Updates on the Diagnosis and Treatment of Peripheral Autonomic Neuropathies (original) (raw)
Related papers
Muscle & Nerve, 2020
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. S...
Peripheral Autonomic Neuropathy
Journal of Neuropathology & Experimental Neurology, 2012
Skin biopsy has gained widespread use for the diagnosis of somatic small-fiber neuropathy, but it also provides information on sympathetic fiber morphology. We aimed to ascertain the diagnostic accuracy of skin biopsy in disclosing sympathetic nerve abnormalities in patients with autonomic neuropathy. Peripheral nerve fiber autonomic involvement was confirmed by routine autonomic laboratory test abnormalities. Punch skin biopsies were taken from the thigh and lower leg of 28 patients with various types of autonomic neuropathy for quantitative evaluation of skin autonomic innervation. Results were compared with scores obtained from 32 agematched healthy controls and 25 patients with somatic neuropathy. The autonomic cutoff score was calculated using the receiver operating characteristic curve analysis. Skin biopsy disclosed a significant autonomic innervation decrease in autonomic neuropathy patients versus controls and somatic neuropathy patients. Autonomic innervation density was abnormal in 96% of patients in the lower leg and in 79% of patients in the thigh. The abnormal findings disclosed by routine autonomic tests ranged from 48% to 82%. These data indicate the high sensitivity and specificity of skin biopsy in detecting sympathetic abnormalities; this method should be useful for the diagnosis of autonomic neuropathy, together with currently available routine autonomic testing.
Autonomic dysfunction in peripheral nerve disease
2003
Autonomic neuropathies are inherited or acquired neuropathies in which autonomic nerve fibers are selectively or disproportionately affected. Generally, sympathetic and parasympathetic fibers are both affected but there are exceptions. Acquired cases can be autoimmune; due to diabetes, amyloidosis, drugs, or toxins; or idiopathic. Autoimmune autonomic neuropathy is often subacute, sometimes associated with a neoplasm, and associated with high titers of antibody to ganglionic nicotinic acetylcholine receptor in about half of the severe cases. The molecular basis of inherited autonomic neuropathies is better known, including recent identification of the loci and genes of hereditary sensory and autonomic neuropathies types I, III, and IV. The inherited amyloid neuropathies are due to mutations of three proteins: transthyretin, apolipoprotein A1, and gelsolin. Non-invasive autonomic testing complements clinical and electrophysiological characterization of the autonomic neuropathies.
Evaluation and Management of Autonomic Disorders
2018
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Idiopathic Autonomic Neuropathy
Archives of Neurology, 2004
Background: The clinical characteristics of autoimmune autonomic neuropathy are only partially defined. More than 50% of patients with high levels of ganglionic acetylcholine receptor (AChR) autoantibodies have a combination of sicca complex (marked dry eyes and dry mouth), abnormal pupillary light response, upper gastrointestinal tract symptoms, and neurogenic bladder. Objective: To compare patients with idiopathic autonomic neuropathy who were seropositive (n=19) and seronegative (n= 87) for ganglionic AChR antibodies. Design: Retrospective review of autonomic programmatic database.
Reflections on the past three decades of autonomic neurology
Clinical Autonomic Research, 2021
I enthusiastically congratulate Clinical Autonomic Research for its growth and recognition on the thirtieth anniversary of its founding. In such a relatively small field, the journal now enjoys a readership, influence and impact factor that is achieved by few subspecialty journals. This growth and development in no small part reflects the dynamic and visionary leadership that has steered this important journal. I take this opportunity to make some observations on the growth of autonomic neurology and autonomic medicine from a “Cinderella” of medicine to a respected and established subspecialty. What are the major motive forces that have led us from what we had thirty years ago to what we are today? These observations are of course, my personal opinion. Neurologists and clinicians have long recognized that the autonomic nervous system is relevant, since some important diseases are characterized by autonomic failure or dysfunction. Autonomic failure can be dramatic, leading to faintin...
Autonomic History, Examination, and Laboratory Evaluation
PURPOSE OF REVIEW: Autonomic disorders offer a fascinating view of the complexity of the nervous system. Their impact on human health ranges from benign to severe. Deciphering autonomic symptoms and signs draws on the cognitive skills and personal interest in the plight of patients that first attracted many physicians to the field of neurology. This article provides tools to sharpen those skills.
The natural history of recently diagnosed autonomic neuropathy over a period of 2 years
1998
Background: Patients with diabetic autonomic neuropathy (DAN) have an increased cardiovascular mortality rate compared with normals or diabetic patients without DAN. Indices of standard cardiovascular autonomic function tests and heart rate variability (HRV) are reliable markers of the presence and severity of DAN. Objective: The present prospective study investigated the natural history of values of HRV indices and cardiovascular reflex tests in patients with recently diagnosed asymptomatic DAN, over a period of 2 years, at 3 month intervals. Patients and methods: A total of 30 consecutive patients (nine men and 21 women), of median age 51 (range 25-65) years, eight with type 1 and 22 with Type 2 diabetes mellitus, were included in the study, at the time that the presence of DAN was confirmed, as this was established if at least two of cardiovascular autonomic function tests became abnormal. The expiration/inspiration (E/I) ratio, S.D. and mean circular resultant of R-R intervals, the Valsalva index, the 30:15 ratio, and the blood pressure response to standing as well as normalised spectral power indices of HRV were used. Results: All measured indices, except the Valsalva index, deteriorated in all 30 patients during the 2 year follow-up. Most of HRV indices values deteriorated significantly in comparison to baseline at month 12, while the values of cardiovascular reflex tests displayed significant deterioration, in comparison to baseline, between months 15 and 21. Fourteen patients developed symptoms of DAN during the 2 year period. Patients with better glycemic control exhibited deterioration of DAN markers at the same time period with those with poor glycemic control. Conclusions: Our data suggest that the progression of DAN is significant during the 2 years subsequent to its discovery. This was defined by the deterioration of the mean values of HRV indices and standard cardiovascular autonomic function tests, and by the development of autonomic symptoms in some patients. HRV indices are the earlier markers of DAN deterioration.
Autonomic Function Evaluaton in Chronic Polyneuropathies
IOSR Journals , 2019
Introduction: Chronic polyneuropathies were most common neuropathies with major emphasis on motor and sensory symptoms with autonomic symptoms overlooked which may be the cause of poor quality of life and mortality.Aims and Objectives of the Study: 1.To study the frequency of occurrence of autonomic dysfunction in chronic polyneuropathies.2.To study the pattern of autonomic dysfunction in chronic polyneuropathies.3.To quantitate the severity of autonomic dysfunction using Ewing's criteria. Methods: A sample of 97 chronic polyneuropathycases fulfilling the inclusion and exclusion criteria were taken and autonomic functions were analysed using CAN 504 analyser, sympathetic skin response. Results: Out of 97 cases,DM with polyneuropathy (DPN) were 35 cases (36%, M:F=54:46), CIDP were 26 cases (27%, M:F=69:31) and other polyneuropathies were 36 cases (37%, M:F=67:33). Majority of the cases were having autonomic symptoms (61%).Most common symptomwas orthostatic dizziness. Only mild dysfunction was seen in 48% cases,early diagnosis and treatment may prevent progression to severe dysfunction in majority of chronic polyneuropathies.Definite and severe autonomic dysfunction was more common in CIDP (43%). Among heart rate variable components of autonomic tests highest involvement was seen in standing 30:15 ratio (48%). Both parasympathetic (cardio vagal) and sympathetic (adrenergic) systems were affected, parasympathetic (73%) being more involved than sympathetic system (59%) in the total study population. Conclusions: Autonomic dysfunction in chronic polyneuropathies is common, under diagnosed and potentially treatable disease.Mild autonomic dysfunction is more common than definite and severe dysfunction. Early diagnosis and treatment of underlying cause may prevent further progression. Early treatment would prevent morbidity, mortality and can improve the quality of life.
Autonomic Function Tests: Some Clinical Applications
Journal of Clinical Neurology, 2013
Modern autonomic function tests can non-invasively evaluate the severity and distribution of autonomic failure. They have sufficient sensitivity to detect even subclinical dysautonomia. Standard laboratory testing evaluates cardiovagal, sudomotor and adrenergic autonomic functions. Cardiovagal function is typically evaluated by testing heart rate response to deep breathing at a defined rate and to the Valsalva maneuver. Sudomotor function can be evaluated with the quantitative sudomotor axon reflex test and the thermoregulatory sweat test. Adrenergic function is evaluated by the blood pressure and heart rate responses to the Valsalva maneuver and to head-up tilt. Tests are useful in defining the presence of autonomic failure, their natural history, and response to treatment. They can also define patterns of dysautonomia that are useful in helping the clinician diagnose certain autonomic conditions. For example, the tests are useful in the diagnosis of the autonomic neuropathies and distal small fiber neuropathy. The autonomic neuropathies (such as those due to diabetes or amyloidosis) are characterized by severe generalized autonomic failure. Distal small fiber neuropathy is characterized by an absence of autonomic failure except for distal sudomotor failure. Selective autonomic failure (which only one system is affected) can be diagnosed by autonomic testing. An example is chronic idiopathic anhidrosis, where only sudomotor function is affected. Among the synucleinopathies, autonomic function tests can distinguish Parkinson's disease (PD) from multiple system atrophy (MSA). There is a gradation of autonomic failure. PD is characterized by mild autonomic failure and a length-dependent pattern of sudomotor involvement. MSA and pure autonomic failure have severe generalized autonomic failure while DLB is intermediate.