Acute dysautonomia secondary to autoimmune diseases: Efficacy of intravenous immunoglobulin and correlation with a stimulation of plasma norepinephrine levels

Acute dysautonomia is a disorder characterized by severe sympathetic and pa rasympathetic failure with relative preservation of motor and sensory funct ion. The disease is considered to be idiopathic in most cases, but there is now a trend rewards considering the disorder as an uncommon variant of Gui llain Barre syndrome. We report two cases of acute dysautonomia which did n ot fulfill the criteria of the idiopathic form. The first case was associat ed with Sjogren's syndrome and the second with thyroiditis and antiganglios ide antibodies which were correlated with the severity of the disease. Intr avenous gammaglobulin (IVGG) was effective in both cases, as has been repor ted for the idiopathic form, and in one case the treatment was associated w ith an increase in the supine and standing plasma norepinephrine levels, th us substantiating the positive effects of IVGG on the orthostatic blood pre ssure and heart rate. We conclude that the spectrum of acute dysautonomia i s superimposable on that of the inflammatory peripheric neuropathies and sh ould include both the idiopathic form and dysautonomia with autoimmune asso ciated disorders. IVGG are effective and seems to act by increasing plasma norepinephrine levels.