IDIOPATHIC AUTONOMIC NEUROPATHY - CLINICAL, NEUROPHYSIOLOGIC, AND FOLLOW-UP-STUDIES ON 27 PATIENTS

We evaluated the natural history, electrophysiologic characteristics, spectrum of autonomic involvement, pathology, and laboratory features in 27 patients with idiopathic autonomic neuropathy who were followed up for a mean of 32 months. The typical features of idiopathic autonom ic neuropathy include the absence of an associated disease, frequent h istory of preceding infection, and acute or subacute onset with a mono phasic course. The spectrum of autonomic involvement ranges from panau tonomic to selective adrenergic or cholinergic failure. There is infre quent involvement of somatic nerve fibers as assessed by routine nerve conduction studies. Pathologic features include the presence of a sma ll inflammatory mononuclear cell infiltrate in the epineurium. Recover y tends to be gradual and frequently incomplete. The acute onset, freq uent antecedent viral infection, selectivity of involvement by fiber t ype and autonomic level, and presence of perivascular mononuclear cell infiltration suggest that the underlying mechanism is likely to be im mune-mediated. These observations may justify plasma exchange or other immunosuppressive modalities as early therapeutic intervention in pat ients with progressive disability.