Misdiagnosed Seizure-Like Activity in a Patient With Postural Orthostatic Tachycardia Syndrome: A Case Report
Safwat, S. et al.
Sandra Safwat
Fady Safwat
Nivetha Sivanathan
Natashah Daka
Michael Sadek
0
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0
10.7759/cureus.39565
Published in Cureus
Postural orthostatic tachycardia is a syndrome characterized by an elevated heart rate in response to standing. This syndrome typically presents in late adolescence and early adulthood, with a higher percentage occurring in females. This syndrome is often seen following a viral infection, pregnancy, surgery, or intense psychological stress. This condition presents a wide range of symptoms that vary depending on its unclear etiology. We present the case of a 21-year-old woman with convulsions associated with postural orthostatic tachycardia syndrome after being misdiagnosed with a psychiatric condition for many years.
Choices and Challenges With Drug Therapy in Postural Orthostatic Tachycardia Syndrome: A Systematic Review
Vasavada, A.M. et al.
Advait M Vasavada
Deepak Verma
Vineetha Sheggari
Srushti Ghetiya
Punith Chowdary Chirumamilla
Radhika A Kotak
Shruti Sagar Mahapatra
Tirath Patel
Manisha Jain
0
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0
10.7759/cureus.38887
Published in Cureus
The literature on pharmacologic treatments for postural orthostatic tachycardia syndrome (POTS) is inconsistent and unstandardized. Therefore, we aimed to evaluate choices in pharmacologic treatment options for POTS and the challenges encountered in the studies. We searched numerous databases like PubMed, Scopus, Embase, Web of Science, and Google Scholar for literature published before April 8, 2023. The search was done to retrieve potential peer-reviewed articles that explored drug therapy in POTS. Preferred Reporting Items for Systematic Review and Meta-Analysis (PRISMA) guidelines were used to conduct the systematic review. Of the 421 potential articles assessed, 17 met the inclusion criteria. Results demonstrated that pharmacologic treatment options for POTS were effective in reducing symptoms of POTS, but most of the studies were underpowered. Several were terminated due to various reasons. Midodrine ivabradine, bisoprolol, fludrocortisone, droxidopa, desmopressin, propranolol, modafinil, methylphenidate, and melatonin have been studied with positive impact but sample sizes that were low in the range of 10-50 subjects. Therefore, we concluded the treatment options effectively improve symptoms of POTS and increase orthostatic tolerance, but more evidence is needed as most studies had a low sample size and thus are underpowered.
"You're always fighting": the lived experience of people with postural orthostatic tachycardia syndrome (POTS)
Knoop, I. et al.
Iris Knoop
Lynn Dunwoody
0
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0
10.1080/09638288.2022.2071482
Published in Disability And Rehabilitation
Postural orthostatic tachycardia syndrome (POTS) is a debilitating and poorly understood disorder of the autonomic nervous system with many different causes, mostly seen in females of child-bearing age. This study used an illness representation framework to explore the lived experience of those living with a medical diagnosis of POTS. Six individuals (aged 20-42) were recruited from two POTS online support groups. Individual semi-structured interviews were used to explore the five illness representations of identity, cause, consequences, timelines, and cure/controllability. Data were analysed using interpretative phenomenological analysis (IPA). Lived experiences were characterised by four overarching themes: "Fighting to be heard", "My individual self-management toolbox", "A mixed bag of emotions", and "I'm expensive in so many ways". Individuals faced considerable physical, psychosocial and financial challenges and felt underserved by healthcare and support provision. Early diagnosis and recognition of symptoms, along with education on self-management may help reduce associated mental health burdens. A biopsychosocial conceptualisation of this condition may help lead to a more integrated approach to care.Implications for rehabilitationLiving with POTS impacts on all aspects of life, including work, family, and social relationships/activities, and has financial burdens.Family doctors need education on the existence of POTS and the importance of providing biopsychosocial support services.More timely diagnosis of POTS is required, with access to specialists who understand the burden of living with POTS.
Autonomic dysfunction and postural orthostatic tachycardia syndrome in post-acute COVID-19 syndrome
Fedorowski, A. et al.
Artur Fedorowski
Richard Sutton
0
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0
10.1038/s41569-023-00842-w
Published in Nature Reviews. Cardiology
The post-acute sequelae of COVID-19 present major problems for many patients, their physicians and the health-care system. They are unrelated to the severity of the initial infection, are often highly symptomatic and can occur after vaccination. Many sequelae involve cardiovascular autonomic dysfunction, with postural orthostatic tachycardia syndrome in 30% of individuals. Prognosis is unknown, and treatment is still unsatisfactory.
Noteworthy sequelae after COVID-19 pneumonia in a patient with heart failure due to cardiomyopathy
Utsugi, Y. et al.
Yui Utsugi
Shingo Kuwata
Shunichi Doi
Yukio Sato
Norio Suzuki
Masaki Izumo
Tomoo Harada
Yuki Ishibashi
Yoshihiro J Akashi
0
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0
10.1016/j.jccase.2022.12.006
Published in Journal Of Cardiology Cases
Coronavirus disease 2019 (COVID-19) is endemic worldwide. Cardiovascular disease, particularly myocarditis, is one of the most common comorbidities in patients with COVID-19. However, heart failure due to COVID-19-triggered cardiomyopathy is not well understood. Additionally, "pseudo" heart failure symptoms have been reported in patients with a compensated condition, in which the heart works well enough that symptoms are unnoticeable or very easy to manage. Here, we report a case of heart failure due to cardiomyopathy in a patient with COVID-19 and postural orthostatic tachycardia syndrome after heart failure treatment. Postural orthostatic tachycardia syndrome (POTS) symptoms after coronavirus disease 2019 may be mistaken for heart failure symptoms; thus, it is essential to suspect POTS when symptoms such as shortness of breath and palpitations are noted upon standing, along with the relevant physical findings.
Chiari I malformation management in patients with heritable connective tissue disorders
Clarke, J.E. et al.
Jamie E Clarke
Joshua M Reyes
Evan Luther
Vaidya Govindarajan
Jessica D Leuchter
Toba Niazi
John Ragheb
Shelly Wang
0
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0
10.1016/j.wnsx.2023.100173
Published in World Neurosurgery: X
Chiari malformation type I (CMI) is relatively common neurosurgical condition typically treated with posterior fossa decompression. However, the management of CMI in patients with heritable connective tissue disorders (CTDs), such as Ehlers-Danlos Syndrome, Marfan Syndrome, or Osteogenesis Imperfecta, involves a unique set of perioperative challenges. This study aims to define the demographic information, comorbidities, and perioperative course of patients with concomitant CMI and CTD. Patients with CMI admitted for surgical decompression from 2008 to 2015 were captured using the National Inpatient Sample (NIS). Information was collected based on ICD-9 codes. Descriptive and regression analyses were performed in SPSS (version 26). 38,169 CMI patients, 353 of whom had CTD (0.92%), were identified. CMI patients with CTD were more likely to be female ( < 0.001) and present during teenage ( = 0.033) or young adult years ( < 0.001). They had more chronic issues ( < 0.001): systemic comorbidities include postural orthostatic tachycardia syndrome, cardiac dysrhythmias, and gastroparesis (all < 0.001). CNS comorbidities include migraine, tethered spinal cord, and epilepsy (all < 0.001). They have increased joint instability (both < 0.001), as well as craniocervical instability (CCI). More posterior cervical fusion surgeries and application of cervical halo devices were seen during the same inpatient stay (both < 0.001). Patients with concurrent CTD and CMI were more likely to present with complex Chiari and associated CCI. They were also younger, more often female, and had more systemic, CNS, and joint abnormalities. As such, preoperative recognition of an underlying CTD is imperative to achieve optimal outcomes in this patient population.
Active Stand Testing for Identification of Postural Orthostatic Tachycardia Syndrome
Breslin, D. et al.
Deirdre Breslin
Pádraig Synnott
Colm Byrne
0
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0
10.7759/cureus.38281
Published in Cureus
A 67-year-old woman presented to the Emergency Department (ED) of a Level 4 Hospital with a history of "dizziness" on standing. The front-door frailty team, including a consultant geriatrician, reviewed her in the ED. An Active Stand test was performed on the day of attendance, identifying Postural Orthostatic Tachycardia Syndrome (POTS). This was attributed to underlying adrenal insufficiency and managed with oral steroids. "Dizziness" is a common presentation to Emergency Departments and can be challenging to investigate. This case report describes the application of Comprehensive Geriatric Assessment, the novel use of beat-to-beat non-invasive blood pressure monitoring in an Emergency Department setting, and the benefits for an individual patient presenting with a complaint of "dizziness". While the benefits of Comprehensive Geriatric Assessment have previously been described, our case report suggests that Active Stand testing in an ED setting may help clarify this presentation. Further research in this area could prove beneficial to patients.
A predictive model of response to metoprolol in children and adolescents with postural tachycardia syndrome
Xu, B.W. et al.
Bo-Wen Xu
Qing-You Zhang
Xue-Ying Li
Chao-Shu Tang
Jun-Bao Du
Xue-Qin Liu
Hong-Fang Jin
0
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0
10.1007/s12519-022-00677-4
Published in World Journal Of Pediatrics : Wjp
The present work was designed to explore whether electrocardiogram (ECG) index-based models could predict the effectiveness of metoprolol therapy in pediatric patients with postural tachycardia syndrome (POTS). This study consisted of a training set and an external validation set. Children and adolescents with POTS who were given metoprolol treatment were enrolled, and after follow-up, they were grouped into non-responders and responders depending on the efficacy of metoprolol. The difference in pre-treatment baseline ECG indicators was analyzed between the two groups in the training set. Binary logistic regression analysis was further conducted on the association between significantly different baseline variables and therapeutic efficacy. Nomogram models were established to predict therapeutic response to metoprolol. The receiver-operating characteristic curve (ROC), calibration, and internal validation were used to evaluate the prediction model. The predictive ability of the model was validated in the external validation set. Of the 95 enrolled patients, 65 responded to metoprolol treatment, and 30 failed to respond. In the responders, the maximum value of the P wave after correction (Pcmax), P wave dispersion (Pd), Pd after correction (Pcd), QT interval dispersion (QTd), QTd after correction (QTcd), maximum T-peak-to-T-end interval (Tpemax), and T-peak-to-T-end interval dispersion (Tped) were prolonged (all P < 0.01), and the P wave amplitude was increased (P < 0.05) compared with those of the non-responders. In contrast, the minimum value of the P wave duration after correction (Pcmin), the minimum value of the QT interval after correction (QTcmin), and the minimum T-peak-to-T-end interval (Tpemin) in the responders were shorter (P < 0.01, < 0.01 and < 0.01, respectively) than those in the non-responders. The above indicators were screened based on the clinical significance and multicollinearity analysis to construct a binary logistic regression. As a result, pre-treatment Pcmax, QTcmin, and Tped were identified as significantly associated factors that could be combined to provide an accurate prediction of the therapeutic response to metoprolol among the study subjects, yielding good discrimination [area under curve (AUC) = 0.970, 95% confidence interval (CI) 0.942-0.998] with a predictive sensitivity of 93.8%, specificity of 90.0%, good calibration, and corrected C-index of 0.961. In addition, the calibration curve and standard curve had a good fit. The accuracy of internal validation with bootstrap repeated sampling was 0.902. In contrast, the kappa value was 0.769, indicating satisfactory agreement between the predictive model and the results from the actual observations. In the external validation set, the AUC for the prediction model was 0.895, and the sensitivity and specificity were 90.9% and 95.0%, respectively. A high-precision predictive model was successfully developed and externally validated. It had an excellent predictive value of the therapeutic effect of metoprolol on POTS among children and adolescents.
Pathogenic mechanisms of post-acute sequelae of SARS-CoV-2 infection (PASC)
Sherif, Z.A. et al.
Zaki A Sherif
Christian R Gomez
Thomas J Connors
Timothy J Henrich
William Brian Reeves
RECOVER Mechanistic Pathway Task Force
0
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0
10.7554/eLife.86002
Published in E Life
COVID-19, with persistent and new onset of symptoms such as fatigue, post-exertional malaise, and cognitive dysfunction that last for months and impact everyday functioning, is referred to as Long COVID under the general category of post-acute sequelae of SARS-CoV-2 infection (PASC). PASC is highly heterogenous and may be associated with multisystem tissue damage/dysfunction including acute encephalitis, cardiopulmonary syndromes, fibrosis, hepatobiliary damages, gastrointestinal dysregulation, myocardial infarction, neuromuscular syndromes, neuropsychiatric disorders, pulmonary damage, renal failure, stroke, and vascular endothelial dysregulation. A better understanding of the pathophysiologic mechanisms underlying PASC is essential to guide prevention and treatment. This review addresses potential mechanisms and hypotheses that connect SARS-CoV-2 infection to long-term health consequences. Comparisons between PASC and other virus-initiated chronic syndromes such as myalgic encephalomyelitis/chronic fatigue syndrome and postural orthostatic tachycardia syndrome will be addressed. Aligning symptoms with other chronic syndromes and identifying potentially regulated common underlining pathways may be necessary for understanding the true nature of PASC. The discussed contributors to PASC symptoms include sequelae from acute SARS-CoV-2 injury to one or more organs, persistent reservoirs of the replicating virus or its remnants in several tissues, re-activation of latent pathogens such as Epstein-Barr and herpes viruses in COVID-19 immune-dysregulated tissue environment, SARS-CoV-2 interactions with host microbiome/virome communities, clotting/coagulation dysregulation, dysfunctional brainstem/vagus nerve signaling, dysautonomia or autonomic dysfunction, ongoing activity of primed immune cells, and autoimmunity due to molecular mimicry between pathogen and host proteins. The individualized nature of PASC symptoms suggests that different therapeutic approaches may be required to best manage specific patients.
A Case Study of Successful Application of the Principles of ME/CFS Care to an Individual with Long COVID
Petracek, L.S. et al.
Lindsay S Petracek
Camille A Broussard
Renee L Swope
Peter C Rowe
0
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0
10.3390/healthcare11060865
Published in Healthcare (Basel, Switzerland)
Persistent fatigue is one of the most common symptoms of post-COVID conditions, also termed long COVID. At the extreme end of the severity spectrum, some individuals with long COVID also meet the criteria for myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS), raising the possibility that symptom management approaches for ME/CFS may benefit some long COVID patients. We describe the long-term outcomes of a 19-year-old male who developed profound impairment consistent with ME/CFS after a SARS-CoV-2 infection early in the pandemic. We evaluated and treated him using our clinic's approach to ME/CFS. This included a history and physical examination that ascertained joint hypermobility, pathological reflexes, physical therapy maneuvers to look for a range of motion restrictions in the limbs and spine, orthostatic testing, and screening laboratory studies. He was found to have profound postural tachycardia syndrome, several ranges of motion restrictions, and mast cell activation syndrome. He was treated according to our clinic's guidelines for managing ME/CFS, which included manual physical therapy maneuvers and both non-pharmacologic measures and medications directed at postural tachycardia syndrome and mast cell activation. He experienced significant improvement in his symptoms over 30 months. His case emphasizes how the application of the principles of treating ME/CFS has the potential to provide a direction for treating long COVID.
Impact of the COVID-19 pandemic on clinical autonomic practice in Europe A survey of the European Academy of Neurology (EAN) and the European Federation of Autonomic Societies (EFAS)
Fanciulli, A. et al.
Alessandra Fanciulli
Fabian Leys
Magdalena Krbot Skorić
Diogo Reis Carneiro
Giovanna Calandra-Buonaura
Jennifer Camaradou
Giacomo Chiaro
Pietro Cortelli
Cristian Falup-Pecurariu
Roberta Granata
Pietro Guaraldi
Raimund Helbok
Max J Hilz
Valeria Iodice
Jens Jordan
Evert C A Kaal
Anita Kamondi
Anne Pavy Le Traon
Isabel Rocha
Johann Sellner
Jean Michel Senard
Astrid Terkelsen
Gregor K Wenning
Elena Moro
Thomas Berger
Roland D Thijs
Walter Struhal
Mario Habek
Collaborators of European Network of Neurological ANS laboratories
0
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0
10.1111/ene.15787
Published in European Journal Of Neurology
To investigate the impact of the coronavirus-disease-2019 (COVID-19) pandemic on European clinical autonomic practice. Eighty-four neurology-driven or interdisciplinary autonomic centers in 22 European countries were invited to fill in a web-based survey between September and November 2021. Forty-six centers completed the survey (55%). During the first pandemic year, the number of performed tilt-table tests, autonomic outpatient and inpatient visits decreased respectively by 50%, 45% and 53%, and every-third center reported major adverse events due to postponed examinations or visits. The most frequent newly-diagnosed or worsened cardiovascular autonomic disorders after COVID-19 infection included postural orthostatic tachycardia syndrome (POTS), orthostatic hypotension, and recurrent vasovagal syncope, deemed likely related to the infection by ≥50% of the responders. Forty-seven percent of the responders also reported about people with new-onset of orthostatic intolerance, but negative tilt-table findings, and 16% about people with psychogenic pseudosyncope after COVID-19. Most patients were treated non-pharmacologically and symptomatic recovery at follow-up was observed in ≥45% of cases. By contrast, low frequencies of newly-diagnosed cardiovascular autonomic disorders following COVID-19 vaccination were reported, most frequently POTS and recurrent vasovagal syncope, and most of the responders judged a causal association unlikely. Non-pharmacological measures were the preferred treatment choice, with 50-100% recovery rates at follow-up. Cardiovascular autonomic disorders may develop or worsen following a COVID-19 infection, while the association with COVID-19 vaccines remains controversial. Despite the severe pandemic impact on European clinical autonomic practice, a specialized diagnostic work-up was pivotal to identify non-autonomic disorders in people with post-COVID-19 orthostatic complaints.
Long COVID: major findings, mechanisms and recommendations
Davis, H.E. et al.
Hannah E Davis
Lisa McCorkell
Julia Moore Vogel
Eric J Topol
0
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0
10.1038/s41579-022-00846-2
Published in Nature Reviews. Microbiology
Long COVID is an often debilitating illness that occurs in at least 10% of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infections. More than 200 symptoms have been identified with impacts on multiple organ systems. At least 65 million individuals worldwide are estimated to have long COVID, with cases increasing daily. Biomedical research has made substantial progress in identifying various pathophysiological changes and risk factors and in characterizing the illness; further, similarities with other viral-onset illnesses such as myalgic encephalomyelitis/chronic fatigue syndrome and postural orthostatic tachycardia syndrome have laid the groundwork for research in the field. In this Review, we explore the current literature and highlight key findings, the overlap with other conditions, the variable onset of symptoms, long COVID in children and the impact of vaccinations. Although these key findings are critical to understanding long COVID, current diagnostic and treatment options are insufficient, and clinical trials must be prioritized that address leading hypotheses. Additionally, to strengthen long COVID research, future studies must account for biases and SARS-CoV-2 testing issues, build on viral-onset research, be inclusive of marginalized populations and meaningfully engage patients throughout the research process.
COVID-19 Induced Postural Orthostatic Tachycardia Syndrome (POTS): A Review
Mallick, D. et al.
Deobrat Mallick
Lokesh Goyal
Prabal Chourasia
Miana R Zapata
Kanica Yashi
Salim Surani
0
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0
10.7759/cureus.36955
Published in Cureus
POTS (Postural Orthostatic Tachycardia Syndrome) is a multisystem disorder characterized by the abnormal autonomic response to an upright posture, causing orthostatic intolerance and excessive tachycardia without hypotension. Recent reports suggest that a significant percentage of COVID-19 survivors develop POTS within 6 to 8 months of infection. Prominent symptoms of POTS include fatigue, orthostatic intolerance, tachycardia, and cognitive impairment. The exact mechanisms of post-COVID-19 POTS are unclear. Still, different hypotheses have been given, including autoantibody production against autonomic nerve fibers, direct toxic effects of SARS-CoV-2, or sympathetic nervous system stimulation secondary to infection. Physicians should have a high suspicion of POTS in COVID-19 survival when presented with symptoms of autonomic dysfunction and should conduct diagnostic tests like the Tilt table and others to confirm it. The management of COVID-19-related POTS requires a comprehensive approach. Most patients respond to initial non-pharmacological options, but when the symptoms become more severe and they do not respond to the non-pharmacological approach, pharmacological options are considered. We have limited understanding and knowledge of post-COVID-19 POTS, and further research is warranted to improve our understanding and formulate a better management plan.
Experiences of living with postural tachycardia syndrome
Waterman, S. et al.
Samantha Waterman
Morwenna Opie
Debbie Waterman
Dawn Langdon
0
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0
10.1177/17423953211054032
Published in Chronic Illness
Postural tachycardia syndrome (PoTS) is a disorder of the autonomic nervous system which involves a range of symptoms, worsened when adopting an orthostatic (upright) position. Symptoms can include tachycardia, dizziness, fainting, nausea as well as many others which, as is typical of a syndrome, vary from person to person. Although research is increasing into this condition, the unifying experiences of managing it on a daily basis have not been extensively investigated. This study aimed to capture participants' experiences of living with PoTS. A longitudinal digital ethnographic approach was employed. Eight participants recorded daily video diaries discussing their experiences of PoTS and its impact for 17 days. Interpretative phenomenological analysis was utilised to analyse the data and identify connections across participants' accounts. Four superordinate themes emerged: 'loss of control and lack of agency over body', 'identity changes', 'lack of understanding from others' and 'adapting to cope with PoTS'. The findings demonstrated the complex and widespread impact these participants experience from their PoTS symptoms, including the consequent emotional difficulties that result from managing this condition. An overall lack of understanding about PoTS by others was emphasised, suggesting the requirement for better education and support services for this condition.
Transcutaneous vagus nerve stimulation attenuates autoantibody-mediated cardiovagal dysfunction and inflammation in a rabbit model of postural tachycardia syndrome
Deng, J. et al.
Jielin Deng
Hongliang Li
Yankai Guo
Gege Zhang
Hayley Fischer
Stavros Stavrakis
Xichun Yu
0
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0
10.1007/s10840-022-01144-w
Published in Journal Of Interventional Cardiac Electrophysiology : An International Journal Of Arrhythmias And Pacing
Previous studies demonstrated M2 muscarinic acetylcholine receptor-activating autoantibodies (M2R-AAb) were present in some patients with postural tachycardia syndrome (POTS). This study examines how these autoantibodies might contribute to the pathophysiology of POTS, and whether low-level tragus stimulation (LLTS) can ameliorate autoantibody-mediated autonomic dysregulation in the rabbit. Five New Zealand white rabbits were immunized with a M2R second extracellular loop peptide to produce cholinomimetic M2R-AAb. Tilt test and infusion studies were performed on conscious rabbits before immunization, 6 weeks after immunization, and 8 weeks after immunization with 2-week daily LLTS treatment. Each rabbit served as its own control. Compared to preimmune state, an enhanced heart rate increase and decreased parasympathetic activity upon tilting were observed in immunized rabbits. Furthermore, these rabbits demonstrated an attenuated heart rate-slowing response to infusion of the M2R orthosteric agonist arecaidine propargyl ester (APE), suggesting an inhibitory allosteric effect of M2R-AAb. There was also a significant increase in serum inflammatory cytokines in immunized rabbits. LLTS treatment suppressed the postural tachycardia, improved the sympathovagal balance with increased acetylcholine secretion, reduced the levels of inflammatory cytokines, and reversed the attenuated heart rate response to APE in immunized rabbits. No suppression of M2R-AAb expression by LLTS was found during this short-term study period. Receptor-modulating activity of M2R-AAb produced in immunized rabbits was confirmed with in vitro bioassay. Autoantibody inhibition of cholinergic ligand activity may be involved in the development of cardiovagal dysfunction and inflammation associated with POTS, both of which can be improved by vagal stimulation.
Skin biopsy reveals generalized small fibre neuropathy in hypermobile Ehlers-Danlos syndromes
Igharo, D. et al.
Denver Igharo
Joana C Thiel
Roman Rolke
Merve Akkaya
Joachim Weis
Istvan Katona
Jörg B Schulz
Andrea Maier
0
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0
10.1111/ene.15649
Published in European Journal Of Neurology
Ehlers-Danlos syndromes are hereditary disorders of connective tissue that are characterized by joint hypermobility, skin hyperextensibility and tissue fragility. The most common subtype is the hypermobile type. In addition to symptoms of small fibre neuropathy (SFN) due to damage to the small peripheral nerve fibres, with degeneration of the distal nerve endings, autonomic disorders such as postural tachycardia syndrome (PoTS) are frequently reported features in patients with hypermobile Ehlers-Danlos syndrome (hEDS). To date, the underlying pathophysiological mechanisms are still not completely understood. To better understand pathophysiological mechanisms of small fiber neuropathy and autonomic neuropathy in hypermobile Ehlers-Danlos Syndromes. We prospectively investigated 31 patients with hEDS compared to 31 healthy controls by using skin biopsy, quantitative sensory testing, tilt-table testing, the painDetect, Small Fibre Neuropathy Screening List and the COMPASS-31 (Composite Autonomic Symptom Score 31) questionnaire. Nineteen (61%) patients with hEDS were diagnosed with SFN, and 10 (32%) fulfilled the criteria for PoTS. Patients with hEDS had significantly higher heart rates than controls. According to quantitative sensory testing, these patients had generalized thermal and tactile hypesthesia. Skin biopsy revealed significantly reduced intraepithelial nerve fibre density proximally (thigh) and distally (lower leg) in patients compared to controls. This was consistent with various complaints of pain and sensory disturbances in both the proximal and distal body regions. These results confirm histologically proven SFN as a common feature in patients with hEDS, revealing a generalized distribution of nerve fibre loss. Regarding the frequently reported autonomic and neuropathic dysfunctions, the findings support SFN as an important, but not the only, underlying pathomechanism.
Post-acute sequelae of SARS-CoV-2 syndrome presenting as postural orthostatic tachycardia syndrome
Diekman, S. et al.
Sarah Diekman
Tae Chung
0
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0
10.15441/ceem.22.409
Published in Clinical And Experimental Emergency Medicine
The novel SARS-CoV-2 emerged in 2019, and the global COVID-19 pandemic continues into 2022. It has been known that a subset of patients develops chronic, debilitating symptoms after otherwise complete recovery from acute infection of COVID-19. Multiple terms have been used to describe this constellation of symptoms, including long COVID, long-haul COVID, and postacute sequelae of SARS-CoV-2 syndrome (PASC). PASC is broadly defined as a wide range of new, returning, or ongoing symptoms at least four weeks after infection. Those patients are often seen in emergency departments after acute COVID-19 infection, but their symptoms are not adequately managed because the underlying pathophysiology of PASC is not well understood. Among patients with PASC, postural orthostatic tachycardic syndrome (POTS) has been increasingly recognized. POTS is one of the most common forms of autonomic dysfunction and defined by a sustained orthostatic tachycardia during active standing or head-up tilt test in the absence of orthostatic hypotension or other cardiopulmonary diseases. Because POTS is a treatable condition, it is important to recognize POTS among PASC patients. Herein, we reviewed the current literature on POTS and dysautonomia in PASC in order to better understand the overlap and distinction between these pathologies.
Patient-Centered Paradigm for Managing Autonomic Long COVID Symptoms During Sports and Exercise
Messinger-Rapport, B. et al.
Barbara Messinger-Rapport
Blair Grubb
0
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0
10.1097/JSM.0000000000001093
Published in Clinical Journal Of Sport Medicine : Official Journal Of The Canadian Academy Of Sport Medicine
This report highlights a new, patient-centered paradigm for managing post-COVID-19 dysautonomia symptoms during sports and exercise. The patient was a healthcare worker exposed before vaccination. She experienced postural orthostatic tachycardia plus exertional tachycardia, with postexertional fatigue, beginning a few weeks after testing positive for COVID-19. Stress test, echo, and an extensive dysautonomia evaluation were negative. Recommended nonpharmacological and pharmacological interventions were poorly tolerated. Prescription of a novel regimen of "basal-dose" ivabradine, plus very low-dose metoprolol according to an exertional "sliding scale" managed symptoms to an acceptable level for work and recreation.
Manual Therapy and Exercise for the Management of Chronic Neck Pain With Multiple Neurovascular Comorbidities: A Case Report
Mayo, Z. et al.
Zach Mayo
Christopher Keating
0
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0
10.7759/cureus.36961
Published in Cureus
This case describes the clinical decision-making behind the conservative clinical management of an individual presenting with chronic neck pain with myriad neuromuscular comorbidities. The focus of this case report is to support the safe utilization of manual therapy and describe the tolerable prescription of strength and endurance exercise in a patient with numerous complications to improve self-efficacy. A 22-year-old female college student presented with a chief complaint of chronic, non-specific neck pain with comorbid Chiari malformation, migraines, upper cervical spinal fusion, Ehlers-Danlos syndrome (EDS), and postural orthostatic tachycardia syndrome (POTS) to an outpatient physical therapy clinic for evaluation and treatment. Following four sessions of physical therapy treatment, no clinically significant improvement in the individual's symptoms and daily function was achieved. Despite the lack of measurable change, the patient reported the program's value on her ability to self-manage her complex condition. The patient responded well to manual therapy, specifically thrust manipulations. In addition, both endurance and strengthening exercises were well tolerated and provided a measure of self-management that may not have been achieved before physical therapy management. This case report highlights the need for exercise and pain-modulating interventions in highly complex individuals to reduce medical intervention by advancing the patient's self-efficacy. There is a need for further research about the utility of standardized outcome measures, joint manipulations, and the addition of cervico-ocular exercises for those who present with neck pain and pertinent neuromuscular comorbidities.
Orthostatic Intolerance and Chronotropic Incompetence in Patients With Myalgic Encephalomyelitis or Chronic Fatigue Syndrome
Miwa, K.
Kunihisa Miwa
0
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0
10.1253/circrep.CR-22-0114
Published in Circulation Reports
Orthostatic intolerance markedly affects the day-to-day activities of patients with myalgic encephalomyelitis (ME) or chronic fatigue syndrome. Chronotropic incompetence (CI), defined as an impaired chronotropic response or reduced increases in heart rate during exercise and resulting in lower exercise capacity, may also be observed during orthostasis in patients with ME. In this study, the recordings of 101 adult patients with ME (36 men, 65 women; mean [±SD] age 37±12 years) who underwent conventional active 10-min standing tests at least 3 times to determine the presence of CI were analyzed. Recordings were selected for 13 patients who experienced tests both with and without exhibiting postural orthostatic tachycardia syndrome (POTS; an increase in heart rate of ≥30 beats/min or an actual heart rate of ≥120 beats/min) while also both successfully completing and failing to complete 10-min standing on different occasions. Subjects in whom failure without POTS was observed in any test(s) while success was associated with POTS on other occasions were considered positive for CI during orthostasis. Of the 13 patients, 12 (92%) were CI positive, 5 (38%) of whom exclusively failed the tests without experiencing POTS. Some patients with ME were CI positive during standing tests, suggesting impaired sympathetic activation. The presence of POTS appears to be essential for maintaining orthostasis in these patients.
Therapeutic Approaches to Dysautonomia in Childhood, with a Special Focus on Long COVID
Buchhorn, R.
Reiner Buchhorn
0
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0
10.3390/children10020316
Published in Children (Basel, Switzerland)
Dysautonomia seems to be important for the pathophysiology of psychosomatic diseases and, more recently, for long COVID. This concept may explain the clinical symptoms and could help open new therapeutic approaches. We compared our data from an analysis of heart rate variability (HRV) in an active standing test in 28 adolescents who had developed an inappropriate sinus tachycardia (IST, = 13) or postural orthostatic tachycardia syndrome (POTS, = 15) after contracting COVID-19 disease and/or vaccination with 64 adolescents from our database who developed dysautonomia due to psychosomatic diseases prior to the COVID-19 pandemic. We prove the effects of our treatment: omega-3 fatty acid supplementation (O3-FA, = 18) in addition to propranolol (low dose, up to 20-20-0 mg, = 32) or ivabradine 5-5-0 mg ( = 17) on heart rate regulation and heart rate variability (HRV). The HRV data were not different between the adolescents with SARS-CoV-2-related disorders and the adolescents with dysautonomia prior to the pandemic. The heart rate increases in children with POTS while standing were significantly lower after low-dose propranolol (27.2 ± 17.4 bpm***), ivabradine (23.6 ± 8.12 bpm*), and O-3-FA (25.6 ± 8.4 bpm*). The heart rate in children with IST while lying/standing was significantly lower after propranolol (81.6 ± 10.1 bpm**/101.8 ± 18.8***), ivabradine (84.2 ± 8.4 bpm***/105.4 ± 14.6**), and O-3-FA (88.6 ± 7.9 bpm*/112.1/14.9*). The HRV data of adolescents with dysautonomia after COVID-19 disease/vaccination are not significantly different from a historical control of adolescents with dysautonomia due to psychosomatic diseases prior to the pandemic. Low-dose propranolol > ivabradine > omega-3 fatty acids significantly decrease elevated heart rates in patients with IST and the heart rate increases in patients with POTS and may be beneficial in these children with dysautonomia.
10.1016/j.ncl.2022.08.002
Published in Neurologic Clinics
Dysautonomias are a heterogenous group of disorders that can cause variable symptoms ranging from isolated impairment of one autonomic function to multisystem failure. The causes are also diverse and can be central or peripheral and primary (owing to an intrinsic neurologic cause) or secondary (owing to a disorder that secondarily causes damage to the autonomic nervous system). This review covers common phenotypes of dysautonomias, primary and secondary causes, initial clinical workups, interpretation of common autonomic tests, and first-line treatments. A brief review of autonomic impairment associated with acute and long-COVID is also presented.
Sex adjusted standardized prevalence ratios for celiac disease and other autoimmune diseases in patients with postural orthostatic tachycardia syndrome (POTS): A systematic review and meta-analysis
Mannan, H. et al.
Haider Mannan
Clare M Pain
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10.1016/j.heliyon.2023.e12982
Published in Heliyon
Standardised sex-adjusted prevalence ratios (SSPRs) have not been published for any autoimmune diseases (ADs) in patients with Postural Orthostatic Tachycardia Syndrome (POTS), who are predominantly young females. We performed a systematic review according to PRISMA guidelines of POTS cohorts reporting the prevalence of at least one AD. Only four studies were found: two providing data on celiac disease; and two with data on 'any AD', Hashimoto's thyroiditis, rheumatoid arthritis, systemic lupus erythematosus (SLE), Sjögren's syndrome and celiac disease and (one study) antiphospholipid syndrome. All studies were assessed as being at high risk of bias for estimating AD prevalence in POTS patients, with under-reporting of ADs likely due to the lack of rigorous prospective screening for ADs. A literature search found a 'gold standard' general population (GP) comparator only for celiac disease in the United States, leading to a pooled SSPR in POTS patients of 2.75 with 95% confidence interval (1.06-4.40). The lack of recent high-quality studies on GP prevalence for the other ADs was noteworthy. Exploratory pooled SSPRs were calculated for 'any AD' and for the other five ADs using GP comparator data from a comprehensive review. All pooled SSPRs were greater than one and statistically significant, implying a higher prevalence of these ADs, and any AD, in POTS patients. The magnitude of the exploratory SSPRs was very large for SLE, Sjögren's syndrome and antiphospholipid syndrome, perhaps reflecting the use of non-gold standard GP comparators, which may underestimate AD prevalence. Further research in a large POTS cohort with an appropriately age- and sex-matched GP control group is recommended, to confirm the SSPR for celiac disease and to determine whether SLE, Sjogren's syndrome and antiphospholipid syndrome are indeed many times more prevalent in POTS patients than in the GP. The findings are consistent with POTS itself being an AD.
Management of Post-Viral Postural Orthostatic Tachycardia Syndrome With Craniosacral Therapy
Tafler, L. et al.
Leonid Tafler
Aysham Chaudry
Heejin Cho
Angeles Garcia
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10.7759/cureus.35009
Published in Cureus
Postural Orthostatic Tachycardia Syndrome (POTS) is a rare disorder of the autonomic nervous system. The number of people afflicted with this dysautonomia has increased dramatically in recent years due to the long-term effects of coronavirus disease (COVID-19); however, it is largely underdiagnosed. This case report is about a patient with post-viral neuropathic POTS. Neuropathic POTS is believed to be due to the damage of small nerve fibers that regulate the constriction of the blood vessels in the limb and abdomen, which leads to interference with vasoconstriction, and therefore causes tachycardia. Current literature emphasizes a treatment that is based on lifestyle modifications, such as increasing water and salt intake, and symptomatic pharmacological treatment. In this case, the 39-year-old male ptient was treated with osteopathic manipulative treatment (OMT), specifically the compression of the fourth ventricle (CV4), which has been associated with the production of hyperparasympathetic and anti-inflammatory effects and, hence, helps overcome the small-fiber neuropathy caused by the viral illness. We found that the CV4 technique led to the successful remission of the patient's symptoms. Therefore, we propose craniosacral therapy as a successful single management modality in patients with POTS.
Postural orthostatic tachycardia syndrome: New concepts in pathophysiology and management
Grubb, A.F. et al.
Alex F Grubb
Blair P Grubb
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10.1016/j.tcm.2021.10.007
Published in Trends In Cardiovascular Medicine
Postural orthostatic tachycardia syndrome (POTS) is a common and therapeutically challenging condition affecting numerous people worldwide. Recent studies have begun to shed light on the pathophysiology of this disorder. At the same time, both non-pharmacologic and pharmacologic therapies have emerged that offer additional treatment options for those afflicted with this condition. This paper reviews new concepts in both the pathophysiology and management of POTS.