therapy. For management of orthostatic hypotension, we review the use of mineralocorticoids, direct and indirect sympathomimetic agents, other pressors, dopamine-blocking agents, vasopressin receptor agonists, and others. Treatment of urinary incontinence and retention is addressed, with attention to drugs that modulate bladder contractility and bladder outlet resistance. Therapies for bowel dysmotility syndromes (such as gastroparesis, diarrhea, and fecal incontinence) are described, including bulk agents, laxatives, prokinetic agents, and antidiarrheal drugs.

Autonomic Nervous System Diseases↗
PubMed2005

Serotonin transporter abnormality in the dorsal motor nucleus of the vagus in Rett syndrome: potential implications for clinical autonomic dysfunction.

Autonomic dysfunction is prevalent in girls with Rett syndrome, an X-chromosome-linked disorder of mental retardation resulting from mutations in the gene encoding methyl-CpG-binding protein 2 (MeCP2). This gene plays a role in regulating neuronal activity-dependent gene expression, including brain-derived neurotrophic factor (BDNF), which is a potent serotonergic (5-HT) neuronal growth factor. We analyzed selected parameters of the 5-HT system of the medulla in autopsied patients with Rett syndrome because of the role of BDNF in 5-HT cell development and because 5-HT plays a key role in modulating autonomic control. 5-HT neurons were identified by immunostaining for tryptophan hydroxylase, the biosynthetic enzyme for 5-HT. We quantitated the number of 5-HT cells in the medulla at 2 standardized levels in 11 Rett and 7 control cases. There was no significant difference in 5-HT cell number between the groups. We analyzed binding to the serotonin transporter (SERT) using the radioligand [(125)I]-RTI-55 with tissue autoradiography in 7 Rett and 5 controls in 9 cardiorespiratory-related nuclei. In the dorsal motor nucleus of the vagus (DMX) (preganglionic parasympathetic outflow), SERT binding for the control cases decreased significantly over time (p = 0.049) but did not change in the Rett cases (p = 0.513). Adjusting for age, binding between the Rett and control cases differed significantly in this nucleus (p = 0.022). There was a marginally significant age versus diagnosis interaction (p = 0.06). Thus, altered 5-HT innervation and/or uptake in the DMX may contribute to abnormal 5-HT modulation of this major autonomic nucleus in patients with Rett syndrome. These data suggest hypotheses concerning 5-HT modulation of vagal function for testing in MeCP2 knockout mice to understand mechanisms underlying autonomic dysfunction in patients with Rett syndrome.

Adolescent↗
PubMed2007

Rapid gastric emptying is more common than gastroparesis in patients with autonomic dysfunction.

OBJECTIVES: Autonomic dysfunction is associated with a wide variety of gastrointestinal symptoms. It is unclear how many patients with autonomic dysfunction have slow or rapid gastric emptying. The aim of this study was to determine the prevalence of rapid and delayed solid phase gastric emptying in patients with autonomic dysfunction referred for evaluation of gastrointestinal symptoms and the association of emptying rate with clinical symptoms. METHODS: Retrospective review of all patients with autonomic dysfunction who had a gastric emptying test from January, 1996 to March, 2005. Demographic data, clinical symptoms, composite autonomic scoring scale (CASS) score, and gastric emptying parameters were analyzed. RESULTS: Sixty-one subjects (women 49, age 42 [16-74] yr) with autonomic dysfunction were reviewed. Patients had mild-to-moderate (mean CASS score 3) autonomic dysfunction. Twenty-seven, 17, and 17 patients had rapid, normal, and delayed gastric emptying t(1/2), respectively. In addition, 10 patients had initially rapid emptying in phase 1, with subsequent slowing in phase 2 to produce an overall normal or delayed t(1/2). There was no difference in demographic data or CASS score among the three groups. More patients with initial or overall rapid emptying had diarrhea (70%) compared to patients with normal (33%) or delayed (33%) emptying (P= 0.018). CONCLUSIONS: Unexpectedly, more patients with autonomic dysfunction have rapid rather than delayed gastric emptying. The presence of diarrhea in patients with autonomic symptoms should prompt consideration for the presence of rapid gastric emptying. Conversely, the finding of rapid gastric emptying in patients with gastrointestinal symptoms should prompt consideration for the presence of underlying autonomic dysfunction.

Adolescent↗
PubMed1998

Evidence of defective cardiovascular regulation in insulin-dependent diabetic patients without clinical autonomic dysfunction.

(1) Autonomic dysfunction is a well recognised complication of diabetes mellitus and early detection may allow therapeutic manoeuvres to reduce the associated mortality and morbidity. We sought to identify early cardiovascular autonomic neuropathy using spectral analysis of heart rate and systolic blood pressure variability. (2) Thirty patients with Type 1 (insulin-dependent) diabetes mellitus (DM) and 30 matched control subjects were studied. In addition to standard tests of autonomic function, heart rate and systolic blood pressure variability were assessed using power spectral analysis. From the frequency domain analysis of systolic blood pressure and R-R interval, the overall gain of baroreflex mechanisms was assessed. (3) Standard tests of autonomic function were normal in both groups. Total spectral power of R-R interval was reduced in the Type 1 DM group for low-frequency (473 +/- 63 vs. 747 +/- 78 ms2, mean +/- S.E.M., P = 0.002) and high-frequency bands (125 +/- 13 vs. 459+/-90 ms2, P < 0.0001). Systolic blood pressure low-frequency power was increased in the diabetic group (9.3 +/- 1.2 vs. 6.6+/-0.7 mmHg2, P < 0.05). The low frequency/high frequency ratio for heart rate variability was significantly higher in the Type 1 DM patients (4.6+/-0.5 vs. 2.9+/-0.5, P = 0.002), implying a relative sympathetic predominance. When absolute powers were expressed in normalised units, these differences persisted. There were significant reductions in baroreceptor-cardiac reflex sensitivity in Type 1 DM patients compared to controls while supine (9.7+/-0.7 vs. 18.5 +/- 1.7 ms/mmHg, P < 0.0001) and standing (2.9+/-0.9 vs. 7.18+/-1.9 ms/mmHg, P < 0.001). (4) Spectral analysis of cardiovascular variability detects autonomic dysfunction more frequently in Type 1 DM patients than conventional tests, and is suggestive of an abnormality of parasympathetic function. The abnormality of baroreceptor-cardiac reflex sensitivity could be explained by this impairment of parasympathetic function and this may predispose to the development of hypertension and increase the risk of sudden cardiac death. Using spectral analysis methods may allow detection of early diabetic cardiac autonomic neuropathy and allow therapeutic intervention to slow the progression.

Adult↗
PubMed2005

Trigeminal cephalgias and facial pain syndromes associated with autonomic dysfunction.

Trigeminal autonomic cephalgias (TACs) include a spectrum of primary headache syndromes associated with cranial autonomic dysfunction. Other types of headache and facial pain syndromes can be associated with marked localized facial or ear autonomic changes. We report on a group of patients suffering from episodic migraine with cranial autonomic features, patients with different presentations of the 'red ear syndrome' (RES), cluster headache with prominent lower facial involvement and crossover cases. In our experience crossover between TACs and migraine, RES and cluster headache is not uncommon. We propose that all these conditions belong to the same group and a unifying causative mechanism is proposed.

Adult↗
PubMed1987

Autonomic dysfunction.

The autonomic nervous system is closely involved in the majority of bodily functions. Different disease processes and a variety of drugs can result in autonomic dysfunction, some with minor but others with major consequences. This review considers factors which relate to the recognition, investigation and principles of management of such disorders.

Autonomic Nervous System Diseases↗
PubMed1996

Autonomic dysfunction after catheter ablation.

Autonomic dysfunction may occur as a consequence of radiofrequency (RF) catheter ablation of a variety of supraventricular tachycardias. Effects suggestive of autonomic dysfunction that may be seen acutely during the ablation procedure include sudden profound slowing of the sinus rate or transient AV block. These abnormalities may occur during application of RF current, typically along the tricuspid or mitral annulus, at sites distant from both the sinus and AV nodes; they resolve quickly when RF current delivery is terminated. The most common long-term indication of autonomic dysfunction after ablation is inappropriate sinus tachycardia. This complication, rarely a lasting significant clinical problem, is seen after AV node modification and after ablation of accessory pathways. It usually resolves within several months. The mechanism appears to be loss of parasympathetic influence on the sinus node. Autonomic dysfunction after ablation of ventricular tachycardia has not yet been described, but could occur as newer catheter technologies capable of producing larger lesions are perfected.

Autonomic Nervous System↗
PubMed2003

Short-term postural reflexes in diabetic patients with autonomic dysfunction.

AIMS/HYPOTHESIS: Assessment of autonomic dysfunction provides prognostic data in diabetic patients. Clinical tests are limited to heart rate variability and blood pressure measurements. We investigated whether a detailed analysis of postural reflexes of patients during short-term head-up tilting and standing erect (both for 3 min) would provide useful clinical data. METHODS: We studied 14 control subjects and 56 patients with Type 2 diabetes, stratified into four matched groups: uncomplicated, micro-albuminuria, macro-albuminuria and autonomic neuropathy, who were tilted 55 degrees three times and were standing erect. Non-invasive finger plethysmography blood pressure measurements, using the Portapres, and impedance cardiac output measurements, using the RheoCardioMonitor, were continuously recorded throughout the study. Wavelets of the response to tilting and standing were drawn. The wavelets for the three tilts were combined to improve definition. The degree of autonomic dysfunction was quantified by calculating the baroreflex sensitivity index from the heart-rate and blood-pressure variability data. RESULTS: Baroreflex sensitivity data confirmed that autonomic dysfunction increased as diabetic complications worsened (p<0.001). Both standing and tilting resulted in an initial decrease in blood pressure followed by recovery, an increase in heart rate which was more pronounced with standing and a decrease in stroke volume. Characteristic and incremental changes in these responses were noted as the complications of diabetes worsened (p<0.001). These were a greater decrease in blood pressure with loss of the recovery phase, a reduced heart-rate response and paradoxically an increase in stroke volume. CONCLUSION/INTERPRETATION: Non-invasive blood-pressure and stroke-volume measurements recorded during short-term tilting and standing promises to be useful in assessing diabetic autonomic dysfunction.

Adult↗
PubMed1997

Time and frequency domain estimates of spontaneous baroreflex sensitivity provide early detection of autonomic dysfunction in diabetes mellitus.

Diabetic autonomic dysfunction is associated with a high risk of mortality which makes its early identification clinically important. The aim of our study was to compare the detection of autonomic dysfunction provided by classical laboratory autonomic function tests with that obtained through computer assessment of the spontaneous sensitivity of the baroreceptor-heart rate reflex (BRS) by time domain and frequency domain techniques. In 20 normotensive diabetic patients (mean age +/- SD 41.9 +/- 8.1 years) with no evidence of autonomic dysfunction on laboratory autonomic testing (D0) blood pressure (BP) and ECG were continuously monitored over 15 min in the supine position. BRS was assessed as the slope of the regression line between spontaneous increases or reductions in systolic BP and linearly related lengthening or shortening in RR interval over sequences of at least 4 consecutive beats (sequence method), or as the squared ratio between RR interval and systolic BP spectral powers around 0.1 Hz. We compared the results with those of 32 age-matched normotensive diabetic patients with abnormal autonomic function tests (D1) and with those of 24 healthy age-matched control subjects with normal autonomic function tests (C). Compared to C, BRS was markedly less in D1 when assessed by both the slope of the two types of sequences (data pooled) and by the spectral method (-71.3% and -60.2% respectively, both p < 0.01). However, BRS was consistently although somewhat less markedly reduced in D0, the reduction being clearly evident for all the estimates (-57.0% and -43.5%, both p < 0.01). The effects were more evident than those obtained by the simple quantification of the RR interval variability. These data suggest that time and frequency domain estimates of spontaneous BRS allow earlier detection of diabetic autonomic dysfunction than classical laboratory autonomic tests. The estimates can be obtained by short non-invasive recording of the BP and RR interval signals in the supine patient, i.e. under conditions suitable for routine outpatient evaluation.

Adult↗
PubMed1982

Autonomic dysfunction in epilepsy: characterization of autonomic cardiac neural discharge associated with pentylenetetrazol-induced epileptogenic activity.

The purpose of this study was to determine if epileptogenic activity is associated with changes in autonomic cardiac neural discharge and the development of arrhythmias. Nine cats, anesthetized with alpha-chloralose, received pentylenetetrazol (PTZ), 10, 20, 50, 100, 200, and 2,000 mg/kg, intravenously at 10 min intervals. The following were monitored: neural discharge from 1 to 3 postganglionic cardiac sympathetic branches (8 cats, 17 nerves) and the vagus (7 cats, 8 nerves); the electrocorticogram; blood pressure; heart rate; and lead II electrocardiogram. Autonomic dysfunction was manifested by: the observation that autonomic cardiac nerves did not always respond in a predictable manner to changes in blood pressure; the development of a marked increase in variability in mean autonomic cardiac sympathetic and parasympathetic neural discharge; and the fact that the very large increase in the variability of the discharge rate of parasympathetic nerves was seen after PTZ, 50 mg/kg, but did not develop until 100 mg/kg in sympathetic neural discharge. This autonomic imbalance was associated with both interictal and ictal epileptogenic activity. Almost invariably, interictal discharge occurred after PTZ, 10 mg/kg; with higher doses, the duration of ictal activity increased although interictal discharges were present. The altered cardiac autonomic neural discharge was associated with minimal epileptogenic activity in the form of interictal discharges and cardiac arrhythmias which may contribute to sudden unexplained death of the epileptic.

Animals↗
PubMed1983

Autonomic dysfunction in palmar hyperhidrosis.

The autonomic (including sudomotor, baroreceptor, and vasomotor) functions were assessed in 3 groups of individuals, comprising normal, hyperhidrotic, and denervated subjects. The normal group had no palmar hyperhidrosis, with intact T2-3 ganglia, the hyperhidrotic group had palmar hyperhidrosis with intact T2-3 ganglia, and the denervated group had palmar hyperhidrosis treated with T2-3 ganglionectomy. Compared with both the normal and hyperhidrotic subjects, the denervated subjects had a much smaller sweating response of both the forehead, the upper chest region and the upper extremities, and a much greater sweating response of both the lateral lumbar and ventral thigh regions in response to body exercise. In addition, cardiovascular responses to either the Valsalva manoeuver, face immersion, or finger immersion were evaluated in these groups of subjects. When compared with those of either the normal or the denervated subjects, the hyperhidrotic subjects had less reflex bradycardia in response to either Valsalva manoeuver or face immersion. In contrast, when compared with those of either the normal or the denervated subjects, the hyperhidrotic subjects had a higher degree of cutaneous vasoconstriction in response to finger (or cold) immersion. The data indicate that the sympathetic fibers passing through the T2-3 ganglia play an important role in the elaboration or modulation of autonomic function elsewhere. Probably, the hyperhidrotic subjects have an over-functioning of the sympathetic nervous fibers which pass through the T2-3 ganglia, which leads to autonomic dysfunction. The autonomic dysfunctions observed in the hyperhidrotic subjects could be eliminated after the interruption of the excessive sympathetic activities passing through the T2-3 ganglia level. A preliminary report of this work was delivered at the 15th Congress of the Pan-Pacific Surgical Association, January 12-18, 1980 and the 12th World Congress of Neurology, September 20-25, 1981.

Adult↗
PubMed1993

Autonomic dysfunction in peripheral nerve disease.

Autonomic dysfunction is a common complication of peripheral neuropathies. Frequently, it is of little clinical importance, but in some conditions there may be profound disturbance of autonomic function, including orthostatic hypotension, impairment of heart rate and bladder control, and impotence. Some autonomic neuropathies are of acute onset, but most are chronic and of gradual onset. Diseases that primarily affect small fibers in peripheral nerves or cause acute demyelination of small myelinated fibers are those most likely to cause autonomic dysfunction. Conditions that cause severe autonomic dysfunction include acute dysautonomia, familial and primary amyloidosis, Guillain-Barré syndrome, diabetes, porphyria, Chagas' disease, and some hereditary sensory and autonomic neuropathies. Management consists of treating the underlying cause and symptomatic therapy.

Autonomic Nervous System↗
PubMed2004

Aortic stenosis and autonomic dysfunction: co-conspirators in syncope.

Autonomic dysfunction and aortic stenosis share several clinical characteristics, including, in severe cases, syncope. Both illnesses tend to manifest later in life, and most cases are idiopathic in origin. In a short period of 4 weeks, the authors noted that three patients out of 36 referrals for autonomic dysfunction also had histories of aortic valve replacement due to stenosis. In each case, similar presenting symptoms of fatigue, light-headedness, and syncope were attributed to aortic stenosis without mention of autonomic failure as a possible contributor. The authors propose that patients for whom symptoms of aortic valve stenosis are not relieved by surgical intervention may have concomitant autonomic dysfunction contributing significantly to their symptoms. Furthermore, the two conditions may comprise a dangerous combination, aortic stenosis causing physical obstruction of ventricular outflow, and autonomic dysfunction causing decreased venous return and insufficient cardiac filling. It may be beneficial for patients with aortic stenosis who present with syncope to be considered for possible autonomic dysfunction to address both potential pathophysiologies contributing to the syncope.

Aged↗
PubMed2005

Heart rate variability and autonomic dysfunction in SSPE.

Autonomic nervous system involvement in subacute sclerosing panencephalitis was studied in 29 patients by analysis of heart rate variability and compared with a control group which consisted of 20 age- and sex-matched healthy subjects. Holter recordings for 24 hours were obtained, and all recordings were analyzed using time-domain parameters. The patients with subacute sclerosing panencephalitis were found to have significantly lower values of standard deviation of all normal sinus intervals and triangular index when compared with the control group. Of 23 patients who had regular follow-up, 15 died in a period ranging from 1 to 13 months. Twelve of these patients had heart rate variability parameters lower than normal, but no significant difference was observed between the heart rate variability values of patients who survived or died. Also, there was no association between brain magnetic resonance imaging findings and heart rate variability indices. It is concluded that patients with subacute sclerosing panencephalitis have autonomic dysfunction; and this was thought to be mainly due to central involvement.

Autonomic Nervous System↗
PubMed1989

[Chronic recurrent-progressive polyradiculoneuritis--Characteristics of autonomic dysfunction].

We examined autonomic dysfunctions in 12 patients with chronic-recurrent-progressive polyradiculoneuritis (C-R-P-PRN), consisting of 6 males and 6 females with the clinical duration of 8 months to 16 years, and with the age ranged 13 and 71 years. Sixteen healthy volunteers, aged 20 to 70 years, were also examined. Thermal sweat rate was recorded on the palm, forearm, upper arm, anterior chest, lateral thigh and lateral aspect of lower leg using a ventilated capsular method in a climatic chamber at 40 degrees C and 40% of relative humidity. After steady state was attained, thermal sweat rate was measured. Local sweating induced by intradermal injection of pilocarpine and nicotine (a concentration of 10(-4), 0.1 ml) was also measured on the forearm and lower lateral leg at 23 degrees C of room temperature and 40% of relative humidity. Other autonomic functions including skin temperature at rest and after cold loading (15 degrees C, 6 minutes), variation in the R-R interval (CV%), pupillary function (response to 1.25% epinephrine, 2(-5) pilocarpine, 5% tyramine), orthostatic hypotension and bladder dysfunction were also monitored. A decrease in sweat rate and recovery rate of skin temperature was seen more frequently in patients with C-R-P-PRN than normal volunteers. Abnormality in the thermal sweat rate and local sweat response against nicotine and pilocarpine was present more frequently in the forearm and distal leg as compared with the chest and thigh.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1999

Muscle sympathetic activity in cerebellar degeneration with or without mild autonomic dysfunction.

To evaluate autonomic function in sporadic cases of cerebellar degeneration (CD) with or without mild autonomic dysfunction, we measured muscle sympathetic nerve activity (MSNA) by microneurography in 12 CD patients and 18 healthy subjects. The burst incidence and mean amplitude of MSNA at rest was significantly decreased in CD patients (P < 0.01). There were no significant differences between patients with CD and controls in heart rate or blood pressure at rest. During head-up tilting, increases in blood pressure and mean amplitude of MSNA were significantly lower in CD patients. In conclusion, a decrease in muscle sympathetic outflow can be observed even in CD patients who are clinically excluded from multiple system atrophy (MSA). It might be difficult to distinguish other CD syndromes from MSA in recordings of muscle sympathetic activity.

Adult↗