Pub Date : 2024-09-28DOI: 10.1007/s10286-024-01072-x
Jean-Baptiste Bonnet, Clotilde Ramillon-Cury, Sarah Tournayre, Ariane Sultan, Antoine Avignon
{"title":"Management of obesity in an individual with ROHHAD syndrome with semaglutide 2.4 mg/week: a case report.","authors":"Jean-Baptiste Bonnet, Clotilde Ramillon-Cury, Sarah Tournayre, Ariane Sultan, Antoine Avignon","doi":"10.1007/s10286-024-01072-x","DOIUrl":"https://doi.org/10.1007/s10286-024-01072-x","url":null,"abstract":"","PeriodicalId":10168,"journal":{"name":"Clinical Autonomic Research","volume":" ","pages":""},"PeriodicalIF":3.9,"publicationDate":"2024-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142342681","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}
Pub Date : 2024-09-23DOI: 10.1007/s10286-024-01064-x
Tarun W Dasari, Michiaki Nagai, Hallum Ewbank, Praloy Chakraborty, Sunny S Po
Purpose: Autonomic dysregulation is observed in heart failure (HF) with reduced ejection fraction (HFrEF). Abnormal heart rate variability (HRV), a measure of such dysregulation, is associated with poor prognosis in HFrEF. It is unknown if novel HRV metrics normalize in the patients with recovered ejection fraction (HFrecEF) compared to persistent HFrEF. The aim of this study was to investigate novel HRV indexes in persistent HFrEF in comparison to HFrecEF METHODS: A standard 10-min electrocardiography measurement was performed in patients categorized in four groups: persistent HFrEF (n = 40), HFrecEF (n = 41), stage A HF (n = 73) and healthy controls (n = 40).
Results: All HRV indexes were significantly different between the four groups. Specifically, novel metrics, such as higher parasympathetic nervous system (PNS) index and lower sympathetic nervous system (SNS) index, were observed in the HFrecEF group compared to the persistent HFrEF group. In multiple logistic regression analysis, higher PNS index (odds ratio [OR] 2.02, 95% confidence interval [CI] 1.17-3.49; p = 0.01) and lower SNS index (OR 0.68, 95% CI 0.52-0.87; p = 0.002) were associated with HFrecEF. Receiver operating characteristic analysis showed that the SNS index had the highest area under the curve (AUC), followed by the PNS index and mean heart rate for the HF phenotype regarding EF recovery (AUC = 0.71, 0.69 and 0.69, respectively).
Conclusion: Myocardial functional recovery in HFrEF is associated with improved parasympathetic activity and reduced sympathetic activity, as reflected in the PNS and SNS indexes. These novel metrics can be potentially used to aid in identifying recovered versus non-recovered phenotypes in patients with HFrEF.
{"title":"Heart rate variability metrics and myocardial recovery in heart failure with reduced ejection fraction.","authors":"Tarun W Dasari, Michiaki Nagai, Hallum Ewbank, Praloy Chakraborty, Sunny S Po","doi":"10.1007/s10286-024-01064-x","DOIUrl":"https://doi.org/10.1007/s10286-024-01064-x","url":null,"abstract":"<p><strong>Purpose: </strong>Autonomic dysregulation is observed in heart failure (HF) with reduced ejection fraction (HFrEF). Abnormal heart rate variability (HRV), a measure of such dysregulation, is associated with poor prognosis in HFrEF. It is unknown if novel HRV metrics normalize in the patients with recovered ejection fraction (HFrecEF) compared to persistent HFrEF. The aim of this study was to investigate novel HRV indexes in persistent HFrEF in comparison to HFrecEF METHODS: A standard 10-min electrocardiography measurement was performed in patients categorized in four groups: persistent HFrEF (n = 40), HFrecEF (n = 41), stage A HF (n = 73) and healthy controls (n = 40).</p><p><strong>Results: </strong>All HRV indexes were significantly different between the four groups. Specifically, novel metrics, such as higher parasympathetic nervous system (PNS) index and lower sympathetic nervous system (SNS) index, were observed in the HFrecEF group compared to the persistent HFrEF group. In multiple logistic regression analysis, higher PNS index (odds ratio [OR] 2.02, 95% confidence interval [CI] 1.17-3.49; p = 0.01) and lower SNS index (OR 0.68, 95% CI 0.52-0.87; p = 0.002) were associated with HFrecEF. Receiver operating characteristic analysis showed that the SNS index had the highest area under the curve (AUC), followed by the PNS index and mean heart rate for the HF phenotype regarding EF recovery (AUC = 0.71, 0.69 and 0.69, respectively).</p><p><strong>Conclusion: </strong>Myocardial functional recovery in HFrEF is associated with improved parasympathetic activity and reduced sympathetic activity, as reflected in the PNS and SNS indexes. These novel metrics can be potentially used to aid in identifying recovered versus non-recovered phenotypes in patients with HFrEF.</p>","PeriodicalId":10168,"journal":{"name":"Clinical Autonomic Research","volume":" ","pages":""},"PeriodicalIF":3.9,"publicationDate":"2024-09-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142281158","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}
Pub Date : 2024-09-19DOI: 10.1007/s10286-024-01051-2
Naome Mwesigwa, Patricio Millar Vernetti, Annet Kirabo, Bonnie Black, Tan Ding, Jose Martinez, Jose-Alberto Palma, Italo Biaggioni, Horacio Kaufmann, Cyndya A. Shibao
Purpose
We previously reported that single doses of the norepinephrine transporter inhibitor, atomoxetine, increased standing blood pressure (BP) and ameliorated symptoms in patients with neurogenic orthostatic hypotension (nOH). We aimed to evaluate the effect of atomoxetine over four weeks in patients with nOH.
Methods
A randomized, double-blind, placebo-controlled crossover clinical trial between July 2016 and May 2021 was carried out with an initial open-label, single-dose phase (10 or 18 mg atomoxetine), followed by a 1-week wash-out, and a subsequent double-blind 4-week treatment sequence (period 1: atomoxetine followed by placebo) or vice versa (period 2). The trial included a 2-week wash-out period. The primary endpoint was symptoms of nOH as measured by the orthostatic hypotension questionnaire (OHQ) assessed at 2 weeks.
Results
A total of 68 patients were screened, 40 were randomized, and 37 completed the study. We found no differences in the OHQ composite score between atomoxetine and placebo at 2 weeks (−0.3 ± 1.7 versus −0.4 ± 1.5; P = 0.806) and 4 weeks (−0.6 ± 2.4 versus −0.5 ± 1.6; P = 0.251). There were no differences either in the OHSA scores at 2 weeks (3 ± 1.9 versus 4 ± 2.1; P = 0.062) and at 4 weeks (3 ± 2.2 versus 3 ± 2.0; P = 1.000) or in the OH daily activity scores (OHDAS) at 2 weeks (4 ± 3.0 versus 5 ± 3.1, P = 0.102) and 4 weeks (4 ± 3.0 versus 4 ± 2.7, P = 0.095). Atomoxetine was well-tolerated.
Conclusions
While previous evidence suggested that acute doses of atomoxetine might be efficacious in treating nOH; results of this clinical trial indicated that it was not superior to placebo to ameliorate symptoms of nOH.
{"title":"Atomoxetine on neurogenic orthostatic hypotension: a randomized, double-blind, placebo-controlled crossover trial","authors":"Naome Mwesigwa, Patricio Millar Vernetti, Annet Kirabo, Bonnie Black, Tan Ding, Jose Martinez, Jose-Alberto Palma, Italo Biaggioni, Horacio Kaufmann, Cyndya A. Shibao","doi":"10.1007/s10286-024-01051-2","DOIUrl":"https://doi.org/10.1007/s10286-024-01051-2","url":null,"abstract":"<h3 data-test=\"abstract-sub-heading\">Purpose</h3><p>We previously reported that single doses of the norepinephrine transporter inhibitor, atomoxetine, increased standing blood pressure (BP) and ameliorated symptoms in patients with neurogenic orthostatic hypotension (nOH). We aimed to evaluate the effect of atomoxetine over four weeks in patients with nOH.</p><h3 data-test=\"abstract-sub-heading\">Methods</h3><p>A randomized, double-blind, placebo-controlled crossover clinical trial between July 2016 and May 2021 was carried out with an initial open-label, single-dose phase (10 or 18 mg atomoxetine), followed by a 1-week wash-out, and a subsequent double-blind 4-week treatment sequence (period 1: atomoxetine followed by placebo) or vice versa (period 2). The trial included a 2-week wash-out period. The primary endpoint was symptoms of nOH as measured by the orthostatic hypotension questionnaire (OHQ) assessed at 2 weeks.</p><h3 data-test=\"abstract-sub-heading\">Results</h3><p>A total of 68 patients were screened, 40 were randomized, and 37 completed the study. We found no differences in the OHQ composite score between atomoxetine and placebo at 2 weeks (−0.3 ± 1.7 versus −0.4 ± 1.5; <i>P</i> = 0.806) and 4 weeks (−0.6 ± 2.4 versus −0.5 ± 1.6; <i>P</i> = 0.251). There were no differences either in the OHSA scores at 2 weeks (3 ± 1.9 versus 4 ± 2.1; <i>P</i> = 0.062) and at 4 weeks (3 ± 2.2 versus 3 ± 2.0; <i>P</i> = 1.000) or in the OH daily activity scores (OHDAS) at 2 weeks (4 ± 3.0 versus 5 ± 3.1, <i>P</i> = 0.102) and 4 weeks (4 ± 3.0 versus 4 ± 2.7, <i>P</i> = 0.095). Atomoxetine was well-tolerated.</p><h3 data-test=\"abstract-sub-heading\">Conclusions</h3><p>While previous evidence suggested that acute doses of atomoxetine might be efficacious in treating nOH; results of this clinical trial indicated that it was not superior to placebo to ameliorate symptoms of nOH.</p><h3 data-test=\"abstract-sub-heading\">Trial registration</h3><p>ClinicalTrials.gov; NCT02316821.</p>","PeriodicalId":10168,"journal":{"name":"Clinical Autonomic Research","volume":"25 1","pages":""},"PeriodicalIF":5.8,"publicationDate":"2024-09-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142260627","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":"Clinical comparison of the 2008 and 2022 diagnostic criteria for early multiple system atrophy-cerebellar type.","authors":"Seoyeon Kim,Kyung Ah Woo,Jung Hwan Shin,Han-Joon Kim,Beomseok Jeon","doi":"10.1007/s10286-024-01061-0","DOIUrl":"https://doi.org/10.1007/s10286-024-01061-0","url":null,"abstract":"","PeriodicalId":10168,"journal":{"name":"Clinical Autonomic Research","volume":"21 1","pages":""},"PeriodicalIF":5.8,"publicationDate":"2024-09-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142204781","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}
Background: The autonomic nervous system (ANS) is critical in regulating involuntary bodily functions, including heart rate. Heart rate variability (HRV) reflects the complex interplay between the ANS and humoral factors, making it a valuable noninvasive tool for assessing autonomic function. While HRV has been extensively studied in adults, normative data for HRV in children, primarily based on long-term rhythm recordings, are limited.
Objective: This study aimed to establish comprehensive normative data for HRV in children.
Methods: In this retrospective study, we examined 24-h Holter monitors of children aged 1 day to 18 years, divided into six age groups, at Nemours Children's Health in Orlando, Florida, spanning the years 2013-2023. HRV analysis encompassed time-domain, frequency-domain, and nonlinear indices.
Results: Holter data for a total of 247 patients in six age groups were included. An age-related uptrend was observed in all time- and frequency-domain variables except the normalized unit of low-frequency power. Entropy analysis revealed contradictory results among different entropy techniques. Sample and approximate entropy analyses were consistent and showed less complexity and more predictability of HRV with decreasing heart rate, while Shannon entropy analysis showed the opposite. Fractal detrended fluctuation analysis exhibited significant decreases across the age groups, suggestive of diminishing self-similarity of HRV patterns.
Conclusion: Control of heart rate and HRV is a highly complex process and requires further study for a better understanding. It seems that no single parameter can fully elucidate the entire process. A combination of time-domain, frequency-domain, and nonlinear indices may be necessary to explain HRV behavior in the growing body.
{"title":"Comprehensive linear and nonlinear heart rate variability normative data in children.","authors":"Bahram Kakavand, Takeshi Tsuda, Aliya Centner, Safia Centner, Timothy Maul","doi":"10.1007/s10286-024-01056-x","DOIUrl":"https://doi.org/10.1007/s10286-024-01056-x","url":null,"abstract":"<p><strong>Background: </strong>The autonomic nervous system (ANS) is critical in regulating involuntary bodily functions, including heart rate. Heart rate variability (HRV) reflects the complex interplay between the ANS and humoral factors, making it a valuable noninvasive tool for assessing autonomic function. While HRV has been extensively studied in adults, normative data for HRV in children, primarily based on long-term rhythm recordings, are limited.</p><p><strong>Objective: </strong>This study aimed to establish comprehensive normative data for HRV in children.</p><p><strong>Methods: </strong>In this retrospective study, we examined 24-h Holter monitors of children aged 1 day to 18 years, divided into six age groups, at Nemours Children's Health in Orlando, Florida, spanning the years 2013-2023. HRV analysis encompassed time-domain, frequency-domain, and nonlinear indices.</p><p><strong>Results: </strong>Holter data for a total of 247 patients in six age groups were included. An age-related uptrend was observed in all time- and frequency-domain variables except the normalized unit of low-frequency power. Entropy analysis revealed contradictory results among different entropy techniques. Sample and approximate entropy analyses were consistent and showed less complexity and more predictability of HRV with decreasing heart rate, while Shannon entropy analysis showed the opposite. Fractal detrended fluctuation analysis exhibited significant decreases across the age groups, suggestive of diminishing self-similarity of HRV patterns.</p><p><strong>Conclusion: </strong>Control of heart rate and HRV is a highly complex process and requires further study for a better understanding. It seems that no single parameter can fully elucidate the entire process. A combination of time-domain, frequency-domain, and nonlinear indices may be necessary to explain HRV behavior in the growing body.</p>","PeriodicalId":10168,"journal":{"name":"Clinical Autonomic Research","volume":" ","pages":""},"PeriodicalIF":3.9,"publicationDate":"2024-09-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142153252","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":"Autoimmune autonomic ganglionopathy and myasthenia gravis: a case report and review of the literature.","authors":"Jingwen Yan, Huaxia Yang, Xiaona Jin, Ying Tan, Yuzhou Guan","doi":"10.1007/s10286-024-01059-8","DOIUrl":"https://doi.org/10.1007/s10286-024-01059-8","url":null,"abstract":"","PeriodicalId":10168,"journal":{"name":"Clinical Autonomic Research","volume":" ","pages":""},"PeriodicalIF":3.9,"publicationDate":"2024-08-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141916217","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}
Pub Date : 2024-08-01Epub Date: 2024-06-25DOI: 10.1007/s10286-024-01040-5
Ryan Solinsky, Kathryn Burns, J Andrew Taylor, Wolfgang Singer
Purpose: This work's purpose was to quantify rapid sympathetic activation in individuals with spinal cord injury (SCI), and to identify associated correlations with symptoms of orthostatic hypotension and common autonomically mediated secondary medical complications.
Methods: This work was a cross-sectional study of individuals with SCI and uninjured individuals. Symptoms of orthostatic hypotension were recorded using the Composite Autonomic Symptom Score (COMPASS)-31 and Autonomic Dysfunction following SCI (ADFSCI) survey. Histories of secondary complications of SCI were gathered. Rapid sympathetic activation was assessed using pressure recovery time of Valsalva maneuver. Stepwise multiple linear regression models identified contributions to secondary medical complication burden.
Results: In total, 48 individuals (24 with SCI, 24 uninjured) underwent testing, with symptoms of orthostatic hypotension higher in those with SCI (COMPASS-31, 3.3 versus 0.6, p < 0.01; ADFSCI, 21.2 versus. 3.2, p < 0.01). Pressure recovery time was prolonged after SCI (7.0 s versus. 1.7 s, p < 0.01), though poorly correlated with orthostatic symptom severity. Neurological level of injury after SCI influenced pressure recovery time, with higher injury levels associated with more prolonged time. Stepwise multiple linear regression models identified pressure recovery time as the primary explanation for variance in number of urinary tract infections (34%), histories of hospitalizations (12%), and cumulative secondary medical complication burden (24%). In all conditions except time for bowel program, pressure recovery time outperformed current clinical tools for assessing such risk.
Conclusions: SCI is associated with impaired rapid sympathetic activation, demonstrated here by prolonged pressure recovery time. Prolonged pressure recovery time after SCI predicts higher risk for autonomically mediated secondary complications, serving as a viable index for more "autonomically complete" injury.
{"title":"Valsalva maneuver pressure recovery time is prolonged following spinal cord injury with correlations to autonomically-influenced secondary complications.","authors":"Ryan Solinsky, Kathryn Burns, J Andrew Taylor, Wolfgang Singer","doi":"10.1007/s10286-024-01040-5","DOIUrl":"10.1007/s10286-024-01040-5","url":null,"abstract":"<p><strong>Purpose: </strong>This work's purpose was to quantify rapid sympathetic activation in individuals with spinal cord injury (SCI), and to identify associated correlations with symptoms of orthostatic hypotension and common autonomically mediated secondary medical complications.</p><p><strong>Methods: </strong>This work was a cross-sectional study of individuals with SCI and uninjured individuals. Symptoms of orthostatic hypotension were recorded using the Composite Autonomic Symptom Score (COMPASS)-31 and Autonomic Dysfunction following SCI (ADFSCI) survey. Histories of secondary complications of SCI were gathered. Rapid sympathetic activation was assessed using pressure recovery time of Valsalva maneuver. Stepwise multiple linear regression models identified contributions to secondary medical complication burden.</p><p><strong>Results: </strong>In total, 48 individuals (24 with SCI, 24 uninjured) underwent testing, with symptoms of orthostatic hypotension higher in those with SCI (COMPASS-31, 3.3 versus 0.6, p < 0.01; ADFSCI, 21.2 versus. 3.2, p < 0.01). Pressure recovery time was prolonged after SCI (7.0 s versus. 1.7 s, p < 0.01), though poorly correlated with orthostatic symptom severity. Neurological level of injury after SCI influenced pressure recovery time, with higher injury levels associated with more prolonged time. Stepwise multiple linear regression models identified pressure recovery time as the primary explanation for variance in number of urinary tract infections (34%), histories of hospitalizations (12%), and cumulative secondary medical complication burden (24%). In all conditions except time for bowel program, pressure recovery time outperformed current clinical tools for assessing such risk.</p><p><strong>Conclusions: </strong>SCI is associated with impaired rapid sympathetic activation, demonstrated here by prolonged pressure recovery time. Prolonged pressure recovery time after SCI predicts higher risk for autonomically mediated secondary complications, serving as a viable index for more \"autonomically complete\" injury.</p>","PeriodicalId":10168,"journal":{"name":"Clinical Autonomic Research","volume":" ","pages":"413-419"},"PeriodicalIF":3.9,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141445722","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}
Pub Date : 2024-08-01Epub Date: 2024-06-12DOI: 10.1007/s10286-024-01039-y
Hebatallah R Rashed, Nathan P Staff, Margherita Milone, Michelle L Mauermann, Sarah Berini, William P Cheshire, Elizabeth A Coon, Robert D Fealey, Eric Sorenson, Jeremy Cutsforth-Gregory, Eduardo E Benarroch, Paola Sandroni, Phillip A Low, Wolfgang Singer, Kamal Shouman
Purpose: Prior studies reported evidence of autonomic involvement in motor neuron disease and suggested more severe dysfunction in upper motor neuron predominant syndromes. Hence, we sought to characterize autonomic impairment in primary lateral sclerosis.
Methods: Neurological evaluations, thermoregulatory sweat tests, and autonomic reflex screens were analyzed retrospectively in 34 primary lateral sclerosis patients (28 definite and 6 probable). Patients with other potential causes of autonomic failure and patients with autonomic testing results compromised by artifact were excluded.
Results: A total of 17 patients reported autonomic symptoms. Orthostatic lightheadedness was most frequent (8 patients), followed by bladder (7), bowel (5), and erectile dysfunction (3). The autonomic reflex screens of 33 patients were reviewed; 20 patients had abnormal studies. The thermoregulatory sweat tests of 19 patients were reviewed; 11 patients had abnormal studies. Composite Autonomic Severity Score was calculated for 33 patients and found abnormal in 20/33 patients (60.6%): 15/20 patients (75%) had mild impairment, and 5/20 patients (25%) had moderate impairment. The frequencies of testing abnormalities were: sudomotor 18/20 (90%), cardiovagal 9/20 (45%), and adrenergic 6/20 (30%). Sweat loss pattern analysis showed global, regional, and mixed patterns to be more common than length-dependent and distal patterns.
Conclusion: We found evidence of frequent autonomic dysfunction in primary lateral sclerosis, which is generally of modest severity akin to prior reports for amyotrophic lateral sclerosis, but more commonly in a pattern consistent with preganglionic/ganglionic localization. This suggests that primary lateral sclerosis, as with amyotrophic lateral sclerosis, is a multisystem disease that affects the autonomic nervous system.
{"title":"Autonomic impairment in primary lateral sclerosis.","authors":"Hebatallah R Rashed, Nathan P Staff, Margherita Milone, Michelle L Mauermann, Sarah Berini, William P Cheshire, Elizabeth A Coon, Robert D Fealey, Eric Sorenson, Jeremy Cutsforth-Gregory, Eduardo E Benarroch, Paola Sandroni, Phillip A Low, Wolfgang Singer, Kamal Shouman","doi":"10.1007/s10286-024-01039-y","DOIUrl":"10.1007/s10286-024-01039-y","url":null,"abstract":"<p><strong>Purpose: </strong>Prior studies reported evidence of autonomic involvement in motor neuron disease and suggested more severe dysfunction in upper motor neuron predominant syndromes. Hence, we sought to characterize autonomic impairment in primary lateral sclerosis.</p><p><strong>Methods: </strong>Neurological evaluations, thermoregulatory sweat tests, and autonomic reflex screens were analyzed retrospectively in 34 primary lateral sclerosis patients (28 definite and 6 probable). Patients with other potential causes of autonomic failure and patients with autonomic testing results compromised by artifact were excluded.</p><p><strong>Results: </strong>A total of 17 patients reported autonomic symptoms. Orthostatic lightheadedness was most frequent (8 patients), followed by bladder (7), bowel (5), and erectile dysfunction (3). The autonomic reflex screens of 33 patients were reviewed; 20 patients had abnormal studies. The thermoregulatory sweat tests of 19 patients were reviewed; 11 patients had abnormal studies. Composite Autonomic Severity Score was calculated for 33 patients and found abnormal in 20/33 patients (60.6%): 15/20 patients (75%) had mild impairment, and 5/20 patients (25%) had moderate impairment. The frequencies of testing abnormalities were: sudomotor 18/20 (90%), cardiovagal 9/20 (45%), and adrenergic 6/20 (30%). Sweat loss pattern analysis showed global, regional, and mixed patterns to be more common than length-dependent and distal patterns.</p><p><strong>Conclusion: </strong>We found evidence of frequent autonomic dysfunction in primary lateral sclerosis, which is generally of modest severity akin to prior reports for amyotrophic lateral sclerosis, but more commonly in a pattern consistent with preganglionic/ganglionic localization. This suggests that primary lateral sclerosis, as with amyotrophic lateral sclerosis, is a multisystem disease that affects the autonomic nervous system.</p>","PeriodicalId":10168,"journal":{"name":"Clinical Autonomic Research","volume":" ","pages":"421-425"},"PeriodicalIF":3.9,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141305552","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}
Pub Date : 2024-08-01Epub Date: 2024-06-27DOI: 10.1007/s10286-024-01044-1
Surat Kulapatana, Andre' Diedrich, Patharapan Lersritwimanmaen, Wattana B Watanapa
Postural orthostatic tachycardia syndrome (POTS) is a heterogeneous autonomic disorder. All patients have exaggerated tachycardia upon standing, but the pathophysiology may be diverse. We present a young adult Thai male with a chief complaint of palpitations while in an upright posture since childhood. The patient underwent a modified Ewing test battery which included standing test, deep breathing, and Valsalva maneuver. His heart rate increased more than 30 beats per minute (bpm) during repeated active stand tests (65 to 110 bpm and 77 to 108 bpm), while upright diastolic blood pressure increased more than 10 mmHg. Normal Valsalva ratio (2.01 and 1.86) and baseline heart rate variability (HFRRI = 4030.24 ms2 and 643.92 ms2) indicated intact vagal function. High low-frequency systolic blood pressure variability (LFSBP = 20.93 mmHg2), increased systolic blood pressure overshoot in phase IV of Valsalva (42 mmHg), and increased upright diastolic blood pressure indicated a hyperadrenergic state. In conclusion, the overall autonomic profile was compatible with hyperadrenergic POTS. Thus, we confirmed the first male POTS case reported in Thailand. We demonstrated the importance of autonomic function testing with continuous measurements to confirm POTS. There is a need for further research in POTS in Thailand.
{"title":"Postural orthostatic tachycardia syndrome in a Thai male patient.","authors":"Surat Kulapatana, Andre' Diedrich, Patharapan Lersritwimanmaen, Wattana B Watanapa","doi":"10.1007/s10286-024-01044-1","DOIUrl":"10.1007/s10286-024-01044-1","url":null,"abstract":"<p><p>Postural orthostatic tachycardia syndrome (POTS) is a heterogeneous autonomic disorder. All patients have exaggerated tachycardia upon standing, but the pathophysiology may be diverse. We present a young adult Thai male with a chief complaint of palpitations while in an upright posture since childhood. The patient underwent a modified Ewing test battery which included standing test, deep breathing, and Valsalva maneuver. His heart rate increased more than 30 beats per minute (bpm) during repeated active stand tests (65 to 110 bpm and 77 to 108 bpm), while upright diastolic blood pressure increased more than 10 mmHg. Normal Valsalva ratio (2.01 and 1.86) and baseline heart rate variability (HF<sub>RRI</sub> = 4030.24 ms<sup>2</sup> and 643.92 ms<sup>2</sup>) indicated intact vagal function. High low-frequency systolic blood pressure variability (LF<sub>SBP</sub> = 20.93 mmHg<sup>2</sup>), increased systolic blood pressure overshoot in phase IV of Valsalva (42 mmHg), and increased upright diastolic blood pressure indicated a hyperadrenergic state. In conclusion, the overall autonomic profile was compatible with hyperadrenergic POTS. Thus, we confirmed the first male POTS case reported in Thailand. We demonstrated the importance of autonomic function testing with continuous measurements to confirm POTS. There is a need for further research in POTS in Thailand.</p>","PeriodicalId":10168,"journal":{"name":"Clinical Autonomic Research","volume":" ","pages":"467-475"},"PeriodicalIF":3.9,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11658283/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141466573","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}
Pub Date : 2024-08-01Epub Date: 2024-07-13DOI: 10.1007/s10286-024-01045-0
Otto Appenzeller
This is a self report. It is age related because I am 96 years old; about 5 years ago I was asymptomatic in this respect. I describe the difficulties to think to have to take a breath without getting evidence of hypoxia such as flashes of light and other evidence of hypoxia such as loss of muscle tone. The problem I have is loss of autonomic function which normally controls breathing without the need to think to take a breath.
{"title":"Breathing difficulties in a nonagenarian: a self report.","authors":"Otto Appenzeller","doi":"10.1007/s10286-024-01045-0","DOIUrl":"10.1007/s10286-024-01045-0","url":null,"abstract":"<p><p>This is a self report. It is age related because I am 96 years old; about 5 years ago I was asymptomatic in this respect. I describe the difficulties to think to have to take a breath without getting evidence of hypoxia such as flashes of light and other evidence of hypoxia such as loss of muscle tone. The problem I have is loss of autonomic function which normally controls breathing without the need to think to take a breath.</p>","PeriodicalId":10168,"journal":{"name":"Clinical Autonomic Research","volume":" ","pages":"477"},"PeriodicalIF":3.9,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141603389","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}