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Current Neurology and Neuroscience... Nov 2023Dysautonomia refers to the dysfunction of the autonomic nervous system and encompasses a wide variety of autonomic symptoms and disorders. The most common autonomic... (Review)
Review
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. 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.
Topics: Humans; Mast Cell Activation Syndrome; Postural Orthostatic Tachycardia Syndrome; Primary Dysautonomias; Comorbidity; Migraine Disorders
PubMed: 37847487
DOI: 10.1007/s11910-023-01307-w -
Ugeskrift For Laeger Apr 2024Spinal anaesthesia is considered an effective and safe method for providing pain relief during procedures below the waist. However, in a small subset of patients,...
Spinal anaesthesia is considered an effective and safe method for providing pain relief during procedures below the waist. However, in a small subset of patients, life-threatening vasovagal reactions may develop leading to severe bradycardia and hypotension or ultimately asystole and complete circulatory collapse. Early recognition and prompt treatment of this condition can be lifesaving as illustrated in this case report where the patient developed asystole for ten seconds shortly after placing the spinal anaesthetic.
Topics: Humans; Anesthesia, Spinal; Bradycardia; Heart Arrest; Syncope, Vasovagal
PubMed: 38704723
DOI: 10.61409/V11230702 -
International Journal of Molecular... Feb 2024Autoimmune autonomic ganglionopathy (AAG) is a disease of autonomic failure caused by ganglionic acetylcholine receptor (gAChR) autoantibodies. Although the detection of... (Review)
Review
Autoimmune autonomic ganglionopathy (AAG) is a disease of autonomic failure caused by ganglionic acetylcholine receptor (gAChR) autoantibodies. Although the detection of autoantibodies is important for distinguishing the disease from other neuropathies that present with autonomic dysfunction, other factors are important for accurate diagnosis. Here, we provide a comprehensive review of the clinical features of AAG, highlighting differences in clinical course, clinical presentation, and laboratory findings from other neuropathies presenting with autonomic symptoms. The first step in diagnosing AAG is careful history taking, which should reveal whether the mode of onset is acute or chronic, followed by an examination of the time course of disease progression, including the presentation of autonomic and extra-autonomic symptoms. AAG is a neuropathy that should be differentiated from other neuropathies when the patient presents with autonomic dysfunction. Immune-mediated neuropathies, such as acute autonomic sensory neuropathy, are sometimes difficult to differentiate, and therefore, differences in clinical and laboratory findings should be well understood. Other non-neuropathic conditions, such as postural orthostatic tachycardia syndrome, chronic fatigue syndrome, and long COVID, also present with symptoms similar to those of AAG. Although often challenging, efforts should be made to differentiate among the disease candidates.
Topics: Humans; Ganglia, Autonomic; Post-Acute COVID-19 Syndrome; Autoimmune Diseases of the Nervous System; Autonomic Nervous System; Autonomic Nervous System Diseases; Autoimmune Diseases; Peripheral Nervous System Diseases; Autoantibodies
PubMed: 38396973
DOI: 10.3390/ijms25042296 -
Clinical Rheumatology Oct 2023Psoriatic arthritis (PsA) is an inflammatory disease with a high prevalence of cardiovascular (CV) events due to traditional cardiovascular risk factors and increased... (Review)
Review
Psoriatic arthritis (PsA) is an inflammatory disease with a high prevalence of cardiovascular (CV) events due to traditional cardiovascular risk factors and increased systemic inflammation. In this review, our objectives were to (i) evaluate the cardiovascular events and risk factors and (ii) investigate the relationship between autonomic dysfunction and CV diseases in PsA. A systematic review of the literature was done on the Medline/PubMed, Scopus, and the Directory of Open Access Journals databases between January 2017 and July 2022. After screening and exclusions, 73 studies were included for the final review. Patients with PsA have a greater risk of CV diseases and increased traditional CV risk factors, including hypertension, diabetes mellitus, obesity, metabolic syndrome, and dyslipidemia. Although autonomic dysfunction is more common in PsA than in the general population, its relationship with increased CV diseases in these patients is still unclear. Limitations in explaining CV risk in these patient groups complicate patient assessment as cardiovascular risk factors are linked to the morbidity and mortality of PsA, and it is essential to improve an optimal screening and management strategy for CV disease. All CV risk scoring systems cannot fully assess the CV risk in these patients, so in addition to scoring systems, carotid ultrasound evaluation may be a part of the CV evaluation.
Topics: Humans; Arthritis, Psoriatic; Cardiovascular Diseases; Heart Disease Risk Factors; Hypertension; Primary Dysautonomias; Risk Factors
PubMed: 36542174
DOI: 10.1007/s10067-022-06484-6 -
Current Opinion in Neurology Aug 2024Multiple system atrophy (MSA) is a rapidly progressive synucleinopathy characterized by autonomic failure, parkinsonism, and cerebellar ataxia. Here, we provide an... (Review)
Review
PURPOSE OF REVIEW
Multiple system atrophy (MSA) is a rapidly progressive synucleinopathy characterized by autonomic failure, parkinsonism, and cerebellar ataxia. Here, we provide an update on α-synuclein's role in MSA pathophysiology and review the new Movement Disorders Society (MDS) diagnostic criteria and the utility of α-synuclein-based biomarkers. We also highlight ongoing efforts toward clinical trial readiness and review potential disease-modifying therapies undergoing clinical trials.
RECENT FINDINGS
A role of urinary tract infections in triggering α-synuclein aggregation and contribution of genes implicated in oligodendroglial development have been suggested in the MSA pathophysiology. The clinically probable MSA category of the new diagnostic criteria shows improved accuracy in early disease stages. Predictors of phenoconversion from pure autonomic failure to MSA are now better defined. Alpha-synuclein strains in CSF and serum, phosphorylated α-synuclein deposits in the skin, and brain α-synuclein pathology visualized using PET ligand [18F]ACI-12589 are emerging as valuable diagnostic tools. Clinical trials in MSA investigate drugs targeting α-synuclein aggregation or preventing α-synuclein expression, along with stem cell and gene therapies to halt disease progression.
SUMMARY
New MSA diagnostic criteria and α-synuclein-based biomarkers may enhance diagnostic accuracy while promising therapies are in development to address disease progression.
Topics: Multiple System Atrophy; Humans; alpha-Synuclein; Biomarkers
PubMed: 38828714
DOI: 10.1097/WCO.0000000000001285 -
Heart Rhythm Sep 2023
Topics: Humans; Syncope, Vasovagal
PubMed: 37379939
DOI: 10.1016/j.hrthm.2023.06.017 -
The Clinical Journal of Pain Sep 2023An increasing number of systematic reviews have been conducted on various conservative management of complex regional pain syndrome (CRPS) targeting different... (Review)
Review
OBJECTIVES
An increasing number of systematic reviews have been conducted on various conservative management of complex regional pain syndrome (CRPS) targeting different rehabilitation interventions and objectives. The intent of this article was to summarize and critically appraise the body of evidence on conservative management of the CRPS and to provide an overall picture of the current state of the literature.
METHODS
This study was an overview of systematic reviews on conservative treatments for CRPS. We conducted a literature search from inception to January 2023 in the following databases: Embase, Medline, CINAHL, Google Scholar, Cochrane Library, and Physiotherapy Evidence Database (PEDro). Two independent reviewers conducted study screening, data extraction, and methodological quality assessment (using AMSTAR-2). Qualitative synthesis was the preferred method for reporting the findings of our review. We calculated the corrected covered area index to account for the proportion of overlapping primary studies that were included in multiple reviews.
RESULT
We identified 214 articles, and a total of 9 systematic reviews of randomized controlled trials were eligible for inclusion. Pain and disability were the most common outcomes evaluated in the reviews. There were 6 (6/9; 66%) high-quality, 2 (2/9; 22%) moderate-quality, and 1 critically low-quality systematic review (1/9;11%), with the quality of the included trials ranging from very low to high. There was a large overlap across primary studies that were included in the systematic reviews (corrected covered area=23%). The findings of high-quality reviews support the effectiveness of mirror therapy (MT) and graded motor imagery (GMI) programs on pain and disability improvement in CRPS patients. The large effect size was reported for the effectiveness of MT on pain and disability (SMD:1.88 (95% CI: 0.73-3.02) and 1.30 (95% CI: 0.11-2.49), respectively) and the effectiveness of GMI program (GMIP) on pain and disability improvement (SMD: 1.36 (95% CI: 0.75-1.96) and 1.64 (95% CI: 0.53-2.74), respectively).
DISCUSSION
The evidence is in favor of adopting movement representation techniques, such as MT and GMI programs, for the treatment of pain and disability in patients with CRPS. However, this is based on a small body of primary evidence, and more research is required to generate conclusions. Overall, the evidence is not comprehensive or of sufficient quality to make definitive recommendations about the effectiveness of other rehabilitation interventions in improving pain and disability.
Topics: Humans; Systematic Reviews as Topic; Complex Regional Pain Syndromes; Physical Therapy Modalities; Imagery, Psychotherapy; Pain
PubMed: 37224001
DOI: 10.1097/AJP.0000000000001133 -
Revue Neurologique 2024Orthostatic hypotension is defined as a drop in systolic blood pressure of at least 20mmHg or a drop in diastolic blood pressure of at least 10mmHg within 3minutes of... (Review)
Review
Orthostatic hypotension is defined as a drop in systolic blood pressure of at least 20mmHg or a drop in diastolic blood pressure of at least 10mmHg within 3minutes of standing. It is a common disorder, especially in high-risk populations such as elderly subjects and patients with neurological diseases, and is associated with markedly increased morbidity and mortality. Its management can be challenging, particularly in cases where supine hypertension is associated with severe orthostatic hypotension. Education of the patient, non-pharmacological measures, and drug adaptation are the cornerstones of treatment. Pharmacological treatment should be individualized according to the severity, underlying cause, 24-hour blood pressure profile, and associated coexisting conditions. First-line therapies are midodrine and fludrocortisone, which may need to be combined for optimal care of severe cases.
Topics: Humans; Aged; Hypotension, Orthostatic; Midodrine; Hypertension; Blood Pressure; Nervous System Diseases
PubMed: 38123372
DOI: 10.1016/j.neurol.2023.11.001 -
European Journal of Pediatrics Nov 2023This paper aims to improve the diagnosis of syncope and transient loss of consciousness (TLOC) in children. Diagnostic problems stem, first, from some causes spanning... (Review)
Review
This paper aims to improve the diagnosis of syncope and transient loss of consciousness (TLOC) in children. Diagnostic problems stem, first, from some causes spanning various disciplines, e.g. cardiology, neurology and psychiatry, while the most common cause, vasovagal syncope, is not embraced by any specialty. Second, clinical variability is huge with overlapping signs and symptoms. Third, the approach to TLOC/syncope of the European Society of Cardiology (ESC) is underused in childcare. We explain the ESC guidelines using an additional paediatric literature review. Classification of TLOC and syncope is hierarchic and based on history taking. Loss of consciousness (LOC) is defined using three features: abnormal motor control including falling, reduced responsiveness and amnesia. Adding a < 5 min duration and spontaneous recovery defines TLOC. TLOC simplifies diagnosis by excluding long LOC (e.g. some trauma, intoxications and hypoglycaemia) and focussing on syncope, tonic-clonic seizures and functional TLOC. Syncope, i.e. TLOC due to cerebral hypoperfusion, is divided into reflex syncope (mostly vasovagal), orthostatic hypotension (mostly initial orthostatic hypotension in adolescents) and cardiac syncope (arrhythmias and structural cardiac disorders). The initial investigation comprises history taking, physical examination and ECG; the value of orthostatic blood pressure measurement is unproven in children but probably low. When this fails to yield a diagnosis, cardiac risk factors are assessed; important clues are supine syncope, syncope during exercise, early death in relatives and ECG abnormalities. Conclusions: In adults, the application of the ESC guidelines reduced the number of absent diagnoses and costs; we hope this also holds for children. What is Known: • Syncope and its mimics are very common in childhood, as they are at other ages. • Syncope and its mimics provide considerable diagnostic challenges. What is New: • Application of the hierarchic framework of transient loss of consciousness (TLOC) simplifies diagnosis. • The framework stresses history-taking to diagnose common conditions while keeping an eye on cardiac danger signs.
Topics: Adult; Adolescent; Child; Humans; Hypotension, Orthostatic; Syncope; Syncope, Vasovagal; Unconsciousness; Heart Diseases
PubMed: 37470792
DOI: 10.1007/s00431-023-05114-w -
CNS Neuroscience & Therapeutics Feb 2024Autonomic dysfunction with central autonomic network (CAN) damage occurs frequently after intracerebral hemorrhage (ICH) and contributes to a series of adverse outcomes.... (Review)
Review
AIMS
Autonomic dysfunction with central autonomic network (CAN) damage occurs frequently after intracerebral hemorrhage (ICH) and contributes to a series of adverse outcomes. This review aims to provide insight and convenience for future clinical practice and research on autonomic dysfunction in ICH patients.
DISCUSSION
We summarize the autonomic dysfunction in ICH from the aspects of potential mechanisms, clinical significance, assessment, and treatment strategies. The CAN structures mainly include insular cortex, anterior cingulate cortex, amygdala, hypothalamus, nucleus of the solitary tract, ventrolateral medulla, dorsal motor nucleus of the vagus, nucleus ambiguus, parabrachial nucleus, and periaqueductal gray. Autonomic dysfunction after ICH is closely associated with neurological functional outcomes, cardiac complications, blood pressure fluctuation, immunosuppression and infection, thermoregulatory dysfunction, hyperglycemia, digestive dysfunction, and urogenital disturbances. Heart rate variability, baroreflex sensitivity, skin sympathetic nerve activity, sympathetic skin response, and plasma catecholamine concentration can be used to assess the autonomic functional activities after ICH. Risk stratification of patients according to autonomic functional activities, and development of intervention approaches based on the restoration of sympathetic-parasympathetic balance, would potentially improve clinical outcomes in ICH patients.
CONCLUSION
The review systematically summarizes the evidence of autonomic dysfunction and its association with clinical outcomes in ICH patients, proposing that targeting autonomic dysfunction could be potentially investigated to improve the clinical outcomes.
Topics: Humans; Autonomic Nervous System; Sympathetic Nervous System; Autonomic Nervous System Diseases; Vagus Nerve; Cerebral Hemorrhage; Heart Rate
PubMed: 38372446
DOI: 10.1111/cns.14544