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Cureus May 2024Background and objective Achalasia cardia is a primary esophageal motility disorder, and the etiopathology of this disease's progression is not known. Moreover,...
Background and objective Achalasia cardia is a primary esophageal motility disorder, and the etiopathology of this disease's progression is not known. Moreover, autonomic dysfunction has not been studied in different types of achalasia. In light of this, we aimed to address this lack of data in this study. Methods The diagnosis of achalasia was done using high-resolution esophageal manometry (HRM)-based Chicago classification v4.0. Autonomic function tests (AFT) such as the head-up tilt test, deep breathing test (DBT), Valsalva maneuver (VM), handgrip test (HGT), and cold pressor test (CPT), as well as the heart rate variability (HRV) test, were performed among the cohort and the results were compared with those of 39 age- and sex-matched healthy controls. Results AFT and HRV tests were done on 62 patients (30 achalasia type I, 28 type II, and 4 type III) and compared with 39 age- and sex-matched healthy controls. The mean duration of symptoms, high Eckardt score, and dysphagia were most common in type I achalasia, followed by type II and III. The results of AFT showed a generalized loss of parasympathetic and baroreflex-independent sympathetic reactivity in all types of achalasia. However, baroreflex-dependent cardiovascular adrenergic reactivity was normal. Regarding cardiac autonomic tone, there was a loss of parasympathetic and sympathetic influence, but sympathovagal balance was maintained. The severity of the loss of autonomic functions was higher in type I, followed by type II. Conclusions In all types of achalasia, parasympathetic reactivity, baroreflex-independent sympathetic reactivity, and cardiac autonomic tone were lower compared to healthy controls, and the severity of dysfunction increased during the progression of the disease from type II to type I.
PubMed: 38826939
DOI: 10.7759/cureus.59444 -
International Journal of Medical... 2024Severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) leads to coronavirus disease-2019 (COVID-19) which can cause severe cardiovascular complications including... (Review)
Review
Severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) leads to coronavirus disease-2019 (COVID-19) which can cause severe cardiovascular complications including myocardial injury, arrhythmias, acute coronary syndrome and others. Among these complications, arrhythmias are considered serious and life-threatening. Although arrhythmias have been associated with factors such as direct virus invasion leading to myocardial injury, myocarditis, immune response disorder, cytokine storms, myocardial ischemia/hypoxia, electrolyte abnormalities, intravascular volume imbalances, drug interactions, side effects of COVID-19 vaccines and autonomic nervous system dysfunction, the exact mechanisms of arrhythmic complications in patients with COVID-19 are complex and not well understood. In the present review, the literature was extensively searched to investigate the potential mechanisms of arrhythmias in patients with COVID-19. The aim of the current review is to provide clinicians with a comprehensive foundation for the prevention and treatment of arrhythmias associated with long COVID-19.
Topics: Humans; COVID-19; Arrhythmias, Cardiac; SARS-CoV-2
PubMed: 38818469
DOI: 10.7150/ijms.94578 -
Neurology India Mar 2024
Topics: Humans; Autonomic Nervous System Diseases; Flushing; Hypohidrosis
PubMed: 38817189
DOI: 10.4103/neurol-india.Neurol-India-D-24-00157 -
Scientific Reports May 2024Despite treatment with levothyroxine, hypothyroidism and autoimmune thyroiditis (AIT) may be associated with reduced quality of life (QoL), an enigmatic condition...
Despite treatment with levothyroxine, hypothyroidism and autoimmune thyroiditis (AIT) may be associated with reduced quality of life (QoL), an enigmatic condition referred to as "syndrome T". Peripheral neuropathy, described in untreated thyroid disease, could be a contributing mechanism. We analysed autonomic and somatosensory function in 29 patients with AIT and treated hypothyroidism and 27 healthy volunteers. They underwent heart rate variability (HRV) analysis and quantitative sensory testing (n = 28), comprising 13 parameters of small and large nerve fibre function and pain thresholds. Autonomic cardiovascular function was assessed in rest, deep respiration and orthostasis. Additionally, biomarkers for autoimmunity and thyroid function were measured. Anxiety, depression and QoL were assessed using validated questionnaires. 36% of the patients showed at least one sign of somatosensory small or large fibre dysfunction. 57% presented with mild hyperalgesia to at least one stimulus. Several markers of autonomic function and some detection thresholds were related to the antibody titres. Anxiety, depression scores and QoL correlated to antibody titres and HRV measures. Autonomic and somatosensory dysfunction indicate that in treated hypothyroidism and AIT a subgroup of patients suffers from neuropathic symptoms leading to impaired QoL. Additionally, mild hyperalgesia as a possible sensitisation phenomenon should be considered a target for symptomatic treatment.
Topics: Humans; Female; Male; Middle Aged; Adult; Autonomic Nervous System; Quality of Life; Thyroiditis, Autoimmune; Heart Rate; Hypothyroidism; Thyroxine; Aged; Somatosensory Disorders; Anxiety
PubMed: 38811750
DOI: 10.1038/s41598-024-63158-w -
Translational Psychiatry May 2024Empirically supported treatments for posttraumatic stress disorder (PTSD) exist, but research suggests these therapies are less effective, acceptable, and feasible to... (Randomized Controlled Trial)
Randomized Controlled Trial
Empirically supported treatments for posttraumatic stress disorder (PTSD) exist, but research suggests these therapies are less effective, acceptable, and feasible to deliver to active duty service members (SMs) compared to civilians. Stellate ganglion block (SGB) procedure, in which a local anesthetic is injected around the cervical sympathetic chain or stellate ganglion to temporarily inhibit sympathetic nervous activity, is gaining popularity as an alternative PTSD treatment in military settings. However, it is unknown whether certain PTSD symptoms are more responsive to SGB than others. The current study involved a secondary analysis of data collected from a previous randomized controlled trial of SGB compared to sham (normal saline) injection (N = 113 SMs). PTSD symptoms were assessed via clinical interview and self-report at baseline and 8 weeks post-SGB or sham. Logistic regression analyses showed that the marked alterations in arousal and reactivity PTSD symptom cluster demonstrated the greatest symptom severity reductions after SGB, relative to sham. The reexperiencing cluster also showed pronounced response to SGB in clinician-rated but not self-reported outcomes. Post-hoc item-level analyses suggested that arousal and reactivity cluster findings were driven by reductions in hypervigilance, concentration difficulties, and sleep disturbance, whereas clinician-rated reexperiencing cluster findings were driven by reductions in physiological reactions to trauma cues, emotional reactions to trauma cues, and intrusions. Our findings align with a burgeoning literature positioning SGB as a potential novel or adjunctive PTSD treatment. Results could guide future hypothesis-driven research on mediators of therapeutic change during SGB for PTSD symptoms in SMs.
Topics: Humans; Stress Disorders, Post-Traumatic; Stellate Ganglion; Male; Adult; Female; Autonomic Nerve Block; Military Personnel; Treatment Outcome; Middle Aged; Arousal; Young Adult; Self Report
PubMed: 38811568
DOI: 10.1038/s41398-024-02926-8 -
World Journal of Clinical Cases May 2024Myasthenia gravis (MG) is an autoimmune disorder that affects the neuromuscular junction. The primary pathology in MG involves the presence of autoantibodies to...
Myasthenia gravis (MG) is an autoimmune disorder that affects the neuromuscular junction. The primary pathology in MG involves the presence of autoantibodies to acetylcholine receptors (AChRs), which results in qualitative and quantitative reductions in the availability of functional AChRs. Cardiac muscles are also affected, resulting in various perioperative cardiac complications. Antistriational antibodies are commonly reported in MG cases with cardiac involvement. In the presence of thymoma, the prevalence of cardiac manifestations in patients with MG increases to approximately 10%-15%. Cardiac involvement in MG may range from asymptomatic electrocardiogram changes to ventricular tachycardia, myocarditis, conduction disorders, heart failure, and sudden death. Increased incidence of atrial fibrillation, ventricular and supraventricular extra systoles, and prolonged QTc have also been reported in patients with MG. Clinicians should consider the evaluation of autonomic dysfunction and risk of cardiovascular disease in patients with MG.
PubMed: 38808348
DOI: 10.12998/wjcc.v12.i13.2147 -
BMJ Open May 2024To document current practice and develop consensus recommendations for the assessment and treatment of paroxysmal sympathetic hyperactivity (PSH) during rehabilitation...
OBJECTIVES
To document current practice and develop consensus recommendations for the assessment and treatment of paroxysmal sympathetic hyperactivity (PSH) during rehabilitation after severe acquired brain injury.
DESIGN
Delphi consensus process with three rounds, based on the Guidance on Conducting and REporting DElphi Studies (CREDES) guidelines, led by three convenors (the authors) with an expert panel. Round 1 was exploratory, with consensus defined before round 2 as agreement of at least 75% of the panel.
SETTING
A working group within the Nordic Network for Neurorehabilitation.
PANEL PARTICIPANTS
Twenty specialist physicians, from Sweden (9 participants), Norway (7) and Denmark (4), all working clinically with patients with severe acquired brain injury and with current involvement in clinical decisions regarding PSH.
RESULTS
Consensus was reached for 21 statements on terminology, assessment and principles for pharmacological and non-pharmacological treatment, including some guidance on specific drugs. From these, an algorithm to support clinical decisions at all stages of inpatient rehabilitation was created.
CONCLUSIONS
Considerable consensus exists in the Nordic countries regarding principles for PSH assessment and treatment. An interdisciplinary approach is needed. Improved documentation and collation of data on treatment given during routine clinical practice are needed as a basis for improving care until sufficiently robust research exists to guide treatment choices.
Topics: Humans; Delphi Technique; Brain Injuries; Consensus; Neurological Rehabilitation; Autonomic Nervous System Diseases; Scandinavian and Nordic Countries; Sweden
PubMed: 38806428
DOI: 10.1136/bmjopen-2024-084778 -
Pain Physician May 2024There are limited therapeutic options to treat complex regional pain syndrome (CRPS). Spinal cord stimulation and dorsal root ganglion stimulation are proven therapies... (Clinical Trial)
Clinical Trial
BACKGROUND
There are limited therapeutic options to treat complex regional pain syndrome (CRPS). Spinal cord stimulation and dorsal root ganglion stimulation are proven therapies for treating chronic low limb pain in CRPS patients. There is limited evidence that stimulation of dorsal nerve roots can also provide relief of lower limb pain in these patients.
OBJECTIVES
To demonstrate that electrical stimulation of dorsal nerve roots via epidural lead placement provides relief of chronic lower limb pain in patients suffering from CRPS.
STUDY DESIGN
Prospective, open label, single arm, multi-center study.
SETTING
The study was performed at the Center for Interventional Pain and Spine (Exton, PA), Millennium Pain Center (Bloomington, IL), and the Carolinas Pain Center (Huntersville, NC). It was approved by the Western Institutional Review Board-Copernicus Group Institutional Review Board and is registered at clinicaltrials.gov (NCT03954080).
METHODS
Sixteen patients with intractable chronic severe lower limb pain associated with CRPS were enrolled in the study. A standard trial period to evaluate a patients' response to stimulation of the dorsal nerve roots was conducted over 3 to 10-days. Patients that obtained 50% or greater pain relief during the trial period underwent permanent implantation of a neurostimulation system. The primary outcome was the evaluated pain level after 3 months of device activation, based on NRS pain score relative to baseline. Patients were followed up for 6 months after activation of the permanently implanted system.
RESULTS
At the primary endpoint, patients reported a significant (P = 0.0006) reduction in pain of 3.3 points, improvement in quality of life, improved neuropathic pain characteristics, improved satisfaction, and an overall perception of improvement with the therapy. Improvements were sustained throughout the duration of the study up to the final 6-month visit.
LIMITATIONS
Due to the COVID-19 pandemic occurring during patient enrollment, only 16 patients were enrolled and trialed, with 12 being permanently implanted. Nine were able to complete the end of study evaluation at 6 months.
CONCLUSIONS
The results of this short feasibility study confirm the functionality, effectiveness, and safety of intraspinal stimulation of dorsal nerve roots in patients with intractable chronic lower limb pain due to CRPS using commercially approved systems and conventional parameters.
Topics: Humans; Feasibility Studies; Prospective Studies; Complex Regional Pain Syndromes; Spinal Nerve Roots; Chronic Pain; Female; Male; Middle Aged; Adult; Electric Stimulation Therapy; Lower Extremity; Aged; Pain, Intractable; Treatment Outcome; Pain Management
PubMed: 38805527
DOI: No ID Found -
Pain Physician May 2024Sympathetic ganglion block (SGB) technique is becoming increasingly prevalent in the treatment of complex regional pain syndromes (CRPS). Given the varied reported... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Sympathetic ganglion block (SGB) technique is becoming increasingly prevalent in the treatment of complex regional pain syndromes (CRPS). Given the varied reported effectiveness of these techniques and the heterogeneity of treatment regimens, there is an urgent need for consistent and high-quality evidence on the efficacy and safety of such procedures.
OBJECTIVES
This study aimed to compare the efficacy of SGB therapy for CRPS-related pain.
STUDY DESIGN
A meta-analysis of randomized controlled trials (RCTs).
METHODS
PubMed, EMBASE, Web of Science, CINAHL, US National Institutes of Health Clinical Trials Registry, Google Scholar, and Cochrane Library Databases were systematically searched between January 1967 and April 2023. A meta-analysis of the included RCTs on SGB was conducted to evaluate the effectiveness and risk of bias (ROBs) of SGB.
RESULTS
After screening 8523 records, 12 RCTs were included in this meta-analysis. Compared with controls, the visual analog pain score decreased by a weighted mean difference (WMD) of -6.24 mm (95% CI, -11.45, -1.03; P = 0.019) in the random-effects model, and the numerical scale score was reduced by a WMD of -1.17 mm (95% CI, -2.42, 0.08; P = 0.067) in the fixed-effects model, indicating a pain relief. The methodological quality of the included RCTs was high, with an average PEDro score of 7.0 (range: 5-9).
LIMITATIONS
The number of included trials was limited.
CONCLUSIONS
SGB therapy can reduce pain intensity in patients with CRPS with few adverse events. However, owing to the relatively high heterogeneity of the included RCTs, a larger sample of high-quality RCTs is needed to further confirm this conclusion.
Topics: Humans; Complex Regional Pain Syndromes; Stellate Ganglion; Autonomic Nerve Block; Randomized Controlled Trials as Topic
PubMed: 38805523
DOI: No ID Found -
Frontiers in Genetics 2024Congenital insensitivity to pain with anhidrosis (CIPA, OMIM #256800), also known as hereditary sensory and autonomic neuropathy type Ⅳ (HSAN-IV), is a rare autosomal...
BACKGROUND
Congenital insensitivity to pain with anhidrosis (CIPA, OMIM #256800), also known as hereditary sensory and autonomic neuropathy type Ⅳ (HSAN-IV), is a rare autosomal recessive disorder characterized by recurrent episodic fevers, anhidrosis, insensitivity to noxious stimuli, self-mutilating behavior and intellectual disability. CIPA can be caused by the variants in gene, which encodes a high-affinity tyrosine kinase receptor for nerve growth factor. To ascertain the hereditary cause of a patient with CIPA accompanied by the additional symptoms of mild growth retardation, prone to fracture, underdeveloped nails of fingers and toes, irregular tooth alignment, enamel hypoplasia, postoperative wound healing difficulty, hand and limb deformity, and dislocation of hip joint, whole exome sequencing was used and revealed a compound heterozygous variant in .
METHODS
DNA was extracted from peripheral blood samples of pediatric patients and their parents, and subjected to comprehensive analysis using whole-exome sequencing (WES), followed by verification of variant sites in the gene through Sanger sequencing. To elucidate the functional impact of the newly discovered variants, an experimental system was established. Splicing analysis was conducted using PCR and Sanger sequencing, while expression levels were assessed through qPCR and Western blot techniques.
RESULTS
One hotspot variant c.851-33T>A(ClinVar ID: 21308) and a novel variant c.850 + 5G>A(ClinVar ID:3069176) was inherited from her father and mother, respectively, identified in the affected individuals. The c.850 + 5G>A variant in resulted in two forms of aberrant mRNA splicing: 13bp deletion (c.838_850del13, p. Val280Ser fs180) and 25bp deletion (826_850del25, p. Val276Ser fs180) in exon 7, both leading to a translational termination at a premature stop codon and forming a C-terminal truncated protein. The expression of two abnormal splicing isoforms was decreased both in the level of mRNA and protein.
CONCLUSION
In conclusion, this study elucidated the genetic cause of a patient with CIPA and identified a novel variant c.850 + 5G>A in , which broadened the and enriched the mutation spectrum.
PubMed: 38798698
DOI: 10.3389/fgene.2024.1345081