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Postural orthostatic tachycardia syndrome (POTS), one of the most common autonomic disorders, is associated with significant morbidity and functional impairment. Although several possible etiologies have been proposed, autoimmunity has emerged as one of the leading causes with various specific and non-specific antibodies identified in patients with POTS. Treatment with intravenous immunoglobulin has been previously described. We present a case series of patients with severe POTS refractory to the standard pharmacologic and non-pharmacologic therapies treated with immunotherapy consisting of either subcutaneous immunoglobulin (SCIG) therapy or plasmapheresis (PLEX) and report their treatment outcomes.Clinical history of 7 patients with POTS who were treated with SCIG or PLEX was reviewed. Response to treatment was assessed using COMPASS 31 and functional ability scale (FAS), completed by patients retrospectively, pre- and 3-12 months post-treatment with SCIG or PLEX.All patients improved following SCIG or PLEX with an average 50% reduction in COMPASS 31 score and 217% increase in FAS scores. Six out of seven patients were able to reduce or discontinue oral medications for POTS, and five patients were able to return to work or school. Four patients had skin biopsy or quantitative sudomotor axon reflex test (QSART)-proven small fiber neuropathy, and five had various positive antibodies at low titers.Patients with severe, treatment-refractory POTS experienced significant functional improvement with reduction in the autonomic symptoms following immunotherapy with SCIG or PLEX. Randomized controlled trials of SCIG and/or PLEX are needed to determine the efficacy and safety of these long-term therapies in patients with POTS.

Objectives Postural orthostatic tachycardia syndrome (POTS) is a chronic disorder of the autonomic nervous system that is associated with orthostatic intolerance (OI) and gastrointestinal (GI) symptoms. We aimed to determine if a gluten-free diet (GFD) may be an effective treatment in patients with POTS. Methods Patients with confirmed POTS and without celiac disease who followed GFD for 4 weeks retrospectively completed pre- and post- GFD COMPASS-31 questionnaires. Paired-samples t-tests were conducted to compare COMPASS-31 scores before and after adopting GFD. Results All 20 patients (all females, age 16–62 years (mean age 33)), had POTS symptoms for 1–30 years (mean 10.8, SD = 10.23 years). Eleven patients had co-morbid mast cell activation syndrome, and 8 had hypermobile Ehlers-Danlos syndrome. Pre-GFD COMPASS-31 scores (mean 57.8) were significantly higher than post-GFD scores (mean 38.2, t(19) = -8.92, p Discussion GFD may be effective in reducing the symptom burden in patients with POTS, particularly in the OI, vasomotor, and GI symptom domains. Large prospective studies are necessary to confirm whether GFD is an effective long-term treatment option for patients.

A Response to Call for “A New Paradigm” for Long COVID in Lancet Respiratory Medicine

To describe clinical features, diagnostic findings, treatments, and outcomes in patients with new-onset postural orthostatic tachycardia syndrome (POTS) and other autonomic disorders following SARS-CoV-2 infection (COVID-19). We retrospectively reviewed medical records for patients who presented with persistent neurologic and cardiovascular complaints between April and December 2020 following COVID-19 infection. Twenty patients (70% female) were included in this study.Fifteen had POTS, 3 had neurocardiogenic syncope, and 2 had orthostatic hypotension. Six patients had abnormalities on cardiac or pulmonary testing, and 4 had elevated autoimmune or inflammatory markers. All patients were treated with non-pharmacologic therapies, and most required pharmacologic therapies. Six to 8 months after COVID-19, 17 (85%) patients had residual autonomic symptoms, with 12 (60%) unable to return to work. POTS can follow COVID-19 in previously healthy patients. Appropriate diagnostic investigations and therapies are necessary to identify and treat autonomic dysfunction after COVID-19.

Background Postural tachycardia syndrome (POTS) is a heterogenous disorder of the autonomic nervous system that is commonly associated with small fiber neuropathy, Ehlers-Danlos Syndrome and autoimmune disorders, but association with rare conditions may also occur. Methods Reported here are clinical features, diagnostic tests and treatment outcomes of 6 unique patients who presented with POTS and were subsequently diagnosed with Fabry disease, McArdle disease, Complex V mitochondrial disease, carcinoid tumor, Hodgkin’s lymphoma and chemotherapy-induced neuropathy. Results All patients (age range 15-57 years, 3 females, 3 males) presented with orthostatic intolerance of at least 6 months duration, and all patients had co-morbid small fiber neuropathy. Five patients presented with symptoms of POTS months to years before the underlying or associated medical condition was discovered, and three out of six patients experienced either complete resolution or significant improvement of POTS after treatment of the underlying or associated medical condition. Conclusion In rare cases, POTS can present as a possible manifestation of genetic, neoplastic or neurotoxic disorders. Unusual clinical features that fall outside of the typical spectrum of dysautonomia can point toward the presence of another disorder and help guide further diagnostic investigation.

Objective Previous observations suggest an association between Ehlers-Danlos syndrome (EDS) and gastrointestinal (GI), cardiovascular, immune, and autonomic nervous system dysfunction. We sought to determine whether a hospital diagnosis of EDS is associated with a higher prevalence of these manifestations vs hospitalized patients without EDS. We also evaluated hospital outcomes. Methods A total of 6,021 cases and matched controls were acquired from the 2016 National Inpatient Sample. In total, 2,007 EDS patients were identified via ICD-10 code. After bivariate analyses, multivariate logistic regression models were used to adjust for potential confounders. Results GI conditions were found in 44% of EDS patients vs 18% of controls [odds ratio (OR) = 3.57, 95% CI: 3.17, 4.02, P < 0.0001], with irritable bowel syndrome, gastroparesis and coeliac disease strongly associated with EDS. Autonomic dysfunction, including postural orthostatic tachycardia syndrome (POTS), neurocardiogenic syncope and orthostatic hypotension was found in 20% of EDS patients vs 6% of controls (OR = 4.45, 95% CI: 3.71, 5.32, P < 0.0001). EDS patients were more likely to have food allergy (OR = 3.88, 95% CI: 2.65, 5.66, P Conclusion GI, cardiovascular, autonomic and allergic manifestations are significantly more prevalent in EDS patients compared with hospitalized patients without EDS. Physicians should consider EDS in patients with unexplained GI, cardiovascular, autonomic and allergic conditions and exercise precautions when treating EDS patients in a hospital setting.

Thiamine (vitamin B1), a water-soluble vitamin that plays a pivotal role in energy metabolism, growth, development and the function of cells. Has been found to be deficient in conditions such as ma...

To describe clinical features, diagnostic findings, treatments, and outcomes in patients with new-onset postural orthostatic tachycardia syndrome (POTS) and other autonomic disorders following SARS-CoV-2 infection (COVID-19).We retrospectively reviewed medical records for patients who presented with persistent neurologic and cardiovascular complaints between April and December 2020 following COVID-19 infection.Twenty patients (70% female) were included in this study.Fifteen had POTS, 3 had neurocardiogenic syncope, and 2 had orthostatic hypotension. Six patients had abnormalities on cardiac or pulmonary testing, and 4 had elevated autoimmune or inflammatory markers. All patients were treated with non-pharmacologic therapies, and most required pharmacologic therapies. Six to 8 months after COVID-19, 17 (85%) patients had residual autonomic symptoms, with 12 (60%) unable to return to work.POTS can follow COVID-19 in previously healthy patients. Appropriate diagnostic investigations and therapies are necessary to identify and treat autonomic dysfunction after COVID-19.

In the recently published paper, “Autonomic dysfunction and HPV immunization: an overview”, the last name of the lead author is listed incorrectly. The author’s name is Svetlana Blitshteyn.

This article reviews the case series reported from several countries describing patients with suspected severe side effects to the HPV vaccines. The described symptom clusters are remarkably similar and include disabling fatigue, headache, widespread pain, fainting, gastrointestinal dysmotility, limb weakness, memory impairment episodes of altered awareness, and abnormal movements. This constellation of symptoms and signs has been labeled with different diagnoses such as complex regional pain syndrome (CRPS), postural orthostatic tachycardia syndrome (POTS), small fiber neuropathy (SFN), myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS), or fibromyalgia. It is known that autoimmunity and autoantibodies are present in a subset of patients with CRPS, POTS, SFN, ME/CFS, and fibromyalgia. This article proposes that vaccine-triggered, immune-mediated autonomic dysfunction could lead to the development of de novo post-HPV vaccination syndrome possibly in genetically susceptible individuals. Being cognizant that a temporal relationship between vaccination and symptom onset does not necessarily equate to causality, mounting evidence of case series calls for well-designed case-control studies to determine the prevalence and possible causation between these symptom clusters and HPV vaccines. Since personalized medicine is gaining momentum, the use of adversomics and pharmacogenetics may eventually help identify individuals who are predisposed to HPV vaccine adverse events.

In the last decade, a group of chronic disorders associated with fatigue (CDAF) emerged as the leading cause of chronic fatigue, chronic pain, and functional impairment, all of which have been often labeled in clinical practice as chronic fatigue syndrome (CFS) or fibromyalgia. While these chronic disorders arise from various pathophysiologic mechanisms, a shared autoimmune or immune-mediated etiology could shift the focus from symptomatic treatment of fatigue and pain to targeted immunomodulatory and biological therapy. A clinical paradigm shift is necessary to reevaluate CFS and fibromyalgia diagnoses and its relationship to the CDAF entities, which would ultimately lead to a change in diagnostic and therapeutic algorithm for patients with chronic fatigue and chronic pain. Rather than uniformly apply the diagnoses of CFS or fibromyalgia to any patient presenting with unexplained chronic fatigue or chronic pain, it may be more beneficial and therapeutically effective to stratify these patients into more specific diagnoses in the CDAF group.

We describe a young woman who developed POTS with positive serum anti-NMDA receptor antibodies and no evidence of encephalitis after vaccination with HPV vaccine, Cervarix. Her symptoms improved significantly with immunomodulatory therapy and re-occurred after immunomodulatory therapy was stopped, suggesting an autoimmune etiology of POTS after vaccination.

Objective: POTS is a heterogeneous disorder of the autonomic nervous system that can result from multiple etiologies. An increased prevalence of vitamin B12, vitamin D 25-OH and iron deficiencies has been observed in patients with POTS. This study examined the prevalence of vitamin B1 deficiency and assessed response to vitamin B1 supplementation in the deficient POTS patients.Methods and Results: Medical records of 65 consecutive patients with POTS evaluated at our clinic were reviewed. In this cohort (mean age 32, range 13–54 years; 89% female), 6% had vitamin B1 deficiency, and one of four deficient patients experienced significant improvement of POTS after oral vitamin B1 supplementation.Conclusion: A small subset of patients with POTS may have vitamin B1 deficiency. Testing for vitamin B1 deficiency and correcting the deficiency is recommended.

Postural orthostatic tachycardia syndrome (POTS) is a heterogeneous autonomic disorder characterised by orthostatic intolerance and a rise in heart rate by at least 30 bpm or an absolute heart rate value of at least 120 bpm within 10 min of standing or during a tilt table test. Overwhelmingly, POTS affects young Caucasian women, which can lead physicians to miss the diagnosis in men or non-white patients. We describe a case of 29-year-old African-American man who developed lightheadedness, generalised weakness, tachycardia and palpitations and was subsequently diagnosed with POTS. We review its clinical features, differential diagnosis, pathophysiology and treatment options. We also emphasise that POTS should be considered as a differential diagnosis in any patient presenting with typical clinical features, who may not be in the usual demographics of the disorder.

Objective We investigated whether dietary sodium intake from respondents of a national cross‐sectional nutritional study differed by history of migraine or severe headaches. Background Several lines of evidence support a disruption of sodium homeostasis in migraine. Design Our analysis population was 8819 adults in the 1999–2004 National Health and Nutrition Examination Survey (NHANES) with reliable data on diet and headache history. We classified respondents who reported a history of migraine or severe headaches as having probable history of migraine. To reduce the diagnostic conflict from medication overuse headache, we excluded respondents who reported taking analgesic medications. Dietary sodium intake was measured using validated estimates of self‐reported total grams of daily sodium consumption and was analyzed as the residual value from the linear regression of total grams of sodium on total calories. Multivariable logistic regression that accounted for the stratified, multistage probability cluster sampling design of NHANES was used to analyze the relationship between migraine and dietary sodium. Results Odds of probable migraine history decreased with increasing dietary sodium intake (odds ratio = 0.93, 95% confidence interval = 0.87, 1.00, P = .0455). This relationship was maintained after adjusting for age, sex, and body mass index (BMI) with slightly reduced significance (P = .0505). In women, this inverse relationship was limited to those with lower BMI (P = .007), while in men the relationship did not differ by BMI. We likely excluded some migraineurs by omitting frequent analgesic users; however, a sensitivity analysis suggested little effect from this exclusion. Conclusions This study is the first evidence of an inverse relationship between migraine and dietary sodium intake. These results are consistent with altered sodium homeostasis in migraine and our hypothesis that dietary sodium may affect brain extracellular fluid sodium concentrations and neuronal excitability.

ObjectiveIn recent years, there have been a number of studies suggesting that POTS may have an autoimmune etiology. This study examined whether the prevalence of antinuclear antibodies (ANA), other markers of autoimmunity and co-morbid autoimmune disorders is higher in patients with POTS than in the general population.Methods and resultsMedical records of 100 consecutive patients with POTS evaluated at our clinic were reviewed. In this cohort (90% females, mean age 32, range 13–54 years), 25% had positive ANA, 7% had at least one positive aPL antibody and 31% had markers of autoimmunity. When compared to the general population, patients with POTS had a higher prevalence of ANA (25% vs. 16%, OR 1.8, CI 1.1–2.8, p ConclusionPatients with POTS have a higher prevalence of autoimmune markers and co-morbid autoimmune disorders than the general population. One in four patients have positive ANA, almost one in three have some type of autoimmune marker, one in five have a co-morbid autoimmune disorder, and one in nine have Hashimoto’s thyroiditis.

Background Antiphospholipid syndrome (APS) is an autoimmune hypercoagulable disorder that has been shown to cause a large number of cardiac and neurological manifestations. Two recent studies have demonstrated abnormalities in cardiovascular autonomic function testing in APS patients without other cardiovascular or autoimmune disease. However, an association between autonomic disorders such as postural tachycardia syndrome and APS has not previously been described. Methods and results Data were obtained by retrospective chart review. We identified 15 patients who have been diagnosed with APS and an autonomic disorder. The median age of the patients at the time of data analysis was 39 years. The autonomic disorders seen in these patients included postural tachycardia syndrome, neurocardiogenic syncope and orthostatic hypotension. The majority of patients (14/15) were female and the majority (14/15) had non-thrombotic neurological manifestations of APS, most commonly migraine, memory loss and balance disorder. Many also had livedo reticularis (11/15) and Raynaud’s phenomenon (nine of 15). In some patients, the autonomic manifestations improved with anticoagulation and/or anti-platelet therapy; in others they did not. Two patients with postural tachycardia syndrome who failed to improve with the usual treatment of APS have been treated with intravenous immunoglobulin with significant improvement in their autonomic symptoms. Conclusion We believe that autonomic disorders in APS may represent an important clinical association with significant implications for treatment.

Chronic subdural hematomas (CSDHs) typically present with cognitive dysfunction and a history of trauma. Localized dural enhancement on postcontrast MRI scans associated with the surrounding membrane has been described in CSDH. We present an 83-year-old man with rapidly progressing cognitive dysfunction 4 weeks after head trauma related to a fall. MRI showed CSDHs, which in addition to localized dural gadolinium enhancement, showed a marked diffuse, symmetric, contiguous pachymeningeal enhancement of the supratentorial and infratentorial intracranial dural mater. Meningeal biopsy failed to disclose an infectious or neoplastic cause of the enhancement and instead showed fibrocollagenous change. We conclude that diffuse dural enhancement on MRI scans associated with CSDH cause does not necessarily indicate a superimposed process such as infection or malignancy. CSDH should be considered in the differential diagnosis of diffuse dural enhancement, especially when supported by appropriate clinical findings.

Postural tachycardia syndrome (POTS), a disorder of the autonomic nervous system, predominantly occurs in women of child-bearing age. We set out to determine the clinical course and maternal and fetal outcomes in pregnant women with pre-existing POTS.Participants were asked to complete a detailed questionnaire assessing the clinical course of POTS before, during and after pregnancy, as well as complications of pregnancy, labor and delivery and fetal outcomes.Among 10 women with pre-existing POTS (pregnancy age 28 ± 7 years, range 16-39), with a total of 17 live births, two were complicated by pre-eclampsia, 14 were normal vaginal deliveries and three were C-sections. The rate of severe vomiting or hyperemesis gravidarum in the first trimester was 59%. There were no stillbirths or congenital abnormalities. The average birth weight was 3076 ± 733 grams, with two infants born premature. During pregnancy, POTS symptoms were either improved or stable in six of 10 women, and four of these six women utilized medications for POTS. Six months after delivery, POTS symptoms were improved in three, stable in two and worsened in five women compared to before pregnancy.POTS may have a variable clinical course in pregnancy, with 60% of women reporting either improved or stable symptoms during pregnancy, and 50% of women reporting either improved or stable symptoms 6 months after delivery. There may be a higher rate of severe vomiting in the first trimester in women with POTS than in general population.

We describe a patient with a history of recurrent headaches who developed severe and persistent left fronto-orbital and vertex head pain, in conjunction with a maculopapular rash, and was subsequently diagnosed with secondary syphilis. With the incidence of syphilis recently on the rise, we suggest that secondary syphilis should be considered in the differential diagnosis of any patient who presents with headache and a rash. Serologic screening tests for syphilis and a dermatology consultation should be obtained promptly for diagnostic confirmation and institution of treatment with antibiotics.

The authors describe the clinical and diagnostic characteristics of four patients with metastatic CNS disease presenting in de novo nonconvulsive status epilepticus (NCSE). Treatment with anticonvulsants resulted in resolution of NCSE in two patients and a brief improvement in mental status in the other two patients. NCSE should be considered in the differential diagnosis of acute mental status change in patients with metastatic CNS disease.

We describe a patient who presented with a 1-h history of vertical diplopia and nystagmus and was found to have acute left ventrolateral thalamic infarction on the diffusion-weighted magnetic resonance imaging (DWI MRI). This is the first case report demonstrating that vertical diplopia and nystagmus, which typically suggest a lesion in the brainstem or cerebellum, may also occur in acute thalamic infarction. DWI MRI can detect thalamic infarction as early as 1 h after its clinical manifestations.

Pure sensory stroke is a common manifestation of a thalamic stroke and may occur less frequently in the infarction of the brainstem, internal capsule, and parietal cortex. The authors report a 47-year-old man who presented with loss of pain and temperature sensation in the left face, arm, trunk, and leg and was found to have a right lateral medullary infarction on diffusion-weighted magnetic resonance imaging. To the authors' knowledge, this is the first case describing pure sensory stroke as a single, isolated manifestation of the lateral medullary infarction that was detected by diffusion-weighted magnetic resonance imaging. This case suggests that with the availability of diffusion-weighted magnetic resonance imaging, pure sensory stroke may carry a broader spectrum of the anatomical localizations than previously determined and can localize to the lateral medulla. Therefore, a possibility of medullary infarction should be considered when a patient presents with pure sensory stroke, especially when diffusion-weighted magnetic resonance imaging is not immediately available to provide radiographic correlation.