Andrei Gurau, Wasay Nizam, Hamza Khan, Nimisha Seshadri, Christine Kim, Suguru Yamauchi, Rita de Cássia Collaço, Ruilin Ma, Kaitlyn Ecoff, Jolien Vander Cruyssen, Jeannine Forrester, Kristen Rodgers, Andreas S Barth, Frank Bosmans, Jinny S Ha, Malcolm Brock
{"title":"Primary focal hyperhidrosis and autonomic symptom burden: a case-control study using the COMPASS-31 questionnaire.","authors":"Andrei Gurau, Wasay Nizam, Hamza Khan, Nimisha Seshadri, Christine Kim, Suguru Yamauchi, Rita de Cássia Collaço, Ruilin Ma, Kaitlyn Ecoff, Jolien Vander Cruyssen, Jeannine Forrester, Kristen Rodgers, Andreas S Barth, Frank Bosmans, Jinny S Ha, Malcolm Brock","doi":"10.1007/s10286-025-01178-w","DOIUrl":"10.1007/s10286-025-01178-w","url":null,"abstract":"<p><strong>Purpose: </strong>To explore the relationship between primary focal hyperhidrosis (PFH) and generalized autonomic dysfunction, we assessed autonomic symptom burden using a comprehensive and validated measure of autonomic symptoms.</p><p><strong>Methods: </strong>In this case-control study, we compared 132 patients with PFH with 74 matched healthy controls. Autonomic symptoms were assessed using the Composite Autonomic Symptom Score-31 (COMPASS-31) questionnaire. Propensity score matching minimized confounding. Mann-Whitney U tests compared COMPASS-31 scores between groups. A sensitivity analysis using multivariate linear regression accounted for specific hyperhidrosis sites and demographic factors.</p><p><strong>Results: </strong>PFH cases demonstrated significantly higher median COMPASS-31 scores compared to controls (18.7 [IQR 7.7-34.8] vs. 11.2 [IQR 3.5-19.3], p < 0.001). Significant differences were observed in orthostatic (2.0 [0-20] vs. 0 [0-12], p = 0.028), vasomotor (0 [0-0] vs. 0 [0-0], p = 0.005; 24.2% vs. 9.5% non-zero scores, respectively), secretomotor (3.2 [2.1-8.6] vs. 0 [0-3.8], p < 0.001), and gastrointestinal (5.4 [1.8-8] vs. 1.8 [0.9-5.4], p = 0.004) domains. Sensitivity analysis confirmed PFH status as a significant predictor of higher COMPASS-31 scores (additional 7.5 points on average, 95% CI 1.6-13.4, p = 0.012) after adjusting for demographic factors and hyperhidrosis sites. Craniofacial and truncal hyperhidrosis were associated with higher autonomic symptom burden.</p><p><strong>Conclusion: </strong>PFH is associated with a broader spectrum of autonomic symptoms beyond localized sweating, establishing a link to more generalized autonomic dysfunction. These findings underscore the need for a comprehensive approach to evaluating and managing patients with PFH.</p>","PeriodicalId":10168,"journal":{"name":"Clinical Autonomic Research","volume":" ","pages":"245-255"},"PeriodicalIF":4.6,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145741625","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Theodora R Barkoula, Christiana Ioannou, Martina Rekatsina, Kassiani Theodoraki, Panagiotis Zis
{"title":"Cutoffs, sensitivity and specificity of the Ewing battery in evaluating autonomic nervous system disorders: a systematic review.","authors":"Theodora R Barkoula, Christiana Ioannou, Martina Rekatsina, Kassiani Theodoraki, Panagiotis Zis","doi":"10.1007/s10286-025-01185-x","DOIUrl":"10.1007/s10286-025-01185-x","url":null,"abstract":"<p><strong>Purpose: </strong>Many methods have been developed for the assessment of dysautonomia, but they are often unreliable and not readily applicable to daily practice. Among the available investigation methods, the Ewing battery, which includes five non-invasive cardiovascular reflex tests, have become the reference standard in assessing dysautonomia and have been utilized for more than four decades. This systematic review evaluates the diagnostic thresholds and diagnostic performance of Ewing tests across studies in identifying autonomic nervous system (ANS) disorders.</p><p><strong>Methods: </strong>We conducted a comprehensive multi-database literature search, including PubMed, EMBASE, Web of Science, Scopus, and the Cochrane Library, using a pre-defined search strategy, for studies that applied the Ewing tests to assess autonomic dysfunction. We focused on studies that used continuous recordings and those reporting extractable numerical data, including either normative values or diagnostic cutoff thresholds for the Ewing tests. The Ewing parameters that were tested were Valsalva manoeuvre, heart rate variability during deep breathing (E/I ratio), the isometric handgrip, heart rate response to standing (30:15 ratio) and blood pressure response to standing.</p><p><strong>Results: </strong>Our analysis demonstrates good diagnostic performance of the Ewing tests, with sensitivity for individual components reaching up to 91% for Valsalva ratio (VR) in some cohorts. The normative data and respective cutoff points are influenced by age and sex. Optimal diagnostic performance was achieved when the Ewing battery was interpreted using the conventional criterion of ≥ 2/5 abnormal tests particularly when age-adjusted cutoffs were applied.</p><p><strong>Conclusion: </strong>The Ewing battery remains a highly effective tool for diagnosing ANS disorders, especially when age-adjusted normative thresholds are used. To further enhance its diagnostic performance, each laboratory should establish its own normative data reflective of the specific population it serves.</p>","PeriodicalId":10168,"journal":{"name":"Clinical Autonomic Research","volume":" ","pages":"155-173"},"PeriodicalIF":4.6,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13068710/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146008963","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Autonomic characteristics of periodic limb movements: comparison of whole-night and stage N2 linear and non-linear heart rate variability.","authors":"Elif Simin Issı, Selahattin Ayas, Elif Göksu Yiğit Tekkanat","doi":"10.1007/s10286-025-01184-y","DOIUrl":"10.1007/s10286-025-01184-y","url":null,"abstract":"<p><strong>Purpose: </strong>This study aimed to investigate autonomic alterations associated with periodic limb movements during sleep (PLMS) by comparing linear and non-linear heart rate variability (HRV) parameters across whole-night recordings and stage N2 non-rapid eye movement (NREM) sleep (N2).</p><p><strong>Methods: </strong>From 8082 polysomnographic (PSG) recordings, we identified 21 patients with PLMS and 28 age- and sex-matched controls. Linear and non-linear HRV indices were analyzed for whole-night recordings and the longest N2 segment. Periodic limb movement (PLM) indices and arousal-related parameters were also evaluated.</p><p><strong>Results: </strong>Compared with controls, patients with PLMS showed significantly higher standard deviation of normal-to-normal intervals (SDNN) and root mean square of successive differences (RMSSD), increased low-frequency (LF) power, and reduced approximate entropy (ApEn) and sample entropy (SampEn) across the whole night, together with a lower Stress Index (SI). No significant group differences were observed in high-frequency (HF) power or in the composite sympathetic/parasympathetic nervous system (SNS/PNS) indices. During stage N2, the PLM group exhibited significantly greater SDNN, Poincaré plot long-axis standard deviation (SD2), detrended fluctuation analysis alpha-1 exponent (DFA α1), and very-low-frequency (VLF) power, along with lower ApEn values. A significant increase in the SD2/SD1 ratio was also observed specifically during N2, whereas the LF/HF ratio showed only a non-significant upward trend. PLMS counts, indices, and arousal-related parameters were markedly elevated during stage N2.</p><p><strong>Conclusions: </strong>PLMS are characterized by increased autonomic variability (SDNN, SD2, DFA α1) but reduced complexity (ApEn, SampEn), particularly during stage N2. Stage-specific HRV assessment may provide novel insights into the cardiovascular implications of PLMS.</p>","PeriodicalId":10168,"journal":{"name":"Clinical Autonomic Research","volume":" ","pages":"213-221"},"PeriodicalIF":4.6,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13068670/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145959062","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Matthias Amprosi, Fabian Leys, Jean Pierre Ndayisaba, Gerald Fischer, Markus Kofler, Elke Pucks-Faes, Elisabetta Indelicato, Wolfgang Nachbauer, Christina Tiller, Sebastian J Reinstadler, Stefan Kiechl, Gregor Wenning, Sylvia Boesch, Alessandra Fanciulli
{"title":"Autonomic profiling in SPG11 hereditary spastic paraplegia.","authors":"Matthias Amprosi, Fabian Leys, Jean Pierre Ndayisaba, Gerald Fischer, Markus Kofler, Elke Pucks-Faes, Elisabetta Indelicato, Wolfgang Nachbauer, Christina Tiller, Sebastian J Reinstadler, Stefan Kiechl, Gregor Wenning, Sylvia Boesch, Alessandra Fanciulli","doi":"10.1007/s10286-025-01177-x","DOIUrl":"10.1007/s10286-025-01177-x","url":null,"abstract":"","PeriodicalId":10168,"journal":{"name":"Clinical Autonomic Research","volume":" ","pages":"305-309"},"PeriodicalIF":4.6,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145687020","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Svetlana Blitshteyn, Sanjana Suresh, Lucia M Lorenzi
{"title":"Significant improvement of postural orthostatic tachycardia syndrome (POTS) with semaglutide: a case report.","authors":"Svetlana Blitshteyn, Sanjana Suresh, Lucia M Lorenzi","doi":"10.1007/s10286-026-01197-1","DOIUrl":"10.1007/s10286-026-01197-1","url":null,"abstract":"","PeriodicalId":10168,"journal":{"name":"Clinical Autonomic Research","volume":" ","pages":"315-317"},"PeriodicalIF":4.6,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147282532","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Jungyeun Lee, Hyelim Lee, Soon Young Hwang, Seong-Beom Koh
{"title":"Correlation between right vagus nerve cross-sectional area and abnormal heart rate with deep breathing in Parkinson's disease.","authors":"Jungyeun Lee, Hyelim Lee, Soon Young Hwang, Seong-Beom Koh","doi":"10.1007/s10286-025-01180-2","DOIUrl":"10.1007/s10286-025-01180-2","url":null,"abstract":"<p><strong>Purpose: </strong>Autonomic dysfunction, including early cardiovascular parasympathetic involvement, is sustained throughout Parkinson's disease (PD). The vagus nerve, shown by ultrasonography, is atrophic in PD patients. We examined vagus nerve cross-sectional area (CSA) in controls versus PD patients, and its association with autonomic function in early PD.</p><p><strong>Methods: </strong>We investigated 118 de novo PD patients and 29 controls. Vagus nerve CSA was measured using nerve ultrasonography at the bilateral mid-cervical area. Each patient was evaluated once by a randomly assigned sonographer; each control was measured twice by both. For accurate comparison, patients with diabetes were additionally excluded from the PD group. A 1:1 propensity score matching based on age, sex, hypertension, and dyslipidemia was performed. The CSA was compared between matched patients and controls. Clinical interviews, rating scales, and autonomic and olfactory function tests were performed. The association between these results and the CSA was analyzed.</p><p><strong>Results: </strong>Median right and left vagus nerve CSA measured 1.3 and 1.2 mm<sup>2</sup>, respectively, significantly lower than in controls (2.3 and 2.2 mm<sup>2</sup>). Partial correlation analysis showed a negative correlation between the right vagus CSA and abnormal heart rate response to deep breathing (rho = -0.191, P = 0.045).</p><p><strong>Conclusion: </strong>Small right vagus nerve CSA is associated with early parasympathetic dysfunction in PD patients. These findings support the use of vagus nerve ultrasound as a potential non-invasive biomarker for identifying prodromal autonomic involvement and informing future early intervention research.</p>","PeriodicalId":10168,"journal":{"name":"Clinical Autonomic Research","volume":" ","pages":"205-212"},"PeriodicalIF":4.6,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145741611","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Abhishek Lenka, Chi-Ying R Lin, William Dauer, Denis Dickson, Steven Vernino
{"title":"A wolf in synuclein clothing: Creutzfeldt-Jakob disease disguised as MSA.","authors":"Abhishek Lenka, Chi-Ying R Lin, William Dauer, Denis Dickson, Steven Vernino","doi":"10.1007/s10286-025-01179-9","DOIUrl":"10.1007/s10286-025-01179-9","url":null,"abstract":"","PeriodicalId":10168,"journal":{"name":"Clinical Autonomic Research","volume":" ","pages":"301-304"},"PeriodicalIF":4.6,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145755058","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Katharina Müller, Joana C Thiel, Lena Schopen, Bruno Fimm, Jörg B Schulz, Andrea Maier
{"title":"Impaired attention and cognitive deficits associated with pain and autonomic symptoms in hypermobile Ehlers-Danlos syndrome: a pilot study.","authors":"Katharina Müller, Joana C Thiel, Lena Schopen, Bruno Fimm, Jörg B Schulz, Andrea Maier","doi":"10.1007/s10286-026-01191-7","DOIUrl":"10.1007/s10286-026-01191-7","url":null,"abstract":"<p><strong>Purpose: </strong>Patients with hypermobile Ehlers-Danlos syndrome (hEDS) frequently present with circulatory dysfunction, including postural orthostatic tachycardia syndrome (POTS), and cognitive impairments, leading to substantial disability and limitations in daily functioning. Few studies have examined attention, and concentration and associated conditions in hEDS. In this case-control study, we used a comprehensive cognitive test battery to assess whether cognitive performance is impaired in individuals with hEDS and depends on different body positions.</p><p><strong>Methods: </strong>Twenty-nine patients and 29 healthy controls (HC) were enrolled. Baseline cognitive assessments included the Performance Scale of an intelligence test (LPS), Montreal Cognitive Assessment (cognitive deficit screening), and Test of Attentional Performance (TAP). The main cognitive tests for assessing the effects of body position were conducted in randomized conditions (supine, standing, and standing legs-crossed) and included the Stroop test, Corsi block-tapping test, Trail Making Test Part B, and Wechsler Memory Scale-revised.</p><p><strong>Results: </strong>Compared to HC, patients with hEDS had higher intellectual performance (p < .050), but besides relevant comorbid conditions, also significantly impaired attention in the TAP (p < .010) and an impairment of executive function assessed by the Stroop test (p < .010) in the legs-crossed compared to the supine position.</p><p><strong>Conclusion: </strong>Attention in the hEDS group was impaired compared to HC, and executive performance was dependent on body position. Individuals performed worse when standing legs-crossed. Hence, impaired proprioception as present in hEDS may, along with comorbid conditions such as pain, be a contributing factor affecting executive function.</p>","PeriodicalId":10168,"journal":{"name":"Clinical Autonomic Research","volume":" ","pages":"223-232"},"PeriodicalIF":4.6,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13068739/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147389504","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Martin Miranda-Hurtado, Rashmin Hira, Kate M Bourne, Shaun Ranada, Jacquie R Baker, Robert S Sheldon, Satish R Raj
{"title":"Stroke volume reduction impairs cerebrovascular regulation through ETCO<sub>2</sub> in postural orthostatic tachycardia syndrome.","authors":"Martin Miranda-Hurtado, Rashmin Hira, Kate M Bourne, Shaun Ranada, Jacquie R Baker, Robert S Sheldon, Satish R Raj","doi":"10.1007/s10286-025-01181-1","DOIUrl":"10.1007/s10286-025-01181-1","url":null,"abstract":"<p><strong>Background: </strong>Patients with postural orthostatic tachycardia syndrome (POTS) experience disabling symptoms such as brain fog related to reduced cerebral perfusion.</p><p><strong>Objective: </strong>The objective of this study is to determine the mediating role of carbon dioxide in the relationship between stroke volume and cerebral blood flow.</p><p><strong>Methods: </strong>A total of 15 female patients with POTS underwent head-up tilt testing under two conditions: with lower-body compression (higher stroke volume) and without (lower stroke volume). We analyzed cerebral blood flow velocity, respiratory, and cardiovascular responses using linear mixed-effects and mediation models to examine stroke volume-cerebral blood flow interactions. Granger causality and wavelet coherence assessed cerebral autoregulation.</p><p><strong>Results: </strong>Lower-body compression attenuated the reduction in stroke volume (-34 ml versus -23 ml; p < 0.01), end-tidal CO<sub>2</sub> (-6.4 mmHg versus -3.2 mmHg; p < 0.01), and mean middle cerebral artery blood flow velocity (-11.2 cm/s versus -4.2 cm/s; p < 0.01) during tilt. Mediation analysis revealed that carbon dioxide completely mediated the relationship between stroke volume and middle cerebral artery blood flow velocity, with a significant indirect effect (0.18 cm/s/ml, 95% confidence interval (CI) 0.058-0.33) and a nonsignificant direct effect (0.04 cm/s/ml, p = 0.5). Compression attenuated the association between stroke volume and carbon dioxide (-0.07 mmHg/ml; 95% CI -0.12 to -0.010; p = 0.02), as shown by the linear mixed-effect model, and reduced the directional influence of blood pressure on cerebral blood flow (ΔGranger causality: 0.12 (0.05-0.18) versus 0.05 (0.02-0.08); p < 0.01).</p><p><strong>Conclusions: </strong>Reduction in stroke volume leads to reduced cerebral perfusion in POTS, an effect likely mediated by decreased carbon dioxide.</p>","PeriodicalId":10168,"journal":{"name":"Clinical Autonomic Research","volume":" ","pages":"257-269"},"PeriodicalIF":4.6,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145762330","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}