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Methodist DeBakey Cardiovascular Journal 2018Cardiovascular autonomic neuropathy (CAN) is a severely debilitating yet underdiagnosed condition in patients with diabetes. The prevalence can range from 2.5% (based on... (Review)
Review
Cardiovascular autonomic neuropathy (CAN) is a severely debilitating yet underdiagnosed condition in patients with diabetes. The prevalence can range from 2.5% (based on the primary prevention cohort in the Diabetes Control and Complications Trial) to as high as 90% of patients with type 1 diabetes. Clinical manifestations range from orthostasis to myocardial infarction. The diagnosis is made using multiple autonomic function tests to assess both sympathetic and parasympathetic function. The pathophysiology of CAN is complex, likely multifactorial, and not completely understood. Treatment is limited to symptomatic control of orthostatic hypotension, which is a late complication, and current strategies to reverse CAN are limited. This review explores the epidemiology, pathophysiology, clinical manifestations, diagnosis, and complications of CAN as well as current treatment options.
Topics: Autonomic Nervous System; Autonomic Nervous System Diseases; Diabetes Mellitus, Type 1; Diabetes Mellitus, Type 2; Diabetic Neuropathies; Heart; Humans; Predictive Value of Tests; Risk Factors; Treatment Outcome
PubMed: 30788010
DOI: 10.14797/mdcj-14-4-251 -
Continuum (Minneapolis, Minn.) Feb 2020This article provides a summary of the autonomic neuropathies, including neuropathies associated with diabetes mellitus, neuropathies due to amyloid deposition,... (Review)
Review
PURPOSE OF REVIEW
This article provides a summary of the autonomic neuropathies, including neuropathies associated with diabetes mellitus, neuropathies due to amyloid deposition, immune-mediated autonomic neuropathies (including those associated with a paraneoplastic syndrome), inherited autonomic neuropathies, and toxic autonomic neuropathies. The presenting features, diagnostic investigations, and natural history of these neuropathies are discussed.
RECENT FINDINGS
Recent findings in autonomic peripheral neuropathy include data on the epidemiology and atypical presentations of diabetic autonomic neuropathy, treatment-induced neuropathy of diabetes mellitus, the presentation of immune-mediated neuropathies, and advances in hereditary neuropathy associated with amyloidosis and other hereditary neuropathies.
SUMMARY
Knowledge and recognition of the clinical features of the autonomic neuropathies, combined with appropriate laboratory and electrophysiologic testing, will facilitate accurate diagnosis and management.
Topics: Autonomic Nervous System; Autonomic Nervous System Diseases; Diabetic Neuropathies; Disease Progression; Genetic Diseases, Inborn; Humans; Peripheral Nervous System Diseases
PubMed: 31996622
DOI: 10.1212/CON.0000000000000825 -
Muscle & Nerve Jan 2021Autonomic neuropathies represent a complex group of disorders that preferentially target autonomic fibers and can be classified as either acute/subacute or chronic in... (Review)
Review
Autonomic neuropathies represent a complex group of disorders that preferentially target autonomic fibers and can be classified as either acute/subacute or chronic in onset. Acute-onset autonomic neuropathies manifest with such conditions as paraneoplastic syndromes, Guillain-Barre syndrome, Sjögren syndrome, infection, or toxins/chemotherapy. When the presentation is acute, immune-mediated, and without a secondary cause, autoimmune autonomic ganglionopathy is likely, and should be considered for immunotherapy. Of the chronic-onset forms, diabetes is the most widespread and disabling, with autonomic impairment portending increased mortality and cardiac wall remodeling risk. Acquired light chain (AL) and transthyretin (TTR) amyloidosis represent two other key etiologies, with TTR amyloidosis now amenable to newly-approved gene-modifying therapies. The COMPASS-31 questionnaire is a validated outcome measure that can be used to monitor autonomic severity and track treatment response. Symptomatic treatments targeting orthostatic hypotension, among other symptoms, should be individualized and complement disease-modifying therapy, when possible.
Topics: Amyloid Neuropathies, Familial; Autoimmune Diseases of the Nervous System; Autonomic Nervous System; Autonomic Nervous System Diseases; Humans; Peripheral Nervous System Diseases; Prealbumin
PubMed: 32926436
DOI: 10.1002/mus.27048 -
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 -
Advances in Experimental Medicine and... 2012Diabetic autonomic neuropathy (DAN) affects each tissue, organ, system and the whole body, and presents with a diverse clinical picture. Originating from endocrine... (Review)
Review
Diabetic autonomic neuropathy (DAN) affects each tissue, organ, system and the whole body, and presents with a diverse clinical picture. Originating from endocrine factors, this neurological disease may cause symptoms, whose differential diagnosis needs a good knowledge of the whole internal medicine. DAN is strongly involved in the development of diabetic foot, ulceration and amputation. The life threatening consequences of cardiac and patient-frustrating sequels of other types of DAN is more difficult to estimate. In this chapter the different clinical aspects of DAN will be discussed, according to the involved system--cardiovascular, gastrointestinal, genitourinary, sudomotor and pupillary dysfunctions and the unawareness and unresponsiveness to hypoglycaemia. The diagnostic tests for DAN are more complicated and time consuming, compared with the somatic tools. There is a need for simple devices and methods for evaluation of autonomic functions in the everyday clinical practice. Tight glycaemic control is the cornerstone of the prevention, progression and retardation of DAN. An effective broad-spectrum pathogenetic treatment of neural deterioration remains to be established. In most cases symptomatic drugs are the treatment of choice.
Topics: Autonomic Nervous System; Autonomic Nervous System Diseases; Diabetic Neuropathies; Humans
PubMed: 23393679
DOI: 10.1007/978-1-4614-5441-0_15 -
Diabetic Medicine : a Journal of the... 1993
Review
Topics: Adrenal Medulla; Autonomic Nervous System; Cardiovascular System; Diabetes Mellitus, Type 1; Diabetic Neuropathies; Digestive System; Female; Humans; Hypoglycemia; Male; Penile Erection; Urinary Bladder; Vagina
PubMed: 8334847
DOI: 10.1111/j.1464-5491.1993.tb00204.x -
Clinical Autonomic Research : Official... Jun 2019Guillain-Barré syndrome (GBS), an inflammatory, usually demyelinating polyradiculopathy, is characterized by ascending symmetrical limb weakness, sensory disturbances,... (Review)
Review
BACKGROUND
Guillain-Barré syndrome (GBS), an inflammatory, usually demyelinating polyradiculopathy, is characterized by ascending symmetrical limb weakness, sensory disturbances, and absent or reduced deep tendon reflexes. There is extensive literature suggesting that GBS is associated with autonomic dysfunction in up to two-thirds of patients. However, it is interesting that there is still no consensus amongst medical professionals regarding whether GBS patients should be routinely screened for autonomic nervous system (ANS) neuropathy. This is an important issue, as the mortality rate from presumed ANS abnormalities now exceeds that of respiratory failure. Given the long interval since this literature was last comprehensively reviewed, an update on this topic is warranted.
METHODS
A PubMed search yielded 193 results with the terms "GBS or Guillain-Barré syndrome and autonomic symptoms" and 127 results with the terms "GBS or Guillain-Barré syndrome and dysautonomia."
RESULTS
This review will summarize the current literature involving GBS and autonomic dysfunction in terms of presentation, management, and a brief discussion of prognosis. We also examine prospective approaches that may be helpful and update a proposed management plan.
Topics: Autonomic Nervous System; Guillain-Barre Syndrome; Humans; Immunotherapy
PubMed: 30019292
DOI: 10.1007/s10286-018-0542-y -
Journal of the Autonomic Nervous System Jul 1990Symptoms of uraemic autonomic neuropathy are often vague and aspecific. A correct diagnosis of autonomic dysfunction is possible by using several simple and non-invasive... (Review)
Review
Symptoms of uraemic autonomic neuropathy are often vague and aspecific. A correct diagnosis of autonomic dysfunction is possible by using several simple and non-invasive tests of parasympathetic and sympathetic control of the cardiovascular system. A defective regulation of the heart rate, due mostly to an afferent lesion, is more common than damage of reflex blood pressure control. The former appears isolated in 14 to 34% of uraemics on haemodialysis and combined to the latter in 18 to 26%. Contradictory results are reported about influence of intermittent haemodialysis, continuous ambulatory peritoneal dialysis and kidney transplantation on disturbed autonomic nervous system. Pathogenesis of uraemic autonomic neuropathy remains unknown and some mechanisms are only putative.
Topics: Autonomic Nervous System; Autonomic Nervous System Diseases; Blood Pressure; Electrocardiography; Heart Rate; Humans; Kidney Failure, Chronic; Posture; Respiration; Uremia; Valsalva Maneuver
PubMed: 2212485
DOI: 10.1016/0165-1838(90)90127-5 -
Diabetologia Oct 1979This review attempts to outline the present understanding of diabetic autonomic neuropathy. The clinical features have been increasinly recognised but knowledge of the... (Review)
Review
This review attempts to outline the present understanding of diabetic autonomic neuropathy. The clinical features have been increasinly recognised but knowledge of the localization and morphology of the lesions and their pathogenesis remains fragmentary. A metabolic causation as postulated in somatic nerves accords best with clinical observations. Most bodily systems, particularly the cardiovascular, gastrointestinal and urogenital, are involved with added disturbances of thermoregulatory function and pupillary reflexes. Possible effects on neuroendocrine and peptidergic secretion and respiratory control await definition. Current interest centres around the development of a new generation of tests of autonomic nerve function that are simple, non-invasive, reproducible and allow precision in diagnosis and accurate quantitation. Most are based on cardiovascular reflexes and abnormality in them is assumed to reflect autonomic damage elsewhere. Probably no single test suffices and a battery of tests reflecting both parasympathetic and sympathetic function is preferable. Little is known of the natural history. The prevalence may be greater than previously suspected and although symptoms are mild in the majority, a few develop florid features. The relation of control and duration of diabetes to the onset and progression of autonomic neuropathy is not clearly established. Once tests of autonomic function become abnormal they usually remain abnormal. Symptomatic autonomic neuropathy carries a greatly increased mortality rate possibly due to indirect mechanisms such as renal failure and direct mechanisms such as cardio-resiratory arrest. Improved treatment of some of the more disabling symptoms has been possible in recent years.
Topics: Autonomic Nervous System; Body Temperature Regulation; Diabetic Neuropathies; Digestive System; Erectile Dysfunction; Heart; Heart Rate; Humans; Hypotension; Male; Urinary Bladder
PubMed: 387501
DOI: 10.1007/BF01235856 -
Pediatric Diabetes Jun 2013Autonomic neuropathy is an under-recognized complication of diabetes, although it affects multiple organ systems and has widespread clinical manifestations including... (Review)
Review
Autonomic neuropathy is an under-recognized complication of diabetes, although it affects multiple organ systems and has widespread clinical manifestations including orthostatic hypotension, exercise intolerance, gastroparesis, diarrhea, constipation, and urinary incontinence. The most severe consequences include hypoglycemia unawareness and cardiovascular dysfunction. Autonomic neuropathy is also implicated in sudden unexplained deaths in otherwise healthy young people--the ‘dead in bed syndrome’. In adults, cardiovascular autonomic neuropathy is an independent predictor of mortality, predominantly due to cardiovascular disease, nephropathy, and hypoglycemia. While overt autonomic neuropathy is rare in childhood and adolescence, subclinical signs of autonomic dysfunction are common, and can be found soon after diabetes diagnosis. Risk factors for autonomic neuropathy in young people include diabetes duration, poor glycemic control, and presence of aldose reductase gene (AKR1B1) polymorphisms, specifically the Z-2/Z-2 genotype. Autonomic dysfunction is accelerated by puberty.
Topics: Adolescent; Autonomic Nervous System; Autonomic Nervous System Diseases; Blood Glucose; Child; Cross-Sectional Studies; Diabetes Mellitus, Type 1; Diabetic Angiopathies; Diabetic Neuropathies; Humans; Incidence; Longitudinal Studies; Prevalence
PubMed: 23627912
DOI: 10.1111/pedi.12039