Clinical Autonomic Research最新文献

{"title":"Harlequin syndrome: using clinical features and autonomic testing to unmask the disorder.","authors":"Hannah Padilla, William P Cheshire, Eduardo E Benarroch, Sarah E Berini, Jeremy K Cutsforth-Gregory, Michelle L Mauermann, Paola Sandroni, Kamal Shouman, Phillip A Low, Wolfgang Singer, Elizabeth A Coon","doi":"10.1007/s10286-025-01151-7","DOIUrl":"https://doi.org/10.1007/s10286-025-01151-7","url":null,"abstract":"<p><strong>Purpose: </strong>Harlequin syndrome is a rare autonomic disorder characterized by unilateral facial flushing and contralateral anhidrosis. We sought to delineate underlying causes, clinical presentations, and autonomic testing profiles of patients with Harlequin syndrome.</p><p><strong>Methods: </strong>Retrospective chart review was performed of the Mayo Clinic electronic health record for patients with a Harlequin syndrome diagnosis from 1998 to 2024. Clinical, laboratory, imaging, and autonomic function testing results, including autonomic reflex screen (ARS) and thermoregulatory sweat test (TST), were reviewed.</p><p><strong>Results: </strong>Of 51 patients with Harlequin syndrome, 39 (76%) were women. Median age of onset was 52 years (range 8-73 years). Harlequin syndrome was often idiopathic (N = 19; 37%), followed by postsurgical (N = 9; 17%), neoplasm (N = 5; 9.8%), trauma (N = 4; 7.8%), small fiber neuropathy (N = 4; 7.8%), systemic causes (N = 3; 5.9%), autoimmune (N = 3; 5.9%), pure autonomic failure (N = 2; 3.9%), and multiple sclerosis (N = 1; 2%). Pupil abnormalities were found in 13 patients (25.5%) with abnormal muscle stretch reflexes in 17 (33.3%). Headache was a comorbidity in 20 patients (39%). Of those with postsurgical onset, various surgeries preceded Harlequin syndrome onset, including heart, lung, and neck operations. Onset was acute or subacute in the majority of postsurgical patients (57%), while insidious onset was most common in nonsurgical patients (89%; p = 0.001). Median anhidrosis on TST was 9% (range 0.6-63%; N = 27). Median composite autonomic severity score was 1 (interquartile range (IQR) 0-3; N = 31).</p><p><strong>Conclusions: </strong>Harlequin syndrome commonly has an insidious onset and occurs without an identifiable cause, which could be considered primary Harlequin syndrome. Secondary Harlequin syndrome can occur following surgeries in the vicinity of sympathetic pathways, which most commonly leads to an acute or subacute presentation.</p>","PeriodicalId":10168,"journal":{"name":"Clinical Autonomic Research","volume":" ","pages":""},"PeriodicalIF":3.4,"publicationDate":"2025-07-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144728289","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}

{"title":"Prevalence and impact of orthostatic hypotension in Parkinson's disease: a systematic review and meta-analysis.","authors":"Hui Wang, Chi Zhang, Dongxun Xu","doi":"10.1007/s10286-025-01146-4","DOIUrl":"https://doi.org/10.1007/s10286-025-01146-4","url":null,"abstract":"<p><strong>Objective: </strong>Parkinson's disease (PD) is frequently associated with orthostatic hypotension (OH). Research on the prevalence of OH in PD and its effects on patients has produced inconsistent findings.</p><p><strong>Methods: </strong>A systematic review and meta-analysis were conducted by searching for studies related to PD and OH in the PubMed, Web of Science, Embase, and Cochrane databases. Data were pooled as necessary to calculate the prevalence of OH in patients with PD, along with odds ratios (OR), weighted mean differences (WMD), or standardized mean differences (SMD) with 95% confidence intervals (CI). Heterogeneity was assessed using the I² statistic.</p><p><strong>Results: </strong>The prevalence of OH in patients with PD was found to be 33.1% (95% CI 29.3-37%) in a pooled sample of 7748 subjects. Patients with PD and OH were significantly older at the time of examination (WMD 2.92 years) and had a longer disease duration (WMD 0.71 years) compared with those without OH. There was no significant difference in the distribution of sex, or in the scores of the Unified Parkinson's Disease Rating Scale (UPDRS)/the Movement Disorder Society-Unified Parkinson's Disease Rating Scale (MDS-UPDRS) parts I and II, as well as the total scores among patients with Parkinson's disease with or without OH. In addition, patients with PD and OH exhibited significantly higher UPDRS/MDS-UPDRS scores across part III section scores (SMD 0.41, 95% CI 0.23-0.59).</p><p><strong>Conclusions: </strong>The prevalence of OH in PD is 33.1%. Patients with PD and OH are generally older at examination, have a longer disease duration, and display more severe motor symptoms compared with those without OH.</p>","PeriodicalId":10168,"journal":{"name":"Clinical Autonomic Research","volume":" ","pages":""},"PeriodicalIF":3.9,"publicationDate":"2025-07-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144717602","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}

{"title":"Acetate concentration correlates with MSNA in patients with resistant hypertension.","authors":"Revathy Carnagarin, Gianni Sesa-Ashton, Natalie C Ward, Janis Nolde, Anu Joyson, Justine Chan, Ancy Jose, Markus P Schlaich","doi":"10.1007/s10286-025-01144-6","DOIUrl":"https://doi.org/10.1007/s10286-025-01144-6","url":null,"abstract":"<p><strong>Purpose: </strong>Short-chain fatty acids (SCFAs), metabolites of colonic microflora fermentation of dietary fibre, have been implicated in experimental models and clinical trials to impact blood pressure (BP) regulation. Dietary interventions increasing serum SCFA levels have been associated with reduced 24-h systolic BP in hypertensive patients. However, the underlying mechanisms remain elusive. Given the role of the gut-brain axis and clear evidence for sympathetic nervous system activation as important modulators of blood pressure, we examined the relationship between sympathetic drive and SCFA concentration in patients with resistant hypertension (RH) and healthy control subjects (HC).</p><p><strong>Methods: </strong>A total of 21 patients with RH (68.6 ± 9.7 years, 47% male) and 28 healthy control subjects (HC) (34.6 ± 16.7 years, 75% male) were recruited to undergo microneurography for determination of muscle sympathetic nerve activity (MSNA), automated office BP (AOBP) and blood collection for serum SCFA.</p><p><strong>Results: </strong>Mean systolic AOBP was 156 ± 21 mmHg and 115 ± 10 mmHg for RH and HC, respectively (p < 0.0001). Serum acetate levels were 1340 ± 115.4 umol/L for HC and 724.5 ± 116.9 umol/L for RH (p < 0.0001). Butyrate and propionate concentrations did not significantly differ between groups. MSNA burst frequency was markedly elevated in RH compared with HCs (p < 0.001), with 25.3 ± 7.4 burst/minute in HC compared with 40.24 ± 8.3 burst/minute in RH. An inverse relationship was evident between serum acetate levels and MSNA burst frequency (p = 0.0267, R² = 0.4) along with increased sympathetic vascular transduction (p = 0.0008, R² = 0.82) in RH.</p><p><strong>Conclusions: </strong>Our findings suggest that the beneficial effects of SCFA levels, in particular acetate, on cardiovascular regulation may at least in part be mediated by sympatho-inhibition and altered sympathetic vascular transduction.</p>","PeriodicalId":10168,"journal":{"name":"Clinical Autonomic Research","volume":" ","pages":""},"PeriodicalIF":3.9,"publicationDate":"2025-07-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144658586","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}

{"title":"Neuronal intranuclear inclusion disease: a dynamic evolution of MRI in 8 years and mimicker of benign paroxysmal positional vertigo.","authors":"Wei-Qin Ning, Yang-Sha Zhuang, Sheng-Peng Diao, Ming-Fan Hong","doi":"10.1007/s10286-025-01143-7","DOIUrl":"https://doi.org/10.1007/s10286-025-01143-7","url":null,"abstract":"","PeriodicalId":10168,"journal":{"name":"Clinical Autonomic Research","volume":" ","pages":""},"PeriodicalIF":3.9,"publicationDate":"2025-07-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144648649","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}

{"title":"Enhanced visual illusions in Parkinson's disease with cardiovascular autonomic failure.","authors":"Tadashi Umehara, Masahiro Mimori, Tatsushi Kokubu, Masakazu Ozawa, Tomotaka Shiraishi, Asako Onda, Hiromasa Matsuno, Shusaku Omoto, Hidetomo Murakami, Yasuyuki Iguchi","doi":"10.1007/s10286-025-01142-8","DOIUrl":"https://doi.org/10.1007/s10286-025-01142-8","url":null,"abstract":"<p><strong>Purpose: </strong>Few studies have examined the association between cardiovascular autonomic failure and minor hallucinations in patients with Parkinson's disease (PD). The aim of this study was to clarify this association.</p><p><strong>Methods: </strong>The subjects were 133 patients with PD without well-structured visual hallucinations. Visual illusory responses were evaluated using the noise pareidolia test. Cardiac ¹²³I-metaiodobenzylguanidine (¹²³I-MIBG) uptake and neurogenic orthostatic hypotension (nOH) and supine hypertension (nSH) on head-up tilt-table testing were examined in association with the incidence of pareidolia.</p><p><strong>Results: </strong>Fifty-one (38%) patients had pareidolia. nOH (β = 0.220, p = 0.008), cognitive impairment (β = -0.228, p = 0.028), and longer symptom duration (β = 0.273, p = 0.006) were associated with an increased incidence of pareidolia independently of age, sex, motor severity, levodopa-equivalent dose, and anticholinergic and cholinesterase inhibitor use. An increased incidence of pareidolia was also associated with nSH (β = 0.214, p = 0.009), while no such association was found with cardiac ¹²³I-MIBG uptake. Patients with severe nOH or nSH tended to have a higher incidence of pareidolia than those with mild nOH (p = 0.063) or nSH (p < 0.003), respectively.</p><p><strong>Conclusion: </strong>nOH and nSH were associated with the severity of pareidolia in early PD patients without well-structured visual hallucinations. Further studies are required to clarify whether this association is attributable to widespread central pathological changes related to cardiovascular autonomic failure, or to degeneration of the sympathetic nervous system.</p>","PeriodicalId":10168,"journal":{"name":"Clinical Autonomic Research","volume":" ","pages":""},"PeriodicalIF":3.9,"publicationDate":"2025-07-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144567147","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}

{"title":"Correction: Autonomic dysfunction after stroke: an overview of recent clinical evidence and perspectives on therapeutic management.","authors":"Anush Barkhudaryan, Wolfram Doehner, Nadja Jauert","doi":"10.1007/s10286-025-01127-7","DOIUrl":"https://doi.org/10.1007/s10286-025-01127-7","url":null,"abstract":"","PeriodicalId":10168,"journal":{"name":"Clinical Autonomic Research","volume":" ","pages":""},"PeriodicalIF":3.9,"publicationDate":"2025-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144526566","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}

{"title":"Characterising Postural Orthostatic Tachycardia Syndrome (POTS) triggered by a viral illness compared to concussion or trauma.","authors":"Gemma Wilson, Marie-Claire Seeley, Pauline Slater, Dennis H Lau, Celine Gallagher","doi":"10.1007/s10286-025-01136-6","DOIUrl":"10.1007/s10286-025-01136-6","url":null,"abstract":"","PeriodicalId":10168,"journal":{"name":"Clinical Autonomic Research","volume":" ","pages":""},"PeriodicalIF":3.9,"publicationDate":"2025-06-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144339959","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}

{"title":"Author Response: \"Re: Comprehensive linear and nonlinear heart rate variability normative data in children. Clinical Autonomic Research (September 2024)\".","authors":"Bahram Kakavand","doi":"10.1007/s10286-025-01141-9","DOIUrl":"10.1007/s10286-025-01141-9","url":null,"abstract":"","PeriodicalId":10168,"journal":{"name":"Clinical Autonomic Research","volume":" ","pages":""},"PeriodicalIF":3.9,"publicationDate":"2025-06-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144332499","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}

{"title":"A reset on our reclined position-a call to prioritize neurogenic supine hypertension in the synucleinopathies.","authors":"Cameron Miller-Patterson, Abhishek Lenka, Stephen Juraschek, Jeanie Park, Paul A Beach","doi":"10.1007/s10286-025-01140-w","DOIUrl":"https://doi.org/10.1007/s10286-025-01140-w","url":null,"abstract":"","PeriodicalId":10168,"journal":{"name":"Clinical Autonomic Research","volume":" ","pages":""},"PeriodicalIF":3.9,"publicationDate":"2025-06-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144324599","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}

{"title":"Hypoxia disrupts neurovascular regulation of blood pressure in normotensive and untreated hypertensive men.","authors":"Qudus A Ojikutu, Jeann L Sabino-Carvalho, Katherine Latham, Marcos Rocha, Joao D Mattos, Monique O Campos, Daniel E Mansur, Lauro C Vianna, Antonio C L Nóbrega, Igor A Fernandes","doi":"10.1007/s10286-025-01135-7","DOIUrl":"https://doi.org/10.1007/s10286-025-01135-7","url":null,"abstract":"<p><strong>Background: </strong>Hypoxia is a common feature of arterial hypertension that does not consistently elevate blood pressure (BP), but triggers exaggerated increases in muscle sympathetic nerve activity (MSNA) and may disturb sympathetic transduction and baroreflex sensitivity in hypertensive individuals. Elevated resting MSNA, enhanced sympathetic transduction, and reduced baroreflex sensitivity are all associated with increased blood pressure variability (BPV), a marker of target organ damage independent of absolute BP levels. We hypothesized that hypoxia would elicit greater BPV in hypertensive individuals compared to normotensive controls METHODS: Nine young- to middle-aged men with untreated stage 1-2 hypertension (HT) and normotensive controls (NT) were exposed to normoxia (21% O₂) and isocapnic hypoxia (IH, 10% O₂). During both conditions, oxygen saturation, beat-to-beat BP, MSNA, and end-tidal CO₂ (PetCO₂) were continuously monitored, with PetCO₂ clamped. BPV was quantified using standard deviation, coefficient of variation, and average real variability for systolic (SBP), diastolic (DBP), and mean BP (MBP). Sympathetic transduction was assessed using a time-domain signal averaging technique. Cardiac baroreflex sensitivity (cBRS) was evaluated using the sequence method, and sympathetic baroreflex sensitivity (sBRS) was calculated via MSNA-DBP regression RESULTS: IH induced comparable oxygen desaturation in both groups (NT: -25.7 ± 3.3% vs. HT: -21.2 ± 4.0%, p > 0.05). Although BP and PetCO₂ remained unchanged, MSNA responses were significantly greater in HT (NT: +8 ± 2 vs. HT: +12 ± 2 bursts/min, p = 0.03). IH increased all indices of BPV and sympathetic transduction, while both cBRS and sBRS were similarly impaired in the two groups.</p><p><strong>Conclusions: </strong>In conclusion, IH similarly exacerbates BPV and disrupts sympathetic transduction and baroreflex function in normotensive and untreated hypertensive men, despite greater MSNA reactivity in the hypertensive group.</p>","PeriodicalId":10168,"journal":{"name":"Clinical Autonomic Research","volume":" ","pages":""},"PeriodicalIF":3.9,"publicationDate":"2025-06-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144324600","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}