Article
Clinical Neurology
Denver Igharo, Joana C. Thiel, Roman Rolke, Merve Akkaya, Joachim Weis, Istvan Katona, Joerg B. Schulz, Andrea Maier
Summary: This study aimed to understand the pathophysiological mechanisms of small fiber neuropathy and autonomic neuropathy in hypermobile Ehlers-Danlos syndromes. By observing and testing 31 patients with hEDS and 31 healthy controls, it was found that SFN was common in patients with hEDS, with accompanying autonomic dysfunctions. Quantitative sensory testing and skin biopsy results supported SFN as an important underlying mechanism.
EUROPEAN JOURNAL OF NEUROLOGY
(2022)
Article
Cardiac & Cardiovascular Systems
Christopher H. Gibbons, Gustavo Silva, Roy Freeman
Summary: The study demonstrated that an unsupervised at-home training regimen showed promising efficacy in treating POTS, leading to significant improvements in cardiovascular function, syncope frequency, and quality of life for patients with POTS.
Article
Clinical Neurology
Denver Igharo, Joana C. Thiel, Roman Rolke, Merve Akkaya, Joachim Weis, Istvan Katona, Joerg B. Schulz, Andrea Maier
Summary: This study aimed to investigate the pathophysiological mechanisms of small fiber neuropathy and autonomic neuropathy in hypermobile Ehlers-Danlos Syndromes (hEDS). Skin biopsy, quantitative sensory testing, tilt-table testing, the painDetect, Small Fiber Neuropathy Screening List, and the COMPASS-31 questionnaire were used to assess 31 hEDS patients and 31 healthy controls. The results revealed that 61% of hEDS patients were diagnosed with small fiber neuropathy and 32% fulfilled the criteria for postural tachycardia syndrome (PoTS). Skin biopsy showed significantly reduced nerve fiber density in both proximal and distal regions of hEDS patients.
EUROPEAN JOURNAL OF NEUROLOGY
(2023)
Article
Medicine, General & Internal
Kate M. Bourne, Derek S. Chew, Lauren E. Stiles, Brett H. Shaw, Cyndya A. Shibao, Luis E. Okamoto, Emily M. Garland, Alfredo Gamboa, Amanda Peltier, Andre Diedrich, Italo Biaggioni, Robert S. Sheldon, David Robertson, Satish R. Raj
Summary: POTS patients, predominantly female, experience employment challenges and substantial income loss due to illness limitations, highlighting the need for earlier diagnosis and improved therapeutic strategies.
JOURNAL OF INTERNAL MEDICINE
(2021)
Article
Clinical Neurology
Lucy Norcliffe-Kaufmann, Jose-Alberto Palma, Jose Martinez, Celeste Camargo, Horacio Kaufmann
Summary: This study suggests that the postural tachycardia syndrome is closely related to fear-conditioned response during standing, indicating that the syndrome may be a functional psychological disorder.
Article
Clinical Neurology
Katrina Kesterson, Jill Schofield, Svetlana Blitshteyn
Summary: 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.
JOURNAL OF NEUROLOGY
(2023)
Article
Clinical Neurology
Keun-Tae Kim, Sun-Uk Lee, Jung-Bin Kim, Jeong-Yoon Choi, Byung-Jo Kim, Ji-Soo Kim
Summary: The purpose of this study was to investigate the association between otolith function and changes in mean orthostatic blood pressure and heart rate in patients with postural orthostatic tachycardia syndrome (POTS). The results showed that the n1-p1 amplitude of oVEMPs was larger in POTS patients compared to healthy participants, indicating a potential correlation between augmented utricular inputs and sympathetic over vagal control of blood pressure and heart rate in POTS.
CLINICAL AUTONOMIC RESEARCH
(2023)
Article
Clinical Neurology
Micaela R. Owens, Divpreet Kaur
Summary: Postural orthostatic tachycardia syndrome (POTS) is a diverse diagnosis with various clinicopathological associations. The appropriate diagnostic approach is not well-established. This study provides guidance on the initial diagnosis and expanding diagnostic testing, emphasizing the importance of shared decision-making and individualized workup.
Article
Medicine, General & Internal
Jasmina Medic Spahic, Viktor Hamrefors, Madeleine Johansson, Fabrizio Ricci, Olle Melander, Richard Sutton, Artur Fedorowski
Summary: Symptom severity in POTS patients is fivefold higher compared to healthy individuals. The newly developed Malmo POTS Score can be used as a tool to assess symptom burden, monitor disease progression, and evaluate pre-test likelihood of disease.
JOURNAL OF INTERNAL MEDICINE
(2023)
Article
Behavioral Sciences
Veronika Potockova, Sarka Mala, Lucie Hoskovcova, Vaclav Capek, Tomas Nedelka, Lucie Riedlbauchova, Daniel Baumgartner, Livie Mensova, Radim Mazanec
Summary: This study aimed to investigate whether thermal quantitative sensory testing (TQST) can be used as a screening tool to identify patients with diabetes at a higher risk of cardiac autonomic neuropathy (CAN). The results showed that TQST can accurately diagnose CAN with high specificity and positive predictive value. Therefore, TQST can be used as a rapid screening method before performing time-consuming cardiovascular autonomic reflex tests (CART).
BRAIN AND BEHAVIOR
(2022)
Article
Immunology
Melody Hermel, Megan Sweeney, Edsel Abud, Kathleen Luskin, Jose P. Criado, Robert Bonakdar, James Gray, Thomas Ahern
Summary: A case of new-onset postural orthostatic tachycardia syndrome after receiving a single dose of the BNT162b2 SARS-CoV-2 vaccine was reported. Symptoms included fatigue, brain fog, headache, sinus tachycardia, and dizziness. Management involved noninvasive therapies, behavioral approaches, and pharmacologic regimens.
Review
Clinical Neurology
Svetlana Blitshteyn
Summary: Postural orthostatic tachycardia syndrome (POTS) should not only be considered as a disorder of the peripheral nervous system, but also as a central nervous system (CNS) disorder. This is supported by significant CNS symptom burden in patients with POTS, structural and functional differences found on neuroimaging in patients with POTS, evidence of cerebral hypoperfusion and alteration in cerebrospinal fluid volume, and positive response to medications targeting the CNS and non-pharmacologic CNS therapies. Redefining POTS as a CNS disorder may open up new possibilities for pharmacotherapy and non-pharmacologic therapeutic interventions for patients affected by this disabling syndrome.
JOURNAL OF NEUROLOGY
(2022)
Review
Endocrinology & Metabolism
Alice Duque, Mauro Felippe Felix Mediano, Andrea De Lorenzo, Luiz Fernando Rodrigues Jr
Summary: Cardiovascular autonomic neuropathy (CAN) is a debilitating microvascular complication that primarily affects long-standing type 2 diabetes patients but can also manifest early in the disease progression. The pathophysiology of CAN is complex, involving various risk factors and clinical complications, and early identification and treatment of CAN may help reduce mortality rates for affected individuals.
WORLD JOURNAL OF DIABETES
(2021)
Article
Clinical Neurology
Emily C. Smith, Jorge Celedonio, Victor C. Nwazue, Emily M. Garland, Sachin Y. Paranjape, Abby H. Chopoorian, Amr Wahba, Italo Biaggioni, Bonnie Black, Cyndya A. Shibao, Andre Diedrich, Luis E. Okamoto, Satish R. Raj, Alfredo Gamboa
Summary: The study aimed to assess the effects of a high-sodium diet on endothelial function in patients with POTS. Results showed that a 6-day high-sodium diet did not induce endothelial dysfunction in POTS patients. Therefore, short-term use of a high-sodium diet for POTS patients does not negatively affect endothelial function.
CLINICAL AUTONOMIC RESEARCH
(2021)
Article
Clinical Neurology
Alexandra Hovaguimian
Summary: Dysautonomias are a group of disorders that have varied clinical presentations. They can cause either widespread autonomic impairment or milder symptoms based on which systems are affected. The diagnosis involves a detailed history and examination, with autonomic testing aiding in confirming the diagnosis and guiding treatment decisions. Treatment focuses on addressing underlying disorders, optimizing lifestyle factors, and considering pharmacologic options to improve symptoms. Patient-centered care, including setting expectations and providing appropriate counseling, is crucial for effective management.
NEUROLOGIC CLINICS
(2023)