Clinical autonomic research : official journal of the Clinical Autonomic Research Society
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Hereditary transthyretin amyloidosis (hATTR amyloidosis) is a progressive disease primarily characterized by adult-onset sensory, motor, and autonomic neuropathy. In this article, we discuss the pathophysiology and principal findings of autonomic neuropathy in hATTR amyloidosis, the most common methods of assessment and progression, and its relation as a predictive risk factor or a measure of progression in the natural history of the disease. ⋯ Autonomic nerves are often affected before motor nerve impairment, and dysautonomia may support the diagnosis of hATTR amyloidosis when differentiating from other adult-onset progressive neuropathies and from other types of amyloidosis. Most of the progression of autonomic dysfunction is seen in early stages of the disease, commonly before motor impairment or affection of the overall quality of life. Unfortunately, there is no current single standardized approach to evaluate dysautonomia in hATTR amyloidosis.
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Electrochemical skin conductance (ESC) is a non-invasive test of sweat function developed as a potential marker of small fiber neuropathy. Here we systematically review the evolution of this device and the data obtained from studies of ESC across different diseases. ⋯ Although many papers report significant differences in ESC values between disease and control subjects, the compiled data assessed in this review raises questions about the technique. Many of the published results violate biologic plausibility. A single funding source with a vested interest in the study outcomes has supported most of the studies. Normative values are inconsistent across publications, and large combined data sets do not support a high sensitivity and specificity. Finally, there is insufficient evidence supporting the claim that Sudoscan tests sudomotor or sensory nerve fiber function.
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Currently available techniques for the evaluation of small fiber neuropathy and related sudomotor function remain suboptimal. Electrochemical skin conductance (ESC) has recently been introduced as a simple noninvasive and fast method for the detection of sudomotor dysfunction. The purpose of this review is to synthesize and appraise research using ESC measurements for sudomotor evaluation in adults. ⋯ ESC measurements expand the arsenal of available tests for the evaluation of sudomotor dysfunction. The advantages and disadvantages of ESC versus established tests for evaluating sudomotor/small fiber function reviewed herein should be used as evidence to inform future guidelines on the assessment of sudomotor function.
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Meta Analysis
Does resistance training modulate cardiac autonomic control? A systematic review and meta-analysis.
To systematically evaluate the literature on the effects of resistance training (RT) on cardiac autonomic control in healthy and diseased individuals. ⋯ This rigorous systematic analysis revealed that RT has no or minimal effects on cardiac autonomic control of healthy individuals, but RT leads to improvement in cardiac autonomic control of diseased individuals.
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The initial symptoms of multiple system atrophy (MSA) and, in particular, early autonomic symptoms, have received less attention than motor symptoms. Whereas pathognomonic motor signs are essential to diagnostic specificity, early symptoms important to recognition of a neurodegenerative disorder may be less apparent or diagnostically ambiguous. This observational study sought to identify the very earliest symptoms in the natural history of MSA. ⋯ The first symptoms of MSA are frequently autonomic and may predate recognition of motor manifestations. Orthostatic hypotension and, in men, erectile failure are among the first symptoms that, when evaluated in the context of associated clinical findings, may facilitate accurate and earlier diagnosis.