Neurology 2000;54:615
© 2000 American Academy of Neurology
Articles
Distal acquired demyelinating symmetric neuropathy
J. S. Katz, MD,
D. S. Saperstein, MD,
G. Gronseth, MD,
A. A. Amato, MD and
R. J. Barohn, MD
From the Department of Neurology (Dr. Katz), Palo Alto VA Medical Center, CA; Stanford University (Dr. Katz), Palo Alto, CA; Wilford Hall Medical Center (Drs. Saperstein and Gronseth), San Antonio; the University of Texas (Drs. Saperstein and Barohn), Southwestern Medical Center, Dallas; and the University of Texas Medical Center (Dr. Amato), San Antonio, TX.
Address correspondence and reprint requests to Dr. Jonathan Katz, Department of Neurology (127), Department of Veterans Affairs, 3801 Miranda Avenue, Palo Alto, CA 94304.
OBJECTIVE: To characterize an acquired, symmetric, demyelinating neuropathic variant with distal sensory or sensorimotor features.
BACKGROUND: Classic chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) patients have prominent proximal and distal weakness. However, chronic demyelinating neuropathies may present with different phenotypes. An approach that distinguishes these disorders primarily according to the pattern of weakness may be useful to the clinician.
METHODS: A total of 53 patients with acquired symmetric demyelinating polyneuropathies were classified primarily according to the pattern of the neuropathy and secondarily according to the presence and type of monoclonal protein (M-protein) in this retrospective review. The authors distinguished between patients with distal sensory or sensorimotor involvement, designated as distal acquired demyelinating symmetric (DADS) neuropathy, from those with proximal and distal weakness, who were designated as CIDP.
RESULTS: M-proteins were present in 22% of patients with CIDP. There were no features that distinguished clearly between CIDP patients with or without an M-protein, and nearly all of these patients responded to immunomodulating therapy. In contrast, nearly two-thirds of the patients with DADS neuropathy had immunoglobulin M (IgM) kappa monoclonal gammopathies, and this specific combination predicted a poor response to immunomodulating therapy. Antimyelin-associated glycoprotein (anti-MAG) antibodies were present in 67% of these patients.
CONCLUSION: Distinguishing acquired demyelinating neuropathies by phenotype can often predict the presence of IgM kappa M-proteins, anti-MAG antibodies, and responses to immunomodulating therapy.
Key words: Chronic inflammatory demyelinating polyradiculopathy—Distal acquired demyelinating symmetric neuropathy—Monoclonal gammopathy of uncertain significance—Terminal latency index—Myelin-associated glycoprotein
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