급성 범발성 자율신경병 1예

Abstract

Dysfunction of autonomic nervous system had been reported in a number of neurologic and medical diseases, but acquired primary dysautonomia is uncommon and usually tends to show a chronic progressive pattern. 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 and since then, about 26 additional cases had been reported in the literatures, but there have been no such reports in Korea. The present report describes a 22 year-old female patient who had been admitted to Severance hospital because of abruptly developed fever, generalized myalgia, hyperesthesia and difficulties in swallowing and voiding. On admission, physical and neurological examinations revealed orthostatic hypotension, unreactive pupils to light and accomodation, dereased gag reflex, and decreased sensation of pain and temperature on the face and neck. A few days following admission, progressive loss of deep tendon reflew was noticed, followed by distal muscle weakness and atrophy. The serial electrophysiologic studies revealed progressive peripheral axonal polyneuropathy. Autonomic function tests suggested pandysautonomia of postganglionic lesion. Spinal fluid protein was 16mg/dl on admission, but was increased up to 80mg/dl without pleocytosis on the twenty first hospital day. Recovery was poor over a four -month period and she died of disseminated pneumonia.