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The article published in Frontiers in Neurology emphasizes that postural orthostatic tachycardia syndrome (POTS) and other autonomic disorders are distinct from functional neurologic disorders (FND). It highlights the objective diagnostic criteria for common autonomic disorders, such as POTS, which include orthostatic intolerance and specific vital sign parameters. The text also discusses the common comorbidities of POTS, the importance of distinguishing autonomic disorders from autonomic symptoms in psychiatric diseases, and the different pathophysiology and treatments for POTS and FND. The authors stress the need for accurate diagnosis and appropriate therapeutic pathways for patients with autonomic disorders. [Extracted from the article]

Purpose: Multiple system atrophy (MSA) is a rare neurodegenerative disease, often presented with orthostatic hypotension (OH), which is a disabling symptom but has not been very explored. Here, we investigated MSA patients with OH by using positron emission tomography (PET) with 18F-fluorodeoxyglucose (18F-FDG) and 11C-N-2-carbomethoxy-3-(4-fluorophenyl)-tropane (11C-CFT) for in vivo evaluation of the glucose metabolism and dopaminergic function of the brain. Methods: Totally, 51 patients with MSA and 20 healthy controls (HC) who underwent 18F-FDG PET/CT were retrospectively enrolled, among which 24 patients also underwent 11C-CFT PET/CT. All patients were divided into MSA-OH(+) and MSA-OH(-) groups. Then, statistical parametric mapping (SPM) method was used to reveal the regional metabolic and dopaminergic characteristics of MSA-OH(+) compared with MSA-OH(-). Moreover, the metabolic networks of MSA-OH(+), MSA-OH(-) and HC groups were also constructed and analyzed based on graph theory to find possible network-level changes in MSA patients with OH. Results: The SPM results showed significant hypometabolism in the pons and right cerebellar tonsil, as well as hypermetabolism in the left parahippocampal gyrus and left superior temporal gyrus in MSA-OH(+) compared with MSA-OH(-). A reduced 11C-CFT uptake in the left caudate was also shown in MSA-OH(+) compared with MSA-OH(-). In the network analysis, significantly reduced local efficiency and clustering coefficient were shown in MSA-OH(+) compared with HC, and decreased nodal centrality in the frontal gyrus was found in MSA-OH(+) compared with MSA-OH(-). Conclusion: In this study, the changes in glucose metabolism in the pons, right cerebellar tonsil, left parahippocampal gyrus and left superior temporal gyrus were found closely related to OH in MSA patients. And the decreased presynaptic dopaminergic function in the left caudate may contribute to OH in MSA. Taken together, this study provided in vivo pathophysiologic information on MSA with OH from neuroimaging approach, which is essential for a better understanding of MSA with OH. [ABSTRACT FROM AUTHOR]

Purpose of Review: Dysautonomia refers to the dysfunction of the autonomic nervous system and encompasses a wide variety of autonomic symptoms and disorders. The most common autonomic disorders are postural orthostatic tachycardia syndrome (POTS), neurocardiogenic syncope (NCS), and orthostatic hypotension (OH), which may be encountered in clinical practice as part of a triad of dysautonomia, hypermobility spectrum disorders (HSD), and mast cell activation syndrome (MCAS). Migraine is one of the most common comorbidities of POTS, HSD, and MCAS; conversely, these conditions are also prevalent in patients with migraine, especially in those with multiple systemic symptoms, such as chronic dizziness, lightheadedness, orthostatic intolerance, joint pain, and allergic symptoms. Diagnostic criteria, pathophysiologic mechanisms, and therapeutic considerations in patients with migraine and comorbid dysautonomia, HSD, and MCAS are reviewed. Recent Findings: Numerous studies indicate a significant overlap and shared pathophysiology in migraine, dysautonomia, HSD, and MCAS. In clinical setting, dysautonomia, HSD, and MCAS may present a diagnostic and therapeutic challenge in patients with migraine and require a high index of suspicion on the part of the neurologist. Summary: Diagnosis and treatment of these complex disorders in patients with migraine is essential to comprehensive patient-centric care, reduced symptom burden, and improved functional impairment secondary to both migraine and comorbidities. [ABSTRACT FROM AUTHOR]

Background and objective: Loss of intermediolateral nucleus (IML) neurons is considered to play a major role in orthostatic hypotension (OH) of multiple system atrophy (MSA). In Parkinson disease (PD) and dementia with Lewy bodies (DLB), autonomic phenomena such as OH are common and attributed to dysfunction of sympathetic, parasympathetic, and visceral autonomic neurons. However, apart from MSA, fewreports have focused on the neuropathologic aspects in PD and DLB. Here we assessed IML degeneration as well as the fine myelinated fibers (FFs; maximum diameter less than 3 m) considered to be preganglionic sympathetic nerve fibers derived from IML neurons in PD, DLB, MSA, and age-matched normal controls (NC). Methods: We counted IML neurons and measured the diameter and number of myelinated fibers of the ventral root at the level of the 12th thoracic segment. Results: Compared to NC, number of IML neurons and density of FF were significantly reduced in PD (53% and 67%), DLB (47% and 71%) and MSA (27% and 42%). Compared to combined group of PD and DLB without OH (OH-), IML neurons in combined group of PD and DLB with OH (OH+) were significantly reduced (77%). Compared to NC, FF densities in OH-, OH+ were significantly reduced (74% and 59%). The mean ratio of small to large myelinated fibers in OH+ (1.18), but not that in OH- (1.58), was significantly lower than that in NC (3.17). Conclusions: We present neuropathological evidence that IML neurons and FFs in the ventral root are reduced in PD and DLB and that the reduction was more severe in the combined group of OH+ than in OH-. [ABSTRACT FROM AUTHOR]

Background Rapid eye movement (REM) sleep behavioral disorder (RBD), orthostatic hypotension (OH), and cardiac sympathetic denervation were commonly observed in PD and are related in both the premotor and motor periods. This study is intended to evaluate if the OH and cardiac sympathetic denervation found in PD are associated with RBD. Methods Among 94 non-medicated and mild PD patients, 53 had RBD. Orthostatic vital signs and ambulatory 24-hour blood pressure values were recorded. 123 I-metaiodobenzylguanidine (MIBG) cardiac scintigraphy as obtained in all patients. The association between orthostatic hypotension, supine hypertension, nocturnal hypertension, non-dipping, myocardial MIBG uptake, and RBD was analyzed. Results RBD was associated with orthostatic hypotension. Patients with RBD had higher systolic blood pressure changes during orthostasis and lower myocardial MIBG uptake than patients without RBD and controls. Patients with OH also had lower mean H/M ratios those in the non-OH group. Conclusion This study showed that RBD was closely associated with OH and cardiac sympathetic denervation in patients with early and mild PD. The result also suggests that impaired cardiac sympathetic innervation could be the mechanism behind OH in PD. This association may be closely correlated with Braak alpha-synuclein pathogenetic sequences, which would account for the clinical spectrum of PD. [ABSTRACT FROM AUTHOR]

Abstract: In the spectrum of cognitive impairment, ranging from “pure” vascular dementia to Alzheimer's disease (AD), clinical interest has recently expanded from the brain to also include the vessels, shifting the pathophysiological focus from the leaves of synaptic dysfunction to the sap of cerebral microcirculation and the roots of cardiovascular function. From a diagnostic viewpoint, a thorough clinical evaluation of individuals presenting cognitive impairment might systematically include the assessment of the major cardiovascular rings of the chain linking regional perfusion to brain function: 1) lung (with assessment of asthma, chronic obstructive pulmonary disease, obstructive sleep apnea syndrome); 2) heart function (with clinical examination and echocardiography) and cardiovascular risk factors; 3) orthostatic hypotension (with medical history and measurement of heart rate and blood pressure in supine and upright positions); 4) aorta and large artery stiffness (with assessment of pulse wave velocity); 5) large cerebro-vascular vessel status (with neuroimaging techniques); 6) assessment of microcirculation (with cerebrovascular reactivity testing with transcranial Doppler sonography or MRI perfusion imaging); and 7) assessment of venous cerebral circulation. The apparent difference in approaches to “brain” and “vascular” environmental enrichment with physical, cognitive and sensorial training is conceptually identical to that of a constant gardener caring for an unhealthy tree, watering the leaves (“train the brain”) or simply the roots (“mind the vessel”). The therapeutic difference probably consists in the amount and quality of water added to the tree, rather than by where one pours it, with either a top-down (leaves to roots) or bottom-up (roots to leaves) approach. [Copyright &y& Elsevier]

Abstract: Objective: To describe physical capacity, autonomic function, and perceptions of exercise among adults with subacute spinal cord injury (SCI). Design: Cross-sectional. Setting: Two inpatient SCI rehabilitation programs in Canada. Participants: Participants (N=41; mean age ± SD, 38.9±13.7y) with tetraplegia (TP; n=19), high paraplegia (HP; n=8), or low paraplegia (LP; n=14) completing inpatient SCI rehabilitation (mean ± SD, 112.9±52.5d postinjury). Interventions: Not applicable. Main Outcome Measures: Peak exercise capacity was determined by an arm ergometry test. As a measure of autonomic function, orthostatic tolerance was assessed by a passive sit-up test. Self-efficacy for exercise postdischarge was evaluated by a questionnaire. Results: There was a significant difference in peak oxygen consumption and heart rate between participants with TP (11.2±3.4;mL·kg−1·min−1 113.9±19.7beats/min) and LP (17.1±7.5mL·kg−1·min−1; 142.8±22.7beats/min). Peak power output was also significantly lower in the TP group (30.0±6.9W) compared with the HP (55.5±7.56W) and LP groups (62.5±12.2W). Systolic blood pressure responses to the postural challenge varied significantly between groups (−3.0±33.5mmHg in TP, 17.8±14.7mmHg in HP, 21.6±18.7mmHg in LP). Orthostatic hypotension was most prevalent among participants with motor complete TP (73%). Results from the questionnaire revealed that although participants value exercise and see benefits to regular participation, they have low confidence in their abilities to perform the task of either aerobic or strengthening exercise. Conclusions: Exercise is well tolerated in adults with subacute SCI. Exercise interventions at this stage should focus on improving task-specific self-efficacy, and attention should be made to blood pressure regulation, particularly in individuals with motor complete TP. [Copyright &y& Elsevier]

Abstract: Objectives: To determine the effect of an escalating dose of droxidopa (100, 200, and 400mg) compared with placebo on seated blood pressure (BP) in hypotensive individuals with spinal cord injury (SCI). Secondarily, we aimed to determine the effect of droxidopa on (1) supine BP and heart rate, (2) the change in BP and heart rate when these individuals were transferred from the supine to the seated position, and (3) adverse event (AE) reporting. Design: Open-label dose titration trial. Setting: A Veterans Administration Medical Center. Participants: Participants with SCI (C3-T12) (N=10) were studied during 4 laboratory visits. Subjects visited the laboratory for about 5 hours on each visit, which incorporated a 30-minute seated baseline, a 30- to 60-minute supine, and a 4-hour seated postdrug observation. Interventions: Placebo on visit 1, droxidopa 100mg on visit 2, droxidopa 200mg on visit 3, and droxidopa 400mg on visit 4. Main Outcome Measures: BP and heart rate changes from baseline to the postdrug period, orthostatic heart rate and BP responses, and subjective AE reporting. Results: Seated BP was significantly elevated with 400mg droxidopa compared with placebo and 100mg droxidopa for 3 hours and was elevated for 2 hours compared with 200mg droxidopa. Increase in supine BP was not worsened following droxidopa, and the expected fall in BP when transferred to the seated position was prevented with droxidopa 200 and 400mg. There were no significant differences in the heart rate response or AE reporting among the study visits. Conclusions: Our preliminary findings suggest that droxidopa, at the doses tested, does not cause excessive increases in supine BP and the 400-mg dose appears to be effective at increasing seated BP for up to 3 hours in persons with SCI. [Copyright &y& Elsevier]

Abstract: Objectives: To identify medically relevant aspects of blood pressure dysregulation (BPD) related to quality of life in individuals with spinal cord injury (SCI), and to propose an integrated conceptual framework based on input from both individuals with SCI and their clinical providers. This framework will serve as a guide for the development of a patient-reported outcome (PRO) measure specifically related to BPD. Design: Three focus groups with individuals with SCI and 3 groups with SCI providers were analyzed using grounded-theory based qualitative analysis to ascertain how blood pressure impacts health-related quality of life (HRQOL) in individuals with SCI. Setting: Focus groups were conducted at 2 Veterans Affairs medical centers and a research center. Participants: Individuals with SCI (n=27) in 3 focus groups and clinical providers (n=25) in 3 focus groups. Interventions: Not applicable. Main Outcome Measures: Not applicable. Results: Qualitative analysis indicated that all focus groups spent the highest percentage of time discussing symptoms of BPD (39%), followed by precipitators/causes of BPD (16%), preventative actions (15%), corrective actions (12%), and the impact that BPD has on social or emotional functioning (8%). While patient/consumer focus groups and provider focus groups raised similar issues, providers spent more time discussing precipitators/causes of BPD and preventative actions (38%) than patient/consumer groups (24%). Conclusions: These results suggest that BPD uniquely and adversely impacts HRQOL in persons with SCI. While both individuals with SCI and their providers highlighted the relevant symptoms of BPD, the SCI providers offered additional detailed information regarding the precipitators/causes and what can be done to prevent/treat BPD. Further, the results suggest that persons with SCI are aware of how BPD impacts their HRQOL and are able to distinguish between subtle signs and symptoms. These findings exemplify the need for a validated and sensitive clinical measurement tool that can assess the extent to which BPD impacts HRQOL in patients with SCI. [Copyright &y& Elsevier]

Abstract: The aim of this study was to investigate orthostatic reactions in females with Rett syndrome (RTT), and also whether the severity of the syndrome had an impact on autonomic reactions. Based on signs of impaired function of the central autonomic system found in RTT, it could be suspected that orthostatic reactions were affected. The orthostatic reactions in 21 females with RTT and 14 normally developed females matched by age were investigated when they rose from a sitting position, and during standing for 3min. Reactions of the heart, the blood pressure and the time for recovery of systolic blood pressure, were studied in real time, heartbeat by heartbeat, simultaneously. There was no difference between participants with RTT and the normally developed controls regarding general orthostatic reactions (heart rate, systolic and diastolic blood pressure, and mean arterial pressure) when getting up from a sitting position, and when standing erect for 3min. In the specific immediate response by the heart to standing up, the 30:15 ratio, significantly lower values were found for females with RTT. In the RTT group, the maximum fall of systolic blood pressure showed a tendency to a larger decrease, and the initial decrease in systolic blood pressure was significantly faster. The time for recovery of systolic blood pressure from standing erect did not differ between groups. At baseline the females with RTT had significantly lower systolic blood pressure and a tendency to a higher heart rate. The results do not indicate any autonomic limitations for people with RTT in getting up from a sitting position and standing. The participants with RTT had normal orthostatic reactions indicated by the heart and blood pressure responses when standing erect for 3min. A faster initial drop in systolic blood pressure in people with RTT was notable. [Copyright &y& Elsevier]

Abstract: There is bulk of evidence suggesting that blood pressure dysregulation, as low blood pressure (LBP) or hypotension, orthostatic hypotension (OH) and high blood pressure (HPB) or hypertension are associated with alterations in cognitive and emotional domains. Some studies suggest that LBP, neurocardiovascular instability, like the OH, and atherosclerosis resulting from long standing HBP, reduces cerebral blood flow, increasing the risk of cognitive impairment, morbidity and mortality. This study aims to evaluate whether patients with cognitive impairment and cardiovascular disease would show any differences in some anamnestic indicators and/or psychometric measures of cognitive performance and affective symptoms. We recruited 36 patients over 65 years of age admitted to both psycho- and cardio-geriatric ambulatories of our hospital during the last year. The population (mean age of 80.5 years, 72.2% females, 27.8% males) was divided in 2 groups, with OH (25%), and without OH (75%). The first group was subdivided in subgroups: patients with HBP, normal BP and LBP, respectively. Cognitive and depressive domains were assessed with the mini mental state examination (MMSE) and the Italian “scala di valutazione del benessere emotivo nell’ anziano” (SVEBA). Information about the present status, comorbidities (cumulative illness rating scale=CIRS), functional ability (activities of daily living=ADL, instrumental ADL=IADL) and drugs were collected during clinical examination. BP was measured 4 times, at the beginning of examination, then with the patient in clinostatic and orthostatic position (1st and 3rd minute). Data were analyzed by MANCOVA, considering age and gender as covariates, MMSE, SVEBA, CIRS, ADL, IADL and drugs as dependent variables, and presence/absence of OH as factor. Covariates were not significant sources of variance, as well as overall factor. Due to the heuristic aim of the study, we considered of interest the results of subsequent ANOVAs showing significant differences in SVEBA and ADL with respect to the factor. These data give us the basis to develop a longitudinal study to confirm the detrimental effect of OH on a wide range of health domains. [Copyright &y& Elsevier]

Background: This study sought to explore the related factors of orthostatic hypotension (OH) in elderly patients with essential hypertension, and analyze the relationship between early renal damage and OH in elderly hypertensive patients., Methods: The demographic and clinical data of 511 elderly patients with essential hypertension (EH) were collected from September 2017 to September 2018.These patients were divided into group with OH and group without OH. The data were compared between the two groups to analyze correlations between OH and early renal damage indicators [urine microalbumin (mAlb) >30 mg/L]., Results: In the study, 118 were in the OH+ group, and 393 were in the OH-group. The proportion of patients with coronary heart disease, atherosclerosis, grade 3 hypertension, persistent rapid atrial fibrillation, left ventricular diastolic dysfunction, and left ventricular hypertrophy in OH+ group was significantly higher than in OH- group (P<0.05). Further, logistic regression analysis showed that coronary heart disease, carotid atherosclerosis, and grade 3 hypertension are independent associated factors for OH in elderly EH patients. In the 511 patients, 249 had urine mAlb >30 mg/L, and 262 had urine mAlb ≤30 mg/L. The 24-hour systolic blood pressure (SBP)-coefficient of variation (CV), the 24-hour diastolic blood pressure (DBP)-coefficient of variation (CV), the drop difference of both SBP and DBP from supine to standing position in 3-minute were higher in patients with urine mAlb >30 mg/L than in patients with urine mAlb ≤30 mg/L (P<0.05). The urine mAlb of patients with OH was higher than that of patients without OH, and the urine mAlb of patients with elevated blood pressure variability (BPV) was also higher than that in patients with normal BPV (P<0.05). Further groupings revealed that patients with both OH and elevated BPV had the highest urine mAlb levels (P<0.05)., Conclusions: Coronary heart disease, atherosclerosis, and grade 3 hypertension are independent associated factors of OH in elderly EH patients. In elderly hypertensive patients with both OH and elevated BPV, the urine mAlb is much higher than that in patients with OH or elevated BPV alone. OH is associated with early renal damage.