Mechanisms of blood pressure alterations in response to the Valsalva maneuver in postural tachycardia syndrome

The postural tachycardia syndrome (POTS) is characterized clinically by orthostatic lightheadedness and tachycardia. When these patients perform a Valsalva maneuver, there is an excessive blood pressure increment after cessation of the maneuver (phase IV) that is sometimes associated with headaches. It is not known whether excessive phase IV is due to excessive peripheral vascular tone (an -adrenergic mechanism) or is a manifestation of increased -adrenergic tone (hyperadrenergic state). The authors undertook a pharmacologic study evaluating the effect of intravenous phentolamine (-adrenergic antagonist) and propranolol (-adrenergic antagonist) on the different phases of the Valsalva maneuver in a group of patients with POTS and age-matched normal control subjects. Patients with POTS had mean phases, when compared with controls, that were characterized by more negative II-E (p=0.07), smaller II-L (p=0.04), and significantly larger phase IV (p=0.001). The effect of phentolamine was qualitatively and quantitatively different in POTS when compared with controls. Ten mg phentolamine in controls resulted in a significant accentuation of phase II-E (p=0.001), attenuation of phase II-L (p=0.002), and increase of phase IV (57.6 vs 30.7 mm Hg; p=0.025). These changes resembled those of patients with POTS at baseline. In patients with POTS, the phase II abnormalities, already present, were further accentuated (p<0.001), and phase IV became smaller (50.6 vs 73.8 mm Hg; p=0.09). Propranolol had no significant effect on phases II-E and II-L, but significantly reduced phase IV in both controls (p<0.05) and in patients with POTS (p<0.001) and improved the headache symptoms, when present, during and after phase IV. The authors conclude that phase IV is mainly under -adrenergic regulation and that the exaggerated phase IV in POTS is a result of a hyperadrenergic state.     

Certain cardiovascular indices predict syncope in the postural tachycardia syndrome

Patients with postural tachycardia syndrome (POTS) represent a patient population with orthostatic intolerance; some are prone to syncope, others are not. The underlying neurocardiovascular mechanisms are not completely understood. The current study was undertaken to assess if certain cardiovascular indices are predictive of syncope in POTS. We compared the response to tilt-up and the Valsalva maneuver in four groups: POTS patients who fainted (POTS-f;n=11; 31±11 years); POTS patients who did not faint (POTS-nf;n=9; 29±9 years); normal controls (NLS;n=13; 39±11 years); patients with generalized autonomic failure with orthostatic hypotension and syncope (n=10; 59±14 years). Beat-to-beat heart rate (HR), systolic arterial pressure, diastolic arterial pressure (DAP) and pulse pressure (PP) were monitored using Finapres. Cardiac output, stroke volume (SV) and end-diastolic volume (EDV), and calculated total peripheral resistance (TPR) were recorded using thoracic electrical bioimpedance. An autonomic reflex screen which quantitates the distribution and severity of autonomic failure was also done. With the patient supine, all POTS patients (POTS-nf; POTS-f) had increased HR (p<0.001) and reduced SV/EDV (p<0.001) when compared with NLS. On tilt-up, POTS-f patients were significantly different from both NLS and POTS-nf patients; the most consistent alteration was a fall instead of an increase in TPR; other changes were a greater reduction in PP, a reduction (instead of an increment) in DAP, and a different pattern of changes during the Valsalva maneuver (excessive early phase II, attenuated or absent late phase II). Our results suggest -adrenergic impairment with increased pooling or hypovolemia in POTS-f patients. We conclude that it is possible to identify the mechanism of syncope in POTS patients, and perhaps other patients with orthostatic intolerance and an excessive liability to syncope.     

Anxiety in Patients with Postural Tachycardia Syndrome (POTS)

Background: The postural tachycardia syndrome (POTS) is a condition of the autonomic nervous system with symptoms of orthostatic intolerance. In POTS patients, orthostatic stress leads to an overshoot of heart rate increase without a fall in blood pressure. The purpose of this study is to distinguish between anxiety disorders and anxiety as a concomitant phenomenon of orthostatic stress.Methods: 50 patients fulfilling the diagnostic criteria (orthostatic symptoms, heart rate increase of > 30 bpm or up to > 120 bpm by testing with tilt-table) were included. The study design included a thorough medical history as well as standardised questionnaires about anxiety.Results: The average heart rate increase was 36 bpm after ten minutes of standing and 42 bpm after maximal standing time (max. 45 minutes). POTS patients scored significantly higher than a comparison group in a range of anxiety disorders by using anxiety questionnaires like "Beck Angst-Inventar" (BAI) and trait test of "State-Traits-Angstinventar" which include autonomic items. When questionnaires were used that exclude autonomic items (anxiety sensitivity index: ASI; Interaktions-Angst-Fragebogen: IAF) there was no difference.Conclusion: POTS patients do not exhibit signals of anxiety disorders more often than control groups, provided that questionnaires without autonomic items are used.     

Orthostatic intolerance without postural tachycardia: how much dysautonomia?

Background

Chronic symptoms of orthostatic intolerance occur in postural tachycardia syndrome (POTS) and patients with orthostatic intolerance (OI) without tachycardia. We recently reported that deconditioning is almost universal in both patient groups. In this study, we focussed on the question of how much dysautonomia, besides orthostatic tachycardia, is there in POTS vs. OI, and how the two groups compare in regards to clinical, autonomic, laboratory, and exercise variables.

Methods

We retrospectively studied all patients referred for orthostatic intolerance at Mayo Clinic between January 2006 and June 2011, who underwent standardized autonomic and exercise testing.

Results

Eighty-four POTS and 100 OI fulfilled inclusion criteria, 89 % were females. The mean age was 25 and 32 years, respectively. Clinical presentation, autonomic parameters, laboratory findings, and degree of deconditioning were overall similar between the two groups, except for the excessive orthostatic heart rate (HR) rise and mild vasomotor findings observed in POTS but not in OI (slightly larger Valsalva ratio and incomplete blood pressure recovery during Valsalva). Both groups responded poorly to various medications. Severely deconditioned patients were similar to non-deconditioned patients, except for 24 h urine volume (1,555 vs. 2,417 ml), sweat loss on thermoregulatory sweat test (1.5 vs. 0.5 %), and few respiratory parameters during exercise, which are likely clinically insignificant.

Conclusion

Though similar in clinical presentation, POTS and OI are different entities with greater, albeit still mild, dysautonomia in POTS. The clinical and pathophysiological relevance of minimal dysautonomia in the absence of orthostatic tachycardia as seen in OI remain uncertain.     

Gastric emptying in postural tachycardia syndrome: a preliminary report

Purpose

Autonomic neuropathy is widely recognized to be associated with upper gastrointestinal symptoms and abnormal (i.e., rapid or slow) gastric emptying. While patients with postural orthostatic tachycardia syndrome (POTS) may also have gastrointestinal symptoms, our understanding of gastric-emptying disturbances in POTS is very limited. The objectives of this study were to evaluate the relationship between gastric-emptying disturbances and gastrointestinal symptoms in patients with POTS.

Methods

We retrospectively reviewed the medical records of 22 well-characterized patients with POTS and upper gastrointestinal symptoms in whom autonomic (i.e., postganglionic sudomotor, cardiovagal, and adrenergic) functions and gastric emptying were evaluated using standardized techniques and scintigraphy, respectively. Medical records were reviewed retrospectively to assess clinical features, gastric emptying, and autonomic functions.

Results

Over 70 % of patients had nausea and/or vomiting, which was the most common GI symptom; other common symptoms were abdominal pain (59 %), bloating (55 %), and postprandial fullness/early satiety (46 %). Over one-third of patients had abnormal [i.e., rapid (27 %) or delayed (9 %)] gastric emptying. Gastric-emptying disturbances were not significantly associated with GI symptoms, autonomic symptoms or autonomic dysfunction.

Conclusions

Over one-third of patients with POTS and gastrointestinal symptoms have abnormal, more frequently rapid than delayed gastric emptying. These findings need to be confirmed in a larger cohort of patients.     

Symptoms of autonomic failure in Parkinson’s disease: prevalence and impact on daily life

Abstract Frequency and clinical importance of autonomic failure in idiopathic Parkinsons disease (PD) are discussed controversially. 141 patients with PD and 50 healthy age-matched control subjects were interviewed for symptoms of autonomic failure and their influence on daily life using a questionnaire. In PD patients, the prevalence of orthostatic dizziness, bladder dysfunction, erectile dysfunction and hyperhidrosis was significantly higher compared with controls. About 50% of PD patients rated the impact of the symptoms of autonomic failure on their daily lives as a lot or very much due to orthostatic dizziness, bladder dysfunction and constipation, which were more statistically significant than in age-matched controls. Prevalence and number of autonomic symptoms were not correlated with duration and severity of PD. In 32% of patients, impaired cardiovascular regulation was found by standardized cardiovascular function tests. If testing showed abnormal findings, orthostatic dizziness, bladder dysfunction, constipation and erectile dysfunction were significantly more frequent than in patients with normal regulation, but the impact on daily life due to these symptoms differed significantly only for bladder dysfunction between groups. It is concluded that autonomic failure is a clinically relevant, pervasive problem in PD and compromise patients daily life activities in all stages of the disease. This underlines the necessity to adequately search for and treat these non-dopaminergic symptoms during the whole course of the disease.     

Contrasting effects of carbohydrate and water on blood pressure responses to postural maneuvers in patients with posturally related (vasovagal) syncope

Abstract. Crouching then standing induces large changes in arterial blood pressure. Ingestion of carbohydrate and water are known to have contrasting effects on cardiovascular control in patients with various forms of autonomic dysfunction. We tested the hypothesis that, in patients with attacks of posturally related (neurogenic or vasovagal) syncope, postural maneuvers would cause greater changes in blood pressure than in normal controls and that they would be affected differently by carbohydrate and water. We studied 7 patients with histories indicating posturally related syncope and who we had shown to have abnormal responses to an orthostatic stress test, and 7 healthy volunteers with normal orthostatic responses. Responses of blood pressure (Portapres® finger photoplethysmography) were determined to crouching and subsequent standing, before and after ingestion of a high carbohydrate meal (2.5MJ) and (on a different day) water (500 ml). Before the meal or water there were no differences between groups in baseline blood pressures or in the responses to crouching and standing. In controls, neither carbohydrate nor water had any significant effect on baseline blood pressure or on the responses to the maneuvers. In the patients, the standing pressures were also unaffected by carbohydrate but they did increase after water. In the patients the increases in pressure during the crouch were larger after carbohydrate, but smaller after water. These results show that, in patients with posturally related syncope, unlike in control subjects, carbohydrate ingestion and water result in opposite effects on blood pressure during postural maneuvers. These results raise the possibility that these patients might have a mild form of autonomic dysfunction.     

“Echoing approval”: A new speech disorder

We report the cases of two patients presenting a peculiar speech disorder, which we have named echoing approval, in which the patients echo, in replying to questions in a dialogue with short phrases, the positive or negative syntactical construction of a question, or its positive or negative intonation, but without any repetition of whole or part of sentences. When asked about their symptoms, the patients replied 80% of the time with yes, yes, that's right, or exactly to positive questions and no, no or absolutely not to negative questions, regardless of their actual symptoms and oblivious to self-contradiction. In addition, when the examining doctor was speaking to a medical colleague in the patient's presence and using medical terminology that the patient did not understand, he/she agreed or disagreed with any sentence and technical word uttered in a way entirely dependent on the syntax or intonation used. To distinguish this speech disorder from echolalia or verbal perseverations, with which it may be superficially confused, we suggest that it be called echoing approval, as it may be part one of the manifestations of the environment-dependency syndrome. This clinical picture was found to be associated with features of transcortical motor aphasia and frontal lobe signs. One patient had a bilateral callosofrontal malignant glioma and the other a probable multiple system atrophy with global deterioration, pre-eminent frontal release signs, diffuse leukoencephalopathy and multiple lacunes. On the basis of these clinical deficits and neuroimaging features, we are unable to delineate the common, or minimal, lesioned network required for this symptomatology to occur, especially in the absence of a series of patients, and with such a difference in both the location and causes of the lesions. However, bilateral frontosubcortical dysfunction was pre-eminent in the clinical picture in both patients, even though more diffuse brain pathology was seen in one, and it might be speculated that dysfunction of the bilateral orbitofrontal and frontomesial motor frontosubcortical circuits might be involved in the aetiology of this peculiar speech disorder.     

“Sporadic” prealbumin-related amyloid polyneuropathy: report of two cases

Summary Two sporadic cases of amyloid polyneuropathy are reported. There was no family history or plasma cell dyscrasia. Both showed sensorimotor and autonomic polyneuropathy with onset in the seventh decade. Amyloid deposits in both cases reacted with anti-human prealbumin sera but not with antisera to human AA and anti-human immunoglobulin light-chain amyloids, including A and A. One patient had the abnormal serum prealbumin and abnormal DNA sequence found in type I familial amyloid polyneuropathy (FAP) (Japanese type). Investigations in sporadic amyloid polyneuropathy should include immunohistochemistry, using antisera to the different amyloid proteins, and the radioimmunoassay and recombinant DNA techniques for diagnosis of FAP.     

Postural tachycardia syndrome in syringomyelia: Response to fludrocortisone and β-blockers

Orthostatic intolerance is occasionally reported by patients with syringomyelia and is usually attributed to vestibular symptoms or neurogenic orthostatic hypotension. Postural tachycardia syndrome has not been previously described in syringomyelia. A patient with long-standing syringomyelia and a Chiari type I anomaly developed disabling panic-like attacks associated to orthostatic intolerance five years after posterior fossa decompression and shunting of the syrinx. A headup tilt test showed an early phase of postural orthostatic tachycardia followed by progressive arterial hypotension and bradycardia as seen in neurally mediated syncope. A magnetic resonance imaging scan showed a collapsed syrinx from the 3rd cervical to the 12th thoracic vertebra without syringobulbia. Fludrocortisone and -blockers led to resolution of symptoms. Partial sympathetic denervation of the legs in syringomyelia might explain the occasional occurrence of postural tachycardia syndrome. Postural tachycardia syndrome may be included as a possible cause of orthostatic symptoms in syringomyelia patients.     

Serum IgG,IgA and IgM concentration in 1,038 patients with various neurological disorders

Summary Concentration of serum IgG, IgA and IgM in 1,038 unselected patients with various neurological diseases was determined. In 521 instances, the levels of CSF IgG, IgA and IgM were also established. In serum, the most frequent selective quantitative abnormality was found in the IgA concentration. In CSF, an increased level of IgG with normal IgA concentration and undetectable IgM was established about 8 times more frequently than isolated increase of CSF IgA. Selective quantitative abnormalities in serum IgG were observed in 35% of patients with subacute sclerosing panencephalitis as compared to 5% in instances in the definite MS group. Abnormal bands in the serum gamma-globulin field were most frequently seen in electropherograms from patients with subacute sclerosing panencephalitis, in cases of malignant lymphoproliferative disorders and in patients with definite MS. No correlation between the concentration of serum and CSF immunoglobulins could be established. Most frequent quantitative abnormalities in serum IgG, IgA and/or IgM were established in malignant lymphoproliferative disorders, in patients with myopathies including myositides and in subacute or chronic inflammatory CNS disorders. Highest incidence of increased CSF IgG, IgA and/or IgM concentration was detected in patients with inflammatory CNS disease, in instances of definite MS and in malignant lymphoproliferative disorders with CNS symptomatology. Increased CSF IgG and IgA concentration with detectable levels of IgM in patients with elevated CSF total proteins indicated alterations in the blood/CNS barrier structures.Supported by PHS grants NB05450 and 06793.     

Diagnosis of tachycardia syndromes associated with orthostatic symptoms

The postural tachycardia syndrome (POTS) is characterized by excessive tachycardia only in upright position without evidence of a cardiac or metabolic disease in combination with orthostatic symptoms like dizziness, lightheadedness or syncope but without relevant falls in blood pressure. The cause is unknown. A specific diagnostic marker has not been found so far. Eighteen patients with typical symptoms of POTS were examined. They underwent standard autonomic function tests with continuous measurement of heart rate (HR) and blood pressure. All fulfilled the inclusion criteria of pathologically increased HR activation during passive tiltor standing over 90 seconds. The upper limits of normal were based on data from 137 healthy volunteers between 18 and 85 years of age. Actively standing up induced more POTS-typical HR increases and lead to more consistent results than passive tilt. HR responses during Valsalva manoeuvre and deep breathing were normal in all except one patient each, indicating that assessment of HR during these tests does not contribute to the diagnosis of POTS. Frequency of symptoms reducing overall well-being and the degree of impairment of life quality by symptoms typical of POTS were measured with a self-assessment scale. The majority of patients reported a permanent reduction of overall wellbeing and a relevant impairment of life quality due to dizziness, tachycardia, and syncopes. This underlines the importance of considering POTS as a differential diagnosis of orthostatic syndromes and the necessity of treating it adequately.     

Customer, Client, Consumer, Recipient, or Patient

Since the arrival of managed care, there has been a trend toward changing the basic terminology used to address clinicians and patients. Instead of the term patient, third party payors frequently use terms such as customer, client, consumer or recipient. One study demonstrated that patients prefer to be called patients. To investigate the preferred term to refer to patients and to be referred to by patients, we mailed a questionnaire to 100 physicians in four medical specialties each and to 100 psychologists. The overall response rate was 61%. Physicians overwhelmingly preferred to refer to patients by the patient's last name, their second preference was the patient's first name. Psychologists preferred to refer to the patients by first name, their second preference was the patient's last name. No group favored using terms such as client, customer, consumer, or recipient. Most physicians and psychologists preferred being referred to as doctors and nobody favored the term provider.     

Hemodynamic and symptomatic effects of acute interventions on tilt in patients with postural tachycardia syndrome

A variety of approaches have been used to alleviate symptoms in postural tachycardia syndrome (POTS). Drugs reported to be of benefit include midodrine, propranolol, clonidine, and phenobarbital. Other measures used include volume expansion and physical countermaneuvers. These treatments may influence pathophysiologic mechanisms of POTS such as -receptor dysfunction, -receptor supersensitivity, venous pooling, and brainstem center dysfunction. The authors prospectively studied hemodynamic indices and symptom scores in patients with POTS who were acutely treated with a variety of interventions. Twenty-one subjects who met the criteria for POTS were studied (20 women, 1 man; mean age, 28.7±6.8 y; age range, 14–39 y). Patients were studied with a 5-minute headup tilt protocol, ECG monitoring, and noninvasive beat-to-beat blood pressure monitoring, all before and after the administration of an intervention (intravenous saline, midodrine, propranolol, clonidine, or phenobarbital). The hemodynamic indices studied were heart rate (ECG) and systolic, mean, and diastolic blood pressure. Patients used a balanced verbal scale to record any change in their symptoms between the tilts. Symptom scores improved significantly after the patients received midodrine and saline. Midodrine and propranolol reduced the resting heart rate response to tilt (p<0.005) and the immediate and 5-minute heart rate responses to tilt (p<0.002). Clonidine accentuated the immediate decrease in blood pressure on tilt up (p<0.05). It was concluded that midodrine and intravenous saline are effective in decreasing symptoms on tilt in patients with POTS when given acutely. Effects of treatments on heart rate and blood pressure responses generally reflected the known pharmacologic mechanisms of the agents.     

Orthostatic heart rate does not predict symptomatic burden in pediatric patients with chronic orthostatic intolerance

Clinical Autonomic Research - Postural orthostatic tachycardia syndrome (POTS) in adults is defined as symptoms of chronic orthostatic intolerance (COI) and autonomic dysfunction (AD) with heart...     

Attitudes toward mental health professionals in a hospital-based community mental health center

This study examined the attitudes of several diverse subject groups in a large medical center toward various mental health professionals. The groups consisted of: 1) general hospital staff; 2) professional mental health workers; and 3) psychiatric in-patients. Subjects evaluated a selection of 11 professional health related role titles (clinical psychologist, physician, psychiatrist, etc.) and the categories me and mental patient by marking a series of 19 seven-step rating scales, each composed of bipolar anchoring adjectives. Additionally, a familiarity rating for each of the role titles was obtained. An understanding and a value cluster were derived from the 19 adjectives along with an overall favorability-unfavorability score for each role title. It was expected that subjects would value mental health professional roles more strongly than they would indicate an understanding of these same roles. Secondly, it was expected that the hospital setting itself, the subject's role within that setting, and the degree of familiarity with the role being rated would have a significant impact on the subject's attitude. Results generally supported the above expectations. Overall ratings of the professional groups were consistently high, with less difference between the health designations (physician, nurse) and the psych designations than has been previously reported in the literature.     

Types of authority and two problems of psychiatric wards

This paper concerns the relationship between authority structures and two problems reported in the literature as common to milieu or therapeutic community wards. Psychiatric wards with rational-legal and charismatic authority structures are found more likely to experience mood and morale swings on the part of patients and staff and to spend excessive time and energy changing ward rules.     

Immunoglobulin heavy chain gene associations in myasthenia gravis: new evidence for disease heterogeneity

Summary Susceptibility to myasthenia gravis (MG) is known to involve genes residing in the major histocompatibility complex class I and II regions (HLA-B8 and DR3). Immunoglobulin heavy chain constant region (IgCH) allotypes have also shown some associations with MG. We have used restriction fragment length polymorphism analysis with probes to the IgCH switch (S) regions and 1 and the downstream marker D14S1 to investigate 189 Caucasoid patients with well-defined MG. A highly significant increase in the frequency of the 2.6 kilobase (kb) S homozygous genotype and the 2.6 kb S allele was found in patients with disease onset after the age of 40 years (late onset) compared with normal controls (P<0.00075 andP<0.025 respectively). No association was found at the S1 or D14S1 loci. In patients with an associated thymoma there was a moderate increase in the frequency of the 2.6 kb S and 7.4 kb S1 genotypes. These results independently support the previous separation of the late-onset subgroup. Finally, the stronger associations at S rather than at the downstream Sl, Gm and D14S1 loci suggest that the genes predisposing to MG are located within the variable region of the Ig heavy chain loci.     

Immunotherapy with subcutaneous immunoglobulin or plasmapheresis in patients with postural orthostatic tachycardia syndrome (POTS)

Journal of Neurology - Postural orthostatic tachycardia syndrome (POTS), one of the most common autonomic disorders, is associated with significant morbidity and functional impairment. Although...     

What is the minimum duration of head-up tilt necessary to detect orthostatic hypotension?

Abstract Objective There is uncertainty as to the minimum duration of head-up tilt (HUT) needed to detect orthostatic hypotension (OH). The orthostatic duration has variably been recommended to be 1, 2, 3, and 5 minutes. The purpose of the current study was 1) to determine the minimum duration of HUT necessary to detect OH and 2) to identify different patterns of orthostatic blood pressure (BP) response in patients with OH. Design/methods We evaluated the medical records of 66 consecutive patients (mean age 70.0±10.1 years; 64% male) seen at Mayo Clinic-Rochester from 2000–2001 who fulfilled the criteria for OH (systolic blood pressure [SBP] reduction 20mm Hg within 3 minutes of HUT) during routine clinical autonomic studies. All patients completed an autonomic reflex screen with continuous monitoring of heart rate and BP during supine rest and 5 minutes of 70 degree HUT. Severity of autonomic deficits was quantified with the Composite Autonomic Severity Score (CASS). Results Overall, BP was the lowest at 1 minute with gradual and partial recovery over the following 4 minutes. Eighty-eight percent of patients (N=58) developed OH by 1 minute of HUT, with an additional 11% (N=7) developing OH by 2 minutes and the remaining 1% (N=1) developing OH by 3 minutes. We identified two broad patterns of SBP response to HUT. Forty-eight percent (N=32) of patients demonstrated an initial drop in SBP ( 20 mm Hg),which remained stable until tilt-back. Thirty-six percent (N=24) demonstrated an initial drop ( 20mm Hg) followed by a progressive decline in SBP until tilt-back. Repeated measures analysis of variance confirmed that the SBP change in response to HUT differed significantly among patients with a stable vs. progressive pattern [F(3,32)=25.1, p<0.001). Patients with the progressive pattern also had more severe adrenergic impairment on the CASS (p=0.03) and were more likely to have their tilt test terminated early (prior to 5minutes) due to presyncope (p<0.0001) than patients with the stable pattern. Conclusions One minute of HUT will detect OH in the great majority (88%) of patients and three minutes will detect the balance. Orthostatic stress beyond 2 minutes is necessary to detect the pattern of progressive OH. Since this group has more severe adrenergic deficits than the group with stable OH, we suggest that the progressive pattern is due to greater impairment of compensatory reflexes. Recognition of the group with progressive fall in BP is important since this group may be at greater risk of orthostatic syncope.