Clinical features of autoimmune autonomic neuropathy with onset of acute autonomic nerve dysfunction

Abstract

Background Autoimmune autonomic neuropathy with onset of acute autonomic nerve dysfunction is often misdiagnosed because its main symptoms are syncope and gastrointestinal motility disorder. This study summarized the clinical, electrophysiological and pathological changes of peripheral nerve biopsy of patients of autoimmune autonomic neuropathy with onset of acute autonomic nerve dysfunction, which could provide reference for diagnosis, differential diagnosis and treatment. Methods and Results The clinical data of 3 patients diagnosied with autoimmune autonomic neuropathy with onset of acute autonomic nerve dysfunction who were hospitalized in Department of Neurology, Peking University First Hospital from February 2016 to February 2019 were collected. All of the 3 patients were young women, presented with onset symptoms as acute orthostatic hypotension and gastrointestinal motility disorder. Two patients had dysuria and dyshidrosis, and 2 patients had pupil changes. Electrophysiology revealed decreased sensory and motor nerve conduction velocity in 2 patients. Abnormal F wave and H reflex were found in 2 patients. The conduction velocity of large myelinated fibers was normal in one patient. In the autonomic function test, 2 patients had decreased Valsalva ratio, decreased heart rate variability of deep breathing. The blood pressure changes during tilt test between lying and upright position of 2 patients in acute stage was more than 30/15 mm Hg. Two patients underwent lumbar puncture, and one patient showed albuminocytologic dissociation. Sural nerve biopsies showed moderate to marked loss of small myelinated fibers and unmyelinated fibers. Two patients in acute stage were treated with immunomodulatory therapy (glucocorticoid combined with intravenous immunoglobulin), and their symptoms improved in varying degrees. Conclusions Autoimmune autonomic neuropathy with acute autonomic nerve dysfunction has unique clinical manifestations. Early consideration of autoimmune autonomic neuropathy followed by diagnostic work⁃up and prompt immune treatment will help to improve the prognosis.

DOI:10.3969/j.issn.1672⁃6731.2020.10.005