Idiopathic dysautonomia treated with intravenous gammaglobulin

Lancet. 1996 Jan 6;347(8993):28-9. doi: 10.1016/s0140-6736(96)91559-7.

Abstract

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.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Autonomic Nervous System Diseases / diagnosis
  • Autonomic Nervous System Diseases / therapy*
  • Humans
  • Immunoglobulins, Intravenous / administration & dosage
  • Immunoglobulins, Intravenous / therapeutic use*
  • Male
  • Recurrence
  • Treatment Outcome

Substances

  • Immunoglobulins, Intravenous