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Immunoglobulin improves a model of acute motor axonal neuropathy by preventing axonal degeneration

From the Department of Neurology (Drs. Nishimoto, Koga, Hirata and Yuki), Dokkyo University School of Medicine, Tochigi, and Department of Neurology (Dr. Kamijo), Labor Welfare Corporation, Chubu Rosai Hospital, Aichi, Japan.

Address correspondence and reprint request to Dr. N. Yuki, Department of Neurology, Dokkyo University School of Medicine, Kitakobayashi 880, Mibu, Shimotsuga, Tochigi 321-0293, Japan; e-mail: yuki{at}dokkyomed.ac.jp

Background: The action mechanism of IV immunoglobulin (IVIg) for Guillain-Barré syndrome has yet to be clarified.

Objective: To evaluate clinical, histologic, and immunologic effects in a disease model of acute motor axonal neuropathy (AMAN) treated by IVIg.

Methods: Rabbits were sensitized with gangliosides including GM1 and divided randomly into two groups at disease onset. One group received IV homologous -globulin (400 mg/kg/day) for 5 days (n = 15), and the other received saline (n = 15). Disease severity was scored (0 to 13 points) daily. Sixty days after onset, anti-GM1 antibodies were tested by ELISA, and the number of degenerative axons was counted in spinal anterior roots.

Results: Between both groups at onset, there was no difference in any characteristics including clinical score. The IVIg group had faster recovery than the saline group (p = 0.03). The percentage of rabbits that improved by a score of 4 was higher in the IVIg (53%) than in the saline (13%) group 60 days after onset (p = 0.03). Anti-GM1 IgG titers 60 days after onset did not differ between the groups. The anterior roots of rabbits surviving 60 days after onset showed lower frequency of axonal degeneration in the IVIg-treated (n = 11; mean 4.5%) than in the saline-treated (n = 8; mean 11.1%) rabbits (p = 0.01).

Conclusions: The therapeutic efficacy of IVIg in an AMAN model was confirmed. IVIg may not affect the production or catabolism of anti-GM1 IgG, but it may prevent axonal degeneration of motor nerves.

Received November 5, 2003. Accepted in final form February 2, 2004.

Additional material related to this article can be found on the Neurology Web site. Go to www.neurology.org and scroll down the Table of Contents for the June 8 issue to find the title link for this article.

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