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Current Opinion in Neurology Oct 2021The review focuses on the practical evaluation and management of patients with autonomic neuropathies. (Review)
Review
PURPOSE OF REVIEW
The review focuses on the practical evaluation and management of patients with autonomic neuropathies.
RECENT FINDINGS
Autonomic neuropathies are complex disorders and result in diverse clinical manifestations that affect the cardiovascular, gastrointestinal, urogenital, and sudomotor systems. The autonomic medical history is key when seeing a patient with suspected autonomic neuropathy. The history guides the clinical evaluation, laboratory testing, and autonomic testing in patients with autonomic neuropathies. The treatment of autonomic neuropathies is based on the combination of disease-modifying therapies, symptomatic pharmacologic therapies, and nonpharmacological management. Response to treatment can be assessed with quantitative autonomic biomarkers.
SUMMARY
Treatment of autonomic neuropathies should be individualized, guided by disease state, medications' mechanism of action and adverse event profile as well as cost. Genetic discoveries and pathologic understanding lead to the development of disease-modifying therapies as seen in familial amyloid polyneuropathy.
Topics: Amyloid Neuropathies, Familial; Autonomic Nervous System; Autonomic Nervous System Diseases; Humans
PubMed: 33973903
DOI: 10.1097/WCO.0000000000000962 -
Handbook of Clinical Neurology 2018Multiple sclerosis (MS) is a progressive neurologic disorder that disrupts axonal myelin in the central nervous system. Demyelination produces alterations in saltatory... (Review)
Review
Multiple sclerosis (MS) is a progressive neurologic disorder that disrupts axonal myelin in the central nervous system. Demyelination produces alterations in saltatory conduction, slowed conduction velocity, and a predisposition to conduction block. An estimated 60-80% of MS patients experience temporary worsening of clinical signs and neurologic symptoms with heat exposure (Uhthoff's phenomenon). This heat intolerance in MS is related to the detrimental effects of increased temperature on action potential propagation in demyelinated axons, resulting in conduction slowing and/or block. Additionally, MS may produce impaired neural control of autonomic and endocrine functions. Isolating and interpreting mechanisms responsible for autonomic dysfunction due to MS can be difficult as it may involve sensory impairments, altered neural integration within the central nervous system, impaired effector responses, or combinations of all of these factors. MS lesions occur in areas of the brain responsible for the control and regulation of body temperature and thermoregulatory effector responses, resulting in impaired neural control of sudomotor pathways or neural-induced changes in eccrine sweat glands, as evidenced by observations of reduced sweating responses in MS patients. Although not comprehensive, some evidence exists concerning treatments (cooling, precooling, and pharmacologic) for the MS patient to preserve function and decrease symptom worsening during heat stress. This review focuses on four main themes influencing current understanding of thermoregulatory dysfunction in MS: (1) heat intolerance; (2) central regulation of body temperature; (3) thermoregulatory effector responses; and (4) countermeasures to improve or maintain function during thermal stress.
Topics: Autonomic Nervous System Diseases; Body Temperature Regulation; Fever; Humans; Hypothermia; Multiple Sclerosis
PubMed: 30459034
DOI: 10.1016/B978-0-444-64074-1.00042-2 -
American Journal of Physiology.... Aug 2015Stress- and anxiety-related disorders are on the rise in both military and general populations. Over the next decade, it is predicted that treatment of these conditions,... (Review)
Review
Stress- and anxiety-related disorders are on the rise in both military and general populations. Over the next decade, it is predicted that treatment of these conditions, in particular, posttraumatic stress disorder (PTSD), along with its associated long-term comorbidities, will challenge the health care system. Multiple organ systems are adversely affected by PTSD, and PTSD is linked to cancer, arthritis, digestive disease, and cardiovascular disease. Evidence for a strong link between PTSD and cardiovascular disease is compelling, and this review describes current clinical data linking PTSD to cardiovascular disease, via inflammation, autonomic dysfunction, and the renin-angiotensin system. Recent clinical and preclinical evidence regarding the role of the renin-angiotensin system in the extinction of fear memory and relevance in PTSD-related immune and autonomic dysfunction is also addressed.
Topics: Animals; Autonomic Nervous System; Autonomic Nervous System Diseases; Cardiovascular Diseases; Humans; Inflammation; Inflammation Mediators; Prognosis; Renin-Angiotensin System; Risk Factors; Stress Disorders, Post-Traumatic
PubMed: 26062635
DOI: 10.1152/ajpregu.00343.2014 -
Muscle & Nerve Jul 2021Amyloidosis refers to an etiologically heterogeneous group of protein misfolding diseases, pathologically characterized by extracellular amyloid fibrils producing... (Review)
Review
Amyloidosis refers to an etiologically heterogeneous group of protein misfolding diseases, pathologically characterized by extracellular amyloid fibrils producing congophillic amorphous deposits in organs and tissues, which may lead to severe organ dysfunction and mortality. Clinical presentations vary and are often nonspecific, depending on what organs or tissues are affected. In systemic amyloidosis, the peripheral nervous system is commonly affected, whereas the skeletal muscles are only rarely involved. Immunoglobulin light chain (AL) amyloidosis and hereditary transthyretin (ATTRv) amyloidosis are the most frequent types of systemic amyloidosis involving the neuromuscular system. Localized amyloidosis can occur in skeletal muscle, so-called isolated amyloid myopathy. Amyloid neuropathy typically involves small myelinated and unmyelinated sensory and autonomic nerve fibers early in the course of the disease, followed by large myelinated fiber sensory and motor deficits. The relentlessly progressive nature with motor, painful sensory and severe autonomic dysfunction, profound weight loss, and systemic features are distinct characteristics of amyloid neuropathy. Amyloid myopathy presentation differs between systemic amyloidosis and isolated amyloid myopathy. Long-standing symptoms, distal predominant myopathy, markedly elevated creatine kinase level, and lack of peripheral neuropathy or systemic features are highly suggestive of isolated amyloid myopathy. In ATTR and AL amyloidosis, early treatment correlates with favorable outcomes. Therefore, awareness of these disorders and active screening for amyloidosis in patients with neuropathy or myopathy are crucial in detecting these patients in the everyday practice of neuromuscular medicine. Herein, we review the clinical manifestations of neuromuscular amyloidosis and provide a diagnostic approach to this disorder.
Topics: Amyloid Neuropathies, Familial; Amyloidosis; Autonomic Nervous System Diseases; Humans; Immunoglobulin Light-chain Amyloidosis; Muscular Diseases; Neuromuscular Diseases
PubMed: 33458861
DOI: 10.1002/mus.27150 -
Revue Neurologique 2024Pure autonomic failure (PAF) is a neurodegenerative disease affecting the sympathetic component of the autonomic nervous system and presenting as orthostatic hypotension... (Review)
Review
Pure autonomic failure (PAF) is a neurodegenerative disease affecting the sympathetic component of the autonomic nervous system and presenting as orthostatic hypotension (OH). It is a rare, sporadic disease of adults. Although OH is the primary symptom, the autonomic dysfunction may be more generalised, leading to genitourinary and intestinal dysfunction and sweating disorders. Autonomic symptoms in PAF may be similar to those observed in other autonomic neuropathies that need to be ruled out. PAF belongs to the group of α synucleinopathies and is characterised by predominant peripheral deposition of α-synuclein in autonomic ganglia and nerves. However, in a significant number of cases, PAF may convert into another synucleinopathy with central nervous system involvement with varying prognosis: Parkinson's disease (PD), multiple system atrophy (MSA), or dementia with Lewy bodies (DLB). The clinical features, the main differential diagnoses, the risk factors for "phenoconversion" to another synucleinopathy as well as an overview of treatment will be discussed.
Topics: Adult; Humans; Pure Autonomic Failure; Synucleinopathies; Parkinson Disease; Multiple System Atrophy; Lewy Body Disease; Autonomic Nervous System Diseases
PubMed: 38129276
DOI: 10.1016/j.neurol.2023.11.003 -
Continuum (Minneapolis, Minn.) Oct 2014This article focuses on the most prevalent forms of autonomic neuropathies, but also discusses conditions such as focal and dysfunctional syndromes (altered autonomic... (Review)
Review
PURPOSE OF REVIEW
This article focuses on the most prevalent forms of autonomic neuropathies, but also discusses conditions such as focal and dysfunctional syndromes (altered autonomic function in the absence of structural lesions). The goal of this review is to allow the reader to promptly recognize these disorders, identify potentially reversible or treatable causes, and implement the appropriate treatment as well as supportive care.
RECENT FINDINGS
Secondary forms of autonomic neuropathies (eg, diabetes mellitus, amyloidosis) are much more common than primary forms, of which autoimmune ganglioneuropathies represent a major component. However, the spectrum of the latter is continuously evolving and has diagnostic and therapeutic implications. Testing modalities such as autonomic testing, serum autoimmune antibody testing, and skin biopsies are becoming more widely available.
SUMMARY
Autonomic neuropathies are relatively common conditions, and, because of the prognostic implications as well as impact on patient quality of life, they should be promptly recognized and treated aggressively. Testing is critical as other conditions may mimic autonomic neuropathies. Treatment is symptomatic in many cases, but specific therapies are also available in selected autonomic neuropathies.
Topics: Adult; Autonomic Nervous System Diseases; Female; Humans; Male; Middle Aged
PubMed: 25299288
DOI: 10.1212/01.CON.0000455875.76179.b1 -
Current Pain and Headache Reports Feb 2018Symptoms of autonomic dysfunction are common in patients with migraine, both during and between migraine attacks. Studies evaluating objective autonomic testing in... (Review)
Review
PURPOSE OF REVIEW
Symptoms of autonomic dysfunction are common in patients with migraine, both during and between migraine attacks. Studies evaluating objective autonomic testing in patients have found significant, though somewhat conflicting results. The purposes of this review are to summarize and interpret the key findings of these studies, including those evaluating heart rate variability, autonomic reflex testing, and functional imaging in patients with migraine. The neuroanatomy of the central autonomic network as it relates to migraine is also reviewed.
RECENT FINDINGS
Several studies have evaluated autonomic balance in migraineurs, with conflicting results on the magnitude of sympathetic versus parasympathetic dysfunction. Most studies demonstrate sympathetic impairment, with a lesser degree of parasympathetic impairment. Three trends have emerged: (1) migraine with aura tends to produce more significant autonomic dysfunction than migraine without aura, (2) sympathetic impairment is more common than parasympathetic impairment, and (3) sympathetic impairment is common in the interictal period, with increased sympathetic responsiveness during the ictal period, suggesting adrenoreceptor hypersensitivity.
Topics: Autonomic Nervous System Diseases; Humans; Migraine Disorders
PubMed: 29476276
DOI: 10.1007/s11916-018-0671-y -
Handbook of Clinical Neurology 2024A number of the well-recognized autoimmune and paraneoplastic neurologic syndromes commonly involve the autonomic nervous system. In some cases, the autonomic nerves or... (Review)
Review
A number of the well-recognized autoimmune and paraneoplastic neurologic syndromes commonly involve the autonomic nervous system. In some cases, the autonomic nerves or ganglia are primary targets of neurologic autoimmunity, as in immune-mediated autonomic ganglionopathies. In other disorders such as encephalitis, autonomic centers in the brain may be affected. The presence of autonomic dysfunction (especially gastrointestinal dysmotility) is sometimes overlooked even though this may contribute significantly to the symptom burden in these paraneoplastic disorders. Additionally, recognition of autonomic features as part of the clinical syndrome can help point the diagnostic evaluation toward autoimmune and paraneoplastic etiologies. As with other paraneoplastic disorders, the clinical syndrome and the presence and type of neurologic autoantibodies help to secure the diagnosis and direct the most appropriate investigation for malignancy. Optimal management for these conditions typically includes aggressive treatment of the neoplasm, immunomodulatory therapy, and symptomatic treatments for orthostatic hypotension and gastrointestinal dysmotility.
Topics: Humans; Autoantibodies; Nervous System Diseases; Autonomic Nervous System Diseases; Paraneoplastic Syndromes, Nervous System; Neoplasms; Autonomic Nervous System
PubMed: 38494282
DOI: 10.1016/B978-0-12-823912-4.00005-0 -
Journal of Neurology Nov 2023Multiple system atrophy (MSA) is a sporadic, fatal, and rapidly progressive neurodegenerative disease of unknown etiology that is clinically characterized by autonomic... (Review)
Review
Multiple system atrophy (MSA) is a sporadic, fatal, and rapidly progressive neurodegenerative disease of unknown etiology that is clinically characterized by autonomic failure, parkinsonism, cerebellar ataxia, and pyramidal signs in any combination. Early onset and extensive autonomic dysfunction, including cardiovascular dysfunction characterized by orthostatic hypotension (OH) and supine hypertension, urinary dysfunction characterized by overactive bladder and incomplete bladder emptying, sexual dysfunction characterized by sexual desire deficiency and erectile dysfunction, and gastrointestinal dysfunction characterized by delayed gastric emptying and constipation, are the main features of MSA. Autonomic dysfunction greatly reduces quality of life and increases mortality. Therefore, early diagnosis and intervention are urgently needed to benefit MSA patients. In this review, we aim to discuss the systematic treatment of autonomic dysfunction in MSA, and focus on the current methods, starting from non-pharmacological methods, such as patient education, psychotherapy, diet change, surgery, and neuromodulation, to various drug treatments targeting autonomic nerve and its projection fibers. In addition, we also draw attention to the interactions among various treatments, and introduce novel methods proposed in recent years, such as gene therapy, stem cell therapy, and neural prosthesis implantation. Furthermore, we elaborate on the specific targets and mechanisms of action of various drugs. We would like to call for large-scale research to determine the efficacy of these methods in the future. Finally, we point out that studies on the pathogenesis of MSA and pathophysiological mechanisms of various autonomic dysfunction would also contribute to the development of new promising treatments and concepts.
Topics: Male; Humans; Multiple System Atrophy; Quality of Life; Autonomic Nervous System Diseases; Erectile Dysfunction; Parkinsonian Disorders
PubMed: 37477834
DOI: 10.1007/s00415-023-11876-y -
Clinical Autonomic Research : Official... Feb 2016To review existing evidence regarding interactions between the autonomic nervous system and the immune system functions in multiple sclerosis. (Review)
Review
OBJECTIVE
To review existing evidence regarding interactions between the autonomic nervous system and the immune system functions in multiple sclerosis.
METHODS
We reviewed the literature regarding new insights linking autonomic dysfunction to immune deregulation in multiple sclerosis, with particular focus on the specific influence of sympathetic and parasympathetic dysfunction on inflammatory and neurodegenerative processes.
RESULTS
Autonomic dysfunction is common in multiple sclerosis, representing a significant cause of disability. Several connections between pathologic immune pathways and the autonomic nervous system function were found.
CONCLUSIONS
Autonomic dysfunction may enhance inflammatory and neurodegenerative pathways that are of major importance in multiple sclerosis. Autonomic dysfunction can present with highly variable manifestations. Sympathetic and parasympathetic dysfunction displays different patterns in multiple sclerosis, with specific impact on inflammation and neurodegeneration.
Topics: Autonomic Nervous System Diseases; Humans; Multiple Sclerosis
PubMed: 26691635
DOI: 10.1007/s10286-015-0325-7