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From the Department of Neurology, the University of Chicago, Pritzker School of Medicine, Chicago, IL.
we found significant decrement of the compound muscle action potential recorded from the abductor digiti minimi muscle in response to repetitive nerve stimulation in patients with rapidly developing motor neuron disease (MND), but not in patients with slowly developing disease when measured before exercise (7.6 ± 1.3% versus 1.6 ± 1.6%) and 2 minutes after exercise (13.6 ± 1.2% versus 0.5 ± 0.5%). All patients showed repair of decrement immediately after exercise. The mean amplitude of the compound muscle action potential was significantly lower in both the rapid-disease (2.9 ± 0.9 mV) and slow-disease (5.2 ± 1.6 mV) groups than in controls (14.5 ± 1.2 mV). For individual patients, there was no correlation between amplitude of the compound muscle action potential and decrement. These data indicate that decrement in MND relates to activity of disease of the motor neuron.
Address correspondence and reprint requests to Dr. Bernstein, Department of Neurology, University of Chicago, Pritzker School of Medicine, 950 E. 59th Street, Chicago. IL 60637.
Supported by grants from the Amyotrophic Lateral Sclerosis Society of America and the Muscular Dystrophy Association, Inc. (J.P.A.).
Accepted for publication April 21, 1980.
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