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NEUROLOGY 1990;40:118
© 1990 American Academy of Neurology

A syndrome of asymmetric limb weakness with motor conduction block

C. Krarup, MD, PhD, J. D. Stewart, MB, FRCP(C), A. J. Sumner, MD, A. Pestronk, MD and S. A. Lipton, MD, PhD

Division of Neurology (Dr. Krarup), the Department of Medicine, Brigham and Women's Hospital and the Department of Neurology (Drs. Krarup and Lipton), Harvard Medical School, Boston, MA; the Division of Neurology (Dr. Stewart), Montreal General Hospital and McGill University, Montreal, Quebec, Canada; the Department of Neurology (Dr. Sumner), University Hospital of Pennsylvania, Philadelphia, PA; the Department of Neurology (Dr. Pestronk), the Johns Hopkins University, Baltimore, MD; and the Department of Neurology (Dr. Lipton), Children's Hospital, Beth Israel Hospital, and Brigham and Women's Hospital Boston, MA.

We describe 3 patients with asymmetric limb weakness, fasciculations (2 patients), relatively preserved reflexes, normal cranial nerves, and few or no sensory abnormalities. The symptoms had been progressive over 1 to 15 years. Detailed motor nerve conduction studies showed conduction block and slowing localized to sharply circumscribed areas 30 to 100 mm long in several nerves in each patient. By contrast, the sensory conduction studies over the same nerve segments were normal, indicating very selective involvement of motor fibers. Sural nerve biopsies showed minor changes that varied among the patients. One patient had high levels of anti-GM1 antibodies, 1 had mildly elevated levels, and 1 had high levels of only asialo- GM1 antibodies. Treatment with immune suppressive therapy has produced minimal improvement in 1 patient.

Address correspondence to Dr. C. Krarup, Department of Neurophysiology, Rigshospitalet, 9 Blegdamsvej, 2100 Copenhagen, Denmark. Address reprint requests to Dr. S.A. Lipton, Department of Neurology, Harvard Medical School, 25 Shattuck Street, Boston, MA 02115.

Presented in part at the 40th annual meeting of the American Academy of Neurology, Cincinnati, OH, April 1988.

Received May 12, 1989. Accepted for publication in final form June 27, 1989.




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