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PubMed2006

[Autonomic dysfunction and orthostatic intolerance. Cardiologic diagnosis and treatment options].

Various disorders of autonomic circulatory control are characterized by symptoms of orthostatic intolerance and syncope. Since the introduction of tilt-table testing as a cardiac diagnostic tool by Kenny et al., definition of these disorders has changed significantly. This review summarizes the current knowledge of diagnosis and treatment of the syndromes of orthostatic intolerance.

Autonomic Nervous System↗
PubMed1998

Intradialytic hypotension in relation to pre-existent autonomic dysfunction in hemodialysis patients.

After having monitored haemodynamics during haemodialysis, we examined autonomic nervous function in rest prior to a next dialysis session in 28 patients on chronic intermittent haemodialysis. The aim was to compare intradialytically hypotensive with stable patients to assess whether blood pressure regulating mechanisms were related to basal autonomic function, assessed as heart rate variability (HRV) tested by means of the deep breathing test, the lying-to-standing test, and the Valsalva maneuver. Impedance cardiography was used to determine stroke volume and cardiac output during dialysis. In addition, blood pressure was registered automatically and systemic vascular resistance calculated. Blood volume variation was monitored by an on-line optical device. Intradialytic hypotension was observed in 10 patients (36%). Systemic vascular resistance in hypotensive patients decreased considerably (-14.0+/-5.9%), while it increased in stable patients (+9.9+/-4.6%, p = 0.004). Heart rate rose significantly in hypotensive patients (11.5+/-3.8%) in comparison to stable patients (-0.2+/-2.8%, p = 0.02). However, no significant differences in autonomic function were observed between hypotensive and stable patients. Although both groups showed impaired autonomic function, no significant correlation between changes in haemodynamics during dialysis and autonomic function at rest could be ascertained. In conclusions, hypotension during haemodialysis is not related to a patient's autonomic function at rest. This suggests that structural neuronal differences are not responsible for the severe decrease in systemic vascular resistance in intradialytic hypotension.

Autonomic Nervous System↗
PubMed1996

Abnormal heart rate variability reflecting autonomic dysfunction in brainstem infarction.

OBJECTIVES: Brainstem infarctions frequently cause disturbances of cardiovascular and other autonomic functions, but the pathophysiologic mechanisms of these prognostically unfavourable complications are not well-known. MATERIAL & METHODS: In order to evaluate the effects of ischemic brainstem infarction on autonomic cardiac regulation, we analyzed the power spectrum of heart rate variability in 15 consecutive patients with brainstem infarction and in 15 age- and sex-matched healthy control subjects. The components of the power spectrum which reflect quantitatively both sympathetic and parasympathetic cardiovascular regulatory functions were measured from 24-hour electrocardiogram in the acute phase and at 1 month and 6 months after the infarction. RESULTS: All the measured components of heart rate variability, i.e., total power (p < 0.01), very-low-frequency power (p < 0.001), low-frequency power (p < 0.01), and high-frequency power (p < 0.05), were significantly lower in the patients with medullary brainstem infarction than in the control subjects in the acute phase of the infarction. By 6 months, these abnormalities had been reversed. On the contrary, heart rate variability in pontine brainstem infarct patients did not differ significantly from that in the control subjects. CONCLUSIONS: These results suggest that brainstem infarction located in the medulla oblongata causes cardiovascular autonomic dysregulation manifesting as impaired heart rate variability. Medullary brainstem infarction seems to cause both sympathetic and parasympathetic dysfunction, which may contribute to the occurrence of cardiac complications in stroke.

Adult↗
PubMed1996

Left ventricular performance and autonomic dysfunction in patients with long-term insulin-dependent diabetes mellitus.

Cardiac autonomic neuropathy (CAN) is a very frequent complication of insulin-dependent mellitus type 1, affecting the sympathetic or parasympathetic sections or both. The different impairment in the two sections might modify left ventricular function early. To evaluate this relationship, we studied 61 patients (mean age 39.6 +/- 7 years) with type 1 diabetes for more than 10 years, without coronary artery disease (CAD); negative ergometric stress test) and without other pathologies that could interfere with ventricular function. All patients underwent MONO-, 2-dimensional and Doppler echocardiographic examination and radionuclide angiography with 99Tc (RNA). According to the outcome of the Ewing tests, patients were divided into two groups: group A with two or more tests altered (26 patients with CAN) and group B with one or no tests altered (35 patients without CAN). No significant differences between the two groups were found in the systolic function parameters with either technique. In contrast, a pattern of abnormal relaxation was found for the diastolic function parameters: in group A a decrease in E-wave velocity and its time-velocity integral and an increase in A-wave and its time-velocity integral were detected with echocardiography. Moreover, RNA showed a reduced peak filling rate and an increased isovolumic relaxation time. When compared with normal values, an abnormal diastolic filling, defined as two independent echocardiography plus one RNA variable impairment, was found in 15 patients (57.6%) in group A and in only 4 patients (11.4%) in group B (P < 0.001). Our findings suggest an early involvement of diastolic function in patients with CAN.

Adolescent↗
PubMed2001

Characterisation of breathing and associated central autonomic dysfunction in the Rett disorder.

AIM: To investigate breathing rhythm and brain stem autonomic control in patients with Rett disorder. SETTING: Two university teaching hospitals in the United Kingdom and the Rett Centre, Sweden. PATIENTS: 56 female patients with Rett disorder, aged 2-35 years; 11 controls aged 5-28 years. DESIGN: One hour recordings of breathing movement, blood pressure, ECG R-R interval, heart rate, transcutaneous blood gases, cardiac vagal tone, and cardiac sensitivity to baroreflex measured on-line with synchronous EEG and video. Breathing rhythms were analysed in 47 cases. RESULTS: Respiratory rhythm was normal during sleep and abnormal in the waking state. Forced and apneustic breathing were prominent among 5-10 year olds, and Valsalva breathing in the over 18 year olds, who were also most likely to breathe normally. Inadequate breathing peaked among 10-18 year olds. Inadequate and exaggerated breathing was associated with vacant spells. Resting cardiac vagal tone and cardiac sensitivity to baroreflex were reduced. CONCLUSIONS: Labile respiratory rhythms and poor integrative inhibition in Rett disorder suggest brain immaturity. Linking this to an early monoaminergic defect suggests possible targets for the MECP2 gene in clinical intervention. Exaggerated and inadequate autonomic responses may contribute to sudden death.

Adolescent↗
PubMed1988

[Autonomic dysfunction in patients with temporal lobe epilepsy].

In 102 patients with temporal epilepsy autonomic (tone, responses, effectiveness) and sympathoadrenal (urine excretion of catecholamines and its changes under effects of epinephrine, insulin and L-DOPA administration) systems were investigated. Some of the indices under study showed reliable correlations with clinical data. Pronounced alterations of autonomic functions were found. The data suggest an important role of catecholamines in the pathogenesis of temporal epilepsy.

Adolescent↗
PubMed2005

Pediatric disorders with autonomic dysfunction: what role for PHOX2B?

Hirschsprung disease, neuroblastomas, and congenital central hypoventilation syndrome can occur in combination, and familial cases have been reported in all three conditions. This suggests variable expression of a single genetic abnormality as the common cause to these neural crest disorders. Because the PHOX2B gene is pivotal in the development of most relays of the autonomic nervous system, including all autonomic neural crest derivatives, it was considered a candidate gene for the above conditions. Recent studies have shown that 1) PHOX2B is the main disease-causing gene for congenital central hypoventilation syndrome, an autosomal dominant disorder with incomplete penetrance; 2) PHOX2B is the first gene for which germline mutations have been demonstrated to predispose to neuroblastoma; and 3) Hirschsprung disease was associated with an intronic single-nucleotide polymorphism of the PHOX2B gene in a case-control study. For clarifying the variable clinical expression of the autonomic nervous system dysfunction observed in neural crest disorders, international databases of clinical symptoms and molecular test results should be established. Furthermore, the development of genetic mouse models should help to improve our understanding of the molecular mechanisms underlying neural crest disorders.

Animals↗