Pattern Of Autonomic Dysfunction Research Articles

Pattern of Autonomic Involvement in Adult Patients with Guillain Barre Syndrome in a Tertiary Hospital

Background: Guillain Barre Syndrome (GBS) is an acute post infectious immune mediated peripheral neuropathy with a marked variation in pathology, clinical presentation and prognosis. Autonomic dysfunction is one of the important manifestations of GBS which may lead to significant morbidity and mortality. Objective: The aim of the study is to assess the autonomic involvement, to determine its frequency and pattern of involvement in adult patients with GBS Methods: An observational, descriptive, cross sectional study was carried out in the Department of Neurology, BSMMU, Dhaka from March, 2015 to September, 2017. Total 43 patients of GBS and 35 apparently healthy controls were recruited as the study population. On the basis of nerve conduction study patients were classified into different groups: acute inflammatory demyelinating polyneuropathy (AIDP), acute motor axonal neuropathy (AMAN) and acute motor sensory axonal neuropathy (AMSAN) and other variants. Disability status at the time of autonomic testing was measured by Hughes functional grading scale. The following tests of autonomic nervous system were performed in both patients and controls 1) resting heart rate and heart rate on changing posture (30: 15 ratio) 2) supine blood pressure and blood pressure on changing posture 3) heart rate response to valsalva maneuver 4) heart rate response to deep breathing and E: I ratio 5) sphincter disturbance by symptoms questionnaire. Results: The mean age of patients was 35±12 years (range18 to 65 years) and 58.1% were male. Around 88.4 % of patients showed some sort of autonomic dysfunction. Variation of heart rate by different maneuver like posture change, deep breathing and valsalva maneuver was found commonly. Among them 30:15 ratio was abnormal in majority of the patients (82.4%) followed by abnormal max-min HR/min (58.1%) and abnormal valsalva ratio (37.2%). Other abnormalities were postural hypotension (38.2%), sinus tachycardia (25.6%), hypertension (16.3%), hypotension (2.7%), sinus arrhythmia (4.7%), constipation (30%), urinary retention (7%) and urinary incontinence (4.7%). Conclusion: In this study different patterns of autonomic dysfunction was found in 88.4% of patients with GBS involving both sympathetic and parasympathetic components. The present study found no significant association between autonomic dysfunction and motor disability scores. Thus autonomic function assessment is essential in every patient with GBS in addition to motor & sensory function. Bangladesh Medical Res Counc Bull 2022; 48(3): 174-179

Spectrum of Cardiovascular Autonomic Dysfunction and 24-hour Blood Pressure Variability in Idiopathic Parkinson's Disease.

Uncertainty prevails regarding the patterns of autonomic dysfunction in patients with idiopathic Parkinson's disease (IPD). This study was undertaken with the aim of assessing the complete spectrum of cardiovascular autonomic function tests (CAFTs) and blood pressure variability patterns in IPD patients while comparing the same with age-matched controls. Patients with IPD presenting to the Christian Medical College and Hospital from December 2016 to November 2018 along with age-matched controls were prospectively evaluated using CAFTs. The IPD patients also underwent ambulatory blood pressure (BP) monitoring (ABPM), and the diurnal systolic BP differences were used to classify into dippers (10-20%), non-dippers (0-10%), reverse dippers (<0%), and extreme dippers (>20%). Autonomic dysfunction (AD) was prevalent in 41 (68.3%) IPD patients even in early disease (median (inter-quartile range) symptom duration 2 (1-4) years, mean Hoehn and Yahr (H&Y) stage 2 (1.5-2.8). Both sympathetic and parasympathetic parameters were impaired among IPD patients when compared to healthy controls. (E: I ratio 1.17 ± 0.12 vs 1.26 ± 0.14 (P < 0.001), Valsalva ratio (VR) 1.33 ± 0.27 vs 1.55 ± 0.25 (P < 0.001), PRT100 9.6 ± 8.0 vs 3.1 ± 1.8 (P < 0.001), tilt-up SBPAvg change 8.8 (4.2-13.8) vs 1.8 (-2.9-6.1) (P < 0.001), tilt-up HRAvg change 4.8 (2.2-8.2) vs 1.9 (-0.7-5.1) (P < 0.001). BP variability was demonstrated in 47 (79.7%) of IPD patients, with reverse dipping pattern in 28 (47.5%) seen more frequently in this cohort. Timely detection of AD may be helpful not only in recognizing IPD in its pre-motor stages but also in optimizing management for this population of patients. BP variability and abnormal dipping patterns on ABPM can be a potential marker of dysautonomia.

Clinical presentation and autonomic profile in Ross syndrome.

Ross syndrome is a rare disorder characterized by tonic pupils, hyporeflexia, and segmental anhidrosis. We sought to characterize the clinical presentation, associated autoimmune disorders, and autonomic profile in patients with Ross syndrome to further elucidate its pathophysiology. We performed a retrospective chart review of all patients who underwent a thermoregulatory sweat test (TST) between 1998 and 2020 and had confirmation of the diagnosis of Ross syndrome by an autonomic disorders specialist. Standardized autonomic reflex testing was reviewed when available. Twenty-six patients with Ross syndrome were identified. The most common initial reported manifestation was an abnormal segmental sweating response in 16 patients (described as hyperhidrosis in 12 patients and anhidrosis in 4 patients) while a tonic pupil was the initial manifestation in 10 patients. Other commonly reported symptoms included fatigue, chronic cough, and increased urinary frequency. An associated autoimmune disorder was identified in one patient. Positive autoantibodies were found in a minority of patients often with unclear clinical significance. Distributions of anhidrosis encountered were segmental (n = 15), widespread (n = 7), and global (n = 4). Well-circumscribed small areas of preserved sweating within areas of anhidrosis were observed in the majority of patients (88.5%). Anhidrosis progressed slowly over time and sudomotor dysfunction was predominantly (post)ganglionic. Cardiovagal and adrenergic functions were preserved in most patients. The pattern of autonomic dysfunction in Ross syndrome is suggestive of a limited autonomic ganglioneuropathy. Sudomotor impairment is prominent and should be the focus of symptomatic management; however, clinicians should be aware of symptoms beyond the classic triad.

Clinical, Autonomic &amp; Electrophysiological Features in Patients with Guillain Barre Syndrome in a Tertiary Care Hospital of Bangladesh

Background: Guillain-Barre Syndrome (GBS) is the most common cause of acute flaccid paralysis in the adult population. It is an acute post infectious immune mediated peripheral neuropathy with a marked variation in pathology, clinical presentation and prognosis.&#x0D; Objective: The aim of the study were to evaluate clinical profile, to assess autonomic involvement &amp; electrophysiological findings in adult patients with GBS.&#x0D; Methods: An observational, cross sectional study was carried out in the Department of Neurology, BSMMU, Dhaka from March, 2015 to September, 2017. Total 43 patients of GBS fulfilling the inclusion criteria were recruited as the study population. Detailed clinical examination, CSF study &amp; nerve conduction study were done. Disability status was measured by Hughes functional grading scale. For autonomic assessment 35 adult healthy control were also included for comparison. Then following tests of autonomic nervous system were performed in both patient and control group 1) resting heart rate and heart rate on changing posture (30: 15 ratio) 2) supine blood pressure and blood pressure on changing posture 3) heart rate response to valsalva maneuver 4) heart rate response to deep breathing and E: I ratio 5) sphincter disturbance by symptoms questionnaire.&#x0D; Results: The mean age of patients was 35±12 years (range18 to 65 years) with slight male predominance (58.1%). Major clinical presentation was weakness of all 4 limbs followed by sensory complaints (44.2%). 7% of the patient had breathing difficulty and dysphagia. Only 4.7 % had diplopia. Among the symptoms of autonomic dysfunction most common symptoms was constipation (30.2 %) followed by palpitation (14%), urinary retention (7%), lightheadedness and urinary incontinence (4.7%). Cranial nerve palsy was present in 34.9% of cases among them facial palsy was found commonly (27.9%), followed by bulbar palsy (7%) and ophthalmoplegia (4.7%). One patient (2.3%) had both facial palsy and ophthalmoplegia. AIDP, AMAN and AMSAN subtypes comprised 32.6%, 37.2% and 20.9% of cases respectively. Regarding autonomic dysfunction variation of heart rate by different maneuver like posture change, deep breathing and valsalva maneuver was found commonly. 30:15 ratio was abnormal in majority of the patients (82.4%) followed by abnormal max-min HR/min on deep breathing (58.1%) and abnormal valsalva ratio (37.2%). Other abnormalities were postural hypotension (38.2%), sinus tachycardia (25.6%), hypertension (16.3%), hypotension (4.7%), and sinus arrhythmia (4.7%). Bowel bladder dysfunction was another autonomic dysfunction among them constipation 30%, urinary retention 7% and urinary incontinence 4.7% of cases.&#x0D; Conclusion: GBS can be presented with variable presentation including autonomic dysfunction. In this study common clinical presentation was limb weakness &amp; different patterns of autonomic dysfunction was found in patients with GBS. Common electrophysiological subtype was AMAN. So in addition to clinical &amp; electrophysiological analysis autonomic evaluation is essential in every patients with GBS as autonomic dysfunction is one of important cause of mortality.&#x0D; Bangladesh Journal of Neuroscience 2019; Vol. 35 (2): 57-62

Increased sympathetic nerve activity and reduced cardiac baroreflex sensitivity in rheumatoid arthritis.

Key points Rheumatoid arthritis (RA) is a chronic inflammatory condition associated with an increased risk of cardiovascular mortality.Increased sympathetic nerve activity and reduced cardiac baroreflex sensitivity heighten cardiovascular risk, althogh whether such autonomic dysfunction is present in RA is not known.In the present study, we observed an increased sympathetic nerve activity and reduced cardiac baroreflex sensitivity in patients with RA compared to matched controls.Pain was positively correlated with sympathetic nerve activity and negatively correlated with cardiac baroreflex sensitivity.The pattern of autonomic dysfunction that we describe may help to explain the increased cardiovascular risk in RA, and raises the possibility that optimizing pain management may resolve autonomic dysfunction in RA. Rheumatoid arthritis (RA) is a chronic inflammatory condition associated with increased cardiovascular morbidity/mortality and an incompletely understood pathophysiology. In animal studies, central and blood borne inflammatory cytokines that can be elevated in RA evoke pathogenic increases in sympathetic activity and reductions in baroreflex sensitivity (BRS). We hypothesized that muscle sympathetic nerve activity (MSNA) was increased and BRS decreased in RA. MSNA, blood pressure and heart rate (HR) were recorded in age‐ and sex‐matched RA‐normotensive (n = 13), RA‐hypertensive patients (RA‐HTN; n = 17), normotensive (NC; n = 17) and hypertensive controls (HTN; n = 16). BRS was determined using the modified Oxford technique. Inflammation and pain were determined using serum high sensitivity C‐reactive protein (hs‐CRP) and a visual analogue scale (VAS), respectively. MSNA was elevated similarly in RA, RA‐HTN and HTN patients (32 ± 9, 35 ± 14, 37 ± 8 bursts min–1) compared to NC (22 ± 9 bursts min–1; P = 0.004). Sympathetic BRS was similar between groups (P = 0.927), whereas cardiac BRS (cBRS) was reduced in RA, RA‐HTN and HTN patients [5(3–8), 4 (2–7), 6 (4–9) ms mmHg–1] compared to NC [11 (8–15) ms mmHg–1; P = 0.002]. HR was independently associated with hs‐CRP. Increased MSNA and reduced cBRS were associated with hs‐CRP although confounded in multivariable analysis. VAS was independently associated with MSNA burst frequency, cBRS and HR. We provide the first evidence for heightened sympathetic outflow and reduced cBRS in RA that can be independent of hypertension. In RA patients, reported pain was positively correlated with MSNA and negatively correlated with cBRS. Future studies should assess whether therapies to ameliorate pain and inflammation in RA restores autonomic balance and reduces cardiovascular events.

Disturbances of gastrointestinal transit and autonomic functions in postural orthostatic tachycardia syndrome.

Gastrointestinal symptoms are common in the postural orthostatic tachycardia syndrome (POTS). However, few studies have evaluated gastrointestinal transit in POTS. Our primary objectives were to evaluate gastrointestinal emptying and the relationship with autonomic dysfunctions in POTS. We reviewed the complete medical records of all patients aged 18 years and older with POTS diagnosed by a standardized autonomic reflex screen who also had a scintigraphic assessment of gastrointestinal transit at Mayo Clinic Rochester between 1998 and 2012. Associations between specific gastric emptying and autonomic (i.e., cardiovagal, adrenergic, and sudomotor) disturbances were evaluated. Among 163 patients (140 women, mean [± SEM] age 30 [± 1] years), 55 (34%) had normal, 30 (18%) had delayed, and 78 (48%) had rapid gastric emptying. Fifty-eight patients (36%) had clinical features of physical deconditioning, which was associated (p = 0.02) with rapid gastric emptying. Associations with delayed gastric emptying included vomiting, which was more common (p < 0.003), and anxiety or depression, which was less common (p = 0.02). The tilt-associated increase in heart rate and reduction in systolic BP at 1 min was associated (p < 0.05), being greater in patients with delayed gastric emptying. Two-thirds of patients with POTS and GI symptoms had abnormal, most frequently rapid gastric emptying. Except for more severe adrenergic impairment in patients with delayed gastric emptying, the pattern of autonomic dysfunction did not discriminate among gastric emptying groups. Further studies are necessary to ascertain whether extravascular volume depletion and/or deconditioning contribute to POTS in patients with gastrointestinal symptoms.

Autonomic dysfunction in adult-onset alexander disease

Alexander disease (AxD) is an astrogliopathy, resulting from a mutation in the glial fibrillary astrocytic protein gene. Different clinical subtypes have been described, including infantile, juvenile, and adult onset, based upon the age at which symptoms begin. Patients with the adult-onset form, develop a progressive, spastic paraparesis, palatal myoclonus, ataxia, and bulbar weakness. Autonomic nervous system (ANS) dysfunction has been reported as a potential manifestation of adult-onset AxD, but has not been well characterized. We report a case of adult-onset AxD with symptomatic orthostatic hypotension (OH) and heat intolerance that underwent formal autonomic testing. In addition, a comprehensive literature search was conducted to review the frequency and pattern of autonomic dysfunction in this patient population. A 51-year-old patient was diagnosed with AxD at the age of 47, following an 8-year history of vertigo, intermittent diplopia, and sleep disturbance. The patient developed symptoms of OH, erectile dysfunction, and heat intolerance soon after his diagnosis. Autonomic testing demonstrated OH on tilt-table testing (47mmHg decrease in BP with 18 BPM heart rate increment) with absent late phase II and IV responses during the Valsalva maneuver, severe cardiovagal impairment, and preserved postganglionic sympathetic sudomotor function. These findings were interpreted as being consistent with central autonomic failure. The most common autonomic symptoms reported in other AxD cases include constipation, urinary incontinence, and sphincter dysfunction. To our knowledge, this is the first report of formal autonomic testing in AxD. Signs and symptoms of ANS impairment can occur in patients with AxD, and can include orthostatic hypotension and bowel/bladder dysfunction. Autonomic testing in our patient suggests impairment in central autonomic pathways.

Autonomic Dysfunction in Unaffected First-Degree Relatives of Patients Suffering From Schizophrenia

Recent studies revealed cardiac autonomic dysfunction in patients with acute schizophrenia, which appears to be mainly related to reduced vagal and increased sympathetic modulation. To understand the significance of cardiac autonomic function in patients with schizophrenia, we extended these studies to relatives of patients. In this study, we assessed cardiac autonomic modulation in healthy first-degree relatives of patients with schizophrenia (n = 36) to investigate a putative genetic influence. Data were compared with control subjects matched for age, gender, and physical activity as well as to patients suffering from schizophrenia. First-degree relatives showed an attenuated, yet identical pattern in autonomic dysfunction as patients with decreased vagal modulation of heart rate, decreased baroreflex sensitivity, but no difference in blood pressure variability could be detected. The patients' relatives also showed a similar pattern in regards to QT variability. In addition, the subgroup comparison of offspring vs. siblings showed a significant difference in heart rate variability suggesting a higher degree of heritability in offspring. In conclusion, the pattern of autonomic dysfunction seen in patients and relatives might indicate underlying disease-inherent genetic vulnerability, especially because autonomic parameters are heritable. In addition, these findings may be of value to identify the high-risk group of patients' relatives in regards to serious cardiovascular events so that early preventive measures can be taken.

Autonomic dysfunction in systemic lupus erythematosus

The objectives were to study the frequency and pattern of autonomic dysfunction in systemic lupus erythematosus (SLE). Fifty-one patients of SLE and 30 age- and sex-matched healthy controls were studied prospectively using a standard battery of noninvasive tests. Autonomic symptoms were seen in 37% patients. On laboratory testing incipient dysfunction was seen in 9 (18%) cases and 1 (3%) control, while atypical involvement was seen in 11 (21%) cases and 6 (20%) controls. Autonomic dysfunction did not correlate with disease duration, lupus activity, disease damage, any particular organ involvement or the presence/absence of peripheral neuropathy. Autonomic neuropathy is not uncommon in lupus and may exist independent of peripheral neuropathy. There are no specific clinical predictors. The clinical significance of autonomic dysfunction detected by laboratory testing warrants longitudinal studies.

Patterns of recovery of autonomic dysfunctions and neurocognitive deficits in schizophrenics after acute psychotic episodes.

In this study, the authors aimed to identify patterns of autonomic dysfunction and neurocognitive deficit recovery. The authors performed laboratory assessments on 66 patients with schizophrenia immediately after an acute psychotic episode and 6, 12, and 18 months later. Shortly after the psychotic episode, the patients displayed cardiovascular hyperarousal at rest, cardiovascular and electrodermal hyporeactivity during 2 Continuous Performance Tasks (CPTs) and deficits in 2 behavioral CPT measures (i.e., reaction time and omission error rate) compared with 29 normal controls. In the subsequent postpsychotic course, changes indicative of a process of recovery occurred in all measurement areas, although with regard to autonomic hyporeactivity amelioration was limited to a subgroup of schizophrenics with complete and persistent symptomatic remission. Neurocognitive improvement in CPTs did not appear to depend on unimpaired autonomic reactivity mechanisms.

Pattern of autonomic dysfunction in time course of complex regional pain syndrome.

The objective of the present investigation was to describe and localize autonomic dysfunction in acute and chronic stages of complex regional pain syndrome (CRPS). Patients were investigated twice: the first investigation was performed as soon as diagnosis was established during the acute stage of CRPS and the second investigation was performed about 2 years later. Twenty-one patients completed the follow-up investigation. The median duration of CRPS was 5 (range 2-21) weeks at first investigation and 94 weeks (22-148) at follow-up. Skin temperature was recorded by thermography, sudomotor function was assessed by thermoregulatory sweat test (TST) and quantitative sudomotor axon reflex test (QSART). Skin temperature was warmer on the affected side at the first investigation (P < 0.001) and colder at follow-up (P < 0.02) compared with the contralateral limb. Sudomotor output was enhanced after both TST (P < 0.005) and QSART (P < 0.05) at the first investigation on the affected side. However, at follow-up, sweating after TST was still increased (P < 0.04) while QSART responses were not different between the affected and unaffected limbs. As compared to controls there was no statistically significant difference, neither in skin temperature nor sweating, neither on the affected nor on the unaffected side. In conclusion, the present investigation proved that vasomotor and sudomotor control are substantially altered in CRPS. In the acute stage vasomotor control is decreased in the affected limb whereas sudomotor function is enhanced. This may be the result of disturbances of thermoregulation, but different secondary peripheral mechanisms, concerning vasomotor and sudomotor function, contribute to clinical presentation of CRPS and affect autonomic function at all stages of CRPS.

Adverse influence of baroreceptor dysfunction on upright exercise in congestive heart failure

To test the hypothesis that baroreceptor autoregulation is an important determinant of exercise performance, the hemodynamic responses to the gravitational stress of head-up tilt and upright exercise were assessed in 41 patients with severe chronic congestive heart failure. Three patterns of autonomic dysfunction during head-up tilt were identified. Group A patients had near-normal circulatory reflexes whereas Group B patients experienced orthostatic hypotension and Group C patients lacked the afferent stimulus of venous pooling, Group A patients had a significantly longer exercise duration than both Groups B and C (607 ± 104 versus 330 ± 86 and 365 ± 53 seconds, respectively, p <0.05) and a significantly higher double product at peak exercise (17,779 ± 956 versus 12,235 ± 869 and 13,760 ± 998, respectively, p <0.05). Thus, baroreceptor abnormalities in the autoregulatory response to postural change are important determinants of the cardiovascular responses required during exercise and influence exercise performance. These abnormalities may help clarify the discrepancies between supine and upright exercise performance and may influence the long-term efficacy of vasodilator therapy in chronic congestive heart failure.