A case of subacute idiopathic pure pan-dysautonomia--recovery with prednisolone therapy

A 52-year-old man had, after an episode of fever in June 1989, developed orthostatic dizziness followed by sexual impotence, dysuria, decreased sweating and weight-loss, which progressed gradually and reached their maximum seven months after the onset. He was given 400 mg of droxydopa and 8 mg of midodrine HCL per day without apparent benefits, and was admitted to our hospital. His blood pressure (mmHg) and heart rate were 167/102 and 68 in supine position, and 74/41 and 62 in sitting position. Skin was dry. Pupillary reactions were sluggish. Left pupil was slightly irregular. Other cranial nerves, sensory and somatic motor functions were normal. Laboratory tests revealed as follows: slight anemia, ESR 42 mm/hour, serum IgG 2236 mg/dl, CSF protein 64 mg/dl and positive tests for non-specific autoantibodies. Nerve conduction studies and electromyogram were normal. Autonomic function tests showed postganglionic impairments of sympathetic and parasympathetic systems. The sural nerve biopsy disclosed normal myelinated fibers, but decreased unmyelinated fiber density to 60% of the control value. No demyelinating lesions, cell infiltration or amyloid deposits were seen. Under the diagnosis of idiopathic pure pan-dysautonomia, prednisolone, initially 60 mg daily, was added. Within 10 days, he showed marked improvement of general conditions. No exacerbation was seen during reduction or after withdrawal of prednisolone. Repeated tests showed normalizing laboratory data and regression of autonomic deficits. A year after onset he regained normal social life.(ABSTRACT TRUNCATED AT 250 WORDS)