[A case of acute idiopathic pandysautonomia]

A 19-year-old male student who complained of orthostatic syncope and low grade fever was admitted to our hospital and diagnosed acute idiopathic pandysautonomia (AIPD). He had dysesthesia and hypalgesia of his face and the upper part of the level of Th4, slight hypalgesia of the limbs and slightly exaggerated jaw jerk and diminished deep tendon reflexes of the limbs, but his main clinical manifestations were orthostatic hypotension (OH), sweating disturbance and alternative diarrhea and constipation. Paroxysmal cough and vomiting attack, severe weight loss and slight emotional change were also observed. There were no abnormal findings in the routine laboratory examinations, but the protein in the cerebrospinal fluid was slightly elevated in the initial state. The results of autonomic function tests, such as OH on tilting, no blood pressure effects on cold pressor test and hand grip test, no overshoot in Valsalva maneuver, low norepinephrine (NE) level at resting and poor NE response on tilting, exaggerated response to a small dose of NE infusion (denervation supersensitivity), suggested that the sympathetic post-ganglionic sites were chiefly affected. The parasympathetic nervous functions were seemed to be almost intact by the results of Aschner test, Czermak test and coefficient variation of R-R intervals. Sural nerve biopsy revealed a marked loss of small myelinated and unmyelinated fibers, suggesting axonal degeneration. These findings were different from those of the Guillain-Barré syndrome (GBS), although the allergic or immunological mechanisms are suggested as the cause of both AIPD and GBS. In this case, amezinium metilsulfate, an indirect sympathomimetic agent, was most effective to OH and cough attack.