-
Ugeskrift For Laeger Aug 2023Guillian-Barré syndrome (GBS) is an immune mediated disease which is most commonly caused by infections. Symptoms are rapidly progressive and may include servere...
Guillian-Barré syndrome (GBS) is an immune mediated disease which is most commonly caused by infections. Symptoms are rapidly progressive and may include servere weakness of the extremities, bulbar weakness, autonomic dysfunction and respiratory insufficiens. In rare cases paraneoplastic syndrome may mimic GBS, which is important to know as treatment will fail unless the underlying malignancy is treated. This is a case report of paraneoplastic non-small cell lungcancer mimicking GBS with effect of the checkpoint inhibitor pembrolizumab.
Topics: Humans; Paraneoplastic Syndromes; Autonomic Nervous System Diseases; Extremities
PubMed: 37615229
DOI: No ID Found -
Clinical Autonomic Research : Official... Jun 2023
Topics: Humans; Autonomic Nervous System Diseases; Obesity; Autonomic Nervous System
PubMed: 37273037
DOI: 10.1007/s10286-023-00957-7 -
Lakartidningen Dec 2021Paroxysmal sympathetic hyperactivity (PSH) is a condition mainly described in patients after traumatic brain injury and it is also known under the terms "autonomic...
Paroxysmal sympathetic hyperactivity (PSH) is a condition mainly described in patients after traumatic brain injury and it is also known under the terms "autonomic storm" and "dysautonomia". It affects between 8-10% of patients after traumatic brain injury and can also affect patients after other neurological diseases, such as anoxic brain injury, stroke, tumors or infections. PSH manifests with six main symptoms: tachycardia, tachypnea, hypertension, hyperthermia, hyperhidrosis and increased muscle tonus. It is of outmost importance to exclude other causes for the symptoms and there are diagnostic criteria established to identify and diagnose PSH. The treatment is pharmacological and non-pharmacological and often multimodal. PSH is probably underdiagnosed and increased awareness is needed.
Topics: Autonomic Nervous System Diseases; Brain Injuries; Brain Injuries, Traumatic; Humans; Hypertension; Tachycardia
PubMed: 34914088
DOI: No ID Found -
Physical Medicine and Rehabilitation... Aug 2023Persistence of symptoms beyond the initial acute phase of coronavirus disease-2019 (COVID-19) is termed postacute SARS-CoV-2 (PASC) and includes neurologic, autonomic,... (Review)
Review
Persistence of symptoms beyond the initial acute phase of coronavirus disease-2019 (COVID-19) is termed postacute SARS-CoV-2 (PASC) and includes neurologic, autonomic, pulmonary, cardiac, psychiatric, gastrointestinal, and functional impairment. PASC autonomic dysfunction can present with dizziness, tachycardia, sweating, headache, syncope, labile blood pressure, exercise intolerance, and "brain fog." A multidisciplinary team can help manage this complex syndrome with nonpharmacologic and pharmacologic interventions.
Topics: Humans; SARS-CoV-2; COVID-19; Autonomic Nervous System Diseases; Syncope; Syndrome
PubMed: 37419532
DOI: 10.1016/j.pmr.2023.04.003 -
Autonomic Neuroscience : Basic &... Dec 2020Although there is considerably more data showing an association between type 2 diabetes mellitus (T2DM) and autonomic neuropathy, accumulating evidence indicates that... (Review)
Review
Although there is considerably more data showing an association between type 2 diabetes mellitus (T2DM) and autonomic neuropathy, accumulating evidence indicates that cardiovascular autonomic neuropathy (CAN) is common in persons with impaired glucose tolerance (IGT). Furthermore, CAN may occur early after a metabolic insult and obesity, especially among mean, and seems to play an important role in the early pathogenesis of CAN. Autonomic symptoms are common in subjects with IGT. In addition to defects in CAN, in subjects with IGT, there is impaired sudomotor function and abnormalities of endothelial peripheral vasoreactivity. At the present time, the only interventions that may be effective in preventing or reversing IGT associated autonomic neuropathy are lifestyle improvement. These include a tailored diet and exercise program. Other approaches that may be beneficial include modulation of oxidative stress and improvement of metabolic regulation in subjects with IGT. Interventions are most likely to be effective early in the course of disease and therefore it is extremely important to have early diagnosis of IGT and autonomic neuropathy.
Topics: Autonomic Nervous System Diseases; Diabetic Neuropathies; Heart Diseases; Humans; Prediabetic State
PubMed: 33011523
DOI: 10.1016/j.autneu.2020.102722 -
Revue Medicale de Liege May 2023Complex regional pain syndrome is a clinical entity that usually occurs following trauma, surgery, or other triggering event. Patients complain of pain described as...
Complex regional pain syndrome is a clinical entity that usually occurs following trauma, surgery, or other triggering event. Patients complain of pain described as burning, associated with sensory, vasomotor, sudomotor, motor and/or trophic disorders. The pain appears disproportionate to the initial lesion. The diagnosis is purely clinical and based on the Budapest criteria. It is a diagnosis of exclusion. The pathophysiology is still poorly understood and is thought to be multifactorial. Several mechanisms have been described: an inflammatory state, an overactivation of the sympathetic autonomic nervous system and a poor perception of pain at the central level. The management of signs and symptoms is therefore global. It involves physiotherapy and occupational therapy, often combined with analgesic medications. Psychological treatment may be proposed in certain circumstances. The natural evolution is rather favourable.
Topics: Humans; Complex Regional Pain Syndromes; Pain; Physical Therapy Modalities; Medicine
PubMed: 37350208
DOI: No ID Found -
Kardiologia Polska Nov 2019Orthostatic hypotension (OH) is a cardinal sign of cardiovascular (CV) autonomic dysfunction as a result of autonomic nervous system failure to control the postural... (Review)
Review
Orthostatic hypotension (OH) is a cardinal sign of cardiovascular (CV) autonomic dysfunction as a result of autonomic nervous system failure to control the postural hemodynamic homeostasis. The proportion of individuals with OH increases with aging and chronic conditions, such as neurodegenerative diseases, hypertension, heart failure, diabetes, renal dysfunction, autoimmune diseases, and cancer. In individuals over 70 years of age, more than 20% can be affected. It is now increasingly recognized that there is a direct relationship between OH and each step of the CV disease continuum, eventually leading to end‑stage heart disease and CV death. In particular, prevalent OH is associated with cardiac functional and structural remodeling, left ventricular hypertrophy, elevated levels of circulating markers of inflammation, increased intima‑media thickness, subclinical atherosclerosis, and thrombosis. Beyond subclinical changes, the presence of OH independently predicts coronary events, stroke, atrial fibrillation, heart failure, and CV mortality. Furthermore, OH is associated with syncope, falls, and fragility fractures, presenting hurdles to be overcome in the delivery of the best management of CV risk factors. Taken together, OH heralds disruption of global circulatory homeostasis and flags overt autonomic dysfunction. The presence of OH is also an independent risk factor for mortality and CV disease; however, until now, the importance of this highly prevalent disorder has been given insufficient attention by clinicians and other healthcare providers. Consequently, more studies are needed to find effective treatment for this troublesome condition and to identify preventive measures that could reduce the burden of CV risk in OH and autonomic dysfunction.
Topics: Autonomic Nervous System; Cardiovascular Diseases; Humans; Hypotension, Orthostatic
PubMed: 31713533
DOI: 10.33963/KP.15055 -
Pediatric Neurology Aug 2019Pathologic mutations in cyclin-dependent kinase-like 5 cause CDKL5 deficiency disorder, a genetic syndrome associated with severe epilepsy and cognitive, motor, visual,... (Review)
Review
BACKGROUND
Pathologic mutations in cyclin-dependent kinase-like 5 cause CDKL5 deficiency disorder, a genetic syndrome associated with severe epilepsy and cognitive, motor, visual, and autonomic disturbances. This disorder is a relatively common genetic cause of early-life epilepsy. A specific severity assessment is lacking, required to monitor the clinical course and needed to define the natural history and for clinical trial readiness.
METHODS
A severity assessment was developed based on clinical and research experience from the International Foundation for CDKL5 Research Centers of Excellence consortium and the National Institutes of Health Rett and Rett-Related Disorders Natural History Study consortium. An initial draft severity assessment was presented and reviewed at the annual CDKL5 Forum meeting (Boston, 2017). Subsequently it was iterated through four cycles of a modified Delphi process by a group of clinicians, researchers, industry, patient advisory groups, and parents familiar with this disorder until consensus was achieved. The revised version of the severity assessment was presented for review, comment, and piloting to families at the International Foundation for CDKL5 Research-sponsored family meeting (Colorado, 2018). Final revisions were based on this additional input.
RESULTS
The final severity assessment comprised 51 items that comprehensively describe domains of epilepsy; motor; cognition, behavior, vision, and speech; and autonomic functions. Parental ratings of therapy effectiveness and child and family functioning are also included.
CONCLUSIONS
A severity assessment was rapidly developed with input from multiple stakeholders. Refinement through ongoing validation is required for future clinical trials. The consensus methods employed for the development of severity assessment may be applicable to similar rare disorders.
Topics: Autonomic Nervous System Diseases; Child; Clinical Trials as Topic; Cognition Disorders; Delphi Technique; Epileptic Syndromes; Humans; Movement Disorders; Severity of Illness Index; Spasms, Infantile; Speech Disorders; Surveys and Questionnaires; Symptom Assessment; Vision Disorders
PubMed: 31147226
DOI: 10.1016/j.pediatrneurol.2019.03.017 -
Current Neurology and Neuroscience... Dec 2022Autonomic neuropathies are a complex group of disorders and result in diverse clinical manifestations that affect the cardiovascular, gastrointestinal, urogenital, and... (Review)
Review
PURPOSE OF REVIEW
Autonomic neuropathies are a complex group of disorders and result in diverse clinical manifestations that affect the cardiovascular, gastrointestinal, urogenital, and sudomotor systems. We focus this review on the diagnosis and treatment of peripheral autonomic neuropathies. We summarize the diagnostic tools and current treatment options that will help the clinician care for individuals with peripheral autonomic neuropathies.
RECENT FINDINGS
Autonomic neuropathies occur often in conjunction with somatic neuropathies but they can also occur in isolation. The autonomic reflex screen is a validated tool to assess sympathetic postganglionic sudomotor, cardiovascular sympathetic noradrenergic, and cardiac parasympathetic (i.e., cardiovagal) function. Initial laboratory evaluation for autonomic neuropathies includes fasting glucose or oral glucose tolerance test, thyroid function tests, kidney function tests, vitamin-B12, serum, and urine protein electrophoresis with immunofixation. Other laboratory tests should be guided by the clinical context. Reduced intraepidermal nerve density on skin biopsy is a finding, not a diagnosis. Skin biopsy can be helpful in selected individuals for the diagnosis of disorders affecting small nerve fibers; however, we strongly discourage the use of skin biopsy without clinical-physiological correlation. Ambulatory blood pressure monitoring may lead to early identification of patients with cardiovascular autonomic neuropathy in the primary care setting. Disease-modifying therapies should be used when available in combination with nonpharmacological management and symptomatic pharmacologic therapies. Autonomic function testing can guide the therapeutic decisions and document improvement with treatment. A systematic approach guided by the autonomic history and standardized autonomic function testing may help clinicians when identifying and/or counseling patients with autonomic neuropathies. Treatment should be individualized and disease-modifying therapies should be used when available.
Topics: Humans; Autonomic Nervous System Diseases; Blood Pressure Monitoring, Ambulatory; Peripheral Nervous System Diseases; Autonomic Nervous System; Norepinephrine; Diabetic Neuropathies
PubMed: 36376534
DOI: 10.1007/s11910-022-01240-4 -
Autonomic Neuroscience : Basic &... Dec 2020Diabetic autonomic neuropathy affects the entire autonomic nervous system and can lead to dysfunction of the cardiovascular, gastrointestinal, and genitourinary organ... (Review)
Review
Diabetic autonomic neuropathy affects the entire autonomic nervous system and can lead to dysfunction of the cardiovascular, gastrointestinal, and genitourinary organ systems. Genitourinary dysfunction associated with diabetic autonomic neuropathy includes diabetic bladder dysfunction, sexual dysfunction, and recurrent urinary tract infections. Urological complications in diabetes mellitus are very common; in fact, genitourinary complications are more common than diabetic neuropathy or nephropathy. While several studies have reported on genitourinary dysfunction in individuals with diabetes, UroEDIC, an ancillary study to the Diabetes Control and Complications Trial (DCCT) and its observational follow up, the Epidemiology of Diabetes Interventions and Complications study (EDIC), comprehensively characterized the association between urologic complications and cardiovascular autonomic neuropathy. UroEDIC demonstrated significant associations between autonomic neuropathy and urologic complications in type 1 diabetes, specifically erectile dysfunction, female sexual dysfunction, and lower urinary tract symptoms. In this narrative review, we review the current literature on urological complications in diabetes.
Topics: Autonomic Nervous System Diseases; Diabetes Complications; Female; Humans; Male; Sexual Dysfunction, Physiological; Urinary Bladder Diseases
PubMed: 33197694
DOI: 10.1016/j.autneu.2020.102736