Idiopathic dysautonomia treated with intravenous gammaglobulin

doi:10.1016/S0140-6736(96)91559-7

The Lancet

Volume 347, Issue 8993, 6 January 1996, Pages 28-29

https://doi.org/10.1016/S0140-6736(96)91559-7 Get rights and content

Abstract

Summary

Background A previously healthy 23-year-old man presented with a short history of abdominal pain and diarrhoea followed by blurred vision, severe postural hypotension, reduced sweating and unremitting fever.

Methods Examination revealed fixed dilated pupils, impaired sweating and postural hypotension. Clinical and neurophysiological examination showed no motor or sensory deficit. A diagnosis of idiopathic autonomic neuropathy was made. He became gravely ill with profound life-threatening hypotension and a prolonged ileus.

Findings Within 36 h of receiving intravenous gammaglobulin (IVGG) his pupillary areflexia and severe hypotension resolved. 2 weeks later the autonomic failure recurred but again responded to treatment with IVGG. IVGG is a recognised treatment for Guillain-Barré syndrome.

Interpretation This case report demonstrates that IVGG is also effective in the rare pure dysautonomic variant.

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To provide an update on recent advances in the treatment of autoimmune autonomic ganglionopathy (AAG). AAG is an immune-mediated disorder characterized by prominent and selective involvement of autonomic nerve fibers or ganglia. Treatment with intravenous immunoglobulin (IVIG) or plasma exchange (PE) has been reported to be effective in single case reports and recent case series. This review summarizes current treatment options including IVIG, PE, and immunosuppressants agents, alone or in combination.
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Idiopathic dysautonomia treated with intravenous gammaglobulin

Abstract

Lancet

Mayo Clin Proc

Pure pandysautonomia with recovery

Trans Am Neurol Assoc

Acute pandysautonomia. Clinical and morphological study

Arch Neurol

Acute autonomic neuropathy

Neurology