Acute pandysautonomia. Clinical and morphologic study.

Abstract

A man with acute pandysautonomia was examined clinically on two occasions four years apart. Complete autonomic paralysis was demonstrated at first examination, with almost complete recovery subse quently. A sural nerve biopsy 12 years after onset of the disorder showed a striking increase in the number of miniature unmyelinated axons consistent with regeneration. Myelinated fibers showed some morphologic features suggestive of remyelination; these were not prominent and no degeneration of myelinated axons was found. Morphologic features and results of autonomic function tests were consistent with a primary, if not exclusive, involvement of unmyelinated postganglionic fibers. Pathogenesis of this disorder is unclear but clinical features and natural history are most compatible with an immunologic basis.