Abstract

Some peripheral nerve disorders have prominent involvement of autonomic nerves or ganglia; these include amyloid neuropathy; diabetes mellitus; autoimmune or paraneoplastic autoimmune autonomic ganglionopathy; connective tissue disorders; Fabry disease; hereditary sensory and autonomic neuropathies; and toxic or infectious neuropathies. The most common are amyloidosis and diabetes mellitus. The autonomic manifestations of amyloid neuropathy commonly reflect adrenergic vasomotor failure leading to orthostatic hypotension and vagal gastrointestinal failure. Other manifestations include dry eyes or dry mouth; abnormal sudomotor function, urinary retention; and sexual dysfunction leading to erectile dysfunction in men. In most diabetic patients with autonomic neuropathy, the autonomic deficit is mild. However, the potential autonomic manifestations of diabetic neuropathy are vast and can include orthostatic hypotension, gastrointestinal dysmotility, and genitourinary disturbances...In both amyloid and diabetic autonomic neuropathies, recognition is important for adequate treatment of the underlying disease process and for symptomatic management.

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