Rim D, Pham W, Fatouleh R
… +4 more, Hennessy A, Schlaich M, Henderson LA, Macefield VG
Clin Auton Res
· 2025 Dec · PMID 41075070
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PURPOSE: Hypertension is characterised by both enlarged perivascular spaces (ePVS) and chronically elevated resting sympathetic outflow. ePVS is associated with heart rate variability, suggesting links to autonomic outfl...PURPOSE: Hypertension is characterised by both enlarged perivascular spaces (ePVS) and chronically elevated resting sympathetic outflow. ePVS is associated with heart rate variability, suggesting links to autonomic outflow; however, heart rate variability offers limited information on sympathetic nerve activity. Here, we assessed whether ePVS are associated with muscle sympathetic nerve activity (MSNA) in 25 hypertensive patients and 50 healthy normotensive adults. METHODS: T1-weighted MRI anatomical brain images were analysed for ePVS using a deep learning-based segmentation algorithm (nnU-Net). Spontaneous bursts of MSNA were recorded from the right common peroneal nerve via a tungsten microelectrode immediately before the MRI scan in a supine position. A backward regression analysis was conducted to test the relationship between ePVS and MSNA. RESULTS: Significant associations were found between MSNA and ePVS in the white matter (β = 1.02, p = 0.007), basal ganglia (β = 0.43, p = 0.001), and hippocampus (β = 0.24, p = 0.010) in healthy normotensive adults. Similar associations were observed in individuals with hypertension. Notably, the association between MSNA and midbrain ePVS cluster was only observed in the hypertensive group (β = 0.41, p = 0.005). CONCLUSION: ePVS were associated with MSNA in both normotensive and hypertensive patients. These findings warrant further research into the causal relationship between MSNA and ePVS and highlight the potential for ePVS as a neuroimaging biomarker for sympathetic nerve activity.
Woo T, Kim Y, Kim J
… +6 more, Park JW, Lee SU, Park E, Kim GJ, Kim BJ, Kim JS
Clin Auton Res
· 2026 Feb · PMID 41045395
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BACKGROUND AND OBJECTIVE: The association between blood pressure variability (BPV) or heart rate variability (HRV) and the baroreflex is well established. However, the role of the vestibular-autonomic reflex in regulatin...BACKGROUND AND OBJECTIVE: The association between blood pressure variability (BPV) or heart rate variability (HRV) and the baroreflex is well established. However, the role of the vestibular-autonomic reflex in regulating BP and HR stability has primarily been explored only in experimental studies. We aimed to delineate the association of BPV and HRV with otolith function in postural orthostatic tachycardia syndrome (POTS). METHODS: We retrospectively analyzed data from consecutive patients with POTS recruited between April 2021 and April 2025 at a tertiary referral-based hospital in South Korea. All patients underwent a head-up tilt table test using a Finometer device and cervical (cVEMP) and ocular vestibular evoked-myogenic potentials (oVEMP). The Finometer data were analyzed using a power spectral analysis. The Finometer, cVEMP, and oVEMP data were compared with those of 32 age- and sex-matched healthy participants. RESULTS: A total of 47 patients with POTS (mean age standard deviation [SD] = 33 10 years; 30 female patients) and 32 age- and sex-matched healthy participants were included for analysis. The n1-p1 amplitude of oVEMP was larger in patients with POTS than in healthy participants (p = 0.002). p13 latency was negatively correlated with the SD of heart rate in the supine position in patients with POTS (p = 0.001), a trend not observed in healthy participants. The n1-p1 amplitude (odds ratio [95% confidence interval] = 1.27 [1.08-1.49], p = 0.004) and root mean square of successive differences (RMSSD) during tilting (0.82 [0.72-0.93], p = 0.001) were associated with POTS after adjusting for other covariates. CONCLUSIONS: Otolithic function may play a role in accentuating BPV and HRV in POTS by contributing to enhanced sympathetic outflow.
Saren J, Lieten S, Petrovic M
… +3 more, Islamaj E, Bautmans I, Debain A
Clin Auton Res
· 2025 Dec · PMID 41028404
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PURPOSE: This study evaluated the effectiveness of morning versus bedtime antihypertensive medication administration in reducing ambulatory blood pressure (BP) in older adults aged ≥ 65, and to assess whether administrat...PURPOSE: This study evaluated the effectiveness of morning versus bedtime antihypertensive medication administration in reducing ambulatory blood pressure (BP) in older adults aged ≥ 65, and to assess whether administration timing influences conversion from a non-dipper to a dipper BP profile. METHODS: Eight randomized controlled trials were identified through systematically screening of the PubMed and Web of Science databases. Risk of bias was assessed using the Cochrane Risk of Bias tool. Meta-analyses were conducted with Review Manager version 5.4 to compare the efficacy of morning versus bedtime administration on ambulatory BP indices. RESULTS: Bedtime administration resulted in significantly greater reductions in nocturnal systolic BP (mean difference [MD] - 4.52 mmHg, [lower and upper 95% confidence intervals [CI] - 7.15; - 1.90]; p = 0.0007) and diastolic BP (MD - 2.00 mmHg, [95% CI - 2.90; - 1.10]; p < 0.0001). No significant differences were observed in diurnal systolic BP (MD 1.28 mmHg, [95% CI - 0.17; 2.72]; p = 0.08), diastolic BP (MD 0.34 mmHg, [95% CI - 0.49; 1.16]; p = 0.42), 24/48-h systolic BP (MD - 0.02 mmHg, [95% CI - 1.37; 1.33]; p = 0.98), or 24/48-h diastolic BP (MD - 0.50 mmHg, [95% CI - 1.45; 0.45]; p = 0.30). Sensitivity analysis excluding the controversial data from Hermida confirmed significantly greater reductions in nocturnal systolic and diastolic BP with bedtime administration. Two of three studies reported that bedtime administration was associated with a lower proportion of non-dippers than morning treatment. CONCLUSION: Bedtime antihypertensive administration improves control of nocturnal BP in older adults aged ≥ 65 and may facilitate restoration to a dipper BP profile. No significant differences were observed in diurnal or 24/48-h mean BP reductions compared with morning administration.
Hamner JW, Draghici A, Martinez-Magallanes D
… +1 more, Taylor JA
Clin Auton Res
· 2026 Feb · PMID 40971103
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PURPOSE: Spontaneous indices have been widely used to assess baroreflex gain despite their numerous limitations and concerns regarding their validity, reliability, and reproducibility. In this retrospective study, we inv...PURPOSE: Spontaneous indices have been widely used to assess baroreflex gain despite their numerous limitations and concerns regarding their validity, reliability, and reproducibility. In this retrospective study, we investigated whether spontaneous baroreflex indices reflect cardiovagal baroreflex gain assessed by the neck-chamber technique in those with spinal cord injury (SCI) and in uninjured individuals. SCI represents a model of preserved cardiovagal baroreflex control coupled with impaired sympathetic effects on the vasculature. METHODS: We derived three spontaneous indices of baroreflex sensitivity (sequence method, low-frequency (LF), and high-frequency (HF) transfer function) and compared them with baroreflex gain obtained via the neck-chamber technique in adults with SCI (n = 29; neurological level C1-T10, ≤ 2 years since injury) and uninjured adults (n = 14). RESULTS: In both groups, spontaneous indices were highly correlated with each other (all p < 0.01). In uninjured participants, neck suction baroreflex gain did not relate to any spontaneous index. In individuals with SCI, neck-chamber gain correlated significantly with spontaneous indices (all r > 0.43, p < 0.05); these relationships were significantly stronger in individuals with neurologically complete injuries (sequence: r = 0.67, p < 0.01; LF: r = 0.79, p < 0.001; HF: r = 0.76, p < 0.001). However, Bland-Altman analysis revealed a strong proportional bias, with spontaneous indices consistently and progressively overestimating neck-chamber gain (all r > 0.91, p < 0.001). CONCLUSIONS: These results suggest that sympathetic activity is largely responsible for the lack of correspondence between spontaneous and neck-chamber baroreflex gains. However, even in individuals with a neurologically complete SCI, where sympathetic influences are minimal, spontaneous indices may not consistently reflect baroreflex gain derived from other methods.
Clin Auton Res
· 2025 Dec · PMID 40963088
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BACKGROUND: The autonomic synucleinopathy multiple system atrophy (MSA) can be difficult to distinguish clinically from Parkinson disease with orthostatic hypotension (PD+OH). F-Dopamine positron emission tomography sepa...BACKGROUND: The autonomic synucleinopathy multiple system atrophy (MSA) can be difficult to distinguish clinically from Parkinson disease with orthostatic hypotension (PD+OH). F-Dopamine positron emission tomography separates these conditions based on cardiac noradrenergic deficiency in PD+OH and not in MSA but is available only at the NIH Clinical Center. 3,4-Dihydroxyphenylglycol (DHPG) is the main neuronal metabolite of norepinephrine. This retrospective observational study examined whether DHPG levels in cerebrospinal fluid (CSF) or plasma differentiate MSA from PD+OH. METHODS: We reviewed CSF and plasma neurochemical data from all patients referred for evaluation at the NIH Clinical Center between 1995 and 2024 for chronic autonomic failure or parkinsonism. A concurrently studied comparison group included healthy volunteers or patients with orthostatic intolerance. RESULTS: CSF DHPG was decreased in MSA (N = 67, p < 0.0001) compared to the controls but also tended to be decreased in PD+OH (N = 31, p = 0.0776). Antecubital venous plasma DHPG was decreased in PD+OH (N = 47, p = 0.0064) but not in MSA. CSF/plasma concentration ratios of DHPG were lower in MSA than in PD+OH (p = 0.0005). Cardiac arteriovenous increments in plasma DHPG and cardiac norepinephrine spillovers were strikingly decreased in PD+OH (N = 6) and were lower than in MSA (N = 20, p < 0.0001 each). Combining cardiac arteriovenous increments in plasma DHPG with norepinephrine spillovers completely separated PD+OH from MSA. CONCLUSIONS: CSF/plasma ratios of DHPG, cardiac arteriovenous increments in plasma DHPG, and cardiac norepinephrine spillovers separate MSA from PD+OH. On the basis of our results we propose that biomarker combinations involving DHPG in biofluids may enable a clinical laboratory distinction of MSA from PD+OH.
Koay S, Provitera V, Vichayanrat E
… +8 more, Caporaso G, Valerio F, Stancanelli A, Borreca I, Manganelli F, Santoro L, Nolano M, Iodice V
Clin Auton Res
· 2026 Feb · PMID 40938512
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PURPOSE: Cardiovascular autonomic failure and neurogenic orthostatic hypotension (nOH) are common and disabling in Parkinson's disease (PD) and multiple system atrophy (MSA). Recent studies have shown evidence of postgan...PURPOSE: Cardiovascular autonomic failure and neurogenic orthostatic hypotension (nOH) are common and disabling in Parkinson's disease (PD) and multiple system atrophy (MSA). Recent studies have shown evidence of postganglionic autonomic denervation in MSA as well as PD. We aimed to characterise the relationship between nOH, autonomic failure and postganglionic denervation in PD and MSA. We hypothesised that postganglionic autonomic denervation contributes to the development of nOH and correlates with the severity of cardiovascular autonomic failure. METHODS: We assessed 57 patients (37 PD, 20 MSA, median 64 [IQR 59-70] years) with cardiovascular autonomic testing; dynamic sweat testing; plasma noradrenaline levels; skin biopsies for quantification of intraepidermal, pilomotor and sudomotor innervation; and autonomic symptom questionnaires. RESULTS: Overall, 78% of patients with MSA and 36% with PD had nOH ≥ 20/10 mmHg. The MSA group had more severe nOH, sudomotor dysfunction and cutaneous denervation, with higher supine noradrenaline than the PD group. Only supine noradrenaline differed between MSA and PD with nOH subgroups (P = 0.04). Overall, patients with nOH demonstrated more severe (1) cardiovascular autonomic failure, with reduced pressor responses to isometric exercise, deep breathing and Valsalva ratio; (2) intraepidermal, pilomotor and sudomotor denervation; and (3) autonomic symptoms and Hoehn-Yahr grade. The severity of nOH and cardiovascular autonomic failure correlated with autonomic denervation, patient symptoms and Hoehn-Yahr grade (ρ ≥ 0.50). CONCLUSIONS: nOH was associated with cutaneous autonomic denervation in both PD and MSA, with correlations between cardiovascular autonomic failure, cutaneous denervation and Hoehn-Yahr grade. Postganglionic autonomic denervation may contribute to nOH in PD and MSA, and affect responses to therapeutic agents.
Saedon NI, Frith J, Wan Ahmad WA
… +1 more, Tan MP
Clin Auton Res
· 2026 Feb · PMID 40920260
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BACKGROUND: Orthostatic hypotension (OH) is prevalent in older adults and is often associated with falls. However, the presence or absence of symptoms in OH may be mediated by cerebral autoregulation, which helps maintai...BACKGROUND: Orthostatic hypotension (OH) is prevalent in older adults and is often associated with falls. However, the presence or absence of symptoms in OH may be mediated by cerebral autoregulation, which helps maintain cerebral perfusion during blood pressure fluctuations. METHODS: We recruited 40 older adults (aged ≥ 55 years) from the Malaysian Elders Longitudinal Research (MELoR) cohort. Participants underwent cerebral blood flow velocity monitoring using transcranial Doppler ultrasonography and beat-to-beat blood pressure recording. Three protocols were used: active stand, mental arithmetic, and Valsalva manoeuvre. Participants were categorized, based on OH (≥ 30 mmHg systolic drop) and fall history, into four groups. Cerebrovascular resistance (CVR) was derived and analysed. RESULTS: Participants with OH but no history of falls demonstrated preserved autoregulatory responses, as reflected by adaptive reductions in CVR. In contrast, fallers-regardless of OH status-had impaired CVR modulation. Significant group differences were found during the active stand test at 165 s and 180 s (p < 0.05). CONCLUSION: Preserved cerebral autoregulation may protect older adults with OH from symptomatic manifestations such as falls. Targeting cerebral autoregulation could offer novel approaches for preventing falls in this population.
Clin Auton Res
· 2025 Dec · PMID 40913641
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PURPOSE: Orthostatic hypotension (OH) is a non-motor feature in people with Parkinson's disease that can lead to falls from syncope. Current knowledge is lacking on the effects of OH on gait function. METHODS: Participan...PURPOSE: Orthostatic hypotension (OH) is a non-motor feature in people with Parkinson's disease that can lead to falls from syncope. Current knowledge is lacking on the effects of OH on gait function. METHODS: Participants enrolled in a prospectively monitored longitudinal cohort who had OH on vitals at one of two consecutive visits approximately 6 months apart were analyzed. Gait measures were compared at the orthostatic versus non-orthostatic visit using the Wilcoxon signed-rank test and a linear mixed model. Motor and non-motor assessments were also compared. RESULTS: Thirty-nine people with Parkinson's disease and seven age-matched controls in the longitudinal study met the inclusion criteria. Mean stride length and foot-strike length were shorter, and stride velocity was slower at the orthostatic visit compared to the non-orthostatic visit in people with Parkinson's disease. Levodopa dose, duration from last dose, motor and total Unified Parkinson's Disease Rating Scale scores, and cognitive and non-motor assessment scores did not differ between visits. The number of people reporting falls was higher at the orthostatic visit, and the fall frequency in fallers also trended higher. Gait measures did not differ between those with and without symptomatic OH. CONCLUSION: In our cohort of people with Parkinson's disease with repeated measures, gait was more parkinsonian (slower velocity, shorter stride, and decreased foot strike to the ground) when they had OH on vitals at the visit than when they did not. Based on our results, future studies exploring the impact of adequate treatment of OH on gait function are warranted.
Zhang X, Dai S, Wang H
… +6 more, Jiang R, Xie Q, Wen J, Han M, Xu Y, Wang G
Clin Auton Res
· 2025 Dec · PMID 40900354
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PURPOSE: Vasovagal syncope (VVS) involves autonomic dysregulation affecting cardiac electrical activity. The Tp-Te interval, reflecting transmural repolarization dispersion, may help predict positive head-up tilt test (H...PURPOSE: Vasovagal syncope (VVS) involves autonomic dysregulation affecting cardiac electrical activity. The Tp-Te interval, reflecting transmural repolarization dispersion, may help predict positive head-up tilt test (HUTT) responses in patients with suspected VVS. METHODS: A total of 179 patients with suspected VVS were included in the study. A HUTT was performed in enrolled patients, which were divided into HUTT-negative and HUTT-positive groups, and the HUTT-positive group was further classified into three subgroups of "vasodepressor," "cardioinhibitory," and "mixed-type" responses to HUTT. QT interval, corrected QT (QTc) interval, and Tp-Te interval were measured by the baseline 12-lead surface electrocardiograph recorded before HUTT. RESULTS: The QT interval, QTc interval, and Tp-Te interval in the HUTT-positive group were higher than those in the HUTT-negative group (P < 0.001). Tp-Te was higher in the cardioinhibitory and mixed-type subgroups than in the vasodepressor subgroup (P < 0.05). Receiver operating characteristic curve analysis showed that Tp-Te higher than 88 ms was a significant predictor of positive HUTT results (71.70% sensitivity and 75.90% specificity), with a predictive value significantly higher than QT and QTc (P < 0.05), and Tp-Te higher than 95 ms predicted cardioinhibitory and mixed-type response to HUTT (75% sensitivity, and 57.10% specificity). CONCLUSION: Baseline myocardial TDR is associated with VVS and susceptibility to VVS. The baseline Tp-Te interval might be used as a novel noninvasive index for differentiating cardioinhibitory, mixed-type, and vasodepressor responses to HUTT and for predicting the occurrence of cardioinhibitory responses in VVS patients.
Clin Auton Res
· 2025 Dec · PMID 40885865
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PURPOSE: We aimed to clarify the mechanism for presyncope, defined as the gradual onset of hypotension, starting some minutes before vasovagal syncope. Although there is a fall in cardiac output and usually vasodilatatio...PURPOSE: We aimed to clarify the mechanism for presyncope, defined as the gradual onset of hypotension, starting some minutes before vasovagal syncope. Although there is a fall in cardiac output and usually vasodilatation, the control of sympathetic activity during presyncope is uncertain. METHODS: We retrospectively compared haemodynamics and muscle sympathetic nerve activity levels from positive tilt tests (without provocation) in patients with known vasovagal syncope (age 41 ± 3 years, 13 female, n = 27) to controls (age 39 ± 3 years, 8 female, n = 13). We used sequence methods to measure vascular sympathetic and cardiovagal baroreflex gain at baseline (lying supine) during tilt, presyncope and recovery. RESULTS: Patients were tilted for 18.1 ± 1 min, and mean arterial pressure fell to 62 ± 3 mmHg before tilt-back. At baseline and early tilt, all haemodynamic variables were similar to controls, however sympathetic baroreflex gain was increased: -2.7 ± 0.2 bursts/100 beats/mmHg versus -2.0 ± 0.3 (p = 0.03). Cardiovagal baroreflex gain was increased at baseline 11.8 ± 0.6 ms/mmHg versus 9.3 ± 0.8 (p = 0.02). During early presyncope (from 8 to 4 min before tilt-back), sympathetic baroreflex gain fell to -2.4 bursts/100 b/mmHg and thereafter to -0.5 ± 0.3 (p = 0.01) during late presyncope, before losing correlation with mean arterial pressure. In some patients, the regression coefficient reversed before correlation was lost (n = 8) but this did not result in lower levels of nerve activity. At tilt-back, nerve activity fell below baseline levels in at least 63% of patients. CONCLUSION: Presyncope appeared to be initiated by a fall in sympathetic baroreflex gain despite increased levels at baseline and early tilt.
Mwesigwa N, Williamson H, Turner S
… +5 more, Pouya ME, Ding T, Pedro OJ, Anderson KE, Shibao CA
Clin Auton Res
· 2025 Dec · PMID 40856938
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PURPOSE: Postural orthostatic tachycardia syndrome (POTS) is characterized by an excessive heart rate increase upon standing, often associated with dizziness, gastrointestinal symptoms, and decreased functional capacity....PURPOSE: Postural orthostatic tachycardia syndrome (POTS) is characterized by an excessive heart rate increase upon standing, often associated with dizziness, gastrointestinal symptoms, and decreased functional capacity. Acute hepatic porphyrias (AHP) are rare metabolic disorders with nonspecific neurovisceral and autonomic symptoms, some of which overlap with POTS. The purpose of this study was to evaluate AHP by molecular and biochemical testing in patients with POTS. METHODS: We studied 50 patients diagnosed with POTS and gastrointestinal symptoms at the Vanderbilt Autonomic Dysfunction Center. They underwent neuro-hormonal evaluation for POTS and genetic and biochemical screening for AHP. Genetic testing was aimed mainly at the four genes relevant to AHPs. Porphobilinogen (PBG), delta-aminolevulinic acid (ALA), and total porphyrins were measured in urine with normalization to creatinine. RESULTS: The average age of the patients was 33 ± 8.6 years, 96% were female, and the average BMI was 28 ± 7.2 kg/m, average systolic blood pressure was 120 ± 15.5 mmHg, average heart rate was 77 ± 13.6 bpm at baseline, and average SBP was 126 ± 19.1 mmHg. A heart rate of 111 ± 15.8 bpm at 10 min upright, showed normal cardiovascular reflexes. The COMPASS-31 total score was 32 ± 8.4, with a normal autonomic function test. Urine PBG averaged 1 ± 0.7 mg/g creatinine, ALA 2 ± 0.9 mg/g creatinine, and total porphyrins 172 ± 74.2 mmol/g creatinine, which were all normal. None had variants in the four genes associated with AHPs. Three patients were heterozygous for a common low expression ferrochelatase gene variant (FECH). CONCLUSIONS: We found no evidence of AHP in patients with POTS with uncontrolled gastrointestinal symptoms, suggesting that screening for AHP, a rare genetic disorder, may not be warranted.
Russo V, Comune A, Di Nardo G
… +7 more, Parente E, Di Marco GM, De Nigris A, Russo MG, Sarubbi B, Nigro G, Brignole M
Clin Auton Res
· 2025 Dec · PMID 40759876
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BACKGROUND: Syncope is a prevalent issue in pediatric patients. The nitroglycerin (NTG)-potentiated head-up tilt test (HUTT) is widely used in adults for diagnosing reflex syncope; however, few and contrasting data are a...BACKGROUND: Syncope is a prevalent issue in pediatric patients. The nitroglycerin (NTG)-potentiated head-up tilt test (HUTT) is widely used in adults for diagnosing reflex syncope; however, few and contrasting data are available in pediatric populations. The aim of our study was to evaluate the positivity rate and types of responses to NTG-potentiated HUTT in pediatric patients with suspected reflex syncope. METHODS: We conducted a retrospective multicenter analysis of 307 pediatric patients (mean age: 14.4 ± 2.8 years; 57.6% female) who underwent HUTT at two syncope units in Naples, Italy. A group of 16 healthy pediatric subjects (13 ± 3.2 years; 37.5% female) with no history of syncope was used as a control. We described the HUTT overall positivity rate and responses; moreover, the positivity rate, sensitivity, and specificity were evaluated. A multivariate analysis was performed to test the association of positive response to HUTT with a set of clinical covariates. RESULTS: The overall HUTT positivity rate was 74.9%, ranging from 51.5% to 81.6% among pediatric patients with non-classical and classical presentation, respectively. The HUTT positivity rate among healthy control group was 18.7%; consequently the HUTT specificity was 81.3%. Younger age (OR: 0.84; p = 0.005) and female sex (OR: 2.3; p = 0.005) were independent predictors of HUTT positivity; in contrast, the non-classical presentation of syncope (OR: 0.23; p < 0.001) and situational syncope (OR: 0.2; p = 0.006) correlated negatively with HUTT positivity. CONCLUSIONS: NTG-potentiated HUTT showed a high positivity rate, good sensitivity, and specificity in pediatric patients with suspected reflex syncope. Some patients and syncope-related features independently correlated with HUTT positivity. Cardioinhibitory response was more prevalent in pediatric patients with a non-classical presentation of reflex syncope.
Padilla H, Cheshire WP, Benarroch EE
… +8 more, Berini SE, Cutsforth-Gregory JK, Mauermann ML, Sandroni P, Shouman K, Low PA, Singer W, Coon EA
Clin Auton Res
· 2025 Dec · PMID 40721556
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PURPOSE: 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 p...PURPOSE: 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. METHODS: 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. RESULTS: 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). CONCLUSIONS: 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.
Clin Auton Res
· 2025 Dec · PMID 40715652
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OBJECTIVE: 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. METHODS: A systematic...OBJECTIVE: 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. METHODS: 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 I statistic. RESULTS: 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). CONCLUSIONS: 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.