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Neurology India Mar 2024
Topics: Humans; Autonomic Nervous System Diseases; Flushing; Hypohidrosis
PubMed: 38817189
DOI: 10.4103/neurol-india.Neurol-India-D-24-00157 -
Neurology India Mar 2024Morvan's syndrome is a rare, complex autoimmune syndrome comprising peripheral nerve hyperexcitability, dysautonomia, insomnia, and encephalopathy. In this case report,...
Morvan's syndrome is a rare, complex autoimmune syndrome comprising peripheral nerve hyperexcitability, dysautonomia, insomnia, and encephalopathy. In this case report, we highlight the clinical and pathological findings of an elderly gentleman who presented to us with clinical features of Morvan's syndrome associated with anti-contactin-associated protein 2 (CASPR-2) antibodies. Histopathology [Figure 3] revealed cortical atrophy with gliosis and mild microglial proliferation. Microglial activation and gliosis were observed in the hippocampus, hypothalamus, and thalamus. Brainstem showed multifocal inflammation. Mild inflammation was observed in the leptomeninges. Morvan's syndrome is an autoimmune disease with antibodies targeted against CASPR within the voltage-gated potassium channel (VGKC) complex. Early diagnosis and treatment play a key role in the management of patients. Most patients show a good response when treated with plasmapheresis and steroids. This patient presented to us late into the illness and succumbed.
Topics: Humans; Male; Autopsy; Nerve Tissue Proteins; Aged; Syringomyelia; Autoantibodies; Brain; Membrane Proteins
PubMed: 38817173
DOI: 10.4103/ni.ni_692_22 -
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 -
JACC. Cardiovascular Interventions May 2024Fasting before coronary procedures is currently recommended to reduce complications despite the lack of scientific evidence. (Comparative Study)
Comparative Study Randomized Controlled Trial
BACKGROUND
Fasting before coronary procedures is currently recommended to reduce complications despite the lack of scientific evidence.
OBJECTIVES
The TONIC (Comparison Between Fasting and No Fasting Before Interventional Coronary Intervention on the Occurrence of Adverse Events) noninferiority trial investigated the safety and comfort of a nonfasting strategy (ad libitum food and drinks) vs traditional fasting (>6 hours for solid food and liquids) before coronary procedures.
METHODS
In this monocentric, prospective, single-blind randomized controlled trial, 739 patients undergoing coronary procedures were included and randomized to a fasting or a nonfasting strategy. Emergency procedures were excluded. The primary endpoint was a composite of vasovagal reaction, hypoglycemia (defined by blood sugar ≤0.7 g/L), and isolated nausea and/or vomiting. Noninferiority margin was 4%. Secondary endpoints were contrast-induced nephropathy and patients' satisfaction.
RESULTS
Among the 739 procedures (697 elective and 42 semiurgent), 517 angiographies, and 222 angioplasties (including complex and high-risk procedures) were performed. The primary endpoint occurred in 30 of 365 nonfasting patients (8.2%) vs 37 of 374 fasting patients (9.9%), demonstrating noninferiority (absolute between-group difference, -1.7%; 1-sided 95% CI upper limit: 1.8%). No food-related adverse event occurred, and contrast-related acute kidney injuries were similar between groups. Overall, procedure satisfaction and perceived pain were similar in both groups, but nonfasting patients reported less hunger and thirst (P < 0.01). In case of redo coronary procedures, most patients (79%) would choose a nonfasting strategy.
CONCLUSIONS
The TONIC randomized trial demonstrates the noninferiority of a nonfasting strategy to the usual fasting strategy for coronary procedures regarding safety, while improving patients' comfort.
Topics: Humans; Fasting; Male; Female; Prospective Studies; Single-Blind Method; Middle Aged; Treatment Outcome; Aged; Time Factors; Risk Factors; Patient Satisfaction; Percutaneous Coronary Intervention; Coronary Angiography; Hypoglycemia; Syncope, Vasovagal; Blood Glucose; Coronary Artery Disease; Risk Assessment
PubMed: 38811102
DOI: 10.1016/j.jcin.2024.03.033 -
Journal of Neurology May 2024Olfactory dysfunction or dysautonomia is one of the earliest prodromal nonmotor symptoms of Parkinson's disease (PD). We aimed to investigate whether PD patients with...
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.
PubMed: 38806701
DOI: 10.1007/s00415-024-12332-1 -
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 -
Cureus Apr 2024This study examines a four-year-and-one-month-old male with no significant past medical, family, or surgical history who initially presented to the pediatric clinic with...
This study examines a four-year-and-one-month-old male with no significant past medical, family, or surgical history who initially presented to the pediatric clinic with cough, rhinorrhea, conjunctivitis, emesis, leg and arm pain, and increased difficulty ambulating. The patient was transferred to the emergency department and tested positive for a non-COVID-19 coronavirus infection. The patient was stabilized, given intravenous fluids, and discharged only to return to the clinic the next day with the onset of a headache, right eye ptosis, an inability to bear weight, and bilateral upper and lower extremity weakness resulting in an ataxic gait. In addition to the neurological deficits, the patient was found to have an elevated blood pressure and pulse. The patient was promptly transferred to a tertiary care clinic. Through exclusion of various differentials via testing, the patient was diagnosed and managed for atypical Guillain-Barré syndrome. Targeted therapies were initiated to prevent dysautonomia-associated morbidity. Following management, the patient's condition vastly improved and he was admitted to rehabilitation bringing him back to optimal health. This study underlines the importance of prompt identification of atypical presentations of Guillain-Barré syndrome which may aid in avoiding preventable morbidity and mortality.
PubMed: 38800176
DOI: 10.7759/cureus.59068 -
Topics in Spinal Cord Injury... 2024The World Health Organization (WHO) recommends that children be breastfed exclusively for the first 6 months of age. This recommendation may prove challenging for women...
The World Health Organization (WHO) recommends that children be breastfed exclusively for the first 6 months of age. This recommendation may prove challenging for women with spinal cord injury (SCI) who face unique challenges and barriers to breastfeeding due to the impact of SCI on mobility and physiology. Tailored provision of care from health care professionals (HCPs) is important in helping women navigate these potential barriers. Yet, HCPs often lack the confidence and SCI-specific knowledge to meet the needs of mothers with SCI. An international panel of clinicians, researchers, consultants, and women with lived experience was formed to create an accessible resource that can address this gap. A comprehensive survey on breastfeeding complications, challenges, resources, and quality of life of mothers with SCI was conducted, along with an environmental scan to evaluate existing postpartum guidelines and assess their relevance and usability as recommendations for breastfeeding after SCI. Building on this work, this article provides evidence-based recommendations for HCPs, including but not limited to general practitioners, obstetricians, pediatricians, physiatrists, lactation consultants, nurses, midwives, occupational therapists, and physiotherapists who work with prospective and current mothers with SCI.
Topics: Humans; Spinal Cord Injuries; Breast Feeding; Female; Autonomic Dysreflexia; Practice Guidelines as Topic; Mothers; Quality of Life; Adult
PubMed: 38799607
DOI: 10.46292/sci23-00079