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Idiopathic autonomic neuropathy

Clinical, neurophysiologie, and follow-up studies on 27 patients

Departments of Neurology (Drs. Suarez, Fealey, and Low) and Gastroenterology (Dr. Camilleri), Mayo Clinic and Mayo Foundation, Rochester, MN.

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 autonomic neuropathy include the absence of an associated disease, frequent history of preceding infection, and acute or subacute onset with a monophasic course. The spectrum of autonomic involvement ranges from panautonomic to selective adrenergic or cholinergic failure. There is infrequent involvement of somatic nerve fibers as assessed by routine nerve conduction studies. Pathologic features include the presence of a small inflammatory mononuclear cell infiltrate in the epineurium. Recovery tends to be gradual and frequently incomplete. The acute onset, frequent antecedent viral infection, selectivity of involvement by fiber type and autonomic level, and presence of perivascular mononuclear cell infiltration suggest that the underlying mechanism is likely to be immune-mediated. These observations may justify plasma exchange or other immunosuppressive modalities as early therapeutic intervention in patients with progressive disability.

Address correspondence and reprint requests to Dr. Guillermo A. Suarez, Department of Neurology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905.

Presented in part at the 45th annual meeting of the American Academy of Neurology, New York, NY, April 1993.

Received December 1, 1993. Accepted in final form February 21, 1994.

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