Postinfectious pandysautonomia with complete recovery after intravenous immunoglobulin therapy

Acute dysfunction of the autonomic nervous system was first reported as severe parasympathetic and sympathetic failure without other neurologic manifestations by Young et al.1 in 1969. Since then, several other cases have been described.2-6 The characteristic features of this syndrome include severe orthostatic hypotension, impaired gastrointestinal and bladder motility, dry mucous membranes, and loss of sweating ability with impaired pupillary light reflex and accommodation reflex. These features are similar to those seen in Guillain-Barre syndrome (GBS) accompanied by autonomic disturbances. However, in pandysautonomia, there is no clinical or neurophysiological evidence for involvement of the somatic nerve fibers. Acute pandysautonomia is increasingly recognized as a possible variant of GBS. The presence of raised CSF protein and varying degrees of sensory loss and dysesthesias2,3 lends credence to this belief. IV immunoglobulin (Ig) is of benefit in GBS,7 and it seems reasonable to expect that pandysautonomia should also show a favorable response. …