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Baroreflexes play both an adaptive and a homeostatic role, maintaining blood pressure, heart rate, and blood volume within the normal range. Defects in baroreflexes have diverse manifestations and causes. The assessment and management of baroreflex dysfunction are reviewed.

Background Guillain–Barré syndrome (GBS), when severe, involves the autonomic nervous system; our objective was to assess the spectrum and predictors of dysautonomia, and how it may impact functional outcomes. Methods A retrospective review of patients admitted to the Mayo Clinic in Rochester, MN between January 1, 2000, and December 31, 2017, with GBS and dysautonomia was performed. Demographics, comorbidities, parameters of dysautonomia, clinical course, GBS disability score, and Erasmus GBS Outcome Score (EGOS) at discharge were recorded. Results One hundred eighty seven patients were included with 71 (38%) noted to have at least one manifestation of dysautonomia. There are 72% of patients with a demyelinating form of GBS and 36% of patients with demyelination had dysautonomia. Ileus (42%), hypertension (39%), hypotension (37%), fever (29%), tachycardia or bradycardia (27%), and urinary retention (24%) were the most common features. Quadriparesis, bulbar and neck flexor weakness, and mechanical ventilation were associated with autonomic dysfunction. Patients with dysautonomia more commonly had cardiogenic complications, syndrome of inappropriate antidiuretic hormone, posterior reversible encephalopathy syndrome, and higher GBS disability score and EGOS. Mortality was 6% in patients with dysautonomia versus 2% in the entire cohort ( P  = 0.02). Conclusions Dysautonomia in GBS is a manifestation of more severe involvement of the peripheral nervous system. Accordingly, mortality and functional outcomes are worse. There is a need to investigate if more aggressive treatment is warranted in this category of GBS.

The autonomic nervous system (ANS) dynamically regulates the internal environment to maintain homeostasis. The ANS exhibits some forms of synaptic plasticity, including long-term potentiation (LTP) and plastic changes in neurotransmitter distribution, both of which may contribute to autonomic function. Dysautonomia refers to an abnormality in the function of the ANS, with an imbalance between sympathetic and parasympathetic activity. Dysautonomia has been reported in conditions such as stress, hypertension, and metabolic syndrome (MS). MS is a cluster of risk factors for cardiovascular disease, diabetes, and premature death. In MS, the signs of dysautonomia include elevated plasma norepinephrine levels and increased arterial blood pressure. In this study, we characterized the effect of a high-sucrose diet (HSD) on synaptic plasticity in sympathetic ganglia of the rat by measuring LTP expression in the superior cervical ganglion (SCG) and analyzing the expression of acetylcholine (ACh) and GABA, as well as their balance of colocalization/segregation in ganglionic nerve terminals. The HSD consists of adding 30% sucrose to drinking water, which is an accepted model of MS. We observed an impairment in LTP expression, along with a decrease in ACh presence and a reduction in its segregation from GABA. These findings suggest the emergence of an inhibitory effect on synaptic transmission and plasticity within the SCG. We propose that dysautonomia associated with MS might involve changes in sympathetic activity, at least at the level of ganglionic cholinergic transmission. These results may help to improve our understanding of autonomic dysfunction in the context of this metabolic disorder.

BackgroundDue to the lack of long-term studies, this research aimed to explore the changes and predictors of autonomic dysfunction (AD) in people with multiple sclerosis (pwMS) over a 6-year period from disease onset.MethodsAmong the 121 pwMS cohort, 75 underwent autonomic function tests at baseline and year 6. Autonomic symptoms were assessed using the Composite Autonomic System Score-31 (COMPASS-31), while the results of autonomic tests were recorded using the Composite Autonomic Scoring Scale (CASS) at baseline and biennially over 6 years. Symptomatic dysautonomia was identified by a COMPASS-31 score greater than 7.913 and a CASS score greater than 0.ResultsNo significant changes were noted in the COMPASS-31 and CASS scores from baseline to year 6. However, there was a significant decline in the cardiovagal index (p=0.001) and the sudomotor index (p=0.036 and p=0.001, respectively) at years 4 and 6, compared with baseline. The number of participants with symptomatic dysautonomia increased significantly from year 0 to 6 (14 (20.9%) vs 29 (39.2%), respectively; p=0.049). Multivariable logistic regression analysis revealed that experiencing a relapse during the 6 years increased the likelihood of symptomatic dysautonomia (Exp(B) 3.886, 95% CI 1.019 to 14.825, p=0.047). Conversely, transitioning to high-efficacy disease-modifying therapy (HET) reduced the probability of having a CASS score greater than 0 at year 6 (Exp(B) 0.221, 95% CI 0.067 to 0.734, p=0.014).ConclusionsDysfunction of the cardiovagal and sudomotor systems progresses alongside disease duration in pwMS. The early initiation of HET may help mitigate the risk of developing AD.

Background Kleine‐Levin Syndrome (KLS) is a neurological disorder of unknown pathophysiology. It is characterized by relapsing–remitting episodes of hypersomnia, with cognitive symptoms and behavioral disturbances. The diagnosis relies on clinical criteria, which require further standardization. Our main objective was to better phenotype and systematically quantify the severity and impact of cognitive and behavioral symptoms, hypersomnolence and dysautonomia in a well‐characterized population of KLS patients. Methods Forty‐three consecutive KLS patients diagnosed in a National Reference Center for Rare Hypersomnias underwent a standardized clinical and polysomnographic evaluation and completed validated questionnaires with their relatives. The Behavioral Dysexecutive Syndrome Inventory (BDSI) assessed their behavior during most episodes. The Idiopathic‐Hypersomnia Severity Scale (IHSS) assessed different hypersomnolence components (inertia, sleepiness, prolonged sleep), and the Scale for Outcomes in Parkinson's Disease‐Autonomic (SCOPA‐AUT) assessed dysautonomia. The latter two scales were completed twice to differentiate symptoms during and between episodes. Results During symptomatic periods, behavioral changes were observed in all BDSI domains, more than half of patients showing reduced activities and apathy, anticipation–organization–initiation difficulties, disinterest, irritability–aggression, perseveration–stereotypies, and sexual or eating disorders. IHSS scores were severe and higher during episodes (42.6 ± 5.8 vs. 15.6 ± 9.8, p < 0.0001), and each item higher. The SCOPA‐AUT scores were higher during episodes (12.1 ± 8.5 vs. 8.5 ± 8.2, p = 0.0001), particularly in the cardiovascular, pupillomotor, and thermoregulation domains. Conclusions Our findings suggest the relevance of the BDSI for the evaluation of dysexecutive behavioral symptoms in KLS. The IHSS would be useful for phenotyping hypersomnolence and the SCOPA‐AUT for assessing dysautonomia during episodes. Future studies could combine the BDSI with imaging and other biomarkers to explore the neuroanatomical correlates of this enigmatic disorder.

ObjectiveTo identify associated (non-)motor profiles of Parkinson’s disease (PD) patients with hyperhidrosis as a dominant problem.MethodsThis is a cross-sectional, exploratory, analysis of participants enrolled in the Non-motor Longitudinal International Study (NILS; UKCRN No: 10084) at the Parkinson’s Centre at King’s College Hospital (London, UK). Hyperhidrosis scores (yes/no) on question 28 of the Non-Motor Symptom Questionnaire were used to classify patients with normal sweat function (n = 172) and excessive sweating (n = 56) (Analysis 1; n = 228). NMS scale (NMSS) question 30 scores were used to stratify participants based on hyperhidrosis severity (Analysis 2; n = 352) using an arbitrary severity grading: absent score 0 (n = 267), mild 1–4 (n = 49), moderate 5–8 (n = 17), and severe 9–12 (n = 19). NMS burden, as well as PD sleep scale (PDSS) scores were then analysed along with other correlates.ResultsNo differences were observed in baseline demographics between groups in either analysis. Patients with hyperhidrosis exhibited significantly higher total NMSS burden compared to those without (p < 0.001). Secondary analyses revealed higher dyskinesia scores, worse quality of life and PDSS scores, and higher anxiety and depression levels in hyperhidrosis patients (p < 0.001). Tertiary analyses revealed higher NMSS item scores for fatigue, sleep initiation, restless legs, urinary urgency, and unexplained pain (p < 0.001).ConclusionsChronic hyperhidrosis appears to be associated with a dysautonomia dominant subtype in PD patients, which is also associated with sleep disorders and a higher rate of dyskinesia (fluctuation-related hyperhidrosis). These data should prompt the concept of hyperhidrosis being used as a simple clinical screening tool to identify PD patients with autonomic symptoms.

The proportion of different subtypes of Guillain-Barré syndrome (GBS) and their prognosis varied significantly among different regions. This study attempts to investigate the clinical subtypes and outcome of GBS in southwest China. Patients with GBS admitted to The First Affiliated Hospital of Chongqing Medical University from January 2006 to March 2013 were included in our study. Patients were classified into acute inflammatory demyelinating polyneuropathy (AIDP) group, acute motor axonal neuropathy (AMAN) group, Miller-Fisher syndrome (MFS) group, cranial nerve variants(CNV), Bickerstaff's brainstem encephalitis overlaps with GBS (BBE-GBS) group and unclassifiable group based on clinical features and electrophysiological findings. Hughes function grade score (HFGS) was used to assess the prognosis at 3 and 6 months. The prognosis of different subtypes and outcome predictors were analyzed. The most common subtype of GBS was AIDP (57%), followed by AMAN (22%) and MFS (7%). The prognosis of AMAN and BBE-GBS is similar at 3 month(P = 0.0704)and 6 month (P = 0.1614) follow-up. The prognosis of AMAN group was poorer than that of AIDP group at 3 month and 6 month follow-up (P<0.001). Outcome of MFS group and that of CNV group at 6 months were both good(Hughes≤1). Hughes≥3(P<0.0001,OR = 6.650,95%CI = 2.865 to 15.023))and dysautonomia (P = 0.043,OR = 2.820,95%CI = 1.031 to 7.715)) were associated with poor outcome at 6 month follow-up. AIDP is the most common subtype of GBS. Prognosis of AMAN group and BBE-GBS group is poorer than that of AIDP group at 3 month and 6 month follow-up. Hughes≥3 at nadir and dysautonomia are predictors of poor prognosis at 6 month follow-up.

Maintaining cerebral perfusion in the early stages of recovery after stroke is paramount. Autoregulatory function may be impaired during this period leaving cerebral perfusion directly reliant on intravascular volume and blood pressure (BP) with increased risk for expanding cerebral infarction during periods of low BP and hemorrhagic transformation during BP elevations. We suspected that dysautonomia is common during the acute period related to both pre-existing vascular risk factors and potentially independent of such conditions. Thus, we sought to understand the state of the science specific to dysautonomia and acute stroke. The scoping review search included multiple databases and key terms related to acute stroke and dysautonomia. The team employed a rigorous review process to identify, evaluate, and summarize relevant literature. We additionally summarized common clinical approaches used to detect dysautonomia at the bedside. The purpose of this scoping review is to understand the state of the science for the identification, treatment, and impact of dysautonomia on acute stroke patient outcomes. There is a high prevalence of dysautonomia among persons with stroke, though there is significant variability in the type of measures and definitions used to diagnose dysautonomia. While dysautonomia appears to be associated with poor functional outcome and post-stroke complications, there is a paucity of high-quality evidence, and generalizability is limited by heterogenous approaches to these studies. There is a need to establish common definitions, standard measurement tools, and a roadmap for incorporating these measures into clinical practice so that larger studies can be conducted.

Background and objectives Dysautonomia has been associated with paraneoplastic neurological syndrome (PNS)-related mortality in anti-Hu PNS, but its frequency and spectrum remain ill-defined. We describe anti-Hu patients with dysautonomia, estimate its frequency, and compare them to patients without dysautonomia. Methods Patients with anti-Hu antibodies diagnosed in the study centre (1990–2022) were retrospectively reviewed; those with autonomic signs and symptoms were identified. Results Among 477 anti-Hu patients, 126 (26%) had dysautonomia (the only PNS manifestation in 7/126, 6%); gastrointestinal (82/126, 65%), cardiovascular (64/126, 51%), urogenital (24/126, 19%), pupillomotor/secretomotor (each, 11/126, 9%), and central hypoventilation (10/126, 8%). Patients with isolated CNS involvement less frequently had gastrointestinal dysautonomia than those with peripheral (alone or combined with CNS) involvement (7/23, 30% vs. 31/44, 70% vs. 37/52, 71%; P  = 0.002); while more frequently central hypoventilation (7/23, 30% vs. 1/44, 2.3% vs. 2/52, 4%; P  < 0.001) and/or cardiovascular alterations (18/23, 78% vs. 20/44, 45% vs. 26/52, 50%; P  = 0.055). Median [95% CI] overall survival was not significantly different between patients with (37 [17; 91] months) or without dysautonomia (28 [22; 39] months; P  = 0.78). Cardiovascular dysautonomia (HR: 1.57, 95% CI [1.05; 2.36]; P  = 0.030) and central hypoventilation (HR: 3.51, 95% CI [1.54; 8.01]; P  = 0.003) were associated with a higher risk of death, and secretomotor dysautonomia a lower risk (HR: 0.28, 95% CI [0.09; 0.89]; P  = 0.032). Patients with cardiovascular dysautonomia dying ≤ 1 year from clinical onset had severe CNS (21/27, 78%), frequently brainstem (13/27, 48%), involvement. Discussion Anti-Hu PNS dysautonomia is rarely isolated, frequently gastrointestinal, cardiovascular and urogenital. CNS dysfunction, particularly brainstem, associates with lethal cardiovascular alterations and central hypoventilation, while peripheral involvement preferentially associates with gastrointestinal or secretomotor dysautonomia, being the latest more indolent.

Background and purpose Olfactory dysfunction or dysautonomia is one of the earliest prodromal nonmotor symptoms of Parkinson’s disease (PD). We aimed to investigate whether PD patients with dysautonomia and hyposmia at the de novo stage present different prognoses regarding PD dementia (PDD) conversion, motor complication development, and change in levodopa-equivalent doses (LED). Methods In this retrograde cohort study, we included 105 patients with newly diagnosed PD patients who underwent cross-cultural smell identification test (CC-SIT), autonomic function tests (AFT), and dopamine transporter (DAT) scan at the de novo stage. PD patients were divided into Hyposmia + /Dysautonomia + (H + /D +) and Hyposmia − /Dysautonomia − (H − /D −) groups depending on the result of AFT and CC-SIT. Baseline clinical, cognitive, imaging characteristics, longitudinal risks of PDD development and motor complication occurrence, and longitudinal LED changes were compared between the two groups. Results When compared with the H − /D − group, the H + /D + group showed lower standardized uptake value ratios in all subregions, lower asymmetry index, and steeper ventral − dorsal gradient in the DAT scan. The H + /D + group exhibited poorer performance in frontal/executive function and a higher risk of PDD development. The risk of motor complications including levodopa-induced dyskinesia, wearing off, and freezing of gait, was comparable between the two groups. The analysis of longitudinal changes in LED using a linear mixed model showed that the increase of LED in the H + /D + group was more rapid. Conclusions Our results suggest that PD patients with dysautonomia and hyposmia at the de novo stage show a higher risk of PD dementia conversion and rapid progression of motor symptoms.