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      Updates on the Diagnosis and Treatment of Peripheral Autonomic Neuropathies

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          Abstract

          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.

          Summary

          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.

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          Most cited references67

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          Nonbiopsy Diagnosis of Cardiac Transthyretin Amyloidosis.

          Cardiac transthyretin (ATTR) amyloidosis is a progressive and fatal cardiomyopathy for which several promising therapies are in development. The diagnosis is frequently delayed or missed because of the limited specificity of echocardiography and the traditional requirement for histological confirmation. It has long been recognized that technetium-labeled bone scintigraphy tracers can localize to myocardial amyloid deposits, and use of this imaging modality for the diagnosis of cardiac ATTR amyloidosis has lately been revisited. We conducted a multicenter study to ascertain the diagnostic value of bone scintigraphy in this disease.
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            Nervous system consequences of COVID-19

            Neurological symptoms highlight the need to understand pathophysiologic mechanisms Although severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is considered a respiratory pathogen, myriad neurologic complications—including confusion, stroke, and neuromuscular disorders—manifest during acute COVID-19. Furthermore, maladies such as impaired concentration, headache, sensory disturbances, depression, and even psychosis may persist for months after infection, as part of a constellation of symptoms now called Long Covid. Even young people with mild initial disease can develop acute COVID-19 and Long Covid neuropsychiatric syndromes. The pathophysiological mechanisms are not well understood, although evidence primarily implicates immune dysfunction, including nonspecific neuroinflammation and antineural autoimmune dysregulation. It is uncertain whether unforeseen neurological consequences may develop years after initial infection. With millions of individuals affected, nervous system complications pose public health challenges for rehabilitation and recovery and for disruptions in the workforce due to loss of functional capacity. There is an urgent need to understand the pathophysiology of these disorders and develop disease-modifying therapies.
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                Author and article information

                Contributors
                guillaume.lamotte@hsc.utah.edu
                Journal
                Curr Neurol Neurosci Rep
                Curr Neurol Neurosci Rep
                Current Neurology and Neuroscience Reports
                Springer US (New York )
                1528-4042
                1534-6293
                15 November 2022
                : 1-15
                Affiliations
                [1 ]GRID grid.223827.e, ISNI 0000 0001 2193 0096, Department of Neurology, , University of Utah, ; Salt Lake City, UT USA
                [2 ]GRID grid.66875.3a, ISNI 0000 0004 0459 167X, Department of Neurology, , Mayo Clinic, ; Rochester, MN USA
                Author information
                http://orcid.org/0000-0003-4048-6095
                Article
                1240
                10.1007/s11910-022-01240-4
                9663281
                36376534
                56a3744b-d763-4052-b34c-ef32110c9f0f
                © The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature 2022, Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.

                This article is made available via the PMC Open Access Subset for unrestricted research re-use and secondary analysis in any form or by any means with acknowledgement of the original source. These permissions are granted for the duration of the World Health Organization (WHO) declaration of COVID-19 as a global pandemic.

                History
                : 5 October 2022
                Categories
                Autonomic Dysfunction (L.H. Weimer, Section Editor)

                Neurosciences
                autonomic neuropathies,skin biopsy,autonomic reflex screen,amyloidosis,covid-19
                Neurosciences
                autonomic neuropathies, skin biopsy, autonomic reflex screen, amyloidosis, covid-19

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