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Journal of Neuromuscular Diseases 2021Small fiber neuropathy (SFN) is a disorder of the small myelinated Aδ-fibers and unmyelinated C-fibers [5, 6]. SFN might affect small sensory fibers, autonomic fibers... (Review)
Review
Small fiber neuropathy (SFN) is a disorder of the small myelinated Aδ-fibers and unmyelinated C-fibers [5, 6]. SFN might affect small sensory fibers, autonomic fibers or both, resulting in sensory changes, autonomic dysfunction or combined symptoms [7]. As a consequence, the symptoms are potentially numerous and have a large impact on quality of life [8]. Since diagnostic methods for SFN are numerous and its pathophysiology complex, this extensive review focusses on categorizing all aspects of SFN as disease and its diagnosis. In this review, sensitivity in combination with specificity of different diagnostic methods are described using the areas under the curve. In the end, a diagnostic work-flow is suggested based on different phenotypes of SFN.
Topics: Autonomic Nervous System Diseases; Biopsy; Female; Humans; Male; Nerve Fibers, Unmyelinated; Quality of Life; Small Fiber Neuropathy
PubMed: 33337383
DOI: 10.3233/JND-200490 -
Autoimmunity Reviews Jan 2023Chronic fatigue syndrome (CFS), fibromyalgia, silicone breast implants syndrome (SBIs), COVID and post-COVID syndrome (PCS), sick building syndrome (SBS),... (Review)
Review
Autoimmune autonomic nervous system imbalance and conditions: Chronic fatigue syndrome, fibromyalgia, silicone breast implants, COVID and post-COVID syndrome, sick building syndrome, post-orthostatic tachycardia syndrome, autoimmune diseases and autoimmune/inflammatory syndrome induced by adjuvants.
Chronic fatigue syndrome (CFS), fibromyalgia, silicone breast implants syndrome (SBIs), COVID and post-COVID syndrome (PCS), sick building syndrome (SBS), post-orthostatic tachycardia syndrome (POTS), autoimmune diseases and autoimmune/inflammatory syndrome induced by adjuvants (ASIA) are frequently accompanied by clinical symptoms characteristic for dysautonomia: severe fatigue, dizziness, fogginess, memory loss, dry mouth and eyes, hearing dysfunction, tachycardia etc. The recent discovery of an imbalance of autoantibodies against G protein-coupled receptors (GPCR) in some autoimmune diseases, post-COVID syndrome, SBIs allowed researchers to assume the novel mechanism in these conditions - autoimmune autonomic nervous system imbalance. In this review, all data published on an imbalance of autoantibodies against GPCR, clinical symptoms and pathogenic mechanisms in CFS, Fibromyalgia, SBIs, COVID and PCS, SBS, POTS, and some autoimmune diseases were analyzed. Possible criteria to diagnose the autoimmune autonomic nervous system imbalance were created.
Topics: Humans; Fatigue Syndrome, Chronic; Fibromyalgia; Sick Building Syndrome; Breast Implants; COVID-19; Autoimmune Diseases; Autonomic Nervous System; Autoantibodies; Primary Dysautonomias; Tachycardia; Adjuvants, Immunologic; Silicones
PubMed: 36347462
DOI: 10.1016/j.autrev.2022.103230 -
Nature Reviews. Cardiology Jun 2024Cardiovascular autonomic dysfunction (CVAD) is a malfunction of the cardiovascular system caused by deranged autonomic control of circulatory homeostasis. CVAD is an... (Review)
Review
Cardiovascular autonomic dysfunction (CVAD) is a malfunction of the cardiovascular system caused by deranged autonomic control of circulatory homeostasis. CVAD is an important component of post-COVID-19 syndrome, also termed long COVID, and might affect one-third of highly symptomatic patients with COVID-19. The effects of CVAD can be seen at both the whole-body level, with impairment of heart rate and blood pressure control, and in specific body regions, typically manifesting as microvascular dysfunction. Many severely affected patients with long COVID meet the diagnostic criteria for two common presentations of CVAD: postural orthostatic tachycardia syndrome and inappropriate sinus tachycardia. CVAD can also manifest as disorders associated with hypotension, such as orthostatic or postprandial hypotension, and recurrent reflex syncope. Advances in research, accelerated by the COVID-19 pandemic, have identified new potential pathophysiological mechanisms, diagnostic methods and therapeutic targets in CVAD. For clinicians who daily see patients with CVAD, knowledge of its symptomatology, detection and appropriate management is more important than ever. In this Review, we define CVAD and its major forms that are encountered in post-COVID-19 syndrome, describe possible CVAD aetiologies, and discuss how CVAD, as a component of post-COVID-19 syndrome, can be diagnosed and managed. Moreover, we outline directions for future research to discover more efficient ways to cope with this prevalent and long-lasting condition.
Topics: Humans; COVID-19; Autonomic Nervous System Diseases; Cardiovascular Diseases; Post-Acute COVID-19 Syndrome; SARS-CoV-2
PubMed: 38163814
DOI: 10.1038/s41569-023-00962-3 -
Journal of Clinical Neurophysiology :... Jul 2021
Topics: Autonomic Nervous System; Autonomic Nervous System Diseases; Humans
PubMed: 34009849
DOI: 10.1097/WNP.0000000000000756 -
Continuum (Minneapolis, Minn.) Oct 2022Patients with multiple system atrophy (MSA) can present with diverse clinical manifestations, and the clinical care required is complex and requires a thoughtful... (Review)
Review
PURPOSE OF REVIEW
Patients with multiple system atrophy (MSA) can present with diverse clinical manifestations, and the clinical care required is complex and requires a thoughtful approach to emerging symptoms and treatment decisions.
RECENT FINDINGS
Even though it is a rare disease, MSA is often encountered in clinical practice. New developments in biofluid biomarkers and diagnostic assessments offer potential for earlier and more accurate diagnosis. This article describes recent findings, such as the use of skin biopsies, neuroimaging, and novel treatment concepts (eg, central noradrenergic augmentation).
SUMMARY
MSA is a complex disease. This article provides a summary of treatment options for diverse symptoms that include autonomic, sleep, mood, and motor manifestations of the disease to help clinicians care for patients with MSA. Providing comprehensive care for patients with MSA requires an understanding of the diverse symptomatology that patients develop over time and should include an interdisciplinary team.
Topics: Autonomic Nervous System; Autonomic Nervous System Diseases; Biomarkers; Humans; Multiple System Atrophy; Neuroimaging
PubMed: 36222769
DOI: 10.1212/CON.0000000000001154 -
Current Neurology and Neuroscience... Dec 2023Long-COVID is a novel condition emerging from the COVID-19 pandemic. Long-COVID is characterized by symptoms commonly seen in autonomic disorders including fatigue,... (Review)
Review
PURPOSE OF REVIEW
Long-COVID is a novel condition emerging from the COVID-19 pandemic. Long-COVID is characterized by symptoms commonly seen in autonomic disorders including fatigue, brain fog, light-headedness, and palpitations. This article will critically evaluate recent findings and studies on Long-COVID and its physiological autonomic manifestations.
RECENT FINDINGS
Studies have reported on the prevalence of different symptoms and autonomic disorders in Long-COVID cohorts. Autonomic nervous system function, including both the parasympathetic and sympathetic limbs, has been studied using different testing techniques in Long-COVID patients. While numerous mechanisms may contribute to Long-COVID autonomic pathophysiology, it is currently unclear which ones lead to a Long-COVID presentation. To date, studies have not tested treatment options for autonomic disorders in Long-COVID patients. Long-COVID is associated with autonomic abnormalities. There is a high prevalence of clinical autonomic disorders among Long-COVID patients, with limited knowledge of the underlying mechanisms and the effectiveness of treatment options.
Topics: Humans; Post-Acute COVID-19 Syndrome; Pandemics; COVID-19; Autonomic Nervous System Diseases; Autonomic Nervous System; Postural Orthostatic Tachycardia Syndrome
PubMed: 37947962
DOI: 10.1007/s11910-023-01320-z -
Continuum (Minneapolis, Minn.) Feb 2020This article reviews the α-synucleinopathies pure autonomic failure, multiple system atrophy, dementia with Lewy bodies, and Parkinson disease with respect to autonomic... (Review)
Review
PURPOSE OF REVIEW
This article reviews the α-synucleinopathies pure autonomic failure, multiple system atrophy, dementia with Lewy bodies, and Parkinson disease with respect to autonomic failure.
RECENT FINDINGS
The pattern and severity of autonomic involvement in the synucleinopathies is related to differences in cellular deposition and neuronal populations affected by α-synuclein aggregation, which influences the degree and manifestation of autonomic failure. Clinical and laboratory autonomic features distinguish the different synucleinopathies based on pattern and severity. These features also determine which patients are at risk for evolution from pure autonomic failure to the synucleinopathies with prominent motor involvement, such as multiple system atrophy, dementia with Lewy bodies, or Parkinson disease.
SUMMARY
Autonomic failure is a key feature of the synucleinopathies, with varying type and degree of dysfunction from predominantly peripheral involvement in the Lewy body disorders to central involvement in multiple system atrophy.
Topics: Aged; Autonomic Nervous System Diseases; Disease Management; Female; Humans; Male; Middle Aged; Synucleinopathies
PubMed: 31996623
DOI: 10.1212/CON.0000000000000819 -
Hypertension (Dallas, Tex. : 1979) Mar 2024Although orthostatic hypotension (OH) has long been recognized as a manifestation of autonomic dysfunction, a growing body of literature has identified OH as a common... (Review)
Review
Although orthostatic hypotension (OH) has long been recognized as a manifestation of autonomic dysfunction, a growing body of literature has identified OH as a common comorbidity of hypertension. This connection is complex, related to pathophysiology in blood pressure regulation and the manner by which OH is derived as the difference between 2 blood pressure measurements. While traditional therapeutic approaches to OH among patients with neurodegenerative disorders focus on increasing upright blood pressure to prevent cerebral hypoperfusion, the management of OH among patients with hypertension is more nuanced; resting hypertension is itself associated with adverse outcomes among these patients. Although there is substantial evidence that intensive blood pressure treatment does not cause OH in the majority of patients with essential hypertension, some classes of antihypertensive agents may unmask OH in patients with an underlying autonomic impairment. Practical steps to manage OH among adults with hypertension start with (1) a thorough characterization of its patterns, triggers, and cause; (2) review and removal of aggravating factors (often pharmacological agents not related to hypertension treatment); (3) optimization of an antihypertensive regimen; and (4) adoption of a tailored treatment strategy that avoids exacerbating hypertension. These strategies include countermaneuvers and short-acting vasoactive agents (midodrine, droxidopa). Ultimately, further research is needed on the epidemiology of OH, the impact of hypertension treatment on OH, approaches to the screening and diagnosis of OH, and OH treatment among adults with hypertension to improve the care of these patients and their complex blood pressure pathophysiology.
Topics: Adult; Humans; Hypotension, Orthostatic; American Heart Association; Hypertension; Midodrine; Blood Pressure; Antihypertensive Agents; Autonomic Nervous System Diseases
PubMed: 38205630
DOI: 10.1161/HYP.0000000000000236 -
The Journal of Clinical Endocrinology... Jul 2020Rapid-onset obesity with hypothalamic dysfunction, hypoventilation, autonomic dysregulation and neural crest tumor (ROHHHAD[NET]) is a rare and potentially fatal...
CONTEXT
Rapid-onset obesity with hypothalamic dysfunction, hypoventilation, autonomic dysregulation and neural crest tumor (ROHHHAD[NET]) is a rare and potentially fatal disease. No specific diagnostic biomarker is currently available, making prompt diagnosis challenging. Since its first definition in 2007, a complete clinical analysis leading to specific diagnosis and follow-up recommendations is still missing.
OBJECTIVE
The purpose of this work is to describe the clinical timeline of symptoms of ROHHAD(NET) and propose recommendations for diagnosis and follow-up.
DESIGN
We conducted a systematic review of all ROHHAD(NET) case studies and report a new ROHHAD patient with early diagnosis and multidisciplinary care.
METHODS
All the articles that meet the definition of ROHHAD(NET) and provide chronological clinical data were reviewed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analysis individual patient data guidelines. The data were grouped into 7 categories: hypothalamic dysfunction, autonomic dysregulation, hypoventilation, NET, psychiatric symptoms, other clinical manifestations, and outcome.
RESULTS
Forty-three individual patient data descriptions were analyzed. The timeline of the disease shows rapid-onset obesity followed shortly by hypothalamic dysfunction. Dysautonomia was reported at a median age of 4.95 years and hypoventilation at 5.33 years, or 2.2 years after the initial obesity. A NET was reported in 56% of the patients, and 70% of these tumors were diagnosed within 2 years after initial weight gain.
CONCLUSION
Because early diagnosis improves the clinical management and the prognosis in ROHHAD(NET), this diagnosis should be considered for any child with rapid and early obesity. We propose guidance for systematic follow-up and advise multidisciplinary management with the aim of improving prognosis and life expectancy.
Topics: Adrenal Gland Neoplasms; Autonomic Nervous System Diseases; Ganglioneuroblastoma; Ganglioneuroma; Humans; Hypothalamic Diseases; Hypoventilation; Obesity; Prognosis; Syndrome
PubMed: 32407531
DOI: 10.1210/clinem/dgaa247 -
Seminars in Neurology Oct 2020Disorders of sudomotor function are common and diverse in their presentations. Hyperhidrosis or hypohidrosis in generalized or regional neuroanatomical patterns can... (Review)
Review
Disorders of sudomotor function are common and diverse in their presentations. Hyperhidrosis or hypohidrosis in generalized or regional neuroanatomical patterns can provide clues to neurologic localization and inform neurologic diagnosis. Conditions that impair sudomotor function include small fiber peripheral neuropathy, sudomotor neuropathy, myelopathy, α-synucleinopathies, autoimmune autonomic ganglionopathy, antibody-mediated hyperexcitability syndromes, and a host of medications. Particularly relevant to neurologic practice is the detection of postganglionic sudomotor deficits as a diagnostic marker of small fiber neuropathies. Extensive anhidrosis is important to recognize, as it not only correlates with symptoms of heat intolerance but may also place the patient at risk for heat stroke when under conditions of heat stress. Methods for assessing sudomotor dysfunction include the thermoregulatory sweat test, the quantitative sudomotor axon reflex test, silicone impressions, and the sympathetic skin response.
Topics: Autonomic Nervous System Diseases; Body Temperature Regulation; Humans; Hyperhidrosis; Hypohidrosis; Small Fiber Neuropathy
PubMed: 32906168
DOI: 10.1055/s-0040-1713847