체신경이 침범된 급성 범발성 자율 신경병

Abstract

Acute panautonomic neuropathy characterized by selective involvement of sympathetic and parasympathetic nerves with complete or relative sparing of somatic motor and sensory functions was first described by Young et al in 1969. We experienced acute pandysautonomia with peripheral neuropathy. The serial electrophysiologic studies revealed progressive periphear axonal polyneuropathy. Autonomic function test suggested dysautonomia of selective postganglionic lesion in 1 case and pandysautonomia in 3 cases. Our four cases wree Acute panautonomic neuropathies which simultaneously developed sensorimotor polyneuropathy. All Laps were normal except elevated CSF protein without pleocytosis. Recovery was poor in general, but 34-years-old female patient was markedly improved after plasmapheresis. Therefore we recommend immunologic treatment such as plasmapheresis or pulse therapy in early stage of progressive pandysautonomia.