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Idiopathic dysautonomia treated with intravenous gammaglobulin
M T Heafield1, M D Gammage, S Nightingale
1University Department of Clinical Neurology, University Hospital Birmingham Trust, Edgbaston, UK.
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.